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Case 1 was a 60-year-old woman. She was raised in a coastal area in a small town, in what is described as a well-functioning family, and has been trained as a healthcare assistant. Prior to moving into the nursing home, she had been married and had two adult children, with whom she had no contact. She had gradually increased her consumption of alcohol over the years, and in spite of several contacts with outpatient services for alcohol dependence, her drinking had steadily increased. After losing her job, she became increasingly socially isolated. Prior to moving into the nursing home, her home nurses visited several times per day, and they often found her in severe withdrawal, occasionally convulsing. She was underweight and incontinent. Her apartment was untidy and rarely cleaned, smelled of urine and feces and evinced her lack of personal hygiene. She was depressed and talked about suicide.\nAfter moving into the home, she gradually became stable, and was able to manage her personal hygiene with minimal assistance. She ate at meals and began to look better. She was still drinking, but at a level that did not cause problems with other residents. Occasionally, she drank heavily for 1–2 weeks. Her contact with other residents and staff stabilized, and she participated in simple practical activities. She seemed less anxious, and did not go through serious withdrawal.\nDuring the 18-month period before moving into the nursing home, she had been hospitalized nine times for periods ranging from 1 to 19 days; in total, she spent 43 days in hospital, had one outpatient visit and several ER visits. The total cost of her hospital-based care was estimated to be 154,649 DKK (20,798.74 Euros).\nAfter moving into the nursing home, she was admitted to inpatient treatment on two occasions for a total of 4 days. The total cost was 25,226 DKK (3392.64 Euros). She also had two visits at an emergency room (ER) and four outpatient visits. |
A 44-year-old Asian female visited the emergency room complaining of a headache, nausea and vomiting. She had visited the hospital 11 years earlier for gestational hypertension. She had been diagnosed with PCC in the right adrenal gland () and successfully treated with a right adrenalectomy. PCC of the adrenal gland scaled score had been 7, so the tumor had been diagnosed as malignant. For the first 5 years of follow-up, no signs of recurrence had been found, and she had not visited the hospital since then. Four hours before her visiting the present hospital, she took 4 mg of methylprednisolone due to urticaria.\nHer blood pressure (BP) was 180/100 mmHg, and other vital signs were stable. An electrocardiogram and a chest X-ray showed normal findings. She had no history of diabetes, but her glucose level was 330 mg/dL, and bicarbonate level was reduced at 13.5 mmol/L. Other blood tests showed no abnormal findings, and urine dipstick analysis showed ketone 1+. Hydration was performed with normal saline due to the possibility of early phase diabetic ketoacidosis. Abdomen-pelvis computed tomography (CT) was performed, and there was no evidence of local recurrence of PCC ().\nFour hours after visiting the hospital, she complained of severe dyspnea. She showed tachycardia with a pulse of 130 beats/min, severe tachypnea with a respiratory rate of over 35 breaths/min, and a fever of 38°C. Rale was heard from both lung fields, and a chest X-ray revealed a rapidly developed bilateral consolidation (). Intubation and mechanical ventilation were performed because of severe hypoxemia. According to echocardiography conducted at bedside, although the cardiac chamber size was normal, there was severe global hypokinesia of the left and right ventricles. The left ventricular ejection fraction was approximately 20% and the tricuspid annular peak velocity had decreased to 6cm/s, indicating severe ventricular dysfunction of both ventricles. Antibiotics and diuretics were used, considering the possibility of acute respiratory distress syndrome due to atypical pneumonia or acute pulmonary edema caused by heart failure. She was moved to the intensive care unit from the emergency room. Five hours after intubation, her BP was dropped to 60/30 mmHg and pulse gradually slowed. One mg of epinephrine and 1 mg of atropine were injected immediately, but asystole occurred. Cardiopulmonary resuscitation was performed immediately and venoarterial ECMO was prepared. ECMO was initiated 15 minutes after arrest. Asystole and return of circulation were repeated 3 times for 42 minutes. After that, the patient’s rhythm was restored and no further cardiac arrest occurred. She was treated for 5 days with support of ECMO, mechanical ventilation, and inotropic agents. She recovered consciousness and ECMO was removed when her vital signs became stable without inotropics. After 10 days, ventilator weaning and extubation were performed.\nThe results of hormone tests performed at the time of hospitalization showed an overall increase in catecholamine (), and catecholamine-induced cardiomyopathy due to PCC was strongly suspected. In order to confirm the recurrence of PCC, a 131I-metaiodobenzylguanidine (MIBG) scan was performed, but no focal radioactive uptake lesion was shown. The patient’s CT was reviewed closely, and a faint mass-like lesion was detected on her right sacrum. Sacrum magnetic resonance imaging was performed, and a 2.7×2.9×2.8 cm sized well-defined, lobulated, and slightly expansile mass with mainly T1 low T2 high signal intensity and heterogeneous enhancement at right upper sacral ala was seen (). Other small lesions, which are assumed to be metastasis, were also found at the S2 level of the sacrum, right iliac bone, and lower L4 vertebral body. A positron emission tomography scan showed an increase of uptake in the same regions (). For a histological verification, an open bone biopsy was performed. Atypical spindle-shaped tumor cells showing synaptophysin and chromogranin positivity were found upon histopathologic examination (). These results demonstrated that the lesions on the sacrum were recurrent metastatic PCC.\nIn the patient’s follow-up, the cardiac functions, including left and right ventricles, were found to be completely recovered, with 65% of ejection fraction. Coronary angiography performed after ventilator weaning showed no abnormal findings. Currently, her BP is being successfully regulated with an α-blocker (doxazocin) and β-blocker (carvedilol) and treatment for the recurrent PCC is planned. |
A 24-year-old pregnant woman (G2P1) was referred to us due to suspected bilateral ovarian cysts at 8 weeks of gestation. She had undergone ovarian cystectomy twice under open surgery: left and right ovarian cystectomy for mature cystic teratoma and mucinous cystadenoma, respectively. She had no additional medical history or familial medical history. Transvaginal ultrasound and magnetic resonance imaging (MRI) (Figures and ) revealed two pelvic cysts. The left-sided unilocular cyst was 9 cm in diameter. The right-sided multilocular cyst was 5 cm in diameter. We diagnosed this condition as bilateral ovarian cysts.\nAlthough the serum levels of tumor markers (CA125, CA19-9, and CEA) were normal for a pregnant woman, considering the large size of the cyst, cyst resection was attempted at 14 weeks; however, it was converted to probe laparotomy. Marked adhesion around the cysts, posterior uterus, and Douglas' pouch made cyst resection impossible as extensive adhesiolysis may cause uterine damage and also uterine contractions after surgery. Gross examinations revealed no metastatic lesions or lymph node swelling. Abdominal fluid cytology revealed no malignant cells.\nAt 32 weeks of gestation, MRI revealed that the left-sided cyst size had increased to 27 cm in diameter (Figures and ), although she was asymptomatic. As shown in , the right-sided multilocular cyst became very close to the left monocytic cyst. At this stage, the left large monocytic cyst appeared to merge with the smaller right multilocular cyst, forming a large cyst occupying the entire pelvic cavity, which was later confirmed by laparoscopic findings.\nThis large cyst showed no solid-part or papillary growth. The serum levels of tumor markers remained normal. Malignant ovarian tumor could not be ruled out but was considered less likely. We weighed merits and demerits between relaparotomy for tumor resection during pregnancy and a wait-and-see approach for several weeks; the former is likely to require extensive adhesiolysis and may cause preterm delivery. We decided on the latter strategy, since resection should be performed in the event of a size increase or images indicative of malignancy. The fetus normally developed without fetal growth restriction.\nCesarean section and tumor resection were performed at 37+4 weeks of gestation, yielding 3,012-g male infant with Apgar score 8/9 at 1/5 minutes, respectively. The infant did not have congenital abnormalities. After the completion of cesarean section, we ruptured the wall of this large cyst, with care to avoid the cyst content entering into the abdominal cavity. A large amount of serous fluid was drained. This large cyst was a multicystic cyst (5 cm), considered to be the right multicystic ovarian cyst that had been observed from the first trimester. The wall of the large cyst showed marked adhesion to the peripheral peritoneal cavity. We resected it as widely as possible together with right salpingo-oophorectomy (Figures and ). The left ovary was macroscopically normal, and thus there was no evidence of the left ovarian tumor. The resected tumor consisted of a large unilocular cyst with serous fluid and a mucinous cystadenoma (Figures and ). In the former, lining epithelium was absent in many parts () and mucinous epithelium was occasionally found in continuity with the cyst wall of the latter (right ovarian cystadenoma). No malignant cells were found in the resected specimen. Immunohistochemistry revealed focally positive staining for estrogen and progesterone receptors on the resected cyst wall (Figures and ). At 12 months after the delivery, left ovary remained normal and the retention cyst did not recur. An informed consent for this reporting was obtained from this patient. |
An 8 days old male neonate was born to an Asian mother through vaginal delivery at 37 weeks of gestation, weighed 2,380 g, and had APGAR scores of 9 and 10 at 1 and 5 min, respectively. He was admitted to our hospital with a 2 days history of fever of up to 39°C but did not have respiratory or gastrointestinal symptoms. The infant's family denied any medical history and TB contact. His physical examination at admission documented smooth respiration, clear breathing sound, and no hepatosplenomegaly. The complete blood count indicated a total white blood cell count of 17,500/μL with 69% neutrophils, 20% lymphocytes, 9% monocytes, and 2% eosinophils. The C-reactive protein level was 7.3 mg/dL. The findings of the cerebrospinal fluid (CSF) analysis were normal. Bacterial cultures of the blood, urine, and CSF were negative. Intravenous antibiotics, namely cefotaxime and ampicillin, were administered after admission on the basis of suspicion of neonatal fever. Despite the administration of the antimicrobial combination therapy, the fever persisted and the neonate developed abdominal distension when he was 12 days old. Abdominal radiography exhibited nonspecific dilated bowel loops. Because no improvement in the condition of the patient was observed after changing antibiotics, infection caused by some virus and other atypical pathogen, including Mycobacterium tuberculosis, was considered. Tests for herpes simplex virus, Epstein–Barr virus, cytomegalovirus, hepatitis B virus, rubella, Chlamydia trachomatis, and Toxoplasma gondii were all negative. The repeat C-reactive protein level was elevated to 14.4 mg/dL. Coagulopathy with 323.7 μg/mL of abnormal fibrin degradation product and more than 20 mg/L of D-dimer were also noted. Antibiotics were switched to vancomycin and ceftazidime empirically. Chest radiography displayed only increased right lung field infiltration when the infant was 12 days old (), and chest computed tomography (CT) imaging exhibited a large amount of right pleural effusion with mild inflammatory changes in the right lower lobe when the infant was 15 days old (). Pleural effusion drainage was suggested but refused by his parents at that time. Gastric lavages for acid-fast staining and culture were examined when the infant was 20 days old after his parents agreed to further testing, and one of the three acid-fast stains of gastric lavages yielded few acid-fast bacilli. Repeat chest and abdomen CT imaging performed when the infant was 24 days old indicated patchy consolidation in the right upper lung, multiple new nodules in both the lungs, moderate pleural effusion, and multiple low-density nodules in the spleen and hepatic hilar region without hepatomegaly (). Subsequently, pigtail catheter insertion for pleural effusion drainage was performed. The findings of pleural fluid analysis indicated a total white blood cell count of 10,800/μL with 6% neutrophils, 57% lymphocytes, and 37% mesothelial cells; a total protein level of 4.6 g/dL, a lactic dehydrogenase level of 250 IU/L, and a glucose level of 164 mg/dL. TB infection was strongly suspected. The neonate was administered isoniazid (15 mg/kg/day), rifampicin (15 mg/kg/day), and pyrazinamide (20 mg/kg/day) when he was 24 days old. After initiating anti-TB treatment, the neonate's symptoms and signs subsided gradually. Finally, both gastric lavage and pleural effusion cultures showed M. tuberculosis complex.\nThe neonate's mother was 33 years old, gravida 1, para 1. Her Group B streptococcus test was negative. She had been healthy with no previous medical history and TB contact history; however, she developed a mild dry cough 1 week after delivery, experienced persistent general weakness, and was admitted to our medical intensive care unit because of altered mental status 24 days postpartum. Laboratory examinations indicated leukocytosis, thrombocytopenia, coagulopathy, acute hepatic failure, and acute renal failure. The HIV serology test was negative. A chest X-ray exhibited a miliary TB pattern (). A chest CT image displayed diffuse interlobular and intralobular septal thickening with ground-glass opacities (). Because her neonate was highly suspected to have TB infection at that time, acid-fast staining and TB polymerase chain reaction (PCR) of the sputum were performed. Both tests were strongly positive. The mother was administered anti-TB therapy immediately, but she died 3 days after hospitalization. M. tuberculosis infection was confirmed through sputum culture. |
A 28-year-old woman presented to our hospital with numbness in her left arm. Her left arm and back had been larger than the contralateral side since birth and this had increased over time with partial functional difficulty. Sometimes, she felt numbness in the left arm, which worsened when it was lowered. She had been examined at various hospitals and diagnosed with a vascular malformation but had not yet received any treatment.\nThe patient had experienced severe numbness in her left arm for the previous two weeks. This was not relieved by painkillers. It was often necessary to lift the arm above her head in order to feel more comfortable.\nOn physical examination, the entire arm and part of the left back were deformed, being larger than the opposite side (), but the two arms were of equal length. The skin on the left elbow, hand, and back had a bluish tint. The affected region had multiple swollen areas which were different in size, squash, and easily compressible. Some hard round particles could be felt under the skin. The peripheral pulses were symmetrically palpable, and no temperature difference was observed. No abnormalities were found in the cardiovascular system or in other organs.\nBlood tests demonstrated higher than normal levels of D-dimer (> 7.65 mg/L compared to the reference value of < 0.48 mg/L), and the prothrombin time (PT), prothrombin time ratios (PTr), International normalised ratio (INR) values and platelet count were within normal ranges.\nAn ultrasound examination of the left arm revealed numerous subcutaneous tubular structures. These were thin-walled with an anechoic lumen, completely compressible, and colour flow was present with low velocities on Doppler ultrasound. These features were consistent with the telangiectatic superficial veins.\nInside these structures were many echogenic particles with posterior acoustic shadowing consistent with phleboliths (). The axillary, brachial, ulnar, and radial arteries had normal diameters, velocities, and waveforms. The accompanying deep veins did not dilate, were completely compressed, and no thrombosis was observed.\nThe topogram film showed that the humerus, ulna, and radius on the left side were smaller than those on the right side. Multiple well-defined radiopaque lesions were distributed along the left upper limb, chest, and back ().\nA computed tomography scan of the left arm also revealed that the diameter of the left humerus was smaller than that of the right (). The arterial phase showed the axillary, brachial, ulnar, and radial arteries with normal courses and early enhancement of some veins. In addition, there were three vessel plexuses on the left back and posterior aspects of the arm and hand, with enlarged feeding arteries originating from the left third intercostal artery, brachial artery, common palmar digital arteries, with suspected arteriovenous fistulas. In the venous phase, scattered calcified round or oval particles were observed within dilated sinuous superficial veins ( and ).\nThe patient was diagnosed with Servelle-Martorell syndrome and treated with conservative methods, including wearing compression stockings and taking Daflon. However, the blood in the left arm was evacuated to the shoulder when she used a compression sock, making sleep difficult, so she stopped wearing it, and kept her left hand held high to decrease the numbness. |
A 33-year-old, ECOG 0 female was referred to our centre for opinion and management of postoperative locally invasive adenoid cystic carcinoma of the Bartholin's gland (ACCBG). She was nulliparous on an oral contraceptive, with menarche at the age of 15. Gynaecological and family history was otherwise unremarkable.\nShe first presented to a hospital in 2009 for a right vulvar lesion. Biopsy of this lesion was benign, and the lesion resolved without intervention. In 2013, the patient noticed a new right perineal lesion. CT, MRI, and bone scans were organized by her gynaecologist. Investigations were unremarkable except for a lesion in the ischial tuberosity on MRI. The lesion was deemed benign after review by orthopedic surgeons. 4 years later, the patient began to experience dyspareunia associated with a burning sensation. An MRI performed in June 2018 showed soft tissue swelling in the perineal region and a 14x13x13mm rounded soft tissue mass at the right posterolateral margin on the vaginal introitus consistent with a Bartholin's cyst. The lesion appeared to infiltrate the vaginal wall in the anteromedial margin, but this did not cross the midline ().\nA biopsy was performed with histological features consistent with adenoid cystic carcinoma followed by wide local excision of the right vulva. The specimen spanned 43x25x32mm, and the tumour involved the excision margins. On histology, the specimen had foci of perineural invasion and invaded fibrous tissue, fat, and skeletal muscles (). One month later, 8 lymph nodes were dissected, none of which were positive.\nPET scan 2 months after surgery showed FDG uptake consistent with postsurgical changes and uptake in a right axillary node that was likely inflammatory rather than a distant metastasis. Further MRI contrast scans to assess extent of perineural invasion showed linear enhancement along the course of the perineal branch of the right pudendal nerve, terminating before Alcock's canal ().\nThe consensus of the Gynaecologic Oncology Multidisciplinary Tumour Board was to proceed with adjuvant chemoradiation therapy. The patient received radiotherapy postoperatively to the tumour bed to a total dose of 66 Gy and to the right pudendal nerve to 59.4 Gy in 33 fractions by a VMAT technique with concurrent weekly 40 mg/m2 cisplatin chemotherapy which she tolerated well. The ovaries were spared to prevent premature ovarian dysfunction. At her 3 month posttreatment follow-up, there was clinically and radiologically no evidence of locoregional recurrence. The patient is planned for close surveillance. |
A 28-year-old Italian male presented at the ER in August 2011 with diarrhea and an antibiotics refractory fever. Laboratory findings did not show any significant problem, apart from a mild hypertransaminasemia: (ALT 65 u/L AST 46 u/L).\nThe patient was discharged with the indication for further investigations. Abdominal ultrasonography was performed two weeks later, showing normal liver, biliary ducts, and pancreas morphology. Nevertheless a 15 cm increased spleen was found, containing in its cephalic portion a hypoechoic and asymptomatic 70 × 77 mm solid mass. Color Doppler showed blood flow along the edge of the round mass, without relevant signals within the neoformation.\nCT scan was also executed, to better evaluate the splenic lesion: images before contrast material administration displayed a round mass, hypodense compared to the normal splenic parenchyma. After injection of intravenous contrast, it showed a mild contrast enhancement during the arterial phase, followed by a complete washout during the late venous phase, without relevant vascular components inside ().\nAfter these preoperative findings suggestive but not definitive for a well-defined histological lesion, considering the high risk for splenic bleeding in the case of biopsy and the risk for spontaneous rupture, the patient was candidate for totally laparoscopic splenectomy two months after first clinical evaluation. Routine preoperative examination, chest X-ray, blood test, and anaesthesiologic evaluation did not set any contraindication to the operation.\nThe patient was placed in a 30-degree right lateral decubitus, with the help of a cushion positioned along his back.\nRight arm was arranged for intraoperative intravenous fluid infusion, whereas left arm was suspended with bendages above patient's head to allow full access to the left hemiabdomen. A 12 mm disposable Hasson's trocar was placed using an open technique in supraumbilical region for the first induction of pneumoperitoneum and for optical access. A second 10–12 mm disposable trocar was positioned in left hypochondrium, at the intersection between midclavicular line and infracostal line and two more 5 mm accesses, the first in left flank, along anterior axillary line and the second in right hypochondrium, along midclavicular line (). Laparoscopic exploration of the abdominal cavity was negative for any associated or generalized pathology, as well as for accessory spleen. An enlarged 15 cm spleen was identified; omental adhesions and splenocolic ligament were dissected using monopolar and ultrasonic dissector in order to lower the splenic flexure of the colon and to expose the inferior pole of the organ. Dissection was carried on with the section of the inferior and superior gastrosplenic vessels. Thus the lesser peritoneal sac was open, exposing the anterior side of the splenic hilum and the pancreas tail; the stomach was retracted toward the patient's rightside. Vascular splenic pedicle was then isolated at the hilum allowing identification of periferic branches of splenic artery and vein.\nVessels were finally dissected using two vascular 30 mm endostapler refills (EndoGIA, Autosuture, Covidien, Mansfield, MA, USA) obtaining a perfect hemostasis. Spleen was then mobilized toward the patient's right side, to allow dissection of the posterior peritoneal reflection or splenorenal ligament. After placing the totally freed organ inside an EndoBag (EndoCatch II, Autosuture, Covidien, Mansfield, MA, USA) a Pfannestiel suprapubic incision was performed to remove the specimen. The peritoneal-sac access was carried on with a partial surgical section of the rectus muscle in order to allow an easier removal of the unbroken specimen. After clearing the spleen, laparotomic incision was sealed in multiple layers. An accurate hemostasis control and a generous abdominal cavity washing were performed after the recreation of pneumoperitoneum; a single drainage was positioned in the left subphrenic space and major accesses were finally closed.\nA short-term perioperative antibiotic prophylaxis based on cefazolin (1 gr every 8 hours) was administered for the first 24 hours. Pain control was always optimal during the entire hospitalization: during the first 24 hours after surgery pain control was managed with opiates and paracetamol; on the following day opiates were replaced with anti-inflammatory nonsteroidal drugs removed from the 3rd day on; during the same day the drainage was removed. The patient was allowed to eat from the 2nd postoperative day. The complete functionality of the gastrointestinal tract was regained between the 3rd and 4th postoperative day. The patient was dismissed on the 5th day after surgery and stitches were removed 6 days later during an outpatient clinical control. Vaccination against Streptococcus pneumoniae, Haemophilus influenza type B, and Neisseria meningitidis infections was administered 20 days after surgery. Perioperative anticoagulant prophylaxis with subcutaneous heparin (4000 I.U. per day) was applied and continued for 4 weeks after surgery.\nThe anatomopathological analysis of the spleen showed a 14 × 7 × 8 cm organ, weighting 430 gr, containing a 7,5 cm reddish brown nodule of slightly augmented stiffness, compared to the surrounding splenic tissue (). The histopathological diagnosis confirmed the original suspect of splenic hamartoma, also supported by the immunohistochemical stains positive for CD-8 and negative for CD-34. |
A 76-year-old female presented to the ED with family members via private car. On arrival, she was unresponsive with 3 mm pupils, tachypneic (RR= 30), tachycardic (PR=117), hypotensive (80/40 mmHg), and bedside capillary blood glucose was high. She had a background history of diabetes, hypertension, recurrent strokes, and she was bedridden. The patient was attended by a board-certified emergency physician. She was intubated immediately and connected to a mechanical ventilator. She was resuscitated with two liters of crystalloid fluids for low blood pressure and then vasopressors using norepinephrine as well as insulin infusion as her blood sugar was high. The team was able to insert three peripheral intravenous lines at the following sites: right antecubital, left antecubital, and right hand. As her blood pressure and perfusion were not responding to initial crystalloid and low dose norepinephrine, the attending physician decided to insert a left subclavian catheter where a 7.0-French triple lumen CVC was inserted in the left subclavian vein using infraclavicular approach and anatomical landmarks. The norepinephrine was connected to the CVC, and the dose was increased. Chest X-ray showed that the tip of the central line appears to be in the descending aorta as the line seems to have been inserted into the subclavian artery (Figure ).\nThe norepinephrine infusion was stopped, and the central line was removed with compression. She was transferred from the ED to the medical imaging unit for brain computed tomography (CT) then straight admission to the ICU. During the ICU stay and 8 hours since ED arrival, her right upper extremity was found to be cyanosed in color and pulseless. One day later, CT angiography showed a thrombus with complete occlusion in the right subclavian artery (Figure ).\nThe patient’s condition was discussed with a multidisciplinary team including a critical care physician, vascular surgeon, and an interventional radiologist. All had agreed that she is high-risk for surgical intervention, fibrinolysis, and anticoagulation, which was limited given her severe coagulopathy. She remained in the ICU intubated and mechanically ventilated until her demise. |
A 38-year old nulliparous woman experienced headache, nausea, and a defect in the visual field over a period of days. Computed tomography (CT) and magnetic resonance imaging (MRI) of the head detected a tumor with midline shift in the left occipital lobe, 4 cm in diameter (Fig. a). As a result, she had undergone resection of the brain tumor. Histological examination of the resected specimen confirmed pure choriocarcinoma. Serum concentration of human chorionic gonadotropin (hCG) was 5030 IU/L. Serum carbohydrate antigen 125 and α-fetoprotein levels were within normal range. Abdominal and pelvic CT and MRI demonstrated a solid left ovarian tumor, 5.5 cm in diameter (Fig. b). However, no evidence of tumor in the uterus was seen. Chest CT revealed metastatic choriocarcinoma in the left lung (Fig. c). Consequently, her disease was diagnosed as primary pure ovarian choriocarcinoma and two cycles of a combination chemotherapy regimen comprising methotrexate, etoposide, and actinomycin-D (MEA therapy) which is commonly used for gestational choriocarcinoma, were administered. However, each administration of this regimen caused strong bone marrow suppression and a new metastasis appeared in the cerebellum after the second cycle of MEA therapy. Therefore, she was referred to our hospital.\nThe third cycle of MEA therapy was administered with concomitant administration of pegfilgrastim to avoid bone marrow suppression. Gamma knife therapy was also performed for the cerebellar metastatic lesions. However, hCG levels increased continuously and the chemotherapy regimen was changed to a combination chemotherapy regimen of bleomycin, etoposide, and cisplatin (BEP therapy), which is commonly used for nongestational choriocarcinoma (malignant germ cell tumor). Two cycles of BEP therapy were effective and total abdominal hysterectomy was performed with bilateral salpingo-oophorectomy and segmentectomy of the left lung. Histologic examination of the left ovarian tumor demonstrated vast central necrosis and remnant marginal choriocarcinoma with no evidence of other germ cell elements, leading to a diagnosis of pure choriocarcinoma of the ovary (Fig. ). BEP therapy and surgical resection achieved the nadir level of hCG (2.2 IU/L) (Fig. ). However, two additional cycles of BEP therapy failed to achieve complete remission, and hCG level increased steeply when chest CT revealed multiple metastases in the lungs.\nHer clinical history was reevaluated to identify the origin of choriocarcinoma and choose an appropriate chemotherapy regimen. She was unmarried, but had had sexual intercourse with her partner. Her menstrual cycle had been irregular since the time she had suffered subarachnoid hemorrhage due to rupture of an aneurysm on the posterior inferior cerebellar artery 10 months before development of the present illness. Therefore, the possibility of gestational choriocarcinoma subsequent to unrecognized pregnancy could not be ruled out. Consequently, we performed DNA STR analysis using a capillary electrophoresis system. Written informed consent was obtained from the patient for DNA extraction and DNA STR analysis, and this investigation was approved by the Ethics Committee of Nagoya University Graduate School of Medicine. DNA was extracted from oral mucosal cells of the patient and frozen cancer tissue, using a QIAamp® DNA Micro kit (Qiagen, Hilden, Germany) according to the protocol provided by the manufacturer. One microliter of extracted DNA was amplified using an AmpFℓSTR® Identifiler® Plus PCR Amplification Kit (Life Technologies, Waltham MA) for 15 STR loci and the amelogenin locus for gender determination, for a total of 16 DNA markers, as previously described [, ]. One microliter of amplified DNA was capillary electrophoresed on an Applied Biosystems 3130xl Genetic Analyzer (Life Technologies), and the detected peaks were genotyped automatically using GeneMapperID software version 3.2.1 (Life Technologies) as previously described [, ]. Part of the STR analysis is shown in Fig. . Genomic DNA alleles of the tumor were entirely identical to those of the patient, confirming that the choriocarcinoma was nongestational.\nA combination chemotherapy regimen of MEA, paclitaxel and cisplatin (TP), fluorouracil and actinomycin-D (FA), ifosfamide, cisplatin, and etoposide (ICE), etoposide, methotrexate, actinomycin-D/cyclophosphamide and vincristine (EMA/CO) with segmentectomy of the right lung were administered after tumor relapse, but no chemotherapy regimens successfully suppressed tumor activity. The patient died 21 months after the diagnosis of choriocarcinoma. |
In August 2006, a 40-year old female patient was examined for swelling in the lateral side of the right upper arm. Upon resection, the patient was diagnosed with myxofibrosarcoma. A second resection was performed to achieve tumour-free margins and plastic surgery was performed two times on the damaged area for cosmetic purposes.\nScreening for metastatic disease by computer tomography (CT) showed no pulmonary, bone or hepatic metastases, however a lesion (36 × 23 mm diameter), classified as a benign tumour by CT criteria, was detected adjacent to the left adrenal gland. A control CT scan in December 2006 revealed that this lesion had increased in size. Biopsy of the periadrenal tumour indicated a possible mesenchymal tumour, however malignancy was not confirmed.\nIn addition, suspect bilateral mammary lesions were also diagnosed. Histological examination of biopsies taken from both breast tumours, revealed invasive ductal carcinomas on both sides.\nBecause of an unfavourable breast-tumour-relation, neo-adjuvant chemotherapy was applied. After four cycles of an anthracycline-containing regimen, restaging revealed no significant change in the breast tumours, however progression of the periadrenal mass which extended in diameter to 67 × 49 mm, was identified by CT scan. Firstly, a bilateral breast-conserving tumour extirpation in combination with bilateral axillary lymphonodectomy was performed. Both carcinomas were positive for oestrogen receptor (ER) and progesterone receptor (PR) expression in the majority of tumour cells, and also overexpressed the human epidermal growth factor receptor (HER2/neu) (Table ). There were no relevant histological signs of regression 3 months following neo-adjuvant chemotherapy.\nThe periadrenal tumour was subsequently resected. Histological diagnosis revealed a poorly differentiated, pleomorphic, periadrenal liposarcoma. The lipogenic nature of tumour was confirmed by immunohistochemical detection of the S100 protein in the tumour cells. Adjuvant chemotherapy with taxanes was recommended following consultation with an interdisciplinary tumour board, because of the nodal positive breast cancer. Post-operative radiation of the periadrenal region due to RX resection of the periadrenal liposarcoma was also scheduled upon completion of the chemotherapy. Treatment with Trastuzumab and endocrine treatment was included according to the receptor status of the bilateral breast cancers. Moreover, radiation therapy after bilateral breast conserving therapy and radiation of the right upper arm was planned.\nFamily history revealed a paternal uncle who died at 22 years from a malignancy in the splenic region. No further information regarding this tumour was available. Additionally, the paternal grandfather was diagnosed with leukaemia at 70 years. The maternal grandmother died from colorectal cancer diagnosed at 51 years. Criteria for Li-Fraumeni-like syndrome according to Eeles and the updated Chompret criteria were met (Table ) []. Genetic analysis of TP53 was performed by sequencing genomic DNA isolated from peripheral blood leukocytes of our patient. A pathogenic, heterozygous germline mutation (p.Arg282Trp) was identified. Neither parent was shown to be a carrier of this mutation. Paternity was confirmed by using 15 high polymorphic Short-Tandem-Repeats. Taken together, these data indicate that the TP53 mutation in the patient occurred de novo, although a germline mosaicism in one of the parents cannot be excluded. We examined tissue from a different germ layer to identify possible somatic mosaicism in the patient. Analysis of DNA from the oral mucosa revealed the same heterozygous TP53 mutation identified in the leucocytes. Thus, although we cannot rule out somatic mosaicism, this was very unlikely. Predictive testing was performed in the 44 year-old sibling and in both daughters (aged 21 and 18 years) of the patient. All three female subjects were not carriers of the mutation. Analyses of BRCA1 and BRCA2 genes in the index patient did not reveal any pathological findings.\nAfter confirmation of LFS by molecular analysis, the decision of an interdisciplinary consulting panel was to restrict the planned radiotherapy to the location with the highest priority. This decision was based on the suspected radiosensitivity of individuals harbouring a deleterious mutation in TP53. Thus, radiation was scheduled for the periadrenal region with postoperative RX, however the initially intended radiotherapy of both breasts, as well as of the right upper arm was cancelled.\nThe patient was offered secondary, bilateral prophylactic mastectomy, to reduce the risk of recurrent breast cancer. Due to the risk of new primary tumours at other sites, the patient refused this option and opted for a close, post-treatment observation regimen. The surveillance program was adjusted to her elevated risk for other primary malignancies and secondary malignancies after radiotherapy. Biannual ultrasound examination of the breast, annual magnetic resonance imaging (MRI) of the breast, and mammography at larger intervals were recommended. Abdominal ultrasound and MRI were predominantly used to monitor the sites of sarcomas.\nIn June 2008, multiple nodular lesions were detected close to the left kidney by control MRI, inside and adjacent to the former irradiation region. The optimal response to several lines of treatment with chemotherapy was stable disease. A radical resection of the tumour mass was performed in June 2009. Histological analysis confirmed a recurrent, but well-differentiated liposarcoma. The short interval to radiation therapy of the left periadrenal region indicated resistance of the disease to radiation therapy.\nIn June 2010, MRI of the abdomen revealed local recurrence. As of February 2012, the patient has been under long-term treatment with trabectedine at a Karnofsky Index of 80%. No distant metastasis, new primaries or recurrences at other sites have since been identified. |
A 76-year-old Asian man was involved in a road traffic accident. He presented with neck and arm pain on his right side, but motor weakness and paralysis were not observed. His arm pain corresponded to the right C6 and C7 dermatomes.\nWe carried out an X-ray image examination of our patient’s cervical spine and diagnosed a slight cervical spondylolisthesis (Figure ). CT and MRI were not performed. Because our patient did not show motor weakness or paralysis, his neck and arm pain were treated conservatively. However, the pain did not change over the six weeks following the injury. We conducted further X-ray imaging, MRI and CT eight weeks after the injury (Figures a,b,c, and ). Plain X-ray film images obtained at this time showed increased instability at the C5 to C6 joint when compared with those taken immediately after the accident (Figure a,b,c). MRI revealed central spinal canal stenosis at the C5 to C6 joint and high signal intensity in the spinal cord on T2-weighted imaging (Figure ). A sagittal CT showed bilateral dislocation of facet joints (Figure ).\nWe planned posterior-anterior surgery eight weeks after the injury. We performed a partial resection of both C5 to C6 facet joints for reduction, using a posterior approach. Half of the C5 to C6 facet joint was resected on the right side, and one quarter of the C5 to C6 facet joint was resected on the left side.\nLateral mass screws on the left side were used for fixation, and bilateral local bone was grafted between the posterior surface of the C5 and C6 laminae. Lateral mass screws on the right side were not used for fixation because most of the lateral bone mass was resected. Because fixation was insufficient, we added an anterior approach by removing the C5 to C6 intervertebral disc, and grafted iliac bone into the C5 to C6 space. We used a titanium plate for fixation.\nOur patient gradually became symptom-free after surgery. He presented with a transient C5 palsy on his right side; however, he had recovered three months after the surgery. Plain X-ray film images obtained six months after surgery showed good stability (Figure ). MRI revealed recovery of the central spinal canal stenosis at the C5 to C6 joint and showed normal intensity in the spinal cord on T2-weighted imaging (Figures and ). |
63-year-old woman who had suffered from the right medial knee pain for 5 years and was not responsive to conservative treatment was admitted to our clinics. 30° varus-valgus stress X-ray indicated that the patient had an intact MCL and LCL. After the detailed physical examination and reviewing of X-ray images, it was decided that UKR would be the most suitable option for the patient with anteromedial knee osteoarthritis. After spinal anesthesia application and sedation, the UKR surgery was performed with a standard minimal invasive midline vertical incision and a medial parapatellar approach; the patella was removed laterally but not dislocated or everted. The patient received a medial partial knee implant with a mobile-bearing insert (medium size with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). Following the UKR surgery (), weight bearing was allowed as tolerated by the patient and a standard postoperative physiotherapy was started on the first postoperative day. The patient was discharged at postop 2nd day when she met the following criteria: independent ability to get dressed, to get in and out of the bed, and to sit and rise from a chair/toilet; independence in personal care; and mobilization with crutches. After discharge, a home-based exercise program was given to the patient. At postoperative follow-up, our patient acquired a full knee RoM in the postop 1st month and returned to independent daily activities without any external support in the postop 3rd month.\nAt postoperative 1st year after first UKR application, the patient fell down while getting on a public bus; this caused that the right knee of the patient was exposed to the valgus force vector. After that moment, the patient heard a pop sound and felt an incredible pain that prohibited the flexion and/or extension of the medial side of the right knee. And then she was admitted to our emergency department. The first evaluation was performed, and the patient was diagnosed with a grade 3 MCL rupture and the UKR insert dislocation (). Having completed the preoperative preparations, the patient was operated on the same day. After anesthetic administration, a surgery with a standard minimal invasive midline vertical incision and a medial parapatellar approach (to a previous incision site) was performed to change the mobile-bearing insert with the same size (medium-sized mobile-bearing insert with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). After having changed the mobile-bearing insert, the MCL structures were repaired and anchored to its femoral origin with a 5 mm titanium anchor. Following the surgery, weight bearing and full RoM with a hinged knee brace were allowed as tolerated by patient and a standard postoperative physiotherapy was started on the first postoperative day. Crutches were recommended for 2 to 3 weeks to enable the patient to regain a normal gait. The brace was used continuously for 4 weeks and thereafter during the day for 2 weeks. After the physiotherapy program administration, the patient was discharged at postop 1st day.\nThe patients were evaluated regarding pain intensity (Numeric Pain Rating Scale (NPRS)), active range of motion (RoM), and quality of life (Short-Form 12 Health Survey (SF-12 Health Survey)). Functional capacity was evaluated using the Iowa Level of Assistance Scale (ILAS), Iowa Ambulation Velocity Scale (IAVS), Hospital for Special Surgery (HSS) knee score, and Timed Up and Go (TUG) test. Rehabilitation program and outcome evaluation were conducted by one clinical physiotherapist at preoperative period (before the first UKR application), at discharge (postop 2nd day after the first UKR surgery), and at postop 2nd year (after 2 years from the MCL repair and the insert change). The evaluation results are shown in . |
A 5.5-year old girl had undergone surgical patch closure of a large secundum atrial septal defect with valvotomy of a moderate pulmonary stenosis at the age of 2 months; a mild aortic stenosis with a thickened tricuspid aortic valve was not corrected. She was born as the first child to non-consanguineous parents. The family history was negative for sudden cardiac death, but the maternal grandfather had epileptic seizures without fever in his youth. At the age of 12 months the girl suffered from a first afebrile seizure and at the age of 23 months from a first febrile seizure. Four months later she suffered from a series of afebrile seizures. Therefore valproic acid was started and maintained for 2 years. During this treatment she was seizure-free with normal electroencephalogram (EEG). After tapering valproic acid over a period of 2 months, short generalized paroxysmal discharges appeared on the EEG for the first time without clinical correlate. One year later she suffered from two further uncomplicated febrile seizures. Since then she is seizure-free without anticonvulsive treatment but the 24 h-EEG persistently shows subclinical absences. Since the intellectual and physical development of the patient is normal it was decided together with the parents not to restart the antiepileptic medication but to follow her up closely. Cerebral magnetic resonance imaging was normal. Clinical cardiologic, echocardiographic and neurologic examinations of the first degree relatives were normal except for the mother who showed epicanthic folds and hypertelorism. Unfortunately, the maternal grandfather did not consent with any investigations.\nAt inspection, she had bilateral epicanthic folds; broad eyebrows, a broad nasal tip and hypertelorism, however no ptosis or low-set ears (Figures \n and \n). Her body weight was 18 kg (25th percentile), head circumference 51 cm (50th percentile) and height 115 cm (75th percentile). She did not complain about any cardiac symptoms and did not suffer from heart failure. Twelve-lead electrocardiogram showed normal sinus rhythm alternating with an atrio-ventricular-nodal rhythm and an incomplete right-bundle-branch-block. Twenty-four-hour electrocardiogram showed sporadic ventricular and supraventricular ectopic beats. Echocardiography revealed a mild residual valvular pulmonary stenosis with moderate pulmonary valve regurgitation and mild aortic valve stenosis with mild aortic regurgitation. Left ventricular function was normal, however, extensive hypertrabeculation resulting in a two-layered structure of the myocardium was visible in the mid-ventricular and apical segments of the left ventricle, consistent with the diagnosis of LVHT (Figures \n and \n). Review of previous echocardiographic examinations disclosed that LVHT had been present already at age 2 years. As a primary prophylaxis for cardiac embolism aspirin 50 mg/d was started. Since she was symptom-free, no further cardiac medication was prescribed. At the latest follow-up investigation in September 2011, she was in a good cardiac and neurologic condition, no seizures had recurred and her intellectual development was normal. Echocardiography was unchanged.\nMolecular genetic analyses of the PTPN11, KRAS, RAF1 and SOS1 genes were negative. Array CGH using a 1 M oligonucleotide microarray platform (Agilent Technologies, Santa Clara, CA) was performed to screen genome-wide for submicroscopic deletions and duplications \n[]. Genomic positions are given according to genome-build hg18. Array CGH detected five previously not described CNVs as listed in Table \n. FISH analyses were not carried out because of the size of the CNVs. Regarding the duplications it could not be assessed whether they were tandem-duplications on the same locus or whether the duplicated fragment was inserted or translocated in another chromosome. By application of quantitative Real-Time PCR (qPCR) we could show, however, that none of the changes had developed de novo. All five CNVs are currently not listed in the Toronto Database of Genomic Variants (DGV), therefore, have to be considered as non-frequent variants in normal controls. Follow-up and testing of the index patient and her parents by qPCR revealed that all CNVs were inherited from one of the unaffected parents (maternal: deletion 1q42.3, duplication 3q26.32q26.33; paternal: duplication 14q32.11, deletion and duplication 20q13.33). We thus conclude that these changes are most likely not clinically significant CNVs but rare benign variants, although a reduced penetrance of inherited CNVs cannot be excluded. |
A 25-year-old female presented to the emergency department (ED) for evaluation of persistent productive cough of yellowish sputum over the last four week and mild exertional dyspnea over the last two years. Her past medical history was unremarkable and she took no regular medications. There was no personal or family history of multiple endocrine neoplasia type 1 (MEN1) syndrome. She was in no distress on presentation to the ED with a resting hemoglobin oxygen saturation of 97% while breathing room air. Her physical examination was remarkable for absent breath sounds and decreased tactile fremitus on the left middle and lower lung fields. No wheezing or stridor were heard. Laboratory data were within normal limits.\nA chest x-ray (CXR) in the ED demonstrated opacification of the left middle and lower lung fields, hyperinflation of the right lung and deviation of the trachea to the left (Fig. ). A computerized tomography (CT) scan of the chest showed complete left lung atelectasis due to a mass obstructing the left main bronchus and excessive mediastinal deviation to the left with substantial herniation of the hyperdistended right lung into the left hemithorax (Fig. ). There was no evidence of tracheobronchial narrowing in the right lung or esophageal compression. The mass was well demarcated and of soft-tissue quality, demonstrating homogeneous contrast enhancement, starting 2.8 cm distal to the main carina, measuring 4.4 × 2 × 2.8 cm (Fig. ). Abdominal and head CT scans showed no abnormal findings. The patient subsequently underwent a diagnostic flexible bronchoscopy which revealed a pale hypervascular polypoid mass completely obliterating the left main bronchus which was biopsied using forceps (Fig. ). Histopathological examination of endobronchial biopsies disclosed a carcinoid tumor with a Ki-67 index of approximately 10%.\nFollowing thoracic surgery consultation, an open left pneumonectomy with concurrent complete lymph node assessment and dissection was performed. During surgery, the left lung was found completely atelectatic with adhesions between the pericardium and the left pleura which were dissected. No attempt of repositioning the mediastinum or placement of tissue expanders was performed, due to the absence of airway compression in the right bronchial tree during previous bronchoscopy and CT scan. The patient recovered well after surgery and no complications were noted. Post-operative histopathology disclosed an atypical carcinoid with a Ki-67 labelling index of 10% but no areas of necrosis (Fig. ). There was a radical resection of all tumor with clear operative margins, the periphery of the left main bronchus was infiltrated by tumor, but there was no invasion of the visceral pleura, and no infiltration of resected lymph nodes from lymph node stations 5, 7, 9 and 10 by carcinoid cells.\nPre-operative spirometry was as follows: FEV1: 1.51 lit (44% predicted), FVC: 1.54 lit (39% predicted), FEV1/FVC: 98%. Spirometry and static lung volumes 12 months after surgery were as follows: FEV1: 1.93 lit (58% predicted), FVC: 2.34 lit (61% predicted), FEV1/FVC: 82%, TLC: 3.28 lit (63% predicted), RV/TLC: 118% predicted. Although spirometry appears to be significantly improved after surgery, spirometry before surgery triggered fits of coughing and therefore preoperative values might not be representative.\nPostsurgical follow-up has included the following: Initial chest CT scan was carried out 2 months after surgery. Parathyroid hormone (PTH) and prolactin levels were within normal limits 1 year after surgery. The following investigations were carried out at 6 months and then every 6 months for the first 5 years: Chest CT scan, abdominal ultrasound, chromogranin A measurement and standard laboratory testing including complete blood count, renal function, liver function, calcium and glucose. Abdominal CT scan and fiberoptic bronchoscopy were carried out 1 year after surgery and then will be carried out annually for the first 5 years. Bronchoscopy would be performed earlier for any symptoms or imaging findings suggestive of local progression. Repeat chest CT scans after surgery showed no changes in mediastinal rotation compared to those prior to surgery, and no signs of tracheobronchial or esophageal compression. Repeat bronchoscopy showed a normal-appearing surgical stump of left main bronchus and no airway compression of the right bronchial tree. The remaining studies listed above have been normal. The chronic mild exertional dyspnea reported by the patient before surgery completely resolved on hospital discharge, 7 days after pneumonectomy. The patient has been asymptomatic for the last 16 months after surgery with excellent performance status. |
Since March 2002, a 47-year-old woman without any history of sarcoidosis was regularly monitored in consultation for chronic viral hepatitis C (genotype 1). In April 2007, the alanine and aspartate aminotransferase serum levels have increased to 116 and 98 IU/l compared to normal values of 40 IU/l; the serum HCV RNA was 6.2×106 copies/ml. However, the physical and abdominal ultrasound examinations did not show any abnormalities. Because of the presence of biological cytolysis, a percutaneous liver biopsy was performed and revealed severe hepatic fibrosis (Metavir score A2/F3). Then, the antiviral treatment was started, and the patient received once a week the pegylated INF-2a at the rate of 180 μg that injected subcutaneously, and ribavirin 400 mg was administrated orally twice a day. The biological response was good, and the transaminases were standardized after 2 weeks of treatment.\nSix weeks after the beginning of the treatment, the patient noticed weight loss of 5 kg associated with dyspnea and progressive appearance of skin small firm nodules on both her upper and lower extremities. Chest X-ray was normal. However, thoracic computed tomography (CT) scan revealed pulmonary nodules associated with bilateral mediastinal lymphadenopathies, suggesting tuberculosis, lymphoma, and/or sarcoidosis. Abdominal CT scan was normal. The tuberculin skin test was negative. Afterward, bronchoalveolar lavage fluid was performed and showed an increased number of lymphocytes with a normal amount of eosinophils and neutrophils. The histological study of transbronchial lung biopsy revealed a patchy distribution of mild interstitial and perivascular fibrosis, without distinctive granulomas or significant inflammatory cell infiltrations. In addition, cultures for fungi, mycoses, and tuberculosis were all normal. Finally, a biopsy of the skin nodules was performed and found a noncaseating epithelioid granuloma formation strongly suggestive of sarcoidosis []. The serum angiotensin-converting enzyme was significantly elevated (130 U/l for the normal value <40 U/l); the diagnosis of sarcoidosis was retained and oral corticotherapy was started at the dose of 60 mg daily.\nFour days later, the patient suddenly presented a heaviness of the right upper limb predominant distally, associated with a right central facial paralysis and aphasia; however, she was lethargic and her ophthalmologic examination was normal. The cerebral magnetic resonance imaging (MRI) showed a gyriform and nodular left frontal and parietal subcortical enhancement associated with an important perilesional edema [Figures –]. INF-α and ribavirin therapies were discontinued, and intravenous bolus methyl prednisolone was then started at the rate of 10 mg/kg/day for consecutive 3 days; this was followed by prednisone (1 mg/kg/day). Nevertheless, no clinical improvement was noticed. On the contrary, the neurological state of the patient worsened rapidly and the patient died a week later. |
A 54-year-old male experienced a sudden onset of headache which had developed 2 days ago. After the initial diagnosis of subarachnoid hemorrhage (SAH) using brain computed tomography (CT) in another hospital, he was transferred to the emergency room where his Glasgow Coma Scale score was E4V5M5. Despite neck stiffness, there was no focal neurological sign. His medical history was non-contributory and laboratory blood tests found no abnormalities.\nA brain CT scan taken on admission showed focal SAH concentrated in the anterior portion of the basal cistern (). Digital subtraction angiography (DSA) confirmed the presence of the anterior communicating artery (ACoA) aneurysm and 3D rotational angiography (3DRA) showed that the aneurysm had a very narrow neck, which measured 5.9 × 2.7 × 1.7 (width × height × neck diameter) (). Based on the distribution of the SAH, we considered that the ACoA aneurysm had bled. As morphologic features of the aneurysm were adequate for coil embolization, we decided to perform an emergency endovascular coil embolization on the day of admission.\nThe embolization procedure was performed with the patients under general anesthesia. DSA was performed again to assure the aneurysm. After confirming that the result on DSA was identical to the previous one, a microcatheter was carefully guided over a microguidewire into the aneurysm. As the tip of the catheter was kept at the neck of the aneurysm, the first coil (Target® Detachable coil 3 mm × 8 cm; Stryker Neurovascular, Fremont, CA, USA) placement was attempted very slowly. At the last moment of deploying the first coil, the tip of the microcatheter was seen to move back from the aneurysm and the projection of the coil loop outside the aneurysm was observed (). Consequently, the first coil was gently withdrawn from the aneurysm. However, a subsequent angiogram showed near obliteration of the aneurysm without evidence of vasospasm. Only a tiny stump at the neck was noted (). Because acute thrombosis facilitated by an exposed coil within the aneurysm was assumed, and, sooner or later, recanalization could occur, we decided to insert a smaller coil into the remaining stump of the neck. With the coil (HyperSoft® 3D complex Coil 2 mm × 4 cm; MicroVention, Tustin, CA, USA), embolization was performed safely and post-embolization DSA showed complete occlusion of the aneurysm (). There was no occurrence of procedure-related complication during coil embolization and the patient recovered without neurologic deficit after the procedure.\nFollow-up DSA the next day showed persistent occlusion of the aneurysm (). The patient was discharged without neurologic deficit of the modified Rankin scale score 0. Magnetic resonance (MR) angiography at 3-month follow-up showed no evidence of residual neck or recurrence of recanalization. On 1 year follow-up DSA, despite change in the configuration of the coil, the aneurysm remained to be completely obliterated (). |
A 32-year-old Caucasian male presented with sudden onset of a chest and epigastric pain. His past medical history was significant only for a personal and family history of polycystic kidney disease. Upon arrival at the emergency department, the patient was hypoxic and tachycardic. Physical examination was significant for bilateral subcutaneous emphysema present over the lateral aspects of the neck. His abdomen was soft and not distended.\nA computed tomographic scan revealed air and fluid surrounding the esophagus (Fig. ). Esophagogram performed with water-soluble contrast media showed a distal esophageal perforation with a free leak into a large mediastinal cavity to the left of the esophagus (Fig. ). The patient was triaged directly to the operation room. A left chest tube was placed with improvement in his oxygenation, and grossly murky fluid was drained. Esophagogastroduodenoscopy (EGD) was performed next and revealed a very small caliber esophagus with concentric ringed appearance. A tight stricture was noted in the mid-esophagus, which only allowed passage of a pediatric gastroscope. In the lower esophagus, a 2-cm tear with necrotic edges was visualized 3 cm above the gastroesophageal (GE) junction. With this diffusely strictured and very diseased appearance of the esophagus, the decision was made to proceed with stent placement and thoracoscopic drainage of the mediastinum rather than primary surgical repair via thoracotomy. A fully covered, 15 cm × 19 mm, EndoMAXX® esophageal stent was successfully placed covering the perforation site. Mediastinal washout and drainage was performed subsequently via left thoracoscopy. A postoperative esophagram showed no leak and the patient was started on a liquid diet that was tolerated well. His hospital stay was uneventful, and he was discharged on twice daily high-dose proton pump inhibitor (PPI) on post-operative Day 5. He was placed on a mechanical soft diet on discharge. Repeat upper GI study with water soluble contrast postoperatively showed no evidence extravasation. (Fig. )\nA follow-up EGD was performed 6-weeks later for stent removal. Although the stent had migrated slightly, it continued to cover the previous perforation site. The stent was removed, and the esophagus appeared well healed with no residual tear. Contrast study showed no residual leak or significant stricture (Fig. ). On follow-up endoscopy, biopsies from the proximal and distal esophagus revealed 20 eos/hpf and 30 eos/hpf, respectively. The patient was kept on PPI and started on topical steroids and continued to do well with no reported symptoms on the last follow up 9 months and two years and a half after the initial presentation. |
A 76-year-old female admitted to hospital with a two-year medical history of type 2 diabetes mellitus, hypertension, and a diabetic foot ulcer on her left mid-foot that had not healed in over 4 months. The patient sustained a snake bite on the left midfoot at 6 years of age, resulting in a severe foot infection. She recovered from the infection but acquired a residual foot deformity. She has been suffering from numbness and pain in the left mid-foot for many years. To cope with the left foot pain, she attempted to bear more weight in the right foot but reduced activity than the right foot. Two years ago, she was diagnosed with type 2 diabetes mellitus. Subsequently, a recurrent chronic ulcer appeared at the site of the snakebite and for more than 4 months was difficult to heal.\nPhysical examination revealed an ulcer of approximately 1.5cm×0.5cm on the left medial midfoot with purulent discharge (). The ulcer had a 0.8cm deep cavity and a portion of bone was observed at the wound bed with a positive probe-to-bone test. The surrounding tissue was pale. There was no obvious pain and bleeding on the wound surface. The left foot was hypoesthesic and slightly swollen. Light touch sensation was intact in the left foot. The distal pulses, including pedal and posterior tibial pulses, of both feet were palpable.\nLaboratory investigation showed elevated random blood glucose of 14.3 mmol/L and hemoglobin A1c of 7%. The C-reactive protein level was 1.8 mg/L. The erythrocyte sedimentation rate was 17 mm/h. The ankle-brachial index greater than 1.3 in both legs suggested posterior tibial and dorsalis arteriosclerosis. Vibration perception threshold testing indicated severe peripheral neuropathy. Radiographic imaging of the left foot revealed changes in the distal part of the first and second metatarsal bones and abnormal bone morphology. MRI showed multiple bone marrow edema of metatarsal, navicular, cuboid, medial, and lateral cuneiform bones from the first to the fourth toes, indicating infectious disease. Doppler ultrasound results showed that the femoral, popliteal, anterior tibial, and posterior tibial arteries of both limbs were sclerotic, and that the veins were normal.\nThe patient was diagnosed as having diabetic peripheral neuropathy and diabetic peripheral vascular disease. Based on the University of Texas (UT) Diabetic Foot Ulcer Classification System, the classification of the wound on the patient’s left foot was UT Ш D. According to the International Working Group on the Diabetic Foot classification guidelines for diabetic foot wounds, the diabetic foot infection perfusion, extent, depth, infection and sensation grade was Grade 3. The Society for Vascular Surgery Wound, Ischemia, and foot Infection score was: wound 3, ischemia 0, foot infection 2, correlating to clinical stage 3. A series of standard medical treatments including anti-hypertensive treatment and antibiotics were administered, blood glucose control was optimized and peripheral circulation was improved. In addition, the wound was thoroughly debrided, including wound cleaning and removal of all infected and nonviable (necrotic or dead) tissue. Local dressing was performed simultaneously. Intravenous administration of vancomycin was commenced based on microbial culture and drug sensitivity test results from a wound secretion sample.\nAfter treatment for 6-weeks, there was increased exudation from the wound, new granulation formation slowed, and bleeding was minimal, all of which probably suggest lower limb ischemia. Since no individual medical specialty is able to manage all aspects of DFU, a multidisciplinary team in our hospital was launched to manage the patient. The infected dead bone was resected and inflammatory granulation tissues were surgically debrided until healthy granulation wound bed was observed. The space left by bone resection was filled with antibiotic-loaded bone cement (). The antibiotic-loaded bone cement was prepared by mixing 10 g of Palacos G Bone Cement powder (PALACOS® R+G, Heraeus Medical GmbH, Germany) and 0.4 g of vancomycin. Pathological diagnosis showed that nonviable skin, fiber, and bone tissue with a large number of necrotic bones was surgically removed. Eight days later, the vancomycin bone cement bead was removed. Fresh granulation tissue was present on the ulcer surface. The overlying fibrin tissue and excess hardened epithelized tissue were removed, and negative pressure wound therapy (NPWT) with intermittent pressure (−50 to −125 mmHg) was commenced to promote wound healing.\nAfter 5 days, wound bleeding could be observed on the ulcer surface. The appearance of fresh granulation tissues at the bottom of the cleaned wound was minimal. The exposed bone had been successfully closed although there was no significant reduction in the size of ulceration (). After full evaluation and informed consent was obtained, autologous platelet-rich gel (APG) was prepared as described in our previous study () and the gel was administered to the surface of the wound (). After 10 days, granulation tissues gradually grew in the ulcer cavity. However, considering the new granulation tissues were not enough to fill the ulcer cavity, the patient had to receive APG treatment once more. The wound was significantly improved after twice administrations for the treatment of APG ().\nComplete wound closure was achieved nearly 5 months after a combination of treatments (). The patient reported no side effects, and no complications were observed during the entire therapy period. There was no sign of ulcer recurrence at follow-up 1-month () and one-year () after wound closure. Considering her severe left foot deformity, the protection of epithelized, yet fragile, tissue is paramount to reducing the risk of recurrence. After a comprehensive foot assessment, we prescribed therapeutic footwear that had a demonstrated effect on the relief of plantar pressure. Biomechanical parameters, including gait and balance, were assessed before and after therapeutic offloading footwear were worn () (DaveMed LLC, Chongqing, China). After the next 6-month post intervention follow-up, the ulcer had not recurred. |
A 33-year-old married male patient came to our hospital for postchemotherapy surgical removal of seminoma. The patient was a known case of bilateral undescended testis. Patient developed a mass in the abdomen before four months, which on investigation by CT scan and a needle biopsy was confirmed as seminoma.\nThe CT scan showed a heterogenous lesion between the rectum and urinary bladder measuring 13 × 12 × 17 (AP × RL × CC) in size with multiple enlarged retroperitoneal, preaortic and mediastinum lymph nodes.\nNeedle biopsy [] showed nests of malignant epithelial cells separated by fibrous stroma. The cells are large with clear cytoplasm and distinct cytoplasmic borders. The findings were suggestive of seminoma.\nSubsequently two cycles of chemotherapy were given.\nPatient was referred to our hospital for the surgical management. On exploration, a mass was seen adherent to the posterior vesical wall, along with it on the right side was a uterine-like structure with bilateral fallopian tube. The mass surrounded the left iliac vessels and the sigmoid colon. The structures were dissected in total and were submitted for further histopathological evaluation. Grossly, [] the tumor mass was welldefined, measuring 7 × 6.5 × 4 cm. The cut surface was white-tan in color, and was firm. The uterus measured about 6.5 × 5 cm in size, with bilateral fallopian tubes. The right fallopian tube measured 7 cm and left 9 cm.\nTo the right of the uterus is tube like and cord-like structure, and at the end is testis measuring 2.5 × 2.5 cm.\nMicroscopically, the left testis showed seminoma with postchemotherapy changes [Figures and ] with individual cell necrosis, dense fibrosis with focal areas of lymphocytic infiltration.\nThe section from the uterus showed atrophic endometrium [] and measured 0.1 cm in thickness. The myometrium and the cervix were unremarkable. The right tube showed normal tubal histology [] while the left tube showed changes of hydrosalpinx. No ovarian tissue was found on either of the side. Right undescended testis was atrophic with tubular hyalinization and Leydig cell hyperplasia []. The right spermatic cord was identified and was unremarkable.\nA chromosome analysis revealed a normal male karyotype of 46 XY. |
The patient is a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility to be evaluated for CNS vasculitis. She was admitted to this outside facility for a four-week period prior to being transferred to the primary facility for further evaluation over a subsequent 23-day period. Total duration of hospitalization at both the facilities was close to 7.5 weeks. Approximately one week into the initial four-week admission, her family found that she was very lethargic with diminished responsiveness and pronounced difficulty speaking. In the emergency room (ER), her blood pressure was measured at 243/129 mmHg with a blood glucose value greater than 400 mg/dL. She was started on aggressive antihypertensive therapy and underwent a series of diagnostic tests. Dual antiplatelet therapy (DAPT) consisting of aspirin and clopidogrel was initiated in combination with high-dose atorvastatin. With respect to her lethargy and fluctuating cognition, there was concern that she may be experiencing complex partial seizures, so lacosamide was also started.\nA baseline computed tomography (CT) scan of the head without contrast showed multiple indeterminate lacunar infarcts involving the head of the right caudate nucleus and left corona radiata. The same day, a magnetic resonance imaging (MRI) was performed and elicited similar findings with the addition of bilateral punctate infarcts of the left thalamus, right periventricular white matter, and right centrum semiovale. Magnetic resonance angiography (MRA) done on the following day showed high-grade stenosis of the left middle cerebral artery (MCA), in addition to markedly diminished caliber of the right MCA and high-grade stenosis involving the left posterior inferior cerebellar artery (PICA). Bilateral carotid ultrasounds showed very mild plaques. An angiogram exhibited an occluded left posterior cerebral artery (PCA) distally and was also suggestive of advanced intracranial atherosclerosis (more so than would be expected in CNS vasculitis). There was no evident change from day two to day six of this hospital course. A spinal tap performed at the end of the first week demonstrated elevated protein and IgG synthesis rate (16.4), which was concerning for CNS vasculitis. Appreciating the contrast between the imaging and spinal tap findings, CNS vasculitis could not be ruled out. The patient was started on intravenous (IV) corticosteroids briefly, however, the medication was discontinued due to worsening hyperglycemia that was progressively difficult to control. Near the end of the third week of hospitalization, a repeat MRI showed a new small stroke in the left subcortical parietal white matter.\nThe patient was transferred to the primary facility after this initial month of hospitalization, at which time the patient had a National Institutes of Health Stroke Score (NIHSS) of seven. She was alert and oriented to person only and able to follow simple commands. Significant findings on subsequent blood testing revealed leukocytosis (12.2), elevated absolute neutrophil count (ANC) at 11.3, hyperglycemia (314 mg/dL), HbA1c of 9.6%, mildly elevated erythrocyte sedimentation rate (ESR) at 36, positive herpes simplex virus type one (HSV1), and the presence of IgG and hepatitis B core antibody (HBcAb). Workup for hypercoagulable state was negative for Factor V Leiden and antithrombin deficiencies, though notably protein C was elevated. A repeat spinal tap on hospital day one showed elevated levels of protein (122), but also demonstrated an elevation in myelin basic protein (6.09). Otherwise, the patient was afebrile and hemodynamically stable on admission. On hospital day two, rheumatology was consulted. In order to confirm the suspected diagnosis of CNS vasculitis, the specialist recommended a leptomeningeal biopsy and IV corticosteroids in the interim. Although angiography is very sensitive, it is nonspecific as it cannot distinguish between vasculitis and reversible cerebral vasoconstriction syndrome (RCVS). Consequently, the angiography that the patient had undergone earlier in her hospital course could not provide us with a definitive diagnosis, thus warranting the biopsy of the brain.\nA baseline transthoracic echocardiogram (TTE) obtained on hospital day three revealed a left ventricular ejection fraction (LVEF) of 57 +/-5 percent with mild dilation of the left atrial cavity. Repeat imaging showed much of the same findings, however, radiology recommended further workup for underlying CNS vasculitis. Over hospital day four to six, the working diagnosis was “multifocal bihemispheric strokes with no clear etiology with an encephalopathic process.” The patient’s cardiovascular risk factors continued to be treated and monitored (lipids, blood pressure, and sugars), and a more extensive rheumatological workup was ordered. Continuous electroencephalogram (EEG) monitoring on hospital day eight also showed evidence of diffuse encephalopathy although there were no epileptiform changes or seizures recorded. Over the second week of hospitalization, a new left cerebellar infarct was detected on MRI, at which time steroids were tapered down and a transesophageal echocardiogram (TEE) was ordered. The results of this echocardiogram were unchanged in comparison to the baseline TTE. There was no thrombus detected in the left atrium, ruling out cardioembolic etiology of the new stroke. The CT studies of the chest and abdomen were negative for any findings pertinent to the patient’s chief complaint.\nDuring the third week of hospitalization, a repeat head CT without contrast revealed additional recent infarcts. A four-vessel angiogram showed 50% stenosis in the petrous and cavernous segments of the left internal carotid artery (ICA), a completely occluded M1 segment of the left MCA, and multiple alternating foci of narrowing within the M2 and M3 branches of the right MCA as well as the P2 and P3 branches of the PCA. In consideration of these findings and given the fact that there is a considerable overlap between the imaging appearance of vasculitis and atherosclerotic disease, neither diagnosis could be excluded. A second rheumatology consult recommended that a leptomeningeal biopsy be considered prior to starting cyclophosphamide, effectively ruling in CNS vasculitis versus ischemic stroke. The neurosurgery team agreed to conduct the biopsy of the meninges and brain. However, after discussing the details of the procedure with the patient's family, her family decided against her having the procedure due to the risks associated with brain surgery and the debilitating neurologic deficits already suffered by the patient. |
This study was approved by the Ethics Committee of Orthopedic Surgery Department, Imam Khomeini Hospital, Tehran, Iran and a written consent was signed by the parents.\nA 7-yr-old boy, the only child of otherwise healthy parents was referred the Pediatric Orthopedic Clinic, Imam Khomeini Hospital, Tehran, Iran on July 2018 with the diagnosis of CP. The reason for referral was the parents’ concern about the increasing severity of disease despite regular occupational therapy.\nOn physical examination, the patient was developmentally delayed, unable to walk or stand, with obvious cognitional and gross and fine motor retardation. Flexion contractures were noted in elbows, wrists, knees, and hips. There was bilateral equinovarus deformity of feet and increased popliteal angle. Plantar reflexes showed extension response and DTRs were exaggerated. Spastic response of muscles was recorded after continuous stretching. Sitting balance was extremely unstable ().\nThe patient was the result of a consanguine marriage and normal pregnancy. Birth weight was 2950 gr and head circumference and height were 35 and 47, respectively. The few first months of his life showed normal weight gaining and development. He was able to hold his head in 5 months and roll over at 7 months age. The first time the parents had been told about the possibility of an abnormality was in a routine screening at 5 months age. The pediatrician noticed a decreased head circumference growth. Further investigation showed the head circumference reached a plateau (40 cm) in its growth around 12 months age (). His general and developmental condition seemed to experience a sudden pause with progressive delay in growth and development since then. He lost his ability to rolling over and never gained any gross motor milestones. His face became expressionless and his eyes started to sink into the orbits (). Other findings were: apparent cachectic dwarfism, microcephaly, loss of facial adipose tissue, pigmented retinopathy, thoracolumbar kyphosis, multiple joint contractures, senile appearance, photosensitivity, and thin and dry hair.\nAlthough physical examination had a lot of similarity to a patient with CP, the history was inconsistent with the diagnosis of CP in its almost all aspects. This made us reevaluate the diagnosis. After a thorough history taking, some clues were added to our knowledge which was critical to the correct diagnosis. These include rapid regression of all motor functions, regression of language and fine motor functions and facial changes which are not compatible with CP.\nAt 7-yr-old age, he was in a cachectic dwarfism condition. The progeroid appearance narrowed our differential diagnosis.\nOur first diagnosis based on clinical findings and progression of the disease was Cockayne syndrome. The diagnosis was later confirmed by molecular analysis for Cockayne syndrome. The patient was homozygous for ECCR6 gene (genotype: c.2551 T>A /p.W851R- c.2551 T>A /p.W851R). The parents were also heterozygous for the same gene. This was also true for the patient’s only sister. |
An 8 days old male neonate was born to an Asian mother through vaginal delivery at 37 weeks of gestation, weighed 2,380 g, and had APGAR scores of 9 and 10 at 1 and 5 min, respectively. He was admitted to our hospital with a 2 days history of fever of up to 39°C but did not have respiratory or gastrointestinal symptoms. The infant's family denied any medical history and TB contact. His physical examination at admission documented smooth respiration, clear breathing sound, and no hepatosplenomegaly. The complete blood count indicated a total white blood cell count of 17,500/μL with 69% neutrophils, 20% lymphocytes, 9% monocytes, and 2% eosinophils. The C-reactive protein level was 7.3 mg/dL. The findings of the cerebrospinal fluid (CSF) analysis were normal. Bacterial cultures of the blood, urine, and CSF were negative. Intravenous antibiotics, namely cefotaxime and ampicillin, were administered after admission on the basis of suspicion of neonatal fever. Despite the administration of the antimicrobial combination therapy, the fever persisted and the neonate developed abdominal distension when he was 12 days old. Abdominal radiography exhibited nonspecific dilated bowel loops. Because no improvement in the condition of the patient was observed after changing antibiotics, infection caused by some virus and other atypical pathogen, including Mycobacterium tuberculosis, was considered. Tests for herpes simplex virus, Epstein–Barr virus, cytomegalovirus, hepatitis B virus, rubella, Chlamydia trachomatis, and Toxoplasma gondii were all negative. The repeat C-reactive protein level was elevated to 14.4 mg/dL. Coagulopathy with 323.7 μg/mL of abnormal fibrin degradation product and more than 20 mg/L of D-dimer were also noted. Antibiotics were switched to vancomycin and ceftazidime empirically. Chest radiography displayed only increased right lung field infiltration when the infant was 12 days old (), and chest computed tomography (CT) imaging exhibited a large amount of right pleural effusion with mild inflammatory changes in the right lower lobe when the infant was 15 days old (). Pleural effusion drainage was suggested but refused by his parents at that time. Gastric lavages for acid-fast staining and culture were examined when the infant was 20 days old after his parents agreed to further testing, and one of the three acid-fast stains of gastric lavages yielded few acid-fast bacilli. Repeat chest and abdomen CT imaging performed when the infant was 24 days old indicated patchy consolidation in the right upper lung, multiple new nodules in both the lungs, moderate pleural effusion, and multiple low-density nodules in the spleen and hepatic hilar region without hepatomegaly (). Subsequently, pigtail catheter insertion for pleural effusion drainage was performed. The findings of pleural fluid analysis indicated a total white blood cell count of 10,800/μL with 6% neutrophils, 57% lymphocytes, and 37% mesothelial cells; a total protein level of 4.6 g/dL, a lactic dehydrogenase level of 250 IU/L, and a glucose level of 164 mg/dL. TB infection was strongly suspected. The neonate was administered isoniazid (15 mg/kg/day), rifampicin (15 mg/kg/day), and pyrazinamide (20 mg/kg/day) when he was 24 days old. After initiating anti-TB treatment, the neonate's symptoms and signs subsided gradually. Finally, both gastric lavage and pleural effusion cultures showed M. tuberculosis complex.\nThe neonate's mother was 33 years old, gravida 1, para 1. Her Group B streptococcus test was negative. She had been healthy with no previous medical history and TB contact history; however, she developed a mild dry cough 1 week after delivery, experienced persistent general weakness, and was admitted to our medical intensive care unit because of altered mental status 24 days postpartum. Laboratory examinations indicated leukocytosis, thrombocytopenia, coagulopathy, acute hepatic failure, and acute renal failure. The HIV serology test was negative. A chest X-ray exhibited a miliary TB pattern (). A chest CT image displayed diffuse interlobular and intralobular septal thickening with ground-glass opacities (). Because her neonate was highly suspected to have TB infection at that time, acid-fast staining and TB polymerase chain reaction (PCR) of the sputum were performed. Both tests were strongly positive. The mother was administered anti-TB therapy immediately, but she died 3 days after hospitalization. M. tuberculosis infection was confirmed through sputum culture. |
A 58-year-old man was referred to our hospital because of progressive gait disturbance and incontinence. The patient had undergone surgical clipping of a ruptured aneurysm at the right middle cerebral artery bifurcation in another institution 8 months earlier, and the case was complicated by a left-sided hemiparesis (grade IV) after surgery. At that time, an additional unruptured aneurysm at the left PICA origin had been diagnosed postoperatively by angiography. The PICA aneurysm had a wide neck and incorporated the PICA origin. The patient and his family were informed that the aneurysm posed a relatively high risk for rupture because the patient already had a subarachnoid hemorrhage from another aneurysm and the posterior circulation aneurysm could have a higher risk, because it would be very difficult to treat by either a surgical or endovascular approach.\nHydrocephalus was diagnosed with computed tomography (CT) and treated with surgical placement of a ventriculoperitoneal shunt system in our hospital, and an uneventful follow-up period of 31 months. After the Enterprise stent system (Codman Neurovascular, Miami Lakes, FL, USA) became available, which is easier to deliver through the tortuous anatomy than other available stents, we planned a stent-assisted coil embolization of the PICA aneurysm. A follow-up cerebral angiography demonstrated a wide-necked aneurysm with a medially directed distal lobule (). The aneurysm measured 6.1 mm in the long-axis diameter, 2.7 mm in the short-axis diameter, and 3.3 mm in height. There was no significant interval change in aneurysm size and shape. Because the left PICA coursed inferiorly, forming an acute angle with the proximal V4 segment of VA, it was decided that the Enterprise stent should be placed retrogradely with a contralateral (right) VA approach and coils should be placed into the aneurysm through the ipsilateral VA.\nThe patient was premedicated with 75 mg clopidogrel and 100 mg aspirin 72 hours before treatment. Under general anesthesia, we performed bilateral common femoral artery punctures. A Envoy guiding catheter (Codman Neurovascular, Miami Lakes, FL, USA) was introduced into the left VA and a 5 Fr Envoy into the right VA after administration of a 3000 U bolus of IV heparin. A "J-shaped", Prowler Select LP microcatheter (Codman Neurovascular, Miami Lakes, FL, USA) was then advanced through the 5 Fr Envoy guiding catheter into the right distal VA over a Synchro2 micro-guidewire (Boston Scientific, Natick, MA, USA) and navigated retrogradely into the left distal VA through the "acute-angled" VBJ with the help of a microcatheter tip shape (). After the micro-guidewire was exchanged with exchangeable Agility-14 micro-guidewire (Codman Neurovascular, Miami Lakes, FL, USA), the microcatheter was retrieved and the Prowler Plus Select microcatheter (Codman Neurovascular, Miami Lakes, FL, USA) was advanced into the left distal PICA across the aneurysm neck for stent delivery ().\nThrough this microcatheter, a 4.5 × 22 mm Enterprise stent was advanced into the left distal VA-PICA and placed over the aneurysm neck. Before stent deployment, a Prowler Select LP microcatheter was advanced through the ipsilateral VA and placed within the aneurysm. After partial coiling of the aneurysm dome with a 3 mm × 4 cm minicomplex Trulfill DCS Orbit detachable coil (Codman Neurovascular, Miami Lakes, FL, USA), the stent was deployed from the left proximal PICA to the distal VA (). Subsequently, three more detachable coils (3 mm × 4 cm, 2.5 mm × 3 cm, and 2 mm × 2 cm minicomplex Trulfill DCS Orbit coils) were delivered into the aneurysm for further packing. A final control angiogram showed near-complete aneurysm occlusion and preservation of the PICA and VA flow (). The patient recovered from anesthesia with an unchanged neurological status.\nClinical and angiographic follow-up at eleven months revealed complete occlusion of the PICA aneurysm () with no neurological change. |
A 69-year-old White man, having previously undergone right hemicolectomy for colon cancer and with no chronic drug use or any other comorbidities, was admitted to the emergency department of a level-2 hospital for bowel obstruction and was immediately referred to the operating theater to undergo laparoscopic viscerolysis. The postoperative course was complicated on the sixth postoperative day by obstructive cholangitis with jaundice in the presence of bile sand and suspected choledocholithiasis; accordingly, antibiotics were prescribed, and endoscopic retrograde cholangiopancreatography (ERCP) was scheduled to be performed a few days later. Nine days after surgery, he was in good clinical condition. ERCP was performed using a conscious sedation technique; however, during the procedure, the patient suddenly experienced hypoxemia, tachycardia, and hypotension. Because aspiration of gastric contents was diagnosed, tracheal intubation was performed immediately, fluid resuscitation and continuous infusion of norepinephrine was initiated, and the patient was promptly transferred to the intensive care unit (ICU). For the sudden onset of severe ARDS, the patient was sedated and paralyzed to start lung-protective ventilation (LPV). A few hours after ICU admission, the patient experienced further worsening of MOF, and ICU medical staff decided to refer the patient to a level-1 hospital with an ECMO facility. Approximately 6 hours after massive aspiration, the patient was admitted to the authors’ level-1 trauma center hospital, and the ECMO team provided a consultation service in accordance with institutional criteria.\nThe patient arrived at the first evaluation with the ECMO team having undergone maximal medical treatment including deep sedation with neuromuscular blockers, mechanical ventilation over the limits of LPV [], and continuous infusion of vasoactive and inotropic drugs to support circulation. Blood gas analysis revealed a pH of 6.9, a base excess of −11.4 mmol/L, a partial pressure of oxygen of 51.3 mmol/L notwithstanding a fraction of inspired oxygen (FiO2) of 100%, and a positive end-expiratory pressure (PEEP) of 10 cmH2O (Fig. ). Pulmonary compliance was only 21 mL/mbar.\nSystolic blood pressure fell to as low as 60 mmHg, and rapid ultrasound in shock (RUSH) [] revealed bilateral white lung and global systolic dysfunction, as described in the clinical documentation. |
A 46-year-old male presented with progressive, mostly postprandial abdominal pain, and significant weight loss of almost 44 lb. in a six-month period.\nHis medical history was notable for heavy smoking for the past 30 years and cholelithiasis. His medications included 40 mg esomeprazole once a day.\nFour months prior to presentation, he underwent an esophagogastroduodenoscopy (EGD) to evaluate similar complaints and was diagnosed with peptic ulcer disease (PUD) with positive HP. No improvement followed a course of a high dosage of esomeprazole and Helicobacter eradication therapy.\nOn admission the patient was stable, with a heart rate of 65 beats/min., blood pressure of 110/60 mmHg, and oxygen saturation of 98%. His physical examination was unremarkable except for epigastric tenderness and bilateral temporal and extremities wasting. Laboratory tests revealed normal CBC, kidney, and liver function. His C-reactive protein and erythrocyte sedimentation rate were also unremarkable.\nUpon admission, a review of a computed tomography (CT) scan was done in an outpatient setting and revealed fatty liver, thickened gastric wall (), and a hypodense area in the stomach; however no revision of the gastrointestinal arterial vasculature was done at that time. The patient underwent second EGD that revealed mildly erythematous antral mucosa. An immunohistochemical stain for HP was negative. Random gastric biopsies showed mild chronic active gastritis. The patient was discharged with the impression that he is suffering from active nonspecific gastritis. The dosage of esomeprazole was escalated to 40 mg twice a day. Due to the continuity of his symptoms, a third EGD was performed that showed hyperemic erythematous gastric mucosa with few longitudinal ulcerations that were located on the greater curvature on a preantral location. The pathology examination from gastric biopsies revealed a single focus of markedly atypical glands with necrotic material in the lumen, suspicious of a malignancy. A week later, a fourth EGD showed large ulcerations on the preantral greater and lesser curvatures () with hyperemic intervening mucosa. A pathological examination showed acute gastritis and duodenitis, reactive atypia, negative stain for HP, and no signs of intestinal metaplasia or malignancy.\nSix weeks later, he was admitted again due to worsening of epigastric pain and further weight loss, overall losing 66 lbs. over 9 months. During the third hospitalization, a diagnosis of ischemia was considered. A CT angiogram showed significant gastric pyloric wall thickening and surrounding small lymphadenopathy, several new splenic infarcts, and significant narrowing and obstruction in the origin of the superior mesenteric artery (SMA) and the origin of the celiac trunk with mild stenosis of the inferior mesenteric artery (IMA) origin (). A vascular surgery consult was obtained and open surgical revascularization was recommended given our patient's young age and lack of comorbidities. The patient underwent a surgical bypass with expanded polytetrafluoroethylene (ePTFE) graft between the right common iliac artery and the SMA with extension graft to the hepatic artery that resulted in significant clinical improvement and weight gain. Repeat EGD following the surgical bypass revealed mild gastritis and duodenitis with resolution of the gastric ulcers. |
An 8-month-old female child reported with history of seizures since the age of 4 days. She was born to a primigravida, at full term with normal vaginal delivery. There was nothing significant in the ante-partum history. The child looked well and active. Developmental milestones were normal. Head circumference was 42.3 cm (within normal limits). Fontanellae were normally palpable. There were no craniofacial abnormality, organomegaly or neurocutaneous markers.\nComputed tomography (CT) scan (head) was normal. EEG graph included normal drowsy and sleep-state patterns, which were consistent with the age of the patient. Background EEG frequency ranged between 2 and 5 Hz with amplitude of 150-200 μV. At places, sleep markers in the form of V-waves, K-complexes, and sleep spindles were also seen. There was no epileptiform activity.\nSeizure description included initial tonic motor activity, which was followed by asymmetrical facial movements and clonic activity. These were accompanied by uprolling of eye balls and unconsciousness, and the child used to get cyanosed during these episodes. The parents reported that the total duration of the seizure was between 5 and 15 min. Frequency of seizures varied from 3 to 4 per week to 1 per month. Parents were reassured regarding the benign nature and excellent prognosis in this disorder.\nThere was very strong family history of seizures involving nine cases in two successive generations []. Males and females were almost equally involved. The age of onset was remarkably constant. The seizure phenomenology and its occurrence on the 4th day of life were similar among all these cases and it resembled the description as noted above. The age of remission was also similar in five out of nine cases. In these cases, the remission occurred at the age of 1¼ months. In other two cases, remission occurred at the age of 1 year. One patient had remission at the age of 1¼ years. Another patient behaved differently, while he had initial remission at the age of 1¼ years, seizures recurred after 3 months though with different ictal manifestations. Presently, he is 22 years old and is continuing to get seizures. All the patients described above were neurologically normal. The index case presently 2½ years old also got remission of seizures at the age of 1 year and is having normal developmental milestones []. |
An 83-year-old woman with a history of basal cell carcinoma and squamous cell carcinoma presented to our clinic for evaluation of a right midback mass. She initially presented to her dermatologist with what appeared to be an inflamed sebaceous cyst, but after a failed trial of Keflex, a punch biopsy performed at an outside institution revealed dermal involvement by invasive, poorly differentiated carcinoma with epidermoid features. By immunohistochemistry, the tumor cells expressed high-molecular weight keratin, BerEP4, and EMA but were negative for P63, GCDFP15, estrogen receptor, progesterone receptor, TTF-1, CEA, cytokeratin 7, and cytokeratin 20. At this point, the etiology of her mass was unclear; however, it should be noted that the patient was 6 weeks post-op from a laparoscopic-assisted left colectomy at an outside institution for an obstructing, poorly differentiated colon adenocarcinoma with squamous differentiation and 3/25 lymph nodes positive for metastatic carcinoma (pT4aN1b). She declined chemotherapy. Her physical exam on presentation was significant for a 4 × 4 cm firm, nonpigmented mass with a centrally raised 2 × 3 cm area of erythema surrounding a punctate opening from the recent biopsy with serous drainage (). We ordered a PET scan prior to the excision in order to identify the primary tumor. PET scan was remarkable for a large intensely FDG-avid cutaneous/subcutaneous malignant mass in the right midback with FDG-avid right axillary nodal involvement, focal FDG avidity at the left colon anastomotic site, an intensely FDG-avid left peritoneal node in the left lower quadrant adjacent to the surgical bed suspicious for nodal involvement from the colon primary, and an FDG-avid left lateral breast hyperdense mass. The patient subsequently underwent a wide local excision of the cutaneous mass on her back with 1 cm margins and a right axillary sentinel lymph node biopsy in which 3 lymph nodes were removed. Pathology report showed poorly differentiated carcinoma with squamous differentiation, negative margins, and 1/3 lymph nodes positive. Histologically, infiltrating cords and solid nests of malignant epithelial cells were noted ().\nThe patient underwent additional workup for the PET findings. The patient returned to the clinic and reported that her last mammogram was performed over one year ago but was negative for malignancy. She denied breast skin changes, nipple discharge, and noticeable breast masses. Physical exam revealed a round, mobile palpable mass at 1 : 00 approximately 5 cm from the left nipple-areola complex. While the breast mass appeared benign, a bilateral diagnostic mammogram and left breast ultrasound were ordered to rule out malignancy in light of her current presentation of carcinoma with unknown origin. Mammography confirmed a suspicious mass in the left breast, and pathology from the ultrasound-guided biopsy revealed invasive ductal carcinoma, grade 3 (ER 0%, PR 0%, HER-2 1+, Ki-67 80%). She underwent a left lumpectomy with pathology confirming triple-negative invasive ductal carcinoma with associated high-grade DCIS and margins negative for both invasive and in situ disease, staining positive for mammaglobin. There was no suspicion that this was a colon metastasis. She elected to omit sentinel lymph node biopsy, radiation, and breast-specific chemotherapy.\nThe final pathology of the back mass continued to be a diagnostic dilemma. To further characterize the origin, the histologic sections from the back and colon specimens were sent for an expert opinion at another institution. A panel of newly performed immunostains showed the neoplastic cells to be positive for CK20 and negative for CDX2, CK7, p63, GATA3, and mammaglobin, which is compatible with a metastasis from colonic primary. The patient was discussed at our multidisciplinary tumor board. We recommended a colon-specific chemotherapy regimen, which the patient declined. She returned to the clinic 4 months after the initial back mass excision and reported pruritus of her right back along the incision line with an associated lump. She also noted a lump in her right axilla, which was painful with arm movement. Exam revealed a well-healed right upper back scar with a 1.5 × 1.5 cm raised flesh-colored nodule with bluish discoloration proximal to the scar at the midpoint, as well as a well-healed right axillary scar with a firm palpable nodule 5 cm inferior to the scar. The patient was offered a wide resection in the OR, but due to the severe pruritic nature of this lesion, she preferred narrow excision in the office for immediate palliation and diagnosis. Pathology revealed this tissue was morphologically identical to her previous skin lesion, confirming a metastatic poorly differentiated carcinoma of colon primary. Caris testing was performed, and there were no actionable mutations.\nThe patient represented to medical attention one month later with a large bowel obstruction. Imaging showed recurrence at the colonic anastomosis, and this was managed nonoperatively. At this point, the patient agreed to palliative chemotherapy. She completed one cycle of capecitabine and oxaliplatin but tolerated this regimen poorly and was readmitted to the hospital twice. Subsequently, she declined any further treatment and went home on hospice. |
The proband was a 25-yr-old woman, the first child of healthy first cousin parents (), with a height of 156 cm and a body weight of 56 kg. She complained of sparse hair and no menstruation. Her mother’s pregnancy, delivery, and feeding history were normal. Her younger brother had similar symptoms, such as alopecia and gonadal agenesis. She had another younger brother died at 7 mo, so his genetic analysis could not be investigated. It is unknown if the cause of death was related to the mutation of this gene. In addition, generations I and II above the patient’s parents died, and genetic analysis could not be performed (). During her childhood, her parents noticed that her hair was sparse compared to other children’s. In adolescence, she did not show secondary sexual characteristics. Menarche did not occur. Her previous consultations in other hospitals showed that she had abnormal blood glucose, intellectual disability, dysaudia, an immature uterus and ovaries with few follicles, and her bone age was delayed by 9 yr. Over a year of progynova treatment, she had two menstruations. Then, the patient stopped taking the medicine and no longer menstruated. Physical examination on admission showed that her hair was sparse, especially on both temporal sides and forehead. Her face was progeric, with a large forehead and prominent ears. Secondary sexual characteristics were incompletely developed. Axillary or pubic hair was not present, and minimal breast budding was present with marked vulvar hypoplasia (). Unlike some patients in the early literature (), the patient and her younger brother did not have extrapyramidal movement such as dysaudia, dyskinesia with oropharyngeal swallowing disorder, or trouble walking. The patient’s Mini-mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) scale scores were 22 and 19 respectively, which confirmed that she had mental retardation. Interestingly, we did not find abnormal extravertebral movement in this patient or her younger brother, which is common in other patients (). The neurological examination showed that the patient had no dysarthria, dysphagia, tremor and abnormal muscle tension; her coordination movements, posture and gait were also normal; At the same time, her muscle strength was normal and Babinski sign was negative.\nSex hormone examinations disclosed low estradiol level (<5 pg/ml, reference range: 12.4–233 pg/ml) and low testosterone level (<0.025 pg/ml, reference range: 0.07–0.78 pg/ml). Her follicle-stimulating hormone level, prolactin level, and luteinizing hormone level were in the normal range. Other hormone investigations revealed high thyroid-stimulating hormone (6.36 uIU/ml, reference range: 0.27–4.2 uIU/ml), low free thyroxine (7.39 pmol/ml, reference range: 12–22 pmol/ml), and a normal free triiodothyronine level. These data were suggestive of subclinical hypothyroidism. She had a low insulin growth factor 1 (IGF-1) level (45.7 ng/ml, reference range: 69–200 ng/ml) and a high level of HbA1c (8.8%, reference range: 3.6–6%) (). Her C-peptide release test results were as follows: 0 min: 2.25 ng/ml, 30 min: 2.43 ng/ml, 60 min: 2.99 ng/ml, and 120 min: 5.58 ng/ml. A transabdominal ultrasound showed an immature uterus and small ovaries. Her breast ultrasound found tiny gland tissue in the bilateral breasts. A hearing assessment disclosed bilateral mild sensorineural deafness. A left wrist X-ray showed epiphyseal closure. An electrocardiograph (ECG) was unremarkable. Magnetic resonance imaging (MRI) revealed a small pituitary, partially empty sella, no iron deposits in the globus pallidus, and multiple intracranial white matter hyperintensities that may be of vascular origin (). As mentioned above, the patient’s clinical features, metabolic disease, neurological findings, ectodermal appendages, and laboratory tests are summarized in .\nAfter obtaining informed consent from the patient and her family, peripheral blood was sampled from the patient, her younger brother, and their parents. In order to better describe identification of the variant, we have added more detailed steps for genetic testing, data screening and pathogenicity analysis based on previous articles published after the company’s WES technical services (; ). We isolated nucleic acid to capture DNA fragments, then constructed a library, using biotinylated probes to target and capture exons, and amplify the captured targets, qPCR was used for quality control and sequencing, and to analyze the captured information. We analyzed DCAF17 mutatifons and their pathogenicity. We used the MGI-T7 platform (BGI) for sequencing at a depth of 200× and a target coverage of 99.57%. Forward primer: 3′-CAGAATCTCCGAATTTGAAGGAG-5′, reverse primer: 3′-TCTTTAAATCTGAAATGTACATGGG-5’. Her parent’s and younger brother’s samples were tested via Sanger sequencing for verification (). With pathogenicity analysis (), we detected the pathogenic locus (). The patient’s DCAF17 harbored a homozygous mutation c.1111delA, p.(Ile371Term), her younger brother had the same mutation, and her parents were heterozygous for the mutation at this site. The c.1111delA mutation is a nonsense mutation leading to a premature stop codon p.(Ile371Term) (). |
An 82-year-old male was admitted to the emergency department for worsening shortness of breath and hypoxia. He was admitted a week after he was diagnosed with a left ninth rib fracture secondary to a fall. He had long-standing history of chronic obstructive pulmonary disease, coronary artery disease, and peripheral vascular disease. Chest radiographs revealed a left pleural effusion and possible infiltrate. The patient was initially treated with a nebulizer, prednisone, and empiric antibiotic coverage with ceftriaxone and azithromycin. The patient failed to improve with the medical interventions and a therapeutic thoracentesis was performed. The thoracentesis was completed with ultrasound guidance, with the puncture made above the 11th rib at mid chest on the left. The pleural effusion was found to be frank blood. No immediate complications were noted, and the patient was taken to recovery. The next day the patient was found to be in respiratory distress. A chest x-ray revealed an opaque left hemithorax that was likely rapid accumulation of pleural fluid (Fig. ). A follow-up contrast-enhanced computed tomography (CT) of the chest performed during the arterial phase revealed a left intercostal pseudoaneurysm with hemothorax and adjacent compressive atelectasis (Fig. ). Ultrasound of the left chest wall was performed (Fig. ) directly over the thoracentesis site and doppler flow revealed bidirectional fluid flow, indicating the presence of a large pseudoaneurysm (Fig. ).\nFollowing identification of the left intercostal pseudoaneurysm, the patient underwent a thoracic aortogram and multiple-level left intercostal angiogram (Fig. ) under IV conscious sedation. Selective catheterization of the T5, T6, and T7 intercostal arteries was unsuccessful in identifying the pseudoaneurysm. Selective catheterization of T10 and T11 intercostal arteries was performed with a C2 Cobra catheter, following multiple catheter exchanges due to the patient’s atherosclerotic vessels. The pseudoaneurysm was ultimately found to have a left T10 origin and the C2 Cobra catheter was exchanged for a microcatheter. Once access was gained, coil embolization of the pseudoaneurysm was performed with a series of 15 Axium micro coils. Significant room was left on both sides of the pseudoaneurysm and a follow-up angiogram was performed via the microcatheter, then a 5-French Cobra catheter. The follow-up angiogram demonstrated no further filling of the pseudoaneurysm (Fig. ). The catheter was removed and a Perclose device was placed in the left groin for hemostasis. Following completion of the procedure, the patient was taken to recovery. The patient proceeded to return to his baseline following medical management during the remainder of his hospital stay and was discharged home after 5 days. |
We report a 53-year-old man who was previously well, developed fever and malaise and on evaluation was found to be positive for SARS-CoV-2 by RT-PCR. He was hospitalized for isolation and observation. His hemogram and metabolic parameters were within normal limits. However, he had systemic inflammation as evidenced by elevated C-reactive peptide (CRP) levels to the tune of 275 mg/L. He was treated with supportive care and prednisolone (30 mg per day) for a week. He had no infiltrates on the chest x-ray. He became afebrile in a few days and over the next two weeks, his CRP levels reached the normal range. On the 17th day after the onset of symptoms, he developed left foot drop; paresthesias over the dorsum of the left foot and the ulnar border of the left upper limb. He reported blurring of vision in the right eye and his acuity was reduced to 6/24 in the right eye. He was not able to dorsiflex his left ankle (MRC 1/5). The rest of the muscle groups had normal power. His deep tendon reflexes were absent except for normal knee and triceps jerks. Postural tremors were observed in the outstretched hands, suggestive of neurogenic tremors seen in demyelinating neuropathy.\nHe underwent nerve conduction studies which revealed prolonged latencies and conduction block in the left peroneal nerve with reduced SNAPs in the left ulnar nerve (Table ).\nThese features were suggestive of multifocal demyelination. The drop in compound muscle action potential along with conduction block is suggestive of demyelination and not axonal loss. Electromyography was not performed as the patient had a conduction block suggesting a demyelinating pattern. Mononeuritis multiplex can also present with multifocal neuropathy, but it is characterized by an axonal pattern on nerve conduction studies.\nFurther testing for etiology of demyelinating neuropathy was done with IgG and IgM anti-ganglioside antibody profile which was negative. Serum protein electrophoresis and immune fixation electrophoresis did not reveal any monoclonal band. Quantitative kappa and lambda free light chain assay were normal with a free kappa to lambda ratio of 1.11. Anti-aquaporin-4, Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies, anti-nuclear antibody (ANA), antibodies against extractable nuclear antigens and anti-nuclear cytoplasmic antibodies (ANCA) were negative, as were hepatitis B surface antigen (HBsAg) and anti-hepatitis C virus (HCV) antibodies. Serum ACE levels were normal and cryoglobulins were negative. MR neurography and MRI brain including optic nerve imaging were done 10 weeks after onset of initial symptoms including which did not reveal any abnormality. Cerebrospinal fluid analysis was not performed.\nTreatment\nHe was treated with a short course of steroids for two weeks (30 mg oral prednisolone for two weeks followed by tapering by 5 mg weekly), which was started two days after the left foot drop, with which he had a near-complete recovery.\nOutcome and follow-up\nThere were no new deficits. Follow up at 10 weeks after the onset of symptoms, he had recovered significantly. His tremors resolved, his left ankle power both plantar and dorsiflexion improved to 4/5; he was able to stand on his heels, however, not able to walk on his heels. The sensory symptoms resolved completely. His visual acuity also reached a baseline of 6/6 on the right and 6/9 on the left eye. His nerve conduction studies also showed improvement (Table ). At 10-month follow-up, all his symptoms had completely resolved. |
An ~3-year-old female, spayed mixed breed dog, weighing 18.5 kg, was presented for a draining tract in the left TMJ area. The draining tract was associated with extensive scar tissue, reduced mandibular range of motion, progressive oral pain, and frequent head-shaking. The dog was adopted 4 months before presentation after being rescued. Since the adoption, the dog was treated for the draining tract with multiple antibiotics with no apparent resolution of the condition.\nOral and maxillofacial examination revealed a moderately atrophied temporal and masseter musculature, substantial scar tissue, and contraction of the skin on the left side of the face. In addition, a draining tract with hemorrhagic purulent discharge at the left TMJ area was noted (). Multiple tooth fractures were also noted as well as mild generalized dental plaque and calculus accumulation. It was estimated that mouth opening was ~75% of normal opening on both an awake examination and an examination under general anesthesia. Complete blood count, serum biochemistry, and urinalysis were within the normal limits. Ultrasound of the left TMJ area revealed potential osteomyelitis of the zygomatic arch with suspected sequestrum and regional cellulitis with possible TMJ involvement.\nPre- and post-contrast CT images of the skull were obtained as described earlier. Severe chronic septic arthritis of the left TMJ, characterized by full destruction of the articular surfaces as well as osteomyelitis of the zygoma and sequestrum formation, was noted. In addition, secondary cellulitis, abscessation, and draining tract (), as well as enlarged, likely reactive, left mandibular and medial retropharyngeal lymph nodes and chronic otitis at the left ear, were noted.\nA lateral approach to the left TMJ was performed as described earlier. In addition to the procedure described above, zygomectomy of the affected bone was performed with piezosurgery (Piezotome® Cube, Acteon, Mérignac, France) from caudal to the orbital process (and just rostral to the lytic bone defect) and to the rostral aspect of the mandibular fossa (not penetrating the joint). Once the infected and grossly necrotic zygomatic arch was removed, the approach to the TMJ was continued. The removed tissues were submitted for culture and sensitivity testing and histopathology. The dog was discharged with the following medications: fentanyl transdermal patch (50 mcg/h), clindamycin (23 mg/kg, q 12 h until sensitivity results were available), gabapentin (10–20 mg/kg q 8–12 h), and carprofen (2.2 mg/kg q 12 h for 14 days). The owner was informed that the medical therapy might need to be modified based on the culture and sensitivity results.\nHistopathological evaluation revealed lymphoplasmacytic, histiocytic, neutrophilic fasciitis, osteomyelitis, and osteonecrosis (). Culture and sensitivity analysis yielded a small number of P. canis. Anaerobic culture identified a moderate number of mixed growths, including beta-lactamase negative Bacterioides/prevotella species, and a small number of Peptostreptococcus anaerobius species. Given the culture and sensitivity results, and in consultation with a medical pharmacologist, administration of clindamycin was discontinued, and administration of amoxicillin/clavulanic acid was started for 6 weeks (14 mg/kg, q 12 h), along with metronidazole at a dose of 14 mg/kg q 12 h for 6 weeks. The dog was evaluated at 6 days and 3 weeks postoperatively, and was found to have substantial improvement and resolution of the draining tract. No pain was noted on palpation and the dog was noted yawning multiple times without perceived pain.\nAn oral and maxillofacial examination was performed at the 4-month recheck appointment, and CBCT images of the skull were obtained (). The dog exhibited complete resolution of clinical signs, with no recurrence of the draining tract, and increased mouth opening as visually compared to pretreatment (gape angle 49.6 degrees, interincisive measurement 75 mm). CBCT demonstrated static to slightly improved articular surfaces of the left TMJ with persistent irregular articular margins of both the mandibular head of the condylar process and the mandibular fossa. |
A 67-year-old Japanese woman was admitted to our hospital with a duodenal tumor that was incidentally detected on upper gastrointestinal endoscopy for the preoperative examination of sigmoid colon cancer. Endoscopy revealed a slightly elevated lesion at the minor papilla. Histopathologic examination of the biopsy performed by the previous doctor revealed class IV disease. She had no specific symptoms, and there were no abdominal findings on physical examination. The patient's medical history was sigmoid colon cancer and hypertension. Her family history was unremarkable. Results of laboratory tests, including pancreatic tumor markers, were normal (Table ). Ultrasonography (US) failed to detected this lesion. The main pancreatic duct and bile duct were not dilated. Abdominal computed tomography (CT) revealed a 1.0-cm-sized enhancing mass in the minor duodenal papilla with no apparent distant metastasis (Fig. ). Magnetic resonance cholangiopancreatography showed low intensity on a T1-weighted image and no dilation of the main pancreatic duct or bile duct (Fig. ). Endoscopic US (EUS) detected a hypoechoic lesion in the submucosal layer (12 mm in diameter). EUS clearly showed the muscle layer; thus, we determined that the tumor did not invade the muscle layer (Fig. ). Endoscopic retrograde pancreatography findings were normal (Fig. ). We attempted to evaluate the degree of tumor progression using intraductal US (IDUS) through the minor duodenal papilla; however, it was difficult to insert and thus could not be evaluated. Endoscopic sphincterotomy (EST) was performed to enable histological examination of the deeper layer and IDUS insertion; however, histological results were atypical and IDUS insertion remained difficult. A histological examination was performed before treatment, whereas biopsies from the minor papilla demonstrated the histology of adenoma with mild atypia.\nWe determined that the tumor was not invasive; additionally, the patient and her family did not request surgical treatment. Therefore, we performed endoscopic mucosal resection (EMR) after obtaining appropriate written informed consent (Fig. ). First, we performed marking using argon plasma coagulation around the tumor. Next, the lesion was completely elevated by a submucosal injection of saline and then removed using a snare. The excised specimens were collected using a net. No adverse events, such as bleeding or perforation, were observed.\nPapillary proliferation of atypical columnar epithelium cells was observed from within the pancreatic duct of the minor duodenal papilla to the duodenum's surface. Atypical cells had enlarged nuclei with nucleoli. Structural heteromorphism was conspicuous. In the same site, MIB-1 was more frequently positive than the surrounding tissue, while p53 was weakly positive. On immunostaining, MUC-5AC was positive in the surface layer and MUC-6 was positive in the deep layer, suggestive of a gastric-type cancer. Histopathologic examination revealed adenocarcinoma in adenoma and confirmed complete resection (Fig. ). We followed up with yearly upper gastrointestinal endoscopy and CT. Three years after EMR, the patient remains asymptomatic without signs of recurrence. |
Our patient was a 19-year-old woman who was the restrained driver in a roll-over accident at highway speed. Her Glasgow coma scale was 4 with extensor posturing. She was sedated, paralyzed, intubated, and transported to our hospital as a level 1 trauma. Her medical history was unremarkable.\nCT imaging of the head revealed multiple punctate hemorrhagic lesions, early effacement of the gray-white junction and diffuse traumatic subarachnoid hemorrhage (Figure ).\nAn intraparenchymal ICP monitor was placed with initial readings of 23–25 mmHg. Supportive care was instituted with midline placement of the head and elevation of the head to 30°, sedation with propofol and analgesia with fentanyl. She required bolus doses of 20% mannitol for ICP surges with physical stimulation. On hospital day 3 she developed refractory intracranial hypertension and fever which persisted through hospital day 12 despite aggressive medical management. She was very sensitive to any physical stimulation, particularly changes in position or tracheal suctioning which resulted in ICP spikes. Osmotic therapy including alternating boluses of 20% mannitol and 23% saline were used to treat ICP surges. Maintenance fluids were changed to 3% saline solution in order to induce mild hypernatremia and decrease the need for rescue osmotic therapy. We induced mild hypothermia (35°C) using the Arctic Sun device (Bard, Inc., Atlanta, GA, USA) as an adjuvant to osmotic therapy with pharmacological neuromuscular paralysis to control the shivering response. Several attempts at reducing the degree of metabolic suppression by slowly increasing the core body temperature and lightening the degree of neuromuscular paralysis and propofol requirement failed. She consistently required a temperature under 35°C and a dose of Propofol greater than 40 mcg/kg/min to maintain control of her ICP. A repeat CT scan on hospital day 11 (Figure ) did not show significant cerebral edema to explain the persistent and refractory intracranial hypertension. It was noted that despite adequate hydration and apparent intravascular euvolemia, she was consistently tachycardic with intermittent periods of hypertension. This raised the possibility of sympathetic hyperactivity for which she was started on propranolol with only marginal decrease in the heart rate. On hospital day 12 a bolus of 4 mg of morphine was administered following a rise in ICP to 30 mmHg in an effort to determine if the elevation in ICP was due to a sympathetic surge. Immediately following the administration of morphine the ICP rose steadily from a baseline of 30 mmHg up to 55 mmHg over 3 min without any appreciable change in heart rate or blood pressure. The transient but significant rise in ICP was easily managed with hyperventilation and administration of 1 g/kg of 20% mannitol. Following this unexpected surge in ICP after administration of morphine, the fentanyl infusion she had been receiving since hospital day 1 was discontinued with an ensuing decline in ICP over the next 24 h which reached single digits for the first time since monitoring was started. No new interventions were introduced during this period. Parallel with the normalization of ICP was a resolution of her persistent sinus tachycardia and intermittent arterial hypertension. Permitting transient increases of ICPs up to 30 mmHg after stimulation, we were able to rapidly resume normothermia (36.5°C), discontinue neuromuscular paralysis, and transition from propofol to a low dose of midazolam without the need for rescue doses of hyperosmolar therapy (Figure ). Before discontinuation of the ICP monitoring, her ICPs had steadily settled below 15 mmHg. |
A 60 year old female presented to us with a chief complaint of swelling on the right maxillary posterior region of jaw since 3-4 years. It was sometimes associated with draining yellow color fluid which was salty in taste. She also gave a history of the slow growth of the mass since last 3-years to reach the present size. There were no other symptoms and no history of pain. Her medical history was noncontributory.\nThe oral examination showed the presence of a solitary pedunculated mass of size 4 cm × 2 cm × 3 cm which was red in color with lobulated surface on the right maxillary posterior alveolar ridge. The overlying and surrounding gingiva was inflamed []. She was partially edentulous with only four teeth remaining in the oral cavity. On palpation, the mass was firm, nontender, and not fixed to underlying structure. Orthopantomogram (OPG) evaluation showed the presence of a unilocular radiolucent area extending superiorly into the corresponding maxillary sinus, posteriorly to maxillary tuberosity, and anteriorly until the distal end of canine. Few areas of radio-opacities were also seen []. Dento-alveolar scan did not show the extension of mass into maxillary sinus, rather showed the inflamed lining of the sinus. The floor of the maxillary sinus was intact []. A three dimensional construction image done after dento-alveolar scan confirmed the calcifications [] within the mass which was seen as radio-opaque areas in OPG. After clinical and radiographic investigations POF, peripheral giant cell granuloma (PGCG) and pyogenic granuloma (PG) (longstanding) were considered in differential diagnosis.\nA comprehensive explanation was given to the patient and sign was taken on the consent form. Excisional biopsy was carried out, and the tissue was sent for histopathological examination for confirmatory diagnosis. Macroscopically, the gross specimen was measuring 3.5 cm × 2 cm × 1 cm, creamish brown in color, firm to hard in consistency with lobulated, and pebbly surface. The specimen was cut into two halves, and most representative areas were taken for processing [].\nThe specimen was fixed in phosphate-buffered neutral formalin for 1 day, subsequently, five-micron paraffin sections were obtained and stained with hematoxylin and eosin (H and E). Microscopic analysis of the H and E section showed parakeratinized stratified squamous epithelium covering loosely arranged highly cellular connective tissue stroma []. Connective tissue stroma shows few areas of ossification []. Few vascular spaces of varied sizes and inflammatory infiltrate were also seen in underlying connective tissue. Based on these histologic features, the final diagnosis was given as POF. |
A 23-year-old man (weight 65 kg, height 175 cm, and BSA 1.8 m2) with a diagnosis of primitive right atrial enlargement from foetal age was referred to our Centre for cardiological evaluation. Cardiac examination showed increased heart size on percussion and a grade II/VI Levine systolic murmur. No significant pathological findings were found on pulmonary examination. Electrocardiography showed a regular sinus rhythm with a rate of approximately 60 beats/min associated with an abnormal morphology and duration of P wave (enlargement of P wave with duration of 130 msec), together with a low amplitude of QRS complexes in the limb leads. All routine laboratory studies were within normal limits. Chest radiography showed an abnormal cardiac silhouette with increased convexity in the lower half of the right cardiac border and cardiomegaly ().\nTransthoracic two-dimensional echocardiography demonstrated a huge right atrium of about 6.2 cm and a volume of 230 ml/m2, with a thick smoke pattern and mild tricuspid regurgitation. The pulmonary arterial pressure was normal (). The tricuspid valve was normal without significant annular dilation. No stenosis or abnormal displacement of the tricuspid valve leaflets was detected. No significant regurgitation of the tricuspid valve was found despite a partial distortion of the anterior leaflet and compression of the right ventricle inflow. The right ventricle appeared small and compressed anteriorly by the right atrium (area of RV: 11 cm2).\nCardiac magnetic resonance imaging showed a marked right atriomegaly (right atrium area: 66.50 cm2, volume: 220 ml/m2) and normal size of the left atrium (left atrium area: 7.02 cm2). The right ventricle was regular in size and global contractility but was partially compressed and dislocated posteriorly, due to the massive enlargement of the right atrium. The left ventricle was regular in dimension, thickness of the wall, and global/segmental contractility (FE VS = 61%). No evident transvalvular jets or areas of late gadolinium enhancement were found. The pericardium was visualized without focal abnormalities or pericardial effusion ().\nDue to the high risk of arrhythmias and thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, the patient underwent cardiac surgery. Through a median sternotomy, cardiopulmonary bypass was established with standard aorta and bicaval cannulation. After the pericardium was opened, the entire anterior surface of the heart was found to be covered with a thin wall in continuity with the right atrium. No atrial appendage as such was apparent. The right atrium was fully opened. The inferior border of the atriotomy was sewn around the anterior part of the tricuspid annulus, and the superior border was brought over the lateral wall of the right atrium as a flap and sewn near the interatrial groove. This provided adequate reduction of the atrial size and reinforcement of the atrial wall ().\nThe histology of the resected atrial wall showed focal hyperplasic areas of smooth muscle cells with polymorphic nuclei surrounded by a few scattered areas of hypertrophic fibrous tissue.\nPostoperative transesophageal echocardiogram showed a significant reduction of the right atrium area (23 cm2, volume: 93 ml).\nThe patient was extubated 11 hours after surgery. Complications arose postoperatively with the early appearance of pericardial effusion with leukocytosis and elevated inflammatory markers. This was resistant to conventional medical therapy, which in the end required surgical drainage. Medical therapy of the postpericardiotomy syndrome (ibuprofen 600 mg/TID and colchicine 1 mg/OD) was continued over the subsequent 6 follow-up months without further recurrence of pericardial effusion. |
A Caucasian 60-day-old male newborn baby came to our observation. His parents complained that he had bleeding gums in his lower and upper jaws, pain in sucking and consequent difficulties in feeding. He was full-term born and his weight at birth was 2900g. His past medical history was not significant.No noticeable findings were recorded during an extraoral examination. The intraoral examination revealed multiple whitish small masses localized on the alveolar ridge of his maxilla and mandible. His mandibular alveolar ridge showed small nodular lesions in the region of the right deciduous canine and left deciduous canine and first molar (Figure \n). The size of these masses varied from 3 to 4mm in diameter.\nA surgical removal of the lesions was planned in order to solve feeding problems and bleeding.\nThe excisions were performed in different sessions in order to limit the postoperative discomfort to the baby. All oral surgeries were performed under local anesthesia (mepivacaine 3% without adrenalin) with a 15C scalpel blade. After excision of the cysts a sponge of fibrin was used and the wounds were sutured with resorbable polyglactin suture (Vicryl 4-0; Ethicon, Johnson & Johnson, New Brunswick, New Jersey, USA) that was removed after 10 days.The first surgery involved the excision of two lesions on his left mandibular gum (Figure \n).The second surgical session was made after 15 days. The excision of his right mandibular lesions revealed a neonatal tooth. In this step the surgeon decided not to remove it because the tooth incisal margin was below the gingival level and it would not create feeding problems (Figure \n). A follow-up visit 30 days after the surgery showed the neonatal tooth eruption and a new maxillary lesion in the deciduous first molar region. A new surgical cyst excision was necessary and, in the same session, the neonatal tooth extraction was performed in order to allow the baby breastfeeding without hurting the mother (Figure \n).\nThe obtained specimens were fixed in 10% formalin solution and submitted for histological examination.Periodic recall visits were advised to verify the absence of new lesions and to monitor the developing dentition (Figure \n).All three histological examinations revealed that the characteristics of the lesions were compatible with the diagnosis of dental lamina cysts: they were lined with keratinizing, stratified squamous epithelium, developing from islands of basophilic epithelium, which were interpreted as remnants of the dental lamina (Figure \n). |
A male infant was born by cesarean section at 24 weeks and 5 days gestational age, with a birth weight of 700 grams, to a 29-year-old mother (gravida 4, parity 1, preterm 1, miscarriage 1, liveborn 1) with history of pituitary microadenoma and hyperprolactinemia. Mother presented with advanced cervical dilation and footling breech presentation leading to emergency cesarean section. Apgar scores were 4 and 7 at one and five minutes of life, respectively. He was intubated in the delivery room, and he required mechanical ventilation and surfactant for respiratory distress syndrome of prematurity in the NICU. The patient was transferred to our facility at one week of age for escalation of care due to spontaneous intestinal perforation.\nHe had a complicated respiratory course during hospitalization including an episode of pulmonary hemorrhage, prolonged mechanical ventilation, and development of CLD of prematurity. He was finally extubated successfully by 10 weeks of age and gradually weaned off respiratory support to nasal cannula by five months of age. He had a hemodynamically significant patent ductus arteriosus, which required surgical ligation after failed medical management. The patient had spontaneous intestinal perforation at one week of age complicated by fungal peritonitis and complex ascites requiring primary peritoneal drainage, antifungal treatment, and eventual exploratory laparotomy. This resulted in delayed feeding initiation by two months of age. All head sonograms were normal for age. He had progressive retinopathy of prematurity (ROP) for which he received bevacizumab (Avastin®, Genetech, Inc., San Francisco, California, USA) followed by laser photocoagulation. At five months of chronological age, he continued to require respiratory support with nasal cannula (flow: 2 Liters/min; fraction of inspired oxygen: 0.30). He received budesonide and diuretics for the management of chronic lung disease. He was tolerating full enteral feeds of low mineral formula Similac® PM 60/40 (Abbott Nutrition, Columbus, Ohio, USA; for abnormal electrolytes due to multifactorial chronic renal disease), getting vitamin D and calcium supplementation for osteopenia of prematurity, and getting levothyroxine for hypothyroxinemia of prematurity.\nAt chronological age of five months, he developed fever (temperature 38.7° Celsius) prompting a partial sepsis workup including blood and urine culture with initiation of empiric broad-spectrum antibiotics. A respiratory viral panel (using BioFire® FilmArray® respiratory panel, bioMérieux, Salt Lake City, Utah, USA) and a real-time reverse transcription polymerase chain reaction of nasopharyngeal swab for SARS-CoV-2 (BioReference Laboratories, Elmwood Park, New Jersey, USA) were sent in view of widespread community SARS-CoV-2 transmission and history of sick contact with mother and sibling. Both were symptomatic with mild cough and fever in the preceding days but were not tested for SARS-CoV-2. Additionally, the patient’s mother had recently visited the NICU but reported no symptoms at the time of visit. Physical examination of the infant showed mild intermittent tachypnea with no other significant findings, accompanied by no change in his overall respiratory support or oxygen requirement (flow: 2 Liters/min; fraction of inspired oxygen: 0.30). He continued to tolerate feeds without any feeding intolerance. Initial laboratory results were notable for mild leukopenia (white blood cells count: 6 x103/µL) and anemia of prematurity (hemoglobin: 8.8 g/dL). Absolute lymphocyte counts (3,008 cells/ µL, reference: <1,500 cells/ µL) and C-reactive protein (CRP, 5.1 mg/L, reference: <5 mg/L) were normal at the time of partial sepsis workup. Additionally, mild elevation of liver enzymes and creatinine was noted at the time of diagnosis (Figures , , , ). A chest radiograph showed increasing perihilar airspace opacities that could represent underlying viral or atypical pneumonitis (Figure -).\nHe was placed in a negative pressure isolation room with contact, airborne, and eye protection (goggles or face shield) precautions. Patient’s nasopharyngeal swab was positive for SARS-CoV-2 the same day. Respiratory viral panel was negative for other common respiratory viruses. The blood and urine cultures were resulted negative for any growth.\nInitial blood gas analysis at the time of testing for SARS-CoV-2 was within normal limits. Subsequent blood gas analyses showed a trend toward mild metabolic acidosis with respiratory compensation, normal capillary pH, low bicarbonate level of 17.8 mmol/L (reference: 25 to 32 mmol/L), and base excess of negative 6.8 mmol/L (reference: +2.0 to -2.0 mmol/L) on day 5 after testing for SARS-CoV-2, which improved on the following days. The longitudinal measurements of laboratory indices over next few days such as CRP, complete blood cells count, and renal and hepatic function tests remained normal for age (Figures , , , ). Pediatric infectious disease team was consulted regarding management of this patient. Considering his overall stable cardiorespiratory status, no targeted experimental treatment for COVID-19 was initiated. The repeat second and third tests of nasopharyngeal swab for SARS-CoV-2 real-time reverse transcription polymerase chain reaction (RT-PCR) were positive (days 5 and 10 after initial testing) and finally turned negative on the fourth test (day 15 after initial testing). There had been no cases of SARS-CoV-2 infection in the NICU before and after the index case. Following the diagnosis of COVID-19 in this patient, the parents were temporarily restricted from visiting the unit as per our local infection prevention guidelines []. Notably, one healthcare worker developed upper respiratory symptoms a few days after this infant’s diagnosis and tested positive for SARS-CoV-2. A timeline of COVID-19 diagnosis in the beginning of pandemic and evolving infection prevention guidelines within our hospital system and New York City health department is shown in Figure . At discharge from the NICU, he was stable on room air and taking full feeds by mouth of Similac® PM 60/40 (24 calories per fluid ounce) due to his history of chronic kidney disease. He was on Synthroid for his hypothyroidism, vitamin D for osteopenia of prematurity, and amoxicillin for his history of urinary tract infection and chronic renal disease. All head ultrasounds obtained throughout his admission were normal.\nSince his discharge, he had various sub-specialists follow-up visits. He was seen by pediatric pulmonology for his CLD and remains stable on room air. He is followed by pediatric nephrology for chronic renal disease, and his feeds were changed gradually to Enfamil® Neuro Pro™ (Mead Johnson & Company, LLC; Chicago, Illinois, USA) from Similac® PM 60/40. He has been off amoxicillin prophylaxis by 11 months of chronological age. He is followed by pediatric endocrinology for his hypothyroidism and was on Synthroid until 13 months of age, at which time his medication was discontinued with recent thyroid function tests resulting normal for age. He continues to be followed by ophthalmology and had regressed ROP on his last visit at one year of chronological age. By chronological age of 20 months, he can crawl and stand with support, which he started at 18 months of age. He speaks only a few words such as dada and mama. He is getting physical therapy for 30 minutes two times a week via the ‘Early Intervention’ program but is referred for assessing need for occupational or speech therapy. He has had issues with feeding intolerance, with spit up/emesis with feeds because of which he has poor weight gain and growth. He is being followed by nutrition services to help with optimization of growth. He is now eating solid food well with formula supplementation. He is up to date on his vaccines. He has not had any cough, shortness of breath or hospitalizations since his discharge from the NICU. Of note, he has had multiple emergency department (ED) visits for increased irritability, emesis, and subjective fever at home. During those visits, he had no respiratory distress, rash, no vital sign abnormalities in the ED, and no fever documented in ED. He was tested for COVID-19 on those visits with all results being negative. |
Our patient was a 52-year-old male with a past medical history of cerebral palsy, essential hypertension, depression, and knee arthritis status post total knee arthroplasty, who presented to the emergency department after a mechanical fall. He was found to have a left distal femoral shaft fracture above the total knee arthroplasty which was fixed with an intramedullary rod placement. The patient was discharged home in a stable condition. Three weeks later, he developed increased redness and swelling at the surgical site. Vital signs were all stable on admission. Physical examination was significant for a 10 cm post-surgical wound around the left knee with mild dehiscence. Granulation tissue with drainage of serosanguineous fluid was noted along with mild erythema around the surgical site. Laboratory results including white blood cell count, erythrocyte sedimentation rate, and C-reactive protein were all normal at 3,900/µL, 1 mm/h, and 2 mg/L, respectively. Blood cultures were obtained and remained negative at 72 hours. He was taken to the operating room and underwent extensive excisional debridement including skin, muscle, and bone. There was one screw of the nail that was loose and, therefore, removed. Antibiotic (gentamycin)-impregnated cement beads were inserted. Gram stain from intraoperative culture showed no organisms. He was started empirically on cefepime 2 g IV every 8 hours and vancomycin 1 g every 12 hours postoperatively. After four days, there was no growth from the surgical cultures. Microbiology lab was asked to hold the culture for 10 days for the concern of slow-growing organism. The patient was discharged with ceftriaxone 2 g every 24 hours through a PICC line. Five days after discharge, aerobic cultures grew Corynebacterium species. The specimen was sent to Mayo Clinic (Rochester, Minnesota) for further testing. Quantitative antibiotic susceptibility testing was performed to determine the isolate’s minimum inhibitory concentration (MIC) to various drugs. The Corynebacterium species was identified as C. jeikeium, which was resistant to penicillin, ceftriaxone, and doxycycline but susceptible to vancomycin. The susceptibilities and the MICs were as listed in Table .\nThe patient’s antibiotic was switched to vancomycin IV 1.5 g every 12 hours with a target trough level of 15-20 µg/mL. His treatment course remained uneventful, and he completed a six-week course of vancomycin. The patient did not follow up with the orthopedic surgeon or the infectious disease team due to fear of COVID-19. One year later, the patient presented to the emergency department with draining wound and significant fluctuance above his left distal femoral fracture. He was found to be septic with Staphylococcus aureus bacteremia. He underwent irrigation and excisional debridement of his wound along with antibiotic (gentamycin) bead exchange. However, due to persistent sepsis, the patient required above-knee amputation to control his infection. His operating room cultures grew S. aureus as well. The patient completed two weeks of IV cefazolin postoperatively. |
A 47-year-old African American male with no significant medical history presented to his primary care provider for concerns of a two-year history of a slowly enlarging right lower back lesion with infrequent lower back spasms that were well-controlled with cyclobenzaprine and ibuprofen. The patient denied any systemic symptoms or other new or worsening dermatologic conditions. Physical examination was significant for a painless 3 × 2 cm mass located on the right lower back.\nSonographic examination revealed a heterogenous and vascularized tumor that was originally thought to be a simple lipoma based on history and physical examination findings (Figure ). A magnetic resonance imaging (MRI) study of the lesion demonstrated a well-circumscribed mass localized to the subcutaneous tissue without note in the radiology report of distant metastasis or deeper fascial involvement (Figure , Panel a). A subsequent ultrasound-guided biopsy demonstrated histopathologic findings of spindle cells in a storiform pattern with low mitotic activity within the subcutaneous fat, consistent with the diagnosis of DFSP (Figure ). Based on this diagnosis, the patient was referred to a tertiary care center for definitive treatment to include wide local excision; treatment with imatinib mesylate was deferred. A follow-up MRI at six months post-surgery demonstrated no evidence of recurrence at the surgical bed (Figure , Panel b).\nWith the diagnosis of DFSP, the patient was referred to dermatology. At the dermatology clinic, the patient was noted to have at least six hyperpigmented patches consistent with café-au-lait spots scattered over the left frontal scalp, left chest, left flank, left lateral thigh, and left upper back. The physical examination further revealed left axillary freckling and multiple soft 1-2 cm pedunculated plaques consistent with neurofibromas located on the bilateral shoulders and left upper back. Biopsy of one of the lesions confirmed a neurofibroma without plexiform features. These findings confirmed a new diagnosis of NF1. The patient had no other systemic involvement and is now six years from DFSP resection and remains tumor-free. |
A 26-year-old male presented to Accident and Emergency following assault with a baseball bat. His past medical history consisted of longstanding Hepatitis C infection only, he was on no regular medications nor had any known allergies.\nOn admission, the patient had complained of bilateral pleuritic pain on inspiration with visible, tender bruising to the posterior thoracic region bilaterally. There were no urinary, bowel or neurological symptoms reported and no open wounds were visible on examination. The anterior abdomen was soft and non-tender to palpation.\nA CT scan of chest (performed at 30s post i.v. injection) showed acute fractures of left 10th/11thand right 8th/9th ribs, but no evidence of pneumothorax or lung contusion. A splenic laceration was not suspected clinically and the timing of the scan, optimized for the chest, only showed subtle splenic changes which were not identified by the original reporter ().\nLater that day, the patient complained that their pain had spread from the chest down to the right and left flanks. This prompted a CT scan of abdomen/pelvis (performed at 70 s), which demonstrated a contained splenic laceration with no evidence of capsular breach (). Both the rib fractures and splenic injury were treated conservatively with bed rest and analgesia.\nThe patient abruptly deteriorated becoming hypotensive and tachycardic in keeping with hypovolaemic shock. A further CT scan showed splenic capsular rupture, active bleeding and large-volume intraperitoneal haemorrhage ().\nThis was managed urgently with endovascular embolisation. While the patient gave informed consent for the procedure he indicated that he was unlikely to be compliant with some aspects of his aftercare and likely to self-discharge very soon after completion of the procedure.\nFollowing local anaesthesia an ultrasound guided puncture of the right common femoral artery was performed and the splenic artery selectively catheterized with a Sim 1 catheter (a reverse angled catheter). Angiography revealed three pseudoaneurysms, two arising from a second order upper pole branch and one from a third order equatorial branch (). These were then superselectively catheterized and embolized with a series of 3, 4 and 5 mm microcoils (Nester & Vortex, Nester = Cook Medical, Bloomington, IN, USA, Vortex = Boston Scientific, Watertown, MA, USA), delivered through a microcatheter. This abolished filling of the pseudoaneurysms but there was more devascularisation of the upper half of the spleen than envisaged when the decision to perform superselective embolisation was made. The right common femoral artery was then closed with a Starclose vascular closure device (Abbott Medical).\nThe decision to perform superselective embolisation was made with the intention of achieving definitive haemostasis in a patient who was likely to leave hospital at the earliest possible opportunity and not comply well with the procedural aftercare (). There was no further bleeding.\nDay 1 post-procedure, the patient developed a post-embolisation syndrome (PES) characterized by fever, nausea and left abdominal pain. A fourth CT scan was performed. This showed splenic necrosis, parenchymal and intravascular gas, which is an expected appearance post-embolisation. No fluid collection was demonstrated. Conservative resuscitation was initiated with i.v. fluid and antibiotic administration.\nThere was no indication for surgical drainage at this point as the CT scan showed only splenic necrosis, with no evidence of abscess formation. The patient’s temperature and clinical features were also settling, further reassuring stabilisation of his condition ().\nThe patient recovered from this episode a few days later, and deemed medically fit, was subsequently discharged home.\n3 weeks later, the patient presented to Accident & Emergency once again; this time with episodes of frank haemoptysis. He had an associated fever, and left upper quadrant (LUQ) pain.\nBlood tests showed elevated inflammatory markers, suggesting an infection, thought to be of likely respiratory origin at this time. |
A right-handed, 52-year-old woman presented with a two-week history of a tingling sensation on the fifth finger of the right hand, which had started 11 weeks after she had undergone bilateral open carpal tunnel release procedures. After the carpal tunnel releases, the tingling in both hands gradually disappeared, but a new tingling sensation on the right fifth finger commenced 11 weeks after the carpal tunnel release. There was no history of localized, direct trauma around the right wrist after the carpal tunnel release. The physical examination demonstrated mild hypesthesia on the right fifth digit. According to the Medical Research Council scale, the strengths of the right abductor digiti minimi (ADM) and first dorsal interosseous (FDI) muscles were grade 4, whereas other muscle strengths of the right upper extremity, including flexor digitorum profundus III & IV and flexor carpi ulnaris muscles, were grade 5. There was no significant atrophy on the right hypothenar area. Froment's test was negative on both sides, while the nerve percussion sign was positive over the ulnar side of the right wrist. Deep tendon reflexes were normal bilaterally.\nPrior to the carpal tunnel release, nerve conduction studies (NCS) had been performed on both upper extremities (). Bilateral median motor NCS with abductor pollicis brevis (APB) recordings had demonstrated prolonged latency and low amplitude on the left side, with normal responses on the right. Median nerve sensory responses showed prolonged latencies and low amplitudes at the wrist bilaterally. Needle electromyographic findings of the left abductor pollicis brevis muscle revealed abnormal spontaneous activities and polyphasic motor unit potentials with reduced recruitment patterns. The left ulnar motor NCS and bilateral ulnar sensory NCS were normal.\nA follow-up electrodiagnostic study performed 10 weeks after the carpal tunnel release demonstrated the improvement of sensory and motor responses in both median nerves, with improved latencies and amplitudes (, ). Thirteen weeks after the surgery, a third electrodiagnostic study was performed. Bilateral median sensory and motor responses showed no specific interval changes, as compared to the previous study. However, the right ulnar motor NCS with ADM recordings revealed prolonged latency, and the right ulnar sensory NCS with the fifth digit recordings showed low amplitude in comparison to the other side. The right ulnar motor and sensory short segmental studies at the wrist revealed an abnormally prolonged latency difference between the pisiform bone and 2 cm proximal to the pisiform bone (0.7 and 0.8 ms respectively; upper normal limit in ulnar motor and sensory nerves, 0.5 ms) in addition to a conduction block (54.1%) of the ulnar sensory response over the same segment (). Needle electromyographic findings from the right ulnar innervated muscles revealed no spontaneous activity, although the normal motor unit potentials had reduced recruitment patterns in the ADM and FDI muscles. Subsequent ultrasonography (SONOACE 9900, 7.5 MHz linear probe, Medison, Korea) of the right wrist showed ulnar nerve swelling between the pisiform and 2 cm proximal to the pisiform (), as compared to the left side (). Also, compared with the pre-operative ultrasonographic finding, mildly flattened ulnar nerve and artery at the level of pisiform and irregular floor line of Guyon's canal were observed (). Because electrodiagnostic findings suggest a demyelinating process of the ulnar nerve, despite the imaging findings that the nerve was swollen around the wrist, we provided conservative treatment of pain relief and physical therapy (oral non-steroidal anti-inflammatory drug, and infrared heat therapy) after which her symptoms improved. A final follow-up nerve conduction study was performed 4.5 years after the carpal tunnel release and demonstrated spontaneous full recovery of the right ulnar neuropathy at the wrist. There was no abnormally prolonged latency difference or conduction block in the segment between the pisiform and 2 cm proximal to the pisiform along the ulnar motor and sensory segment at the wrist. |
A 62-year-old female with no significant past medical history presented to the emergency department in November of 2017 with complaints of arthralgias, most notably in her right knee, left shoulder, and bilateral thighs that made it difficult for her to ambulate. She was also admitted due to a headache that was triggered primarily by coughing. Vital signs on admission were as follows: a blood pressure of 202/90 mmHg, a heart rate of 137 bpm, a respiratory rate of 20, and a temperature of 36.6 Celsius. Physical exam revealed Janeway lesions. She was found to have a neutrophilic leukocytosis, with white blood cell count at 20.4 cells/mm3 and neutrophils at 17.4 bil/L. Troponin was elevated at 1.85; this was deemed to be noncardiac in nature as the patient's pain was relieved with ibuprofen and her EKG showed no acute findings. ESR and CRP were elevated at 95 mm/hr and 24.8 mg/dL, respectively. A computed tomography of the brain showed a high-density mass in the right occipital lobe, with surrounding vasogenic edema. The patient continued to deny any visual changes or symptoms other than what was discussed above. An ophthalmologist was consulted to perform a dilated fundus exam, which was positive for small intraretinal hemorrhages that were deemed to be secondary to the patient's hypertension and less likely positive for Roth's spots. There was no evidence of disc edema. A brain MRI with and without gadolinium showed multiple small punctate bilateral areas of acute or subacute infarctions indicative of embolic phenomenon. The hemorrhagic area in the right occipital lobe was again identified, with subtle surrounding enhancement; the differential diagnosis consisted of neoplasm, vascular malformation, or embolic infarction with hemorrhagic conversion. A transthoracic 2D echo was without vegetation, so a transesophageal echo was ordered, and vegetation was shown on the posterior leaflet of the mitral valve. Two blood cultures from admission then came back positive for Rothia dentocariosa. Infectious disease was confirmed, and the patient's current antibiotics, which consisted of vancomycin and ceftriaxone, were switched to penicillin G on a continuous pump. The patient remained largely asymptomatic during her admission and was deemed to be stable for discharge from the hospital after a nine-day stay with penicillin G via a continuous pump for a total of six weeks and was planned for a follow-up MRI in three weeks. The repeat MRI came back showing new subacute strokes. The patient was reported, again, to be asymptomatic but was directed to come straight to the emergency department. A repeat transesophageal echo was done and showed the known vegetation on the mitral valve with new vegetation seen on the PICC line and an abscess between the mitral and aortic valves extending into the ascending aorta. The patient then requested transfer to another institution for further evaluation. A repeat transesophageal echo was completed at this outside institution which showed small anterior and posterior mitral leaflet vegetation with no significant destruction and no abscess. A cardiac MRI was then performed which showed a focal delayed enhancement in the apical inferior and lateral wall, likely secondary to coronary arterial embolization. The patient went on to complete the full six weeks of penicillin therapy, remained asymptomatic, and refused a mitral valve replacement. Her follow-up was continued in the cardiology clinic. |
A 40-year-old African American male patient, with a past medical history of chronic achalasia presented with intermittent abdominal pain. He was status-post esophagogastrectomy with colonic interposition and J-tube insertion performed at an outside facility, approximately six months ago. Pain was intermittently present since the time of surgery, moderate intensity, gradually worsening, associated with intractable nausea and vomiting, and located in the left lower quadrant. Patient was well oriented and awake, physical examination was significant for tachycardia and mildly tender left upper quadrant of abdomen. On admission, the computer tomographic (CT) imaging of abdomen/pelvis with contrast showed post-surgical changes and generalized mesenteric edema with no evidence of mesenteric ischemia or internal hernia. Upper gastrointestinal endoscopy was significant for some ulcerative changes at the site of surgical anastomosis, without stenosis and the pathology results were negative for intestinal metaplasia. Through the early course of hospitalization, he was still unable to tolerate diet orally. By hospital day four, he started complaining of blurred right eye vision and horizontal gaze palsy (cranial nerve-VI palsy) was appreciable on physical examination. Computer tomography brain without contrast ruled out intracranial hemorrhage with no evidence of acute pathology. On hospital day six, he was complaining of dizziness that progressively worsened. By the seventh day, his symptoms progressed complicated by confabulation and hallucinations. Cerebrospinal fluid (CSF) examination, as well as magnetic resonance imaging (MRI) of the brain and orbits, were normal.\nBy this time, differential diagnosis included intermittent porphyria and Wernicke's encephalopathy. Urine porphobilinogen was negative; and patient was started on thiamine. On hospital day 9, he was intubated secondary to worsening encephalopathy and inability to maintain his airway. He was then transferred to the intensive care unit (ICU). Of note, the patient had a large volume aspiration event of brownish fluid during the endotracheal intubation. Post intubation ABG was significant for an A-a gradient of 303.8 mmHg and PaO2/fraction of inspired oxygen (FiO2) ratio of 95 while on positive end-expiratory pressure (PEEP) of 8 mmHg. See Figure for more detail of mechanical ventilation from day 1 through day 7 of ICU admission.\nPost intubation patient required two vasopressor support (norepinephrine and vasopressin) to keep mean arterial pressure (MAP) as more than 65 mm of Hg. Chest radiograph showed diffuse bilateral patchy opacities consistent with clinical diagnosis of ARDS and computer tomography chest with and without contrast ruled out the pulmonary embolism. Initially, he was treated as distributive shock and covered with broad-spectrum antibiotics. Due to his clinical decompensation and refractory shock, stress dose steroids, intravenous thiamine, and vitamin C were initiated. Repeat echocardiogram in the setting of refractory shock was significant for essentially normal LV function with an ejection fraction of 50-55% but severe RVD with a tricuspid annular plane systolic excursion (TAPSE) of 12 mm. By early next morning, his shock worsened with multiple organ dysfunction syndromes (MODS) requiring four vasopressors (phenylephrine, norepinephrine, epinephrine and vasopressin) to maintain a MAP above 55 mmHg. He was placed on continuous veno-venous hemodialysis (CVVHD) for refractory acidosis in the settings of gradually declining glomerular filtration rate and persistent lactic academia. He was on mechanical ventilation for his severe ARDS (as per ARDSnet protocol) along with pulmonary vasodilatory therapy with inhaled epoprostenol. Due to his severe clinical instability, he was not a candidate for prone positioning or transfer to an extracorporeal membrane oxygenation (ECMO) center.\nAfter multidisciplinary discussion and review of the clinical case, a decision of device placement was made for mechanical support of the right heart via impella. He was transferred to catheterization laboratory and under fluoroscopy, he was found to have a new left-sided pneumothorax (Figure ), not evident on last chest imaging (Figure ). It was attributed to barotrauma secondary to bag-valve-mask ventilation, required during his transportation to the catheterization laboratory. The event was followed by chest tube placement with complete resolution of pneumothorax. Immediately after the impella placement (Figure ), the need for vasopressors was significantly reduced by nearly 50%. The option to transfer him to ECMO center was re-considered, yet he was not deemed a candidate due to his multi-organ dysfunction and number of days on the ventilator.\nHe continued to have significant improvement in both hemodynamics and multiorgan dysfunction. Vasopressor support, as well as ventilatory support, decreased to a minimum in 48-72 hours post right heart impella placement. Unfortunately, he did not have a significant improvement in his neurological status.\nThe repeat CT brain (Figure ) was consistent with cerebral edema and multifocal hemorrhages in bilateral cerebral hemispheres, with the largest lesion measured approximately 1 centimeter. Possible etiologies included systemic anticoagulation while on CVVHD and mechanical cardiac device, coagulopathy in the settings of shock liver, thrombocytopenia secondary to increased consumption, and possibly compounded by probable thrombasthenia as a sign of worsening uremia. On hospital day 15, he was down to just one vasopressor (vasopressin) and impella was removed on the sixth day of its placement. MRI brain without contrast was performed and was suggestive of extensive damage consistent with Wernicke-Korsakoff’s syndrome (Figure ), with superimposed multifocal hemorrhagic strokes (This was previously deferred while Impella in place).\nGiven the global extent of the underlying neurologic pathology, the patient’s family decided to transition to comfort measures. The patient died soon after weaning off the mechanical ventilation. |
A 59-year-old male presented to the emergency department with complaints of left shoulder pain for 3 weeks. He was moving furniture, which resulted in sudden onset of pain, which was briefly relieved by ibuprofen but became progressive with tingling numbness in the left arm. Eventually, he came to the emergency department for further evaluation. He also mentioned about night sweats. His vital signs in the emergency room showed blood pressure of 106/64 mm Hg, heart rate of 75 beats per minute, respiratory rate of 14 breaths per minute, temperature of 100.6 °F. Physical examination was only remarkable for point tenderness over the left coracoid process with a restricted range of motion and pain on abduction of the shoulder. There was no lymphadenopathy. Initial laboratory investigations revealed mild anemia, elevated lactate dehydrogenase, and alkaline phosphatase, as shown in .\nComputed tomography scan of the left shoulder with contrast demonstrated a 10 × 8 mm lytic lesion along the coracoid of the scapula with enlarged left axillary lymph nodes (). The patient eventually got magnetic resonance imaging of left shoulder, which demonstrated abnormal marrow signal within the coracoid process of the scapula, with a cortical disruption, periosteal reaction, and multiple foci of enhancement within the humerus with no evidence of rotator cuff tear (). Findings were concerning for malignancy. The whole body bone scan showed increased activity in the left glenoid process with no other discrete areas of abnormal uptake ( and ). During hospitalization, the patient had a bone biopsy by interventional radiology with histopathology and immunophenotype () showing atypical intertrabecular lymphohistiocytic infiltrate containing small and large lymphocytes in the background of necrosis. Large cells demonstrated marked cytologic atypia with irregular hyperchromatic nuclei and abundant cytoplasm with few showing Reed-Sternberg–like morphology. Neoplastic cells were positive for CD15, CD20, CD30, CD45, CD79a, MUM1, and PAX5 with partial weak staining of BOB1, OCT2, and BCL2. Neoplastic cells were negative for BCL6 and Epstein-Barr virus. Based on the above findings, diagnosis of lymphoma, unclassifiable with features intermediate between DLBCL and cHL was made.\nThe patient was started on etoposide, prednisone, vincristine, cyclophosphamide, hydroxydaunorubicin, and rituximab (EPOCH-R) chemotherapy regimen consisting of 6 cycles. Bone scan repeated after chemotherapy showed no evidence of residual disease. The patient subsequently received consolidative radiotherapy for 15 sessions with a dose of 30 Gy in 15 fractions over 3 weeks. The patient currently remains disease-free with complete resolution of shoulder pain. |
A 21-year-old male was being investigated for an acute onset of pain involving the left hip for 6–8 weeks. The magnetic resonance imaging revealed a permeative lesion involving the head and upper shaft of the femur. The needle core biopsy from the lesion was performed in another hospital which was reported as suspicious of a primitive neuroectodermal tumor and was referred to our institute. His laboratory workup in our hospital revealed normal complete blood count with Hb concentration – 14.2 g/dl; WBC count – 5.7 × 109/L; platelets – 278 × 109/L; and PBS did not reveal any abnormal cells or blasts. The serum lactate dehydrogenase was mildly raised to 216 U/L. PET scan with F-18 FDG revealed hypermetabolic disease involving the head, neck, and proximal one-third shaft of the left femur with maximum SUV of 7.6 with no obvious cortical destruction or soft tissue mass. In addition, the proximal end of the left fibula also showed an active disease with maximum SUV of 3.59. There was no evidence of active disease elsewhere in the body.\nA biopsy of the lesion was reviewed which showed sheets of medium-sized tumor cells with scant cytoplasm and blastic chromatin with few crushing artifacts. Mild pleomorphism of tumor cells with focal apoptosis was noted. These features were consistent with a high-grade blastic neoplasm. On IHC, the tumor cells showed diffuse membranous expression of MIC2, whereas TdT showed weak, but distinct nuclear reactivity. In B-lymphoid lineage markers, CD10 and CD20 were diffusely positive while PAX5 was negative. In addition, blasts expressed MPO; however, CD117 did not reveal any positive expression.\nThe BM study was omitted initially as there was no evidence of any other sites or marrow involvement or uptake by PET scan. However, BM was done after a month of diagnosis, and it revealed 54% of blasts which were negative for cytochemical MPO. We performed eight-color flow cytometric immunophenotyping (FCI) using a comprehensive antibody panel []. Cells were acquired on Navios (Beckman Coulter [BC]) flow cytometer using Navios software, and data were analyzed using Kaluza software (BC).\nImmunophenotypic analysis [] showed approximately 43% of viable cells were abnormal blasts. The abnormal blasts revealed a B-lymphoid lineage origin expressing CD10, CD19, CD20, and CD22 but negative for cytoplasmic CD79a. It also showed expression of CD34 and human leukocyte antigen–antigen D. In addition, they showed cytoplasmic MPO expression but were negative for rest of the other markers tested. BM trephine biopsy also showed a sheet of blasts expressing CD10, CD20, and MPO. Cytogenetics revealed no evidence of BCR/ABL fusion: t(9;22), AML1/ETO: t(8;21), and MLL rearrangement.\nAll features were consistent with bi/mixed phenotypic blastic hematolymphoid neoplasm as B-lymphoblastic lymphoma with coexpression of the myeloid marker and MPO that progressed to mixed phenotypic acute leukemia (B/myeloid leukemia) during a course of time. |
A previously healthy 39-year-old woman was referred to our hospital because of a cystic lesion in the liver demonstrated by abdominal ultrasonography (US). Laboratory studies, including liver function tests, and tumor markers were also within the normal limits. Serological markers for hepatitis B or C viral infection were undetectable. Abdominal US revealed a well demarcated, heterogeneously low-echoic mass 170 mm in diameter in right lobe of the liver. Abdominal computed tomography (CT) during hepatic arteriography (CTHA) revealed early ring enhancement in the peripheral area in the arterial phase and slight internal heterogeneous enhancement in the delayed phase (Figures and ). Magnetic resonance imaging (MRI) showed that the tumor had low signal intensity on T1-weighted images and some foci of high signal intensity on T2-weighted images. Gadolinium ethoxybenzyl (Gd-EOB) MRI revealed no uptake in the corresponding area (Figures , , and ). Abdominal angiography demonstrated a large avascular region in the liver corresponding to the tumor, although no typical features of cavernous hemangioma were evident (). 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed no abnormal FDG uptake. With these radiological findings, malignant liver tumor could not be excluded, such as biliary cystadenocarcinoma, cholangiocarcinoma, mesenchymal tumors, and hepatocellular carcinoma associated with cystic formation.\nThe patient underwent posterior sectionectomy. Intraoperative examination revealed a relatively soft dark red tumor (); the resected specimen weighed 1.1 kg and measured as 170×100×80 mm. The cut surface of the tumor revealed a white, solid, and cystic mass that was elastic, soft, and homogeneous with a yellowish area considered to be myxoid degeneration (). Histological examination showed that the tumor mostly consisted of sclerotic area and cavernous hemangioma area is partly observed (). Sclerotic area presents diffuse fibrosis () and the typical histology of cavernous hemangioma was confirmed in some parts. In addition, marked increase and dilation of medium sized veins with cavernous form were frequently noted in the surrounding areas of tumor (). The increased and dilated veins show positivity of CD31 immunostaining being a marker of endothelium (). The pathologic features were consistent with sclerosing hemangioma. The postoperative course was uneventful, and the patient was discharged on postoperative day 10. |
A 74-year-old male presented in February 2016 with increasing dysphagia. There was no history of prior abdominal infection or surgery. On esophagogastroscopy, a necrotic and circumferential friable tumor was seen at 33 to 40 cm from the incisors, with an endoscopic appearance of involvement of gastroesophageal (GE) junction and the proximal 2 cm of the stomach. Biopsies of the distal esophageal tumor confirmed poorly differentiated adenocarcinoma. The patient was anemic with a hemoglobin of 89 g/L. Staging endoscopic ultrasound suggested a breach of muscularis propria and four enlarged paraesophageal nodes. Neoadjuvant chemoradiotherapy followed by esophagectomy was initially considered; however, a staging positron emission tomography (PET) scan demonstrated 18-fluorodeoxyglocose (FDG) uptake not only in the primary tumor, but also in the paraesophageal region near the GE junction and upper abdominal lymph nodes extending as far inferiorly as the right renal vessels, in a retrocaval location (Figure ).\nA radiation oncology consultation was sought regarding treatment options of such extensive lymphadenopathy. Palliative radiation therapy (RT) was recommended. The patient was also evaluated by a medical oncologist who advised that chemotherapy may be considered after assessing the response to palliative radiotherapy.\nFrom March 21, 2016 to April 5, 2016, the patient received palliative RT to the symptomatic primary tumor and closest adjacent nodes using a pair of anterior and posterior fields. A total dose of 30 Gray (Gy) was prescribed over 10 daily fractions. As the lymphadenopathy in the lower abdomen was not symptomatic, and would have contributed to increased toxicity, this region was deliberately excluded from the high dose RT volume (Figure ). Other than very mild odynophagia, the patient had no other RT-related side effects. On the first follow-up visit, one month following treatment completion, he had improved swallowing function and a weight gain of six pounds.\nFollow-up computed tomography (CT) scan was obtained on May 24, 2016 to evaluate for the suitability of chemotherapy and to serve as a baseline during systemic therapy. This demonstrated persistent thickening of the lower esophagus, with lymphadenopathy reported to have decreased in size and no significant retroperitoneal adenopathy. When given the option of receiving palliative chemotherapy, the patient declined and chose to continue on observation only. Further CT scans in August and October 2016 showed a complete response in the irradiated primary tumor and nodes, with a stable 10 mm lymph node at the right renal vein.\nIn January 2017, due to symptoms of increasing dysphagia, the patient was assessed by a thoracic surgeon for consideration of esophageal stent placement. Endoscopy on January 12, 2017 noted that there was a possible small amount of residual tumor at the GE junction, but there was no significant narrowing or stricture, and no biopsies were taken. A further CT scan on April 10, 2017 showed minor circumferential thickening of the distal esophagus, but unchanged from previous. Paraesophageal lymphadenopathy was reported to be unchanged. The PET-positive lymph node at the renal vein decreased from 10 mm to 5 mm.\nThe patient’s symptom of dysphagia resolved spontaneously, and an evaluation was made with a further PET scan on May 19, 2017 (Figure ). This demonstrated mild residual FDG activity within the distal esophagus, more likely inflammatory change rather than malignancy. The FDG activity within all the lymph nodes, both treated and untreated, had unexpectedly resolved. |
The patient is a 50-year-old African American female with a history of bilateral breast reduction twelve years ago, iron deficiency anemia, and obesity, who presented to the surgeon's office complaining of tenderness of her right breast. The patient reported that recently she had been developing keloids along the scar of the right breast with some areas having a blue hue; her left breast was unremarkable. She noticed that after wearing a sports bra there was increased pressure and abrasions to the keloid, leading to cellulitis and edema. She was previously treated with two courses of antibiotics for what was presumed to be an infected keloidal scar of her right breast but with minimal improvement. On exam, she had a large 10 cm diameter keloidal region on the inferior and lateral aspect of the right breast with edema and cellulitis. The keloidal area had no palpable fluctuance; she exhibited no nipple discharge or palpable adenopathy of the right axilla ().\nThe patient had a benign-appearing mammogram 8 months prior, and all of her screening mammograms since her breast reduction have been without signs of malignancy. Another mammogram was ordered but was not performed due to patient discomfort. An ultrasound of the breast was preformed and suggested marked edema and skin thickening suggestive of infection but no definitive fluid collection or underlying suspicious mass was observed.\nThe patient underwent a right breast partial mastectomy for cosmesis and resection of the infected keloidal area. Intraoperatively, the mass was highly vascular, firm, but not fixed to the chest wall. Postoperatively, the pathology revealed a high-grade primary angiosarcoma of the breast with negative margins.\nPatient underwent a computed tomography of the chest, abdomen, and pelvis, which did not show any evidence of gross metastatic disease. The patient then underwent completion mastectomy and scheduled for adjuvant chemotherapy with combination gemcitabine and Taxotere, followed by radiation. |
A 62-year-old man was admitted to our hospital with 3 weeks of left eye redness and ipsilateral hemifacial edema, as well as pain and edema of the left arm. The patient’s past medical history was significant for end-stage renal disease of nonestablished etiology, and his past surgical history was significant for surgical creation of left brachiocephalic arteriovenous hemodialysis fistula in 2011 and kidney transplant in 2012. The patient had no history of known prothrombotic disease or cancer. Prior to transplantation, the patient was dialyzed by a right central venous catheter (CVC), and the left upper extremity arteriovenous fistula (AVF) had never been used, as his kidney transplant had normal function.\nOn ophthalmological examination, visual acuity, visual fields, and extraocular movements of the left eye were normal. However, tortuous, dilated and hyperemic conjunctival vessels and proptosis were evident. On the left eye the intraocular pressure was moderately increased (26 mm Hg), and fundoscopy only revealed some arterio-venous crossing points bilaterally. Otherwise, the examination of the right eye was normal.\nPhysical examination revealed a large aneurysmal brachiocephalic AVF, and prominent deep veins over the upper arm and shoulder in the left extremity (). Duplex ultrasonography showed acute thrombosis of the proximal segment of the left subclavian vein, patent AVF with flow volume of 800 mL/min, and blood return from the AVF travelling up in a retrograde fashion through the left external jugular vein. Computed tomography (CT) confirmed the central venous thrombosis and significant retrograde dilatation of the left axillosubclavian and external jugular veins (), with retrograde flow in the cavernous sinus and superior ophthalmic vein and subsequent proptosis of the left eye (). Laboratory findings were within normal limits, including a complete blood count, a coagulation profile, and comprehensive metabolic panel.\nTherefore, the proptosis was attributable to retrograde blood flow in the left jugular venous system, originating from an upper extremity dialysis AVF in the presence of central venous occlusion. We confirmed that the AVF was a major contributor of the proptosis by occluding it with a blood pressure cuff, which rapidly improved the patient’s symptoms. Ligation of the brachiocephalic AVF was then performed, and after surgery the patient presented a striking improvement with a decrease in facial and upper limb edema, as well as complete disappearance of the proptosis. At discharge, oral anticoagulant treatment (acenocoumarol) was indicated for 6 months. |
A 14-year-old previously healthy girl presented 2 weeks after an uncomplicated laparoscopic appendectomy for non-perforated acute appendicitis in a regional hospital. The girl complained of gradually reducing urinary frequency to twice per day and prolonged hesitancy. The micturition stream was initially weak and slow before becoming interrupted. Straining did not produce stronger urinary stream. She had never suffered from urinary tract infections (UTIs) or constipation and opened her bowels daily.\nFollowing an episode of acute cystitis 2 months later, she completely lost her ability to void. She was put on indwelling Foley urinary catheter, and her cystitis was successfully treated with antibiotics. After every attempt to remove the urinary catheter, she had to be catheterized again with 300 to 1200 mL of urine volume registered. She noted loss of urge to urinate and felt only dull pain in suprapubic region and right iliac fossa on extreme bladder distention. The girl was kept on indwelling urethral urinary catheter and referred to a tertiary center to determine the etiology of her urinary retention.\nShe was examined with normal clinical findings and no obvious pathology on abdominal and pelvic ultrasound scan (USS). A pediatric neurologist found nothing abnormal, and magnetic resonance imaging (MRI) of the brain and spine, electromyography (EMG) of the lower extremity, somatosensory-evoked potentials (SEP) of tibial nerve, electroencephalogram (EEG), and lumbar puncture were with no pathology. On USS, the gynecologist described multiple follicular cysts on ovaries bilaterally and found no pathology explaining her urinary retention.\nOur pediatric urologist performed an examination under general anesthesia including a free calibration of the urethra up to 26F followed by normal findings on cystoscopy. Videourodynamic study (VUDS) showed an asensitive and hypotonic bladder. The bladder filling had to be stopped at 360 mL due to the patient's discomfort. Maximum intravesical pressure achieved 11 cmH\n2\nO. When pulling the urodynamic catheter out of the bladder manually, the maximum urethral pressure measured was 120 cmH\n2\nO. On vesicocystourethrogram (VCUG), there was no vesicoureteral reflux, a smooth bladder wall and closed bladder neck (\n).\nPsychologic and psychiatric evaluation identified no major problem. During the following 2 years of repeated admissions to several regional and university hospitals, many of the tests described above were repeated, including an MRI of brain and spine with identical conclusions.\nClean intermittent catheterization (CIC) was recommended to the patient. However, because of poor tolerance of CIC due to frequent macroscopic hematuria and pain, a suprapubic catheter was placed. Thereafter, she suffered recurrent symptomatic afebrile UTIs caused by multi-resistant bacterial strains, e.g.,\nKlebsiella\n,\nPseudomonas\n, or\nEscherichia\n. Finally, after 2 years, based on the history, symptoms, and urodynamic findings, she was diagnosed with Fowler's syndrome (FS).\nFor the treatment of FS, the patient was indicated for S3 neurostimulation. The implantation of two Medtronic S3 neurostimulators, type Interstim II, bilaterally in the upper gluteal region was performed under general anesthesia in two phases. The first phase was a transcutaneous implantation of the electrodes into S3 foramina and their connection to externalized neurostimulators. The first procedure took 30 minutes. As the patient restored her voiding completely back to normal when switching on the neurostimulators and experienced no side effects, she could undergo the second phase 4 weeks later—permanent subcutaneous implantation of the neurostimulators (\n). The second procedure took 15 minutes under general anesthesia.\nWith a transcutaneous remote control, she was able to modify the intensity of stimulating current to avoid any discomfort (\n). On the last follow-up, 4 months after the implantation, she voided four to six times per day with post-void residuals up to 50 mL on USS. Unfortunately, she suffered two prolonged episodes of burning on micturition even after the operation. On both occasions, she was diagnosed with acute cystitis by\nE. coli\n107 that was treated with antibiotics after sensitivity testing. |
A 42-year-old man presented to our pancreas multidisciplinary clinic after a computed tomography (CT) scan (), prompted by a 2-month history of generalized bloating and epigastric discomfort, that demonstrated a 11.2 × 9.6 cm heterogeneous solid appearing mass in the tail of the pancreas. The irregular mass had several small peripheral calcifications and lobulated contours abutting the spleen, stomach, and splenic flexure of colon without any direct invasion. He underwent a distal pancreatectomy and splenectomy with splenic artery lymph node dissection. Intraoperatively the large soft lobular cystic mass at the pancreatic tail was locally contained without any obvious invasion of surrounding structures or gross metastasis. Histopathological assessment of the mass established it as a pT3pN0pMx SPNP (CD56pos nuclear β-cateninpos chromograninneg and synaptophysinneg). Margins were negative without any lymphovascular or perineural invasion. The patient was discharged home after an uneventful period of convalescence in the hospital.\nFour years later, he was referred back to our clinic after discovery of a biopsy-proven recurrence in the splenic fossa (). The bulk of the tumor was densely adherent to the splenic flexure and gastric fundus and was resected with wedge gastrectomy and partial colectomy. A 4 cm nodule of tumor adherent to the diaphragm as well as omentum was removed by dividing the omentum and stripping the superficial layer of diaphragm. The tumor was soft, extremely friable, and fractured with minimal manipulation. It remained densely adherent to the left diaphragm, left kidney, and left adrenal gland. Eventually, we were able to dissect down through the Gerota's fat and strip the anterior capsule of the kidney clean to dissect the tumor off the kidney and the adrenal gland. The other end of the mass remained adherent to the diaphragm and was removed along with a portion of the diaphragm.\nFinal pathology report confirmed the presence of recurrent metastatic SPNP in omentum, diaphragm, accessory spleen tissue, and the gastric fundus. The patient recovered well from his surgery and was discharged home. He underwent CT surveillance at 3-month intervals per his medical oncologist and his first three scans showed stable postoperative changes without any evidence of local recurrence or metastatic spread. However, his next scan showed enlarged retroperitoneal paraaortic nodes that were found to be fluorodeoxyglucose (FDG) avid. He was started on capecitabine with stable disease on recent repeat imaging in April 2018. |
A 68-year-old female was referred to our service for evaluation of erythema and swelling of the right elbow. She was admitted to the hospital 12 days before because of an infected left foot ulcer that required debridement and intravenous antibiotic therapy. Six weeks prior to admission she was seen by a pain management specialist for swelling of the right elbow and she was diagnosed with olecranon bursitis. She was treated with bursa aspiration and corticosteroid injection. Cultures were reportedly negative. One to two weeks after the procedure, she had worsening of swelling, as well as pain and erythema of the olecranon bursa. She did not report fever, weight loss, fatigue, or weakness.\nHer past medical history was remarkable for RA diagnosed at 30 years of age, arterial hypertension, peripheral vascular disease, and asthma. Her surgical history included amputations of the first and second toes of the left foot and two tendon repairs (left hand and left shoulder). She received multiple therapies for RA including auranofin, methotrexate, leflunomide, and etanercept. Her last treatment was golimumab, which she used for 4 years. She did not have further follow-up with her rheumatologist and stopped this treatment about 2 years before onset of olecranon bursitis.\nOn initial evaluation, she had normal vital signs. Scleromalacia was noted bilaterally. Examination of the upper extremities showed ulnar deviation of the metacarpophalangeal joints and swan neck deformities bilaterally. The left wrist had marked limitation of flexion and extension. The right olecranon bursa was tender and swollen, and the overlying skin was erythematous (). Range of motion of the right elbow was not limited. She had multiple subcutaneous nodules in the right leg and a stage IV ulcer on the medial aspect of the left foot. Generalized muscle atrophy was observed. The rest of the examination was unremarkable.\nLaboratory tests showed a white blood cell count of 6.8 × 103/μL, lymphocytic count of 1.2 × 103/μL, hemoglobin of 9.6 g/dL, and platelet count of 344 × 103/μL. Serum creatinine was 0.5 mg/dL and blood urea nitrogen was 17 mg/dL. Wound cultures were negative. Chest X-ray showed mild prominence of the pulmonary markings in the right infrahilar region.\nThe olecranon bursa was aspirated and 9 mL of hemorrhagic fluid was obtained. No crystals were observed under polarized microscopy. Fluid analysis showed a white blood cell count of 3.1 × 103/μL (41% neutrophils, 30% lymphocytes, and 29% monocytes). Bacterial cultures were negative. Fungal culture was positive for Candida parapsilosis; this was sensitive to anidulafungin, micafungin, caspofungin, 5-flucytosine, voriconazole, itraconazole, and fluconazole. The VITEK 2 system (bioMérieux, Inc., Hazelwood, Missouri, USA) was used for fungus identification. The Sensititre YeastOne system (Trek Diagnostic Systems, Cleveland, Ohio, USA) was utilized to determine antifungal susceptibility.\nShe was treated with caspofungin 50 mg intravenously daily for 13 days. Four aspiration procedures were required (every 3–5 days) due to fluid reaccumulation. All samples were cultured but only the first two were positive for Candida parapsilosis. After intravenous therapy, fluconazole 200 mg orally daily was prescribed for one week. She responded well to this therapy but had recurrent swelling of the bursa. Bursectomy was recommended but she did not wish to proceed with the intervention or receive further treatment. After 3 months of follow-up, she continued to have fluid in the olecranon bursa and had some discomfort in the area. |
A 38-year-old man visited our hospital complaining of anterior chest pain. He had no significant medical or family history, and the vital signs were stable. Ischemic events were not observed in electrocardiography, but chest X-ray and computed tomography (CT) showed a cystic lesion (6.0 × 7.0 × 10.0 cm) in the anterior mediastinum (Fig. a). Although the cystic capsule demonstrated contrast enhancement, its fluid component had low radiation absorbance. Based on these findings, we suspected the mass to be a thymic cyst. Blood tests indicated the presence of inflammation (white blood cell count 11,200/μL and C-reactive protein 3.38 mg/dL).\nTwo days after hospitalization, the patient developed dyspnea and his chest pain worsened. Subsequent chest CT showed that the cystic lesion had become inhomogeneous and the radiation absorbance of the cyst’s fluid component had increased (Fig. b). The cyst wall became thickened, and bilateral effusion was observed. Blood tests indicated that hemoglobin levels had decreased from 15.8 to 12.8 g/dL, and levels of inflammatory markers had increased, with the fever exceeding 38.5 °C. Needle aspiration biopsy and tumor wall biopsy with a small skin incision were performed; however, we could not obtain a diagnosis. One week after admission, general condition and laboratory data of the patient gradually improved. A chest CT on day 13 showed that the tumor had become small in size with a thickened wall (Fig. c). The effusion on the right side had decreased and that on the left side had disappeared.\nThe patient had recovered enough to undergo surgery; the tumor was resected by sternotomy on day 18. The tumor was found to be encased in a smooth, yellow, and elastic coat. The tumor was densely adhered to the junction of the left brachiocephalic vein and superior vena cava, and it was required to detach the tumor from the dense adhesion site carefully. The right phrenic nerve was preserved, and the right pleural effusion was serous. The tumor and thymic tissue were resected en bloc. The operative time was 288 min, and the estimated blood loss was 521 mL. The resected tumor was covered with a thick, fibrous capsule, and the lumen was filled with necrotic tissue and hemorrhagic material (Fig. a, b). The postoperative course was uneventful, and he was discharged on day 26.\nThe pathological findings showed a fibrotic cyst wall; the cyst was filled with necrotic tissue. The slight proliferation of lymphocytes was confirmed in the necrotic tissue and around the cyst wall (Fig. a, b). The tumor was diagnosed as type B1 cystic thymoma (Fig. c). As the tumor did not appear to have spread beyond the capsule, it was determined to be at Masaoka stage I. Nevertheless, the dense adherence of the tumor to its surrounding tissue indicated the possibility of invasion, and postoperative radiotherapy (50 Gy) was administered.\nTwo years after the surgery, recurrent metastasis of the tumor was found on the right pleura and the left upper lobe of the lung. The patient was treated with chemotherapy, radiotherapy, and local resection. The patient remains alive 12 years after the first surgery. Following an analysis of the tissue obtained from the resected recurrent tumor, the pathological diagnosis was changed to type B3 thymoma. |
A 77-year-old male patient was diagnosed with prostate cancer on biopsies taken because of a high prostate specific antigen (PSA) serum concentration (10.35 ng/mL). Histological examination revealed prostate cancer with Gleason score 6 (3 + 3) and the clinical stage was determined as T1cN0M0 by magnetic resonance imaging (MRI), computed tomography (CT) and bone scintigraphy. The patient had a previous history of trans-urethral resection of prostate (TURP) for benign prostatic hyperplasia more than ten years prior. He was not suffering from diabetes mellitus, hypertension or neurogenic bladder. He was administered with external beam radiation at a total dose of 50 Gy in 25 fractions and high-dose rate brachytherapy at a total dose of 18 Gy in 2 fractions. After treatment, serum PSA value immediately decreased (nadir value 0.21 ng/mL).\nThree years later, the patient received medical examination due to dysuria during follow up. Cystoscopy revealed that prostatic urethra was obstructed by yellow necrotic tissue, and prostatic and membranous urethral mucous membrane were white and hemorrhagic. These abnormal findings were thought to be caused by previous radiation therapies. As urethral catheterization was difficult because of the obstruction, he received transurethral resection of the necrotic tissue. Resected tissue did not contain viable cancer. However, after the operation, complete urinary incontinence occurred and gross hematuria and pyuria persisted. Several months later, he returned to the hospital due to inguinal swelling and fever. His inguinal and femoral region turned purple with pus present. Leukocytosis was observed (white cell count 12,870 /mm3), and CRP was elevated at 22.28 mg/dL. CT scan showed extensive gas within the femoral and retroperitoneal tissues and pubic bone fracture (Fig. ). He underwent immediate surgical exploration for suspected necrotizing fasciitis. Exploration of both the groin and right thigh revealed severe soft tissue infection involving the skin, subcutaneous fat and fascia. The pubic bone became necrotic and the ventral part of the prostate was absent, thereby exposing the urethral catheter. These operative findings suggested necrotizing fasciitis was caused by urinary fistulation into the pubic symphysis and subsequent pubic symphysis osteomyelitis. Microbiological exam showed growth of E. coli and Enterococcus faecalis and patient was treated with broad-spectrum antibiotics. Simultaneously, negative pressure wound therapy was performed. The patient underwent ureterocutaneous urinary diversion on day 8 after surgery. On day 148 post surgery, the patient’s wound recovered and he regained mobility. |
In a 19-year-old, mentally disabled male, chest radiography was done because of a sudden episode of cough. Metallic, hook-shaped foreign bodies were identified in both the main bronchi.\nThe right-sided FB was removed by fiber-optic bronchoscopy in the regional hospital, whilst the left-sided extraction failed with the left-sided FB persisting in the left main bronchus ().\nUpon urgent admission in a tertiary institution, extraction was attempted by rigid bronchoscope under general anesthesia. Bronchoscopic extraction failed, associated with some bleeding and subcutaneous emphysema immediately after the intervention. The increasing mediastinal and subcutaneous emphysema raised suspicion about an iatrogenic airway lesion, so surgery was indicated. Esophageal injury was previously ruled out by esophagoscopy, revealing many metallic FBs in the stomach. At thoracotomy, a significant mediastinal emphysema (), together with diffuse adhesions, was noticed. After the lung liberation, a proximal 0.5 cm of the noncurved part of the metallic hook was found to protrude through the perforated membranous wall of the left main bronchus, 1 cm away from the descending aorta (, arrow). The part of the FB protruding outside the bronchus was grasped by the clamp and, by following the curved shape of the FB, gentle maneuvers were applied by pulling the sharp end (hook) of the FB in the direction outside the bronchus. The FB was removed from the bronchus (insert on ) without the need for additional bronchotomy. The remaining 10 × 1 mm defect in the bronchial wall, caused both by manipulations during a bronchoscopic extraction attempt and subsequent surgical extraction, was sutured by interrupted PDS 3-0 stitches, and the lung fully inflated. No air leaks appeared during the water test. Having in mind the dimensions of the defect and tensionless suture, no suture-line protection was performed.\nAfter the thoracotomy closure, laparotomy was done and several sharp metallic pieces of different shapes were removed from the stomach (Figures and ). This was followed by an uneventful postoperative course and the discharge after 14 days.\nAfter three years, the patient was urgently readmitted for the new episode of the metallic foreign body aspiration (Figures and ). The abdominal radiography revealed metallic pieces in the digestive tract as well (). With the surgical team on site, rigid bronchoscopy under general anesthesia was done. As the tip of the FB was not impacted in the mucosa, it was possible to grasp it with the rigid biopsy forceps and to withdraw it up to the tip of the bronchoscope. Because of the curved shape of the FB and the impossibility to remove it through the instrument, the FB and bronchoscope were pulled out from the patient together, with the FB firmly grasped, followed by reintubation with the same bronchoscope (Figures and ). After a careful check-up for bleeding and mucosal damage, the patient was extubated.\nMetallic pieces left the digestive tract spontaneously after a couple of days. |
Our patient is a 54-year-old female with a past medical history of hypothyroidism and very severe obesity (BMI 48 kg/m2). She underwent laparoscopic gastric sleeve surgery in the year 2012. Results were non-satisfactory in terms of weight loss with a difference of 6 kg/m2 in BMI post-procedure. So after six years, she underwent a laparoscopic biliopancreatic diversion with a duodenal switch. She had an uneventful postoperative recovery period. An upper gastrointestinal (GI) study contrast post-procedure did not reveal any evidence of obstruction or leak. The patient was discharged home two days after the procedure. A few days later, she started experiencing three episodes of nausea with brown-colored vomitus. She was found to be septic, with a heart rate of 110 beats per minute and temperature of 100.2oF. Her white blood cells count was 12/mm3.The source of infection was presumed to be intraabdominal considering her symptoms. Computed tomography (CT) of the abdomen and pelvis showed mildly dilated proximal small bowel loops. The patient was started on empiric antibiotic therapy with ceftriaxone 1 gm intravenous (IV) daily and metronidazole 500 mg IV every eight hours. Symptoms did not improve, so she was taken back to the operating room for diagnostic laparoscopy. Partial small bowel obstruction was noted along with ischemia of a segment of the ileum that was part of the duodenoileostomy due to mesenteric dissection. She underwent an open revision of the small bowel anastomosis with resection and anastomosis for the obstruction revision of the duodenoileostomy. Her hospital stay post-surgery remained uneventful. Diet was advanced gradually throughout the hospital course and a week later, the patient was discharged home with outpatient follow-up. Three weeks after that procedure, she noticed a productive cough with thick, yellow, foul-smelling phlegm and shortness of breath. She saw her primary care physician. A chest X-ray performed showed a right lung infiltrate with a right-sided pleural effusion. She was started on treatment with augmentin 500 mg/125 mg every eight hours. Her symptoms became worse so she came to the emergency room. Her vitals showed blood pressure 129/79 mmHg, heart rate 86 beats per minute, respiratory rate 20 breaths per minute, and temperature 98.6oF. Pulse oxygen saturation was 97% on room air. Mild leukocytosis was evident (white blood cells count 11.4/mm3 with no bands or left shift). A chest CT showed loculated, right-sided hydropneumothorax with almost total collapse of the right lung (Figure ).\nThere was a fistulous connection evident, extending from the surgical anastomosis in the stomach/bowel in the right upper quadrant through the right hemidiaphragm to the right hemithorax. These CT scan findings were new as compared to a CT scan obtained for this patient six months prior to the duodenal switch when she presented to the emergency department for non-specific left-sided chest pain. To analyze the anatomy of the fistula further, an upper gastrointestinal fluoroscopic contrast study was performed that showed a large fistula from the distal stomach prior to the duodenal bulb opening to the right pleural cavity (Figure ).\nConsultations from gastroenterology and cardiothoracic surgery teams were obtained. Chest tube drains were placed with the plan of eventually performing a video-assisted thoracoscopic surgical decortication. Post-procedure CT showed patent chest tubes draining the right pleural cavity. The drained fluid was exudative in nature as per Light’s criteria (fluid lactate dehydrogenase > 12,000 u/L and total protein ratio = 0.7) and culture from the right lung empyema grew Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, and Candida albicans. An infectious disease consultation was placed at this time. The patient was started on levofloxacin 750 mg IV daily for two weeks as per the sensitivity result obtained for Escherichia coli, Klebsiella pneumoniae, and Pseudomonas aeruginosa. Micafungin 100 mg IV daily was started for the infection with Candida albicans. This was later switched to Diflucan 400 mg IV daily for a total of two weeks. Repeat cultures from the draining fluid were negative toward the end of the second week.\nFor treatment of the fistula, the patient was transferred to another facility for esophagogastroduodenoscopy (EGD) and possible clipping of the fistula due to the unavailability of that particular service in our hospital. As a result, there was a delay of 16 days from admission to the treatment of the fistula. When the EGD was performed, it showed that there was no anastomotic leak from the previous surgery. No evidence of any stricture was identified at the previous anastomosis. A small fistulous tract was noted in the distal part of the antrum likely secondary to ulcer formation that was noted in very close proximation to the fistula tract. An endoscopic clipping was performed. A post-procedure contrast study performed on the same day revealed complete closure of the fistulous opening. The patient was transferred back to our facility after the procedure. An upper GI contrast study performed two days later showed residual leakage from the distal stomach to the right upper quadrant. A repeat EGD was performed along with fulguration of a fistulous opening with argon beam coagulation and repeat orthoscopic clip application with complete obliteration of the fistula tract. This was confirmed by an upper gastrointestinal contrast study showing no persistent fistulous communication between the post-bulbar duodenum and pleural space (Figure ).\nAfter confirmation of fistula obliteration, a video-assisted thoracoscopic surgery was performed followed by the washout of the right pleural space with the placement of a right-sided chest tube. The patient was discharged home after the procedure and has been followed by multidisciplinary teams on an outpatient basis. |
A 36-year-old Brazilian male patient was admitted to the hospital with a palpable lump in his right breast, located at the junction of the upper quadrants of the right breast (Fig. ). On physical examination, the lesion appeared firm with irregular margins. Axillary lymphadenopathy was negative and there were no palpable supraclavicular nodes. On breast imaging, ultrasonography showed a hypoechoic mass with partially defined contours measuring 4.0 × 3.0 cm, located at the upper region of the right pectoralis major muscle at the 12 o’clock position with muscle infiltration (Fig. ). Histological examination of core biopsy samples revealed a malignant tumor. Preoperative exams, such as X-rays and chest CT scan, abdominal US did not show any signs of disease. Radical mastectomy was then performed, due to pectoralis major muscle infiltration, consisting in removal of the breast along with the major and minor pectoralis muscles. Biopsy of the sentinel lymph node was performed. Gross examination revealed a solid tumor measuring 3.7 × 3.5 cm with a yellowish-tan cut surface and local foci of hemorrhage. Histopathology showed intravascular papillary proliferation of endothelial cells, spindle cell areas and necrosis, atypia and prominent mitotic figures, consistent with the diagnosis of high-grade angiosarcoma with areas of infiltration of the pectoralis major muscle (HE staining, magnification of 400×) (Fig. ). Histopathology also demonstrated a surgical specimen with clear margins, absence of angiolymphatic and perineural invasion, in addition to sentinel lymph node free of metastasis. Immunohistochemical study revealed a tumor positive for CD31 marker (Fig. ), confirming the vascular nature of the tumor. At the two-week follow-up of the surgical procedure, adequate wound healing was observed, without any evidence of the disease. The patient was transferred to the clinical oncology department, where he presented with severe headache and seizures after the second cycle of adjuvant chemotherapy with paclitaxel. Magnetic resonance imaging of the brain was ordered, revealing a right frontal parasagittal lesion, measuring 1.3 × 1.1 cm with a hemorrhagic component and perilesional edema, suggestive of brain metastasis. The disease progressed rapidly, culminating in the patient’s death at 20 days after the onset of neurological symptoms. |
A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition. |
A three-year-seven-month-old girl was referred to the Pediatric Dentistry Section, Department of Stomatology, National Cheng Kung University Hospital, to have a swelling and erythematous mass on the facial gingiva of the primary maxillary right central incisor evaluated. According to her parents' statements, the eruption time of this tooth was the latest of all her upper anterior teeth. Immediately after the eruption, it was positioned more palatally than the adjacent incisors. A small, white, tooth-like substance existed on the facial gingiva of this tooth. Then the tooth-like substance spontaneously exfoliated. About 1.5 years ago, the mother became aware of her daughter's gingival mass. She could not remember that her daughter had undergone any trauma. They went to a local dental clinic for evaluation. The dentist performed endodontic treatment for the central incisor because he found caries and speculated that the gingival swelling had an endodontic origin. Five months later, they went to the same clinic because the lesion became larger. The dentist decided to refer the child to our hospital. During her initial examination in our hospital, we noticed a fiery red gingival mass that neither bled nor felt palpation pain (). The probing depths of the tooth were within normal range. The tooth was a little palatally displaced but not mobile (). The periapical radiograph revealed no periapical lesion, root resorption, or radiopaque abnormality (). Our initial but tentative diagnosis was a pyogenic granuloma. After the lesion had been locally debrided and irrigated with 0.2% chlorhexidine, the patient's mother was instructed in oral hygiene techniques. There was no improvement in the patient's condition after 2 weeks of follow-up. After we had consulted with periodontics and oral-maxillofacial surgery specialists, we suggested an excisional biopsy under general anesthesia for the patient. After we had obtained the consent of the parents, we removed the mass using surgical scalpels; bleeding was stopped using electrocautery in the operating room. Amoxicillin and acetaminophen were prescribed for the patient. The surgical specimen was sent to National Cheng Kung University Hospital's Pathology Department for evaluation. The gross specimen measured 10 × 5 × 2 mm. A microscopic examination revealed a small island and cords of odontogenic epithelium in a loose and primitive-appearing connective tissue that resembled dental papilla. The mass contained calcified material and an enamel-like deposit. Abundant small blood vessels surrounded the odontogenic epithelium (). The pathological diagnosis was an ameloblastic fibro-odontoma. A one-year postoperative follow-up revealed no evidence of recurrence, but only a mild gingival inflammation (). The patient's parents were advised to bring her back for continuous follow-ups and to conscientiously provide her with good oral hygiene. |
A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points (). |
A 47-year-old man had intermittent dull pain in the upper abdomen after eating greasy food for 2 days associated with melena twice a day before he was admitted to the local hospital. During hospitalization, the patient experienced hemorrhagic shock and underwent an initial emergency gastroscopy. An ulcer (0.8 cm × 0.8 cm) was identified on the anterior wall of the duodenal bulb (Fig. ). The ulcer had a large area, and its anatomical location was difficult to approach. Moreover, the ulcer caused arterial bleeding, thus traditional endoscopic hemostasis was difficult to achieve; thus, effective hemostasis was not attained. Due to the large amount of bleeding, poor endoscopic field of vision, and small operating space, the ulcer could not be treated. Thus, the patient was immediately transferred to surgery, and a gastroenterostomy (Billroth II) + Bancroft operation was performed. During the operation, ulcers with scabs were observed near the descending part of the duodenal bulb and the ulcerated area was deep. After determining the area for surgical excision, it was still difficult to fully expose the intestinal tubes of the duodenal ulcer from the outside; therefore, the surgeon at the local hospital selected the Billroth II + Bancroft operation. The bleeding stopped after the operation.\nSeven days postoperatively, the patient again experienced melena, which suggested that the ulcer had begun bleeding again. He was immediately transferred to the Department of Gastroenterology at the First Affiliated Hospital of Nanchang University, where he underwent endoscopic hemostasis for the second time. However, no lesion was observed. Digital subtraction angiography (DSA) was also performed, and it revealed no signs of hemorrhage (Fig. ). The patient's condition was stable after blood transfusion and acid inhibition therapy. Five days after he was admitted to our hospital, the patient suddenly vomited a large quantity of blood (approximately 800 mL), and his hemoglobin level dropped to 57 g/L. An emergency endoscopy was performed, and a large amount of fresh blood was observed in the esophagus and residual stomach. Repeated washing of the anastomotic site showed that there were no bleeding points. Then, we used a JI enteroscope (PCF-Q260JI, Olympus, Tokyo, Japan), which can extend deeper than a normal gastroscope. Fortunately, blood clots were observed in the input loop, and residual blood vessels were observed near the blind end (Fig. ). Next, the positioning for the OTSC system was confirmed above the lesion. By rotating the wheel, which was attached to the endoscope, the OTSC was quickly deployed to the target site and the hemostasis process was completed. Repeated flushing revealed no additional blood (Fig. ), indicating that the bleeding had stopped. We performed follow-up examinations at 5 months, 10 months, and 1 year after endoscopy (Fig. ). Rebleeding was not observed in any of these examinations. |
A 62-year-old woman had undergone emergency surgery for a giant ovarian tumor rupture at another hospital in December 2013. A duodenal perforation occurred after surgery but improved with conservative treatment. She had undergone long-term nasogastric tube placement for 4 months because she was on a mechanical ventilator and did not receive proton pump inhibitors (PPIs). Thereafter, the patient experienced dysphagia, and a video fluoroscopic examination of her swallowing revealed the reflux of contrast medium from the stomach to the esophagus in February 2014. An esophagogastroduodenoscopy (EGD) revealed circumferential reflux esophagitis (grade D) and a stricture located 25 to 40 cm from the incisor teeth. She received medical treatment with fasting and PPIs. She underwent an EGD again in March 2014. The reflux esophagitis had improved somewhat, but the esophageal stricture located 33 cm from the incisor teeth had worsened, making it difficult to pass the nasal endoscope. Thereafter, balloon dilatation was attempted, but the stricture did not improve and she was referred to our hospital in April 2014. She had a history of bronchial asthma.\nUpon admission, she had a height of 152.5 cm, a weight of 41.6 kg, a body mass index of 17.8, a blood pressure of 108/58 mmHg, a temperature of 36.4 °C, and pulse rate of 74 beats/minute, with no significant physical findings. Laboratory findings showed a hemoglobin level of 11.9 g/dL, a serum glutamic oxaloacetic transaminase level of 54 U/L, and a serum glutamic pyruvic transaminase level of 119 U/L, indicating mild anemia and liver dysfunction.\nAn upper gastrointestinal series revealed a severe stricture measuring 85 mm along the longitudinal axis from the middle to lower thoracic esophagus (Fig. ). An EGD showed a cicatricial stricture beginning 25 cm from the incisor teeth, making it difficult to pass the endoscope through the esophagus (Fig. ). A contrast-enhanced chest computed tomography (CT) scan examination revealed marked wall thickening from the middle to lower thoracic esophagus (Fig. ). FDG-PET/CT showed a slight accumulation of isotope in the esophagus, and while a malignant disease could not be completely ruled out, a diagnosis of esophagitis seemed more probable. Although a 24-h pH monitoring test is necessary for the diagnosis of gastroesophageal reflux disease (GERD), this test was not performed because the pH catheter was not expected to pass through the esophagus because of the severe stricture. The clinical course and the above findings led to a diagnosis of benign esophageal stricture caused by reflux esophagitis.\nBecause endoscopic balloon dilatation did not improve the stricture, a thoracoscopic esophagectomy was performed []. The patients were placed in a prone position after intubation using a single-lumen endotracheal tube and a bronchial blocker tube. Only the left lung was ventilated, and a pneumothorax in the right chest was created using 6 mmHg of CO2 gas. Five trocars were inserted into the right thoracic cavity. The 12-mm trocar inserted into the ninth intercostal space on the inferior scapular angle line was used for a flexible endoscope. The anterior pleura and the posterior pleura of the upper posterior mediastinum were incised around the esophagus, and the upper esophagus was mobilized. Lymph node dissection was not performed because the patient had been diagnosed as having a benign esophageal stricture. The arch of the azygos vein was divided, and the right bronchial artery was preserved. Next, the anterior pleura and the posterior pleura of the middle and lower posterior mediastinum were incised around the esophagus. When the middle and lower esophagus was also mobilized, a severe fibrotic change between the esophagus and the descending aorta was observed. This fibrotic change seemed to be similar to the change in a case that had received chemoradiation therapy (Fig. ). Finally, the upper esophagus was transected using a stapler device, and the thoracic esophagus was successfully mobilized. At the end of the thoracoscopic procedure, a single 32-Fr chest tube was inserted. The patient was then placed in a supine position. The stomach was mobilized, and a gastric tube was created. An antethoracic route was chosen because the patient had a BMI of 17.8 kg/m2 and was treated with total parenteral nutrition, which posed a surgical risk. An anastomosis between the cervical esophagus and the gastric tube was performed using a circular stapler in the neck, and a tube jejunostomy was created.\nThe gross pathological findings showed a circumferential stricture with white scar formation from the lower esophagus to the cardia. Histologically, the infiltration of inflammatory cells, mainly neutrophils, lymphocytes and plasma cells, and fibrosis were observed. Erosion and ulceration were seen, but there were no malignant findings (Fig. ).\nHer postoperative course was uneventful, and she was discharged on day 19. At present, 1 year and 8 months after surgery, she is attending an outpatient clinic.\nEsophageal strictures have a variety of causes. Makuuchi classified esophageal strictures into functional and organic strictures. The former included esophageal achalasia, diffuse spasm, vigorous achalasia, and nutcracker esophagus, while the latter included malignant neoplasms, reflux esophagitis, esophageal web, and postoperative anastomotic stricture. It was also described that most esophageal strictures caused by reflux esophagitis occur at the lower end of the esophagus []. The majority of esophageal strictures are malignant, and benign esophageal strictures are rare. Recently, the use of PPIs has decreased the number of patients with benign esophageal strictures. The incidence of esophageal stricture is 1.1 among 10,000 persons, and 68 % of esophageal stricture cases are peptic. GERD, hernia, ulcer, and heavy drinking increase the risk of stricture []. Also, patients with esophagitis reportedly have an eight times higher risk of stricture [].\nThe mechanism of stricture formation arising from reflux esophagitis is considered to be as follows: gastric acid reflux causes inflammation in the lamina propria which is then disrupted, leading to stricture formation []. In addition, esophageal stricture caused by long-term nasogastric tube placement has also been reported, and according to the report, the use of a nasogastric tube for more than 2–4 weeks should be avoided []. The present patient had reflux esophagitis, had undergone long-term nasogastric tube placement, and did not receive PPIs, placing her at a high risk for stricture.\nMalignant transformation has to be taken into account in esophageal strictures associated with reflux esophagitis. The reflux of digestive juice is widely known to cause the development of Barrett’s esophagus, eventually leading to Barrett’s esophageal cancer, and controlling the reflux of digestive juice to prevent malignant transformation is of primary importance. In this case, the clinical course and the findings of CT and FDG-PET/CT led to a diagnosis of benign esophageal stricture caused by reflux esophagitis. Postoperatively, the resected specimen revealed no malignant findings and the preoperative diagnosis was proven to be correct.\nvan Boeckel and Siersema previously described an algorithm for treatment selection. According to their report, endoscopic dilatation is successful in more than 80–90 % of cases, but recurrence occurs within 1 year in one third of cases. At the time of recurrence, the combination of dilatation and steroid injection or stenting should be considered. Surgery should be a last-resort treatment option [].\nSurgery for benign esophageal stricture can be classified into two types. In cases where endoscopic dilatation can be performed, antireflux surgery, such as a Toupet, Belsey, or Nissen procedure, is performed following stricture dilatation. In cases where the stricture cannot be dilated, an esophagectomy and reconstruction are performed. In addition, surgery is indicated in (1) patients in whom stricture dilatation is not useful, (2) patients with frequent aphagia, (3) patients with intractable refractory esophagitis, and (4) patients with aspiration pneumonia, etc. []. In the present case, surgery was performed because the patient was refractory to endoscopic treatment and had reflux esophagitis (grade D), and the extended length of the stricture persisted even after conservative treatment.\nThe present case was characterized by an 85-mm-long segment of stenosis extending from the middle to lower thoracic esophagus. As described above, most strictures associated with reflux esophagitis are usually localized at the lower end of the lower esophagus and are found at a distance of 1 to 4 cm from the gastroesophageal junction [, ]. A search of PubMed using the keywords “esophagus,” “stricture,” and “benign” yielded five articles on benign stricture caused by the reflux of digestive juice describing the length of the stricture [–] (Table ). None of the severe strictures measured >8 cm in length in these reports. Accordingly, the present case appears to be relatively rare. |
A 26-year-old woman gravida 1, para 1 came to emergency with severe lower pain abdomen and vomiting since last two hours. She was 18 weeks pregnant and her routine malformation scan done one week back was normal. Her previous delivery was by Cesarean section for transverse lie. She denied history of dysmenorrhoea or of pain earlier in previous pregnancy. The patient had no significant medical or surgical history. No records of previous cesarean section were available. On examination patient was in hypovolemic shock with severe pallor, hypotension, and tachycardia. The abdomen was tense and symphysiofundal height was 24 weeks. Her bowel sounds were normal. On pelvic examination cervix and vagina were healthy, there was no bleeding through os, and size of uterus could not be made out due to intense guarding. Per rectal examination was within normal limits. Immediately two large bore intravenous cannulas were inserted, one liter of fluid was rushed, patient was catheterized (she passed 50 mL of clear urine), and urgent investigations and cross match was sent for four units of blood. Her hemoglobin was 4.6 g%, one unit blood was rushed, and after stabilization urgent emergency ultrasound was done. Her uterus was found to be empty, with a hyperechoic shadow adjacent to it. There was marked free fluid in abdomen. Immediately consultant review was sought, which revealed unicornuate uterus with noncommunicating rudimentary horn (). The rudimentary horn was found to be ruptured on posterolateral wall () with moderate free fluid in peritoneal cavity (). A dead fetus was found floating in the abdominal cavity (Figures and ). The patient was taken for explorative laparotomy. Intraoperatively a unicornuate uterus with rupture of noncommunicating rudimentary horn was confirmed and a dead fetus was found in peritoneal cavity with four liters of hemoperitoneum (Figures and ). There was no endometrial cavity in the noncommunicating horn. Both the ovaries and tubes were normal (Figures and ). Previous cesarean scar was healthy (). Placenta was found separated in abdominal cavity. Excision of rudimentary horn, ipsilateral salpingectomy, and peritoneal toileting was done. Patient received five units of blood transfusion. She had an uneventful recovery and was discharged on day 7 post operative with an advice for hysterosalpingogram and intravenous pyelogram 6 weeks later. |
A 62-year-old male patient with advanced undernutrition caused by a multiple system atrophy for at least 3 years was referred to our tertiary center for PRG. The PRG procedure was performed under local anesthesia with fluoroscopic guidance after informed consent of the patient.\nFirst, the absence of any obstacle in the left hepatic lobe was confirmed under ultrasound examination. Then, local anesthesia was administered with a 22G needle, from the skin to the stomach, with a mixture of 20ml of Lidocaine, Ropivacaine and Bicarbonate. Three anchors were then placed in order to achieve gastropexy. Finally, a 16F gastrostomy tube was placed, using the Seldinger technique.\nThe treatment was performed without early complications and the patient was transferred to the neurology department. Enteral nutrition was initiated 24 hours later. He was readmitted three weeks later for slight hemorrhagia, with a decrease of 5.4 hemoglobin points (from 12.9 to 7.5 g/dl) in 25 days, which required transfusion of two units of red blood cells. Initially, a surgical approach with cauterization under local anesthesia, in the peripheral area of the gastrostomy, was carried out. Subsequently, the bleeding persisted, requiring a computed tomography (CT) scan which showed a correct placement of the three anchors and the gastrostomy tube, without subcutaneous or intra-gastric hematoma (). A 9mm focal dilatation from the left superior epigastric artery, in contact with the gastrostomy tube and one of the three anchors was also vizualized. No active leakage of contrast agent was observed. It was compatible with a pseudoaneurysm. After a multidisciplinary meeting including surgeons and vascular radiologists, percutaneous embolization was decided. The patient was then transferred to the interventional radiology room. An ultrasound confirmed a pulsatile rounded image in color Doppler, into the left straight muscle of the abdomen, in contact with the left superior epigastric artery. First, the pseudoaneurysm was punctured under ultrasound guidance, using a 22G-needle. Under fluoroscopic guidance, iodine-contrast agent was injected, which allowed us to confirm the correct position of the needle. After flushing the needle with 5% dextrose solution, a mixture of 0.5ml of N-butyl cyanoacrylate (NBCA) (Glubran 2, GEM Srl, Viareggio, Italy) and ethiodized oil (Lipiodol, Villepinte, Guerbet, France) was injected in a 1:1 ratio, until complete opacification of the pseudoaneurysm (). The control CT scan showed the persistent permeability of the superior epigastric artery, the absence of contrast leakage, and non-opacification of the pseudoaneurysm (). The patient had no further bleeding and the remote control CT scan showed complete embolization of the pseudoaneurysm. The patient died 6 months later from his underlying disease. |
A 9-month-old female infant with no significant medical or surgical history was admitted due to a febrile UTI. Her urine culture study demonstrated Escherichia coli of more than 100,000 colony forming units/mL. During evaluation, renal ultrasonography showed normal findings, but VCUG was performed to detect VUR by a skilled pediatric radiologist and pediatrician on the seventh day of her hospital stay. A 5-Fr. foley catheter with a balloon was inserted into the urethra, and residual urine was removed. An 80 mL - a mixture of saline and contrast media, was instilled using a 50-mL syringe, under a fluoroscopic guide until the dome of the bladder completely distended. The bladder was normal in contour, and a grade II VUR within the right ureter and a grade III VUR within the left ureter were noted (). Afterwards, the 5-Fr. foley catheter was removed. During the voiding time, she voided spontaneously. However, in just a few seconds, intraperitoneal contrast media was observed (). On physical examination, the patient was found tachypneic with a distended and tender abdomen. Her vital sign was checked as respiratory rate was 32 per minute, and blood pressure was 103/67. The patient was transferred to the urology department. She was immediately sent to the operating room and underwent exploration. Urine, blood clots and contrast media were evacuated from the peritoneum. An approximately 3 cm long bladder rupture from the left side of the bladder wall was found after removal of the contrast media remnant from the peritoneum. The bladder was closed in two layers using an absorbable 5-0 vicryl sutures. A 5-Fr. foley catheter was left per urethra. A suprapubic tube was not used. Perioperative antibiotics were administered, and the foley catheter was removed on postoperative day 7. No leakage of the bladder upon postoperative cystography was noted. The patient's recovery was uneventful and she can void spontaneously to date. |
An 8-year old neutered male Rottweiler with a body weight (BW) of 52 kg and a body condition score (BCS) of 5/9 was presented for a severe flare-up of watery diarrhoea and anorexia. The dog was already under treatment as an outpatient for previously diagnosed CE at a referral animal hospital, and was now admitted for fluid therapy and supportive care at the same animal hospital. On admission, the dog was on maintenance treatment with budesonide (3 mg on 2 days out of 3, Entocort; Tillotts Pharma GmbH, Rheinfelden, Germany) and a single protein source diet. The dog had been clinically stable with normal faeces for 2.5 years. Diarrhoea appeared once when budesonide had been tapered to 3 mg every other day, but was absent on a maintenance dose of 3 mg budesonide 2 days out of 3. Biopsies from the gastrointestinal tract had been collected endoscopically 4 years previously and showed mild to moderate lymphocytic-plasmacytic inflammation. Laboratory and imaging data at the time of admission to the animal hospital are shown in Tables and .\nPrednisolone (Prednisolon; Pfizer, Sollentuna, Sweden) at a dose of 10 mg q 24 h was added to the maintenance dose of budesonide for 3 weeks, which brought the diarrhoea temporarily into remission but led to calcinosis cutis, prompting cessation of prednisolone treatment and tapering of budesonide to 3 mg q 48 h. This was associated with recurrence of diarrhoea. The dog continued to have diarrhoea as well as excessive flatulence and halitosis during the following 12 months. Numerous treatment protocols, including additional immunosuppressive treatments such as cyclosporine and mycophenolate mofetil, metronidazole and dietary changes were tried at different times during this period (Table ). A faecal microbiota transplantation was given once as a rectal enema at a dose of 5 g of frozen donor stool per kg body weight as described by Chaitman and co-workers []. The stool was thawed and blended with 0.9% saline on the day of administration. The donor dog was clinically healthy, free of intestinal parasites, bacterial pathogens and extended beta-lactamase resistant E.coli.\nThe dog was refractory to all treatments and had liquid diarrhoea 4–5 times daily, including once at night, and a canine inflammatory bowel disease activity (CIBDAI) index of 7 (consistent with moderate IBD) []. The poor response to medical and dietary intervention raised the suspicion of BAD. Cholestyramine (Kolestyramin Alternova; Orifarm Generics, Odense, Denmark) treatment was started with 2 g q 24 h for a week. The owners immediately noticed that the faeces became much firmer during 12 h after cholestyramine administration, but watery diarrhoea reappeared later during the day. The dose was increased to 2 g q 12 h, which further improved the quality of the faeces, reduced the frequency of defecation to 2–3 times daily, and markedly reduced flatulence. After increasing the dose to 3 g q 12 h, the diarrhoea stopped, CIBDAI had decreased to 2 (clinically insignificant disease) and faecal scores had improved from 5/5 (liquid diarrhoea) to 3/5 (formed but soft faeces) []. Dose reduction to 2 g cholestyramine q 12 h led to return of unformed feces within 36 h. The cholestyramine dose was again successfully increased to 3 g q 12 h. At the time of writing, diarrhoea had not recurred for 11 months, excessive flatulence had disappeared, and vomiting had ceased. Furthermore, the BCS had increased to 7/9 and the dog owner described the dog as more playful than during the past several years. |
A 29-year-old married woman and home maker, diagnosed with moderate depression, presented for therapy following the discovery of her husband's chats of a sexual nature with his cyber chat partner via Internet, since the last six months. She reported that this was the second time she had caught him engaging in a sex chat with the same partner: Her husband had been friends with the cyber partner for five years. However, chats of a sexual nature had begun since the last one-and-a-half years, which she discovered for the first time eight months ago. She had become increasingly suspicious of her husband as he spent considerable time chatting on the Internet and had on occasions compared her with his chat partner, whom he had described as ‘smarter and more intelligent’. A subsequent search of the recent chat room conversations revealed that they had been sex chatting. When she confronted her husband, he denied they were having an affair, but readily agreed to discontinue chatting with his cyber partner. She was extremely hurt, but decided to put the issue behind them and move on with their lives as before. A few months later the husband again began spending long hours on his computer, late into the night. He explained that it was due to his trying to meet deadlines for some ongoing projects at work. She initially accepted his explanations as he did have a very demanding job. However, she noticed that when she approached near him while he worked on his computer he would immediately close down his computer windows. A search to confirm her suspicions proved correct again when she discovered that her husband had resumed sex-chatting two months later with the same person, who chatted with him using a different name. She was devastated with husband's ‘cheating’ on her. Her distress was heightened by her husband's strong denial of the issue as infidelity on his part.\nIn addition, she expressed her dissatisfaction at being a homemaker and felt inferior to her friends who had been less successful than her in school but were now doing well in their careers.\nThe marital evaluation suggested the possibility of other difficulties in the marital relationship and hence it was deemed necessary to explore this area in detail. The husband was invited to participate for conjoint sessions and he accompanied his wife for the following session. Although he admitted that he had been cybersex chatting, he did not perceive it as infidelity. He felt it was not so, as there had been no real physical contact and he merely engaged in it to ‘unwind and relax’ after work.\nDetailed exploration of the couples overall satisfaction with the quality of their marital relationship revealed that both were dissatisfied with it since the last few years. She reported being increasingly unhappy with her husband's long working hours, which became even longer after his promotion at work (husband returned home by midnight or even later, on most days). Additionally, enquiry about the level of satisfaction with their sexual relationship revealed that husband was sexually dissatisfied. The couple had been resorting to coitus interruptus as husband was allergic to latex and the wife could not tolerate the side effects of contraceptive pills. She did not report sexual dissatisfaction, however. The husband's rationalization of his action as not amounting to infidelity resulted in its continuance, despite awareness of his wife's objection and distress related to it. Differences in the couple's perception of the issue resulted in the current impasse.\nFurther, she had a growing sense of dissatisfaction with the trajectory of her life. She felt capable of achieving much more, but had not actively pursued her ambitions. As her life became increasingly centered around her home and husband, opportunities for developing peer relationships also considerably reduced. This situation greatly eroded her self-esteem and left her with a sense of frustration. Against this backdrop, being let down by her husband deeply hurt and disappointed her and she felt that life had become meaningless.\nMarital therapy was planned in the light of the fact that the current issue was clearly embedded in the ongoing relationship difficulties. The couple was seen for eight sessions over a period of two months. Initial work focussed on helping the wife ventilate her anger and distress. The therapist ‘psycho-educated’ the husband about online infidelity,[] regarding rationalization of his action. The therapist, using a non-confrontational stance pointed out the paradoxical manner in which he had maintained secrecy about his behavior, and at the same time regarded it as ‘not cheating’. The husband was able to acknowledge this and also reflected on the impact of cybersex on their marriage. He subsequently expressed regret over his behavior and apologized to his wife. His justification for his behavior as a way of relaxing or unwinding was also pointed out as paradoxical, as he reported experiencing physical exhaustion from spending excessive time on the computer at work, but also ended up utilizing his leisure time only on the computer. He was encouraged to exercise self-control in using the Internet during leisure time with regard to his choice of sites (for blogging, updating on information, etc.).\nKeeping in mind the practical limitation of her husband's long working hours, the next stage of therapy focused on enhancing intimacy between the couple by encouraging them to identify ways by which they could spend more time together. They were able to think of joint activities that both enjoyed and taking brief vacations during weekends, as methods to enhance emotional bonding.\nSubsequent to enquiry about their sexual relationship, the couple had approached their gynecologist who suggested a newer contraceptive pill, which did not produce side effects and they no longer had to engage in coitus interruptus. Wife's uneasiness about her conservative attitude towards sex was discussed and misconceptions that she held were clarified. She felt more comfortable engaging in certain sexual practices that she was unsure of earlier, resulting in greater sexual satisfaction for the couple.\nAfter the conjoint sessions had been terminated, the wife was seen individually for four sessions to help her constructively pursue the goal of completing her post graduation. This helped to enhance her self-esteem and also ensured that she spent less time brooding about the past.\nAt termination, the wife's depressive symptoms had reduced. She was euthymic and reported adequate sleep and appetite. The couple reported that therapy had a positive impact on their relationship. The husband had accepted responsibility for his actions and was willing to work on rebuilding trust in the relationship. A follow-up two months later revealed that therapeutic gains were maintained. The wife had stopped anti-depressants as her symptoms reduced and also because the couple had planned for a second child. They engaged in several joint activities and reported greater intimacy and improvement in their sexual relationship. The husband had reduced time spent on the Internet while at home, and the wife had started actively pursuing her academic goals and was regularly setting time aside for her studies and looking forward to completing her post graduation. |
A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event. |
A 74-year-old woman (height, 156 cm; weight, 63 kg), with American Society of Anesthesiologists physical status grade II, was diagnosed with malignant fibrous histiocytoma in the left buttock, for which she underwent surgery and left gluteal flap closure. She had a medical history of hypertension and chronic renal failure. The surgery was performed under general anesthesia for a prolonged time (24 h and 55 min), and the patient was intubated with a 7.0-mm-internaldiameter Mallinckrodt™ (Covidien, Dublin, Ireland) cuffed reinforced ETT. The patient was transferred to the intensive care unit (ICU) and remained intubated because of postoperative upper airway edema. An oral airway was inserted as a bite block to prevent collapse of the tube. She was kept in a prone position with the reinforced ETT because the gluteal flap turned blue in the lateral decubitus position. During the ICU stay, she was sedated with dexmedetomidine and remifentanil. The infusion rate of the drugs was controlled on the basis of the level of sedation, with a goal of achieving Richmond Agitation Sedation Scale (RASS) level 2. Although she was weaned from the mechanical ventilator in a pressure support ventilation mode, she could not be extubated because she tested positive on the cuff-leak test and underwent two additional emergency surgeries—one on the same day and another 48 h later. On postoperative day 3, she still tested positive on the cuff-leak test one and a half hours before the event. Also, the charge nurse had difficulties passing a suction catheter through the lumen of the ETT because of the narrow lumen, but the tidal volume and peak inspiratory pressure were not changed. Thirty minutes before the event, the tidal volume and peak inspiratory pressure were still unchanged. Peak airway pressure suddenly increased with a decrease in the tidal volume, and oxygen saturation was reduced to 85% despite the delivery of 100% oxygen. We attempted to pass a suction catheter through the lumen of the ETT, but the catheter was obstructed at the level of the incisor teeth. After placing the patient in a supine position, we found that the ETT had been completely severed and the oral airway had been spat out. Upon inspection of the oral cavity, we discovered that the distal portion of the ETT was missing. Therefore, we performed fiberoptic bronchoscopy after normalizing the oxygen saturation to 98-100% (flow rate, 10 L/min) by using a facial mask. Although the severed distal portion of the ETT was observed beneath the vocal cord, it could not be removed. Chest anteroposterior radiography showed that the severed portion was lodged in the right main bronchus (). Her self-respiration was maintained without upper airway irritation during the time required for exams and the surgical procedure. The patient was taken to the operating room for emergency bronchoscopy. The ETT was surgically removed by an otolaryngologist under general anesthesia. In the operating room, she was preoxygenated with 100% oxygen, induced with intravenous propofol 1.5 mg/kg without the use of a muscle relaxant and anesthesia was maintained with manual ventilation with 100% oxygen and sevoflurane. After achieving adequate depth, a micro-laryngoscope was introduced and the severed ETT was removed with a forceps successfully (). Micro-laryngoscopy revealed a mild subglottic swelling right after removal of the ETT. As the patient recovered spontaneous breathing, she was transferred to the ICU. Additional intubation was not needed and 5 mg dexamethasone was intravenously injected twice a day for 2 days for relieving upper airway edema. The remainder of the patient’s ICU stay was uneventful, and she was transferred to a ward without any difficulty with respiration. Further laryngeal evaluation showed no abnormal findings. However, the gluteal flap did not remain viable and underwent necrosis; hence, the patient had to undergo another surgery for flap removal. She was then transferred to the regional hospital for palliative care. |
We report the case of a 29-year-old male who presented to the emergency department 12 h following blunt injury of the penis during a trivial two-wheeler accident. The two-wheeler which he was driving had a large gap between the petrol tank and the seat. The patient was wearing a “dhoti”, traditional wear in South India which makes the penis more susceptible to injury. While travelling, he had to apply a sudden brake which resulted in a sudden push of his body against the petrol tank and in the course of this event, his erected penis was caught in the gap between the seat and the tank which resulted in this injury. He immediately experienced a sensation of “tearing”, rapid detumescence, severe penile pain and mild swelling at the root of the penis but was able to void well. The patient approached us 12 h after the injury and on examination a tender and swollen penis at the root was seen []. Urethral meatus was normal. No retrograde urethrogram (RGU) was performed as patient was voiding well and urine analysis was normal. The patient was taken to the operating room for urgent exploration. However, a preoperative Doppler ultrasound was done which revealed clots confined to the crus of the left cavernosa; based on this we applied a penoscrotal incision. On exploration, clots were found deep down at the crura of the penis which were cleared []. No local debridement or any vessel ligation was required. After clot evacuation, a deeply located 1-cm transverse rupture of the crus of both corpora cavernosa was discovered []. The defects were repaired in two layers, the inner layer sutured with 3-0 vicryl in a simple running fashion and the outer with 4-0 prolene interrupted sutures [Figure , ]. The urethra was normal, but considering the bilaterality and extent of the injury, an intraoperative RGU was done which was normal. An 18-French Foley catheter was placed into bladder which was removed the next day and discharged. He was advised to abstain from intercourse for at least four weeks. He was called for follow-up after two weeks during which his wounds were completed healed [] and he was started on oral phosphodiesterase Type 5 Inhibitors- Sildenafil 25 mg once a day for one month. During his follow-up after three months, he expressed satisfactory painless erections and his international index of erectile function (IIEF)–ED domain score was 23 and rigidity grade was 3 which were clinically acceptable. A follow-up Doppler ultrasound was done, which showed no leakage during the tumescence phase. |
A 40-year-old female patient with a history of hypothyroidism was presented to the hospital with 5-day long flu-like symptoms (malaise, rhinorrhea and chills) along with cough and fever. By the time of her admission, she presented with dyspnea and an oxygen saturation level of 85%. On examination, the temperature was 37°C, respiratory rate 24 cycles/min, pulse rate 98 bpm, blood pressure 105/60 mm Hg. Rales were present at both pulmonary bases and mild abdominal pain was found on palpation over the upper right quadrant without peritoneal irritation signs. A chest x-ray was performed showing a radiopaque image at the left side of the cardiac silhouette from the second and seventh costal arc, deviation of the airway to the right side, and absence of pleural effusion (). A CT scan revealed a heterogeneous extrapulmonary multiloculated and predominantly fluid density lesion. It was located at the anterior mediastinum with left lateral extension. On intravenous contrast administration, wall enhancement of the lesion was observed, without pericardium involvement ().\nBefore surgery, the cystic tumor was punctured in a separate session with ultrasound-guided technique by a pulmonologist to generate space in the thoracic cavity, obtaining a thick yellowish fluid (). After that, surgical treatment was indicated with a VATS approach. The camera was inserted through a 1 cm incision in the seventh intercostal space (ICS) on the posterior axillary line, a small working incision was made in the fourth ICS between anterior and midaxillary lines for thoracoscopic instruments. Upon entering the left thorax, a 15 cm diameter cystic tumor was observed, the tumor was firmly adherent to the pericardium, contralateral pleura and great vessels. A small part of the lung was resected en bloc together with the cystic tumor, which was removed successfully without intraoperative complications. A 24-French silicon closed drain was left in place.\nAfter surgery, the patient developed right lobar atelectasis (), which was treated with respiratory physiotherapy and rehabilitation. She was discharged 8 days after surgery and the drain was removed during the follow-up examination on the 13th postoperative day. Histologic examination showed a mature cystic teratoma without malignant characteristics (). No recurrence was found during a 24-month follow-up. |
The patient was a 64-year-old female with a past medical history of hypertension, diabetes mellitus type 2, and hyperlipidemia referred to urology clinic for evaluation of a 5 cm left-sided renal mass that was incidentally discovered during evaluation for diarrhea and flank pain. She denied any history of gross hematuria or other urinary symptoms. She had no significant past medical history. The patient had never smoked and denied a family history of renal malignancy. On physical examination, she was found to be in no acute distress, and appeared generally well (ECOG performance status 0). There were no carotid bruits or neck masses palpated. Her chest was clear to auscultation bilaterally and her abdomen was soft, nontender, and nondistended without palpable abdominal masses or costo-vertebral angle tenderness. Laboratory evaluation including comprehensive metabolic panel, complete blood count, and urinalysis were within normal limits.\nComputed tomography of the abdomen and pelvis with contrast demonstrated a 4.9 cm by 5.0 cm by 5.8 cm endophytic interpolar left renal mass (Figures and ). Chest CT with contrast demonstrated a 12 mm right lower lobe pulmonary nodule, which was noted to have been stable on prior imaging up to eleven years previously and was interpreted as a granuloma. Finally, she was found to have a slightly enlarged thyroid gland with several small calcified nodules ().\nThe patient was counseled regarding options for management and underwent hand-assisted laparoscopic radical nephrectomy. The surgical procedure was uncomplicated with an estimated blood loss of 25 cc. She tolerated surgery well, and, following an uncomplicated recovery, was discharged from the hospital on postoperative day three.\nHistopathology revealed a 6.5 cm mass consistent with metastatic follicular variant of papillary thyroid cancer (). The diagnosis was confirmed with immunohistochemistry as the tumor cells were CK7 (+), TTF1 (+), and Thyroglobulin (+) but CK20 (−) and WT1 (−). The soft tissue and vascular surgical margins were negative for tumor. Given the results of the pathologic analysis and evidence of several calcified nodules within the enlarged thyroid, she was referred to endocrine surgery for further evaluation. On thyroid ultrasound, a left-sided thyroid nodule was appreciated. Her endocrine evaluation included thyroid function panel, which demonstrated an elevated thyroid-stimulating hormone (TSH) of 7.57 uIU/mL and normal T4 (0.81 ng/dL). She underwent fine-needle-aspiration of the thyroid, which showed atypia. Subsequent nuclear medicine whole body thyroid scan demonstrated no additional sites of I-131 avidity. She underwent a total thyroidectomy and radioactive iodine 3 months after. Pathology was consistent with a follicular variant of papillary thyroid carcinoma. |
A 66-year-old female with no significant past medical history was brought to the emergency department by emergency medical services (EMS) with a complaint of left-sided chest pain. She was in her usual state of health when she went to a scheduled appointment with a vascular surgeon for outpatient elective sclerotherapy for the cosmetic treatment of varicose veins. Shortly after being administered an injection of polidocanol, she began experiencing severe left-sided chest pain radiating to her left back with left arm numbness. She also complained of nausea, one episode of vomiting, pressure in her jaw, and lightheadedness. Prior to being transported to the emergency department by ambulance, she was given two doses of aspirin 81 mg.\nOn arrival to the emergency department, she reported that her symptoms had subsided. Her initial electrocardiogram (ECG) was remarkable for T-wave inversions in leads V1-V3 and sinus bradycardia at 54 beats per minute (), with no prior ECG available for comparison. No ST-segment abnormalities were present. Initial laboratory studies were also notable for an elevated troponin I level of 0.62 ng/mL.\nThe patient was admitted to the inpatient telemetry unit for suspected acute coronary syndrome. Repeat assessment of her cardiac enzymes six hours after her initial bloodwork revealed that her troponin I level had risen to 2.26 ng/mL, at which point the patient had been started on continuous unfractionated intravenous heparin. She remained asymptomatic. The patient was offered cardiac catheterization but initially declined. In a third set of cardiac enzymes, her troponin I level had trended down to 1.15 ng/mL.\nFurther studies, including echocardiogram and nuclear stress testing, were pursued to better define her cardiovascular function. A transthoracic echocardiogram revealed an ejection fraction of 50%, suggestive of mild systolic dysfunction, in addition to the presence of apical hypokinesis (). Grade II diastolic dysfunction was also noted. Her stress test confirmed an akinetic cardiac apex, as well as a small, severe-intensity, fixed defect, possibly suggestive of myocardial infarction. No areas of reversible ischemia were identified.\nAfter completing her stress test, the patient stated that her left-sided chest pain had returned and was accompanied by shortness of breath. A repeat ECG again showed deeper T-wave inversions in leads V1-V3, in addition to new T-wave inversions in leads I, II, aVL, and V4-V6 (). A CT angiogram of her chest with IV contrast was able to rule out an underlying pulmonary embolism and aortic dissection as potential sources of her chest pain.\nAfter further discussion with the patient and her family, she eventually agreed to undergo cardiac catheterization. The procedure established that all vessels were patent, effectively ruling out an acute ischemic event and atherosclerotic disease as the source of her symptoms. In the setting of left ventricular systolic dysfunction, the absence of coronary artery disease, and elevated cardiac enzymes with T-wave inversions on ECG, the patient was diagnosed with Takotsubo syndrome. Her postprocedure course was uncomplicated, and the patient was ultimately discharged home on aspirin 81 mg and simvastatin 20 mg for preventative purposes, with instructions to follow up for a repeat echocardiogram as an outpatient. |
In May 2001 a 54-year-old man presented to observation with diagnosis of 6.1 mm thick, Clark's level IV, ulcerated cutaneous melanoma of the trunk. Preoperative staging with chest X-ray and abdominal ultrasound (US) did not reveal signs of systemic disease. He underwent wide local excision (WEX) and sentinel lymph node biopsy (SLNB). Preoperative lymphoscintigraphy showed uptake of the radiotracer in one node of the left axilla that was removed and resulted on finally pathology negative for metastatic melanoma. The patient was submitted to regular follow-up every three months with physical examination, chest X-ray, US and blood work, according to the Multicenter Selective Lymphadenectomy Trial []. In January 2002 hepatobiliary US detected the presence of intracholecystic nodule of 1.0 cm. Three months later the patient presented upper abdominal pain mimicking symptomatic cholecystolithiasis. Blood analysis values were normal. US examination showed an increasing in size to 2.0 cm of the intracholecystic nodular image (Figure ). Computerised tomography (CT) confirmed the presence of a nodule in the lumen of the gallbladder measuring about 2.0 cm and a positron emission tomography (PET) revelead uptake in the gallbladder area without evidence of systemic uptake of the radiotracer. In May 2002 the patient underwent laparoscopic cholecystectomy. Intrabdominal exploration showed a normal gallbladder but with an enlarged blue coloured lymph node along the cystic duct, and the presence of a dark spot image of the diaphragmatic peritoneum. The gallbladder was removed together with the lymph node along the cystic duct and put into an endobag to avoid any contamination of the abdominal cavity and extracted. An excisional biopsy of the diaphragmatic peritoneal lesion concluded the surgical procedure. Macroscopic evaluation of the gallbladder showed one vegetant brown coloured polyp adherent to the mucosa, projected into the lumen, measuring about 3,5 cm in size, and a brown lymph node 1.5 cm in diameter. At histological examination, the mucosal (Figure ) and muscular layers of the gallbladder were diffusely infiltrated by epithelioid and spindle heavely pigmentated cells; the epithelium was partially eroded and no junctional activity was evident; the cystic duct lymph node and the peritoneal node were both involved by metastatic cells from melanoma. Immunohistochemical staining of the removed tissues showed a strong positivity for S 100 protein, HMB 45 and MART-1 (Figure , , ). Postoperative course was uneventful and the patient was discharged home on postoperative day two. No adjuvant chemotherapy was offered at this time by our oncologists. Two months later the patient presented clinical evidence of intestinal obstruction. CT revealed intussusception of two segments of the small bowel tract. At laparotomy resection of two portions of the involved small bowel by two stenosing metastases with contiguous involvement of the mesentery and of mesenteric lymph nodes was performed. Pathology confirmed the abdominal recurrence by metastatic melanoma. After surgery he recieved chemoimmunotherapy (fotemustine 100 mg/m2 and dacarbazine 900 mg/m2 intravenously every three weeks for a total of three cycles plus interferon alfa-2b 5 MU/m2 subcutaneously).\nSeventeen months after SLNB, PET showed disseminated disease. The patient died shortly thereafter in April 2003, eleven months after cholecystectomy. |
A 3-year-old girl was referred to a tertiary metabolic bone disease unit for premature loss of primary teeth with roots intact and low serum ALP activity (123 IU/L; reference range: 230–700 IU/L) []. Routine genetic testing revealed compound heterozygosity (c.350A > G, p.Y117C, c.400_401AC > CA, p.T134H) for different TNSALP missense mutations in exon 5 of the ALPL gene, confirming the diagnosis of HPP. On presentation, radiologic assessment of the left hand and arm showed tongue-like lucencies projecting into the metaphyses consistent with childhood HPP. She did not have any clinical features of skeletal involvement of the lower limbs and no motor developmental delay except for a mild waddling gait as a younger child. The patient had a relatively asymptomatic clinical course until she presented at age 11 years with swelling and tenderness of the left ankle that was nonresponsive to paracetamol or ibuprofen. An MRI scan of the ankle suggested a diagnosis of chronic recurrent multifocal osteomyelitis, which was subsequently confirmed by biopsy. The symptoms of pain and swelling of the lower limb joints showed spontaneous transient improvement at age 13 years. Recurring at age 14 years, the symptoms fluctuated and caused significant pain and disability. These symptoms eventually stabilized when the patient was transitioned to adult care at age 17 years. At age 18 years, she successfully underwent radiofrequency ablation for Wolff-Parkinson-White Syndrome, a cardiac disorder unrelated to HPP. The patient is now 27 years of age and has experienced an episode of metatarsal stress fracture; she also suffers from generalized aches and pain.\nOver 22 years, this patient was hospitalized 3 times for a total of 19 days (Table ). Only 1 hospitalization exceeded 3 days, when the patient was admitted for 14 days to receive intravenous antibiotics for suspected osteomyelitis (Table ).\nOutpatient specialist visits, outpatient procedures, and day case procedures represent the majority of healthcare resources used by this patient (Fig. ). Seven specialists provided care for the patient; a pediatric dentist was seen on 40 occasions. Dental procedures, including restorative dentistry (performed on 3 occasions), were the most common of these. This patient was also seen by a pediatric rheumatologist and psychiatrist (Table ). |
A 22-year-old Caucasian lady presented in 2014 with features of pulmonary fibrosis, chronic cutaneous problems, and symmetrical polyarthritis. She had previously been well without any comorbidities. A high-resolution computed tomography (CT) chest and lung biopsy were performed, with a diagnosis of hypersensitivity pneumonitis being made, in the context of significant mould exposure. Skin biopsies demonstrated spongiotic dermatitis (eczema). Echocardiography revealed right ventricular dilatation with mild tricuspid regurgitation. Left ventricular size and function was normal.\nShe was discharged and attended as an outpatient to a specialist centre for pulmonary hypertension, as it was assumed at the time that the right ventricular dilatation was a consequence of undiagnosed pulmonary hypertension. Here, right heart catheterization indicated normal pulmonary arterial pressure. Subsequently, no cardiology follow-up was arranged. The patient was treated with methotrexate, sulfasalazine, and intermittent prednisone over the next 4 years, although a single unifying diagnosis to explain her all of her symptoms was not identified.\nIn 2018, the patient attended a rheumatology clinic. The rheumatologist was concerned that there were clinical signs of pulmonary hypertension detected on physical examination, and thus a routine echocardiogram was performed. This study demonstrated severe right ventricular dilatation (). There was now severe tricuspid valve regurgitation (). The left ventricular size was normal, as was systolic and diastolic left ventricular function. There was no significant left-sided valvular disease. The patient was experiencing dyspnoea on walking 50 m on a flat surface, or at the top of a single flight of stairs. Additionally, she described four episodes of unheralded syncope, all occurring whilst seated. On physical examination, the blood pressure was 130/70 mmHg, the heart rate 88 b.p.m., and regular, with oxygen saturation of 99% on room air. The jugular venous pressure was elevated with prominent V waves. There was a right ventricular heave and a loud pan-systolic murmur audible at the left sternal edge. Pulsatile hepatomegaly was present. Fine crackles were present in both lung bases. Electrocardiogram demonstrated atrial fibrillation with a right bundle branch block ().\nDue to the concerning features on echocardiography and the symptoms of dyspnoea and syncope, the patient was admitted to a monitored cardiac ward for further investigation. Telemetry demonstrated non-sustained ventricular tachycardia (VT) at a rate of 150 b.p.m. (), of which the patient was symptomatic with palpitations but there was no haemodynamic compromise.\nRight heart catheterization revealed normal pulmonary arterial pressures (22/10 mmHg), but elevated right atrial pressures (16/14 mmHg). Cardiac output (5.4 L/min) and pulmonary vascular resistance (88 dynes.s/cm5) were normal.\nThe patient underwent transoesophageal echocardiography (TOE) and successful electrical cardioversion, restoring sinus rhythm. The TOE excluded atrial thrombus, any intracardiac left-to-right shunt, and all four pulmonary veins communicated with the left atrium.\nCardiac magnetic resonance imaging (MRI) did not show any late gadolinium enhancement in either ventricle, and congenital anatomical anomalies were excluded. Cardiac positron emission tomography ( PET) did not reveal increased metabolic activity to indicate inflammation or features of active cardiac sarcoidosis. A CT chest demonstrated unchanged features of pulmonary fibrosis.\nMyocardial biopsy was performed under fluoroscopic guidance. Histopathology revealed a predominantly lymphocytic chronic inflammatory infiltrate (). A single well-formed small granuloma as well as a second focus of scattered giant cells was noted. Moderate to severe myopathic features were seen, including myocyte necrosis with features of myocytolysis and anisonucleosis, and patchy regenerative interstitial fibrosis. The diagnosis was consistent with lymphocytic myocarditis. The morphological pattern that comprised myocyte necrosis and a widespread lymphocytic infiltrate without predominating giant cells and granulomas was less likely to be consistent with cardiac sarcoidosis or giant cell myocarditis. There was a paucity of eosinophils, making the diagnosis of hypersensitivity myocarditis highly unlikely. The classic features of arrhythmogenic cardiomyopathy (AC) were also not seen, i.e. patchy to diffuse irregular replacement of myocytes by adipose tissue, with associated variable amounts of fibrosis, lack of significant lymphocytic infiltrate, and scattered enlarged degenerate vacuolated myocytes. A broad range of serological investigations had also been conducted, with no significant positive results.\nGiven the documented symptomatic VT, and the history of syncope, the patient was referred for a cardiac electrophysiology study, which demonstrated inducible VT originating from the RV apex that lasted for over 30 s. Following a multidisciplinary discussion, the patient consented to the implantation of an automated implantable cardioverter-defibrillator (AICD).\nThe patient was commenced on prednisone 25 mg daily, as well as sotalol 80 mg twice daily and apixaban 5 mg twice daily, prior to discharge. Since discharge, her prednisone has been ceased after being systematically weaned following commencement of azathioprine 100 mg daily. She has been stable thereafter, with no further arrhythmias and has returned to work. Occasional episodes of decompensated mild right heart failure have been treated successfully with oral diuretics, without the need for intravenous therapy or inpatient admission. An echocardiogram performed 12 months after discharge revealed a marginal improvement in right ventricular function, with residual severe tricuspid regurgitation. As the overall clinical condition of the patient has remained stable, repeat myocardial biopsy was not performed.\nThe process is considered chronic based on the first detection of right ventricular abnormalities 4 years prior. In addition, the chronic inflammatory infiltrate and interstitial fibrosis supports this conclusion.\nThus, we describe a case of an immunosuppressed young lady who was suffering from a chronic low-grade lymphocytic myocarditis of the RV resulting in dilatation of the chamber, severe functional tricuspid regurgitation, and arrhythmic complications including atrial fibrillation and VT. All cardiac imaging modalities demonstrated normal left ventricular size and function, suggesting this process demonstrated a preponderance to the RV. |
A healthy, 63 kg, 30-year-old primigravida requested for labor analgesia. As per the institutional protocol, a combined spinal epidural technique (CSEA) was administered. Lumbar puncture was done in L3–L4 interspace and 25 mcg of fentanyl was injected intrathecally. The epidural space was identified with loss of resistance to saline, at a depth of 5 cm to the skin, and a catheter was fixed with the 10-cm mark at the entry point. The analgesic effect of intrathecal fentanyl lasted for 90 min. Following this, 10 mL of 0.125% bupivacaine was given. The patient had satisfactory pain relief and received two epidural top-up doses of 6 mL 0.125% bupivacaine each, at 60 min intervals. About 45 min after the administration of the last dose, it was decided that a cesarean section be performed as the labor had not progressed. In the operating room, the block was extended with a mixture of 15 mL 2% lignocaine with 50 mcg fentanyl titrated over 10 min to achieve a T6 level block. The cesarean section was uneventful and a healthy male baby was delivered. The patient was hemodynamically stable throughout the procedure.\nAt the end of surgery, the patient complained of weakness in the right upper limb. There was no respiratory distress or desaturation. Neurological examination showed right upper limb power of 2/5 (biceps flexion 2/5, shoulder abduction 2/5), full power of 5/5 of the left upper limb, and bilateral lower limb power of 4/5. The sensory examination revealed loss of pin prick sensation at dermatomes C6, C7, C8 and T1 of the right arm. There was no involvement of cranial nerves. A diagnosis of high unilateral block was made and the patient was reassured. The epidural catheter was removed and the patient was kept under observation in the recovery room. The patient regained complete power in the right upper limb 90 min later. Subsequent hospital stay of the patient was uneventful. |
A 40-year-old right-hand-dominant man was offloading beer crates while perched on a box. As he turned round, he lost his balance and fell with his left hand outstretched and elbow extended. He presented with a swollen and deformed elbow joint. It was held in 60° of flexion, and with the forearm in pronation. Distal circulation and motor function were intact, but he complained of pins and needles in the ulnar nerve distribution. His radiographs showed a true lateral displacement of the left proximal radius and ulna in relation to the humerus (Figure ). The olecranon was in contact with the lateral condyle, and in line with the transverse axis of the distal end of the humerus. However, the anatomical relationship of the radius and ulna was maintained and no fractures could be visualised.\nA closed reduction was attempted under light sedation by the casualty registrar using conventional methods, but proved unsuccessful. The patient was re-examined by the orthopaedic team and was placed prone with the affected left arm hanging by the edge of the bed. Ten minutes later, with the patient still sedated, the elbow was first disimpacted by applying longitudinal traction on the forearm with counter-traction on the arm. It was then enlocated applying gentle medial pressure on the olecranon. Valgus and varus instability of the elbow was checked in full extension and 30° of flexion. Normal sensation returned spontaneously in the ulnar nerve distribution.\nThe elbow was then immobilised in plaster in 90° of flexion. Radiographs confirmed a satisfactory reduction with normal joint congruity (Figures and ). The patient was offered a follow-up appointment a week later, but could attend only 3 weeks later on account of personal commitments. His plaster was removed and his elbow stressed to check for medial or lateral ligamentous instability. Nothing untoward was found, but he was started on an intensive physiotherapy regime. He subsequently regained the normal range of elbow movements and was discharged from care after his second follow-up visit at 2 months. |
A previously well 87-year-old Caucasian woman living in a senior assisted care center presented to the neurology clinic with complaints of six months of slowly progressing left sided weakness. Initial difficulty in ambulating and using the stairs progressed to being wheelchair bound. Neurologic exam revealed diffuse 3/5 left sided weakness, left leg drift, and left facial droop. Brain magnetic resonance imaging (MRI) revealed a large confluent white matter T2-hyperintensity in the right frontal lobe with multifocal nodular enhancement of the left cerebral hemisphere (). Foci of enhancement were also identified in the cerebellum and leptomeninges. The radiologic differential diagnosis included vasculitis, lymphoma, and CNS sarcoidosis as the most probable causes of the multifocal disease process, with glial neoplasm, demyelination, and metastases considered less likely.\nAll sample analysis described below were performed on material obtained by brain biopsy as part of clinical care. All samples were obtained with appropriate consent.\nA biopsy of the mass was performed and revealed extensive parenchymal lakes and vascular and perivascular deposition of amorphous, amyloid like material (). Congo-red positive staining and apple-green birefringence (not shown) of the amorphous material upon polarization confirmed that the amorphous material was amyloid (). Also present in the resected tissue were a number of small intraparenchymal blood vessels with perivascular lymphoplasmacytic infiltrates (). The initial histologic differential diagnoses included cerebral amyloid angiopathy-inflammatory type (CAA-I) and lymphoma associated amyloidoma. To identify the underlying etiology of the amyloid accumulation, a number of additional analyses were performed.\nLiquid chromatography tandem mass spectroscopic analysis identified the amyloid as AL λ-type and not β amyloid or an amyloid associated with a hereditary amyloidosis. Further analysis of the perivascular lymphoid populations was undertaken. Histologically, the monotonous populations of perivascular lymphoid cells demonstrated a lymphoplasmacytic appearance (). Immunohistochemical analysis demonstrated that the lymphoid cells were CD20 positive (). Tumor cells were negative for CD3, CD5, BCL1, and CD23. The tumor Ki67 proliferation index was low (3%). The more plasmacytoid appearing cells were CD138 positive and were shown to be lambda light chain restricted by kappa and lambda chromogenic in situ analysis (Figures and ). An immunoglobulin heavy chain (IgH) gene rearrangement analysis of the brain tissue from this case was positive for a clonal process with a 253-base pair peak in the FR2 region. A MYD88 L265P mutation analysis by PCR-based pyrosequencing on the brain tissue from this case was negative. A diagnosis of a low grade, lymphoplasmacytic lymphoma (LPL) was rendered. The identification of this CNS low grade lymphoplasmacytic lymphoma confirmed the cause of the amyloidoma to be a lambda light chain producing lymphoplasmacytic lymphoma.\nTo determine if an extracranial/systemic lymphoplasmacytic lymphoma was the source of the CNS neoplasm, a bone marrow biopsy was performed. The bone marrow biopsy showed normal trilineage hematopoiesis and no evidence of lymphoma, myeloma, or amyloidosis. Cytogenetics and fluorescent in situ hybridization studies on the bone marrow were negative for genetic aberrations. Urine protein and serum immunoglobulin levels were within normal limits. A biopsy of subcutaneous abdominal adipose tissue was negative for amyloid, demonstrating lack of evidence of systemic amyloid deposition. Interestingly, an IgH gene rearrangement analysis on the bone marrow was positive for a clonal gene rearrangement with two peaks: a 282-base pair peak in FR2 region and a 120-base pair peak in FR3 region in a polyclonal background, which importantly were markedly different from the IgH gene rearrangement identified in the CNS lymphoplasmacytic lymphoma. Since the two-small bone marrow clonal peaks are present in a polyclonal background, their significance is uncertain and may be age related.\nOur patient received one cycle of chemotherapy with Rituximab for Primary CNS lymphoplasmacytic lymphoma. Two months after diagnosis, she developed a hemorrhagic infarct on the left frontal white matter and was transferred to hospice care. |
On October 12, 2011, a 57-year-old Chinese female was admitted to our institution with complaint of upper abdominal distension for 2 weeks. She denied any abdominal trauma or surgeries in her life. No specific findings were noted in her family history and personal history. And, she was taking no medications. Before this treatment, she went to the local hospital where an abdominal ultrasound was performed revealing a mass in the head of the pancreas. No abnormalities were detected upon clinical examination and in laboratory investigations. The computer tomography (CT) scan (Fig. ) revealed a 4.0 × 3.2 × 3.0-cm hypodense mass in the porta hepatis, mildly enhanced in arterial phase, and moderately enhanced in portal phase. There was no clear boundary between the mass and the caudate lobe of the liver, and the lower edge of the lesion was close to the neck of pancreas, pressing down on the common hepatic artery. No enlarged hilar lymph nodes or exogenous caudate lobe tumor were identified. The magnetic resonance imaging (MRI) (Fig. ) showed the long T1 and T2 signal mass in the porta hepatis, with 4.0 × 3.0 cm. The imaging feature on MRI was similar to CT. Fine needle aspiration guided by endoscopic ultrasonography revealed spindle cells arranged in bundles and palisades, with no nuclear atypia, suggestive of a spindle cell neoplasia. With a high suspicion of a GIST, diagnostic laparoscopy was performed. The tumor was located in the porta hepatis, tightly pressing the hepatoduodenal ligament, and the caudate lobe and the pancreas were not involved. Then, after careful dissection, the tumor was found to be in close contact with the common hepatic artery and cystic artery. Subsequently, the patient underwent laparotomy, and intraoperative frozen-section examination revealed a mesenchymal tumor in the porta hepatis, highly suspicious of a GIST. So, the tumor was macroscopically resected en-bloc with the invaded common hepatic artery, and the blood supply of the proper hepatic artery was good through the gastroduodenal artery. And, cholecystectomy was also performed. Post-operative detailed histopathological examination showed benign schwannoma (Fig. ): CD117(−), S-100(+), GFAP(+), CD34(−), SMA(−), and Ki-67(+, little). Postoperatively, the patient recovered uneventfully. No symptoms or signs of recurrence have been observed in our patient during the 41 months of follow-up.\nPubMed, Google scholar (), the National Knowledge Infrastructure (/), the Chinese periodical Database of Science and Technology (), and the Wanfang Data Knowledge Service Platform () were searched for cases of porta hepatic schwannoma between 1985 and 2015, adding up to 14 patients. Details of all the 14 cases and the current case shown in Tables and summarized the important available clinicopathological factors. Continuous data are presented as mean ± standard deviation and range.\nSchwannoma, also known as neurilemmoma, is a tumor derived from Schwann cells, which form the inner portion of the peripheral nerve sheath []. The exact etiology and pathogenesis of schwannomas remain unclear, but recent research has indicated that defects in merlin gene are responsible for both sporadic and genetically acquired schwannomas, and the mechanisms by which merlin loss triggers tumor development are being unraveled []. Theoretically, the tumor can affect any nerve trunk or any organ, except the olfactory and optic nerves, which lack Schwann cells []. The most common locations for schwannomas are the upper extremities, trunk, head, and neck, retroperitoneum, mediastinum, pelvis, and peritoneum []. Intraperitoneal schwannomas are relatively rare and mostly located in solid organs such as the liver and the pancreas. However, porta hepatic schwannomas are extremely rare, with only 14 cases reported in the literature to date. The sympathetic and parasympathetic fibers are distributed along the hepatic and gastroduodenal arteries, with their branches interwoven into a network, which is the anatomical location of occurrence of the porta hepatic schwannoma [].\nSchwannomas most commonly occur in patients between the ages of 20 and 50 years with equal frequency in men and women []. For cases of porta hepatic schwannoma, the mean age of the patients was 48 years (range 29–74 years), and the male-female ratio was 6:9. Symptoms caused by schwannomas vary with the site and size of the tumors. In the 15 cases, the lesion was located in the porta hepatis in nine patients (60 %), hepatoduodenal ligament in five patients (33 %), and proper hepatic artery in one patient (7 %). The literature depicts tumors varying from 2.2 cm (the average tumor diameter line) to 7.5 cm in size, with a median of approximately 4.7 cm. Patients with porta hepatic schwannomas may exhibit symptoms by compressing adjacent structures such as the bile duct and the gastrointestinal tract. In the literature, 40 % of the patients were asymptomatic and 60 % of the patients were symptomatic. The most common symptom was abdominal distension/abdominal discomfort (5/15 patients or 33 %), followed by abdominal pain (4/15 patients or 27 %), jaundice/dark urine/colorless stool/pruritus (2/15 patients or 13 %), nausea/vomiting/anorexia (1/15 patients or 7 %), and weight loss (1/15 patients or 7 %).\nPatients with porta hepatic schwannomas usually present with a porta hepatis or hepatoduodenal ligament mass on imaging studies, and it is very challenging for surgeons to make a preoperative diagnosis due to its rarity and nonspecific clinical and imaging characteristics. The ultrasound imaging often shows isoechoic or hypoechoic solid masses with well-defined limits. Generally, a CT scan shows a well-defined hypodense heterogenous mass with peripheral enhancement []. Schwannomas on the MRI are usually masses with hypointensity on T1-weighted images and heterogenous hyperintensity or, sometimes internal heterogeneous mixed hypo and hyperintensity on T2-weighted images []. Author Cohen has presented histologic evidence suggesting that the hypo-density areas and inhomogeneities in schwannomas are due to hypocellular areas adjacent to more cellular regions and/or hypocellular areas adjacent to dense bundles of collagen, xanthomatous formation, and cystic degeneration within the tumors []. In the present case, the CT and MRI showed a homogeneous mass with peripheral enhancement in the porta hepatis. When a solitary, well-demarcated mass occurs in the porta hepatis, differential diagnosis should be made including the following diseases: lymphoma, giant lymph node hyperplasia (GLNH), lymph node tuberculosis, and lesser sac benign smooth muscle tumors such as leiomyomas, nerve sheath tumors, and hemangiomas originating in the lesser sac as well as stromal tumors of the gastrointestinal tract protruding into the lesser sac []. Considering that imaging manifestations of porta hepatic schwannomas are nonspecific, patients are usually misdiagnosed preoperatively, unless by biopsy. In the 15 cases, the FNA was operated only for three patients, including the present case. However, the result of FNA may be inconsistent with final pathologic results []. Therefore, postoperative pathologic examination is still the gold standard in the diagnosis of schwannoma.\nOverall, it is essential for the diagnosis of schwannoma to combine histological examination with immunohistochemistry. Macroscopically, small schwannomas tend to be spheroidal, whereas larger tumors can be sausage-shaped, ovoid, or irregularly lobulated []. Larger schwannomas have a tendency to undergo secondary degeneration such as pseudocystic regression, calcification, and hemorrhage [], which can explain inhomogeneities in a portion of schwannomas. In the 15 cases, seven cases (47 %) underwent cystic changes or calcification. Microscopically, the hallmark of schwannoma is the pattern of alternating Antoni A and B areas, with varying relative amounts []. The Antoni A area is hypercellular and characterized by closely packed spindle cells with occasional nuclear palisading and Verocay bodies, whereas the Antoni B area is hypocellular and is occupied by loosely arranged tumor cells []. Two kinds of structures often coexist in the same tumor, but most with one main type. There were two cases of cellular schwannoma in 15 cases characterized by predominantly Antoni A areas histologically [, ].\nImmunohistochemical staining is strongly and diffusely positive for S-100 protein in a schwannoma consistent with the finding of a nerve sheath tumor []. A few gastrointestinal stromal tumors are positive for S-100; however, they are also positive for either CD34 or CD117 []. As a contrast, a schwannoma is negative for both CD34 and CD117 []. A leiomyoma would be negative for S-100 and positive for desmin or smooth muscle actin []. In addition, we also demand to make a differential diagnosis with malignant peripheral nerve sheath tumor (MPNST), although malignant transformation of these tumors is very rare []. MPNSTs are usually characterized by high cell density, obvious cell atypia, more pathological mitotic, diverse cellular components, and it is common to see tumor necrosis, CK(+), EMA(+), and CEA(+) [].\nThe main treatment of porta hepatic schwannomas is complete excision with free margins and no lymph node dissection. In the 15 cases, seven patients underwent complete tumor excision. But, two patients underwent tumor excision en bloc with hepatic artery resection because of the intraoperative finding of tumor invasion of the hepatic artery (including the present case) []. In addition, one patient underwent tumor or bile duct excision and Roux-en-Y hepaticojejunostomy because the tumor was attached to the extrahepatic bile duct []. One patient underwent tumor excision en bloc with T-tube drainage because a small rupture of the bile duct wall caused bile leakage during the dissection of the extrahepatic bile duct []. One patient underwent tumor excision en bloc with right hepatectomy because the right anterior portal vein was invaded [], and the details of surgical resection were not specified in three patients. Postoperative pathological results indicated 13 cases of benign schwannoma and two cases of cellular schwannoma which is considered as a subtype of schwannoma [, ]. The complete excision of the tumor is curative and most cases do not relapse and additional treatments are not necessary. The overall prognosis is very good []. In the follow-up of 6/15 patients, there was no recurrence with a mean follow-up of 16 months (range 3–41 months). |
A 30-year-old female patient reported to the Department of Oral Pathology, with a swelling over the left side of the palate. Past history revealed that the patient had initially noticed the swelling 6 weeks ago. She had consulted a private dentist when the swelling was approximately 1.5 × 1.5 cm in size and had no associated symptoms (Figures and ). She was advised a biopsy, report of which revealed a histopathological diagnosis of Hemangioendothelioma. She then reported to our hospital for management of the same. On taking an elaborate history, difficulty eating and brushing was revealed. On extra oral examination, a firm swelling extending 1 cm from ala of the nose on the left side anteriorly up to 3 cm from the tragus of the left ear posteriorly was noted. On intraoral examination, a massive, solitary proliferative growth measuring 2.5 cm × 3 cm with irregular margins, extending from the left maxillary canine region up to the posterior part of the hard palate, was evident. The lesion was crossing the midline at the midpalatal region. Mucosa over the swelling was erythematous in appearance and the labial, buccal, and palatal sulci were obliterated due to buccopalatal expansion. It appeared that, at this stage, the swelling had increased in size from its initial description. Computed tomography (CT) findings revealed a heterogenous, well-defined, intensely enhancing lesion measuring 3 × 4.1 × 4.3 cm (cc × ap × trans) seen involving the left side of buccal mucosa and hard palate (Figures and ). Laterally, an erosion of alveolar process of maxilla on left side and involvement of levator anguli oris muscle were seen, with no evidence of neovascularity. The H&E section (provided by the previous hospital of consultation) did not reveal a concrete picture of Hemangioendothelioma. An IHC analysis for CD 34 of the incisional biopsy also revealed a negativity for the tumor cells ruling out the provisional diagnosis of Hemangioendothelioma (Figures , , and ). Based on the clinical manifestations and investigatory findings, the patient was referred to the Department of Oral and Maxillofacial Surgery for further surgical management. Partial alveolectomy of left maxillary region was planned.\nPatient was placed in supine position and GA was administered. Right nasotracheal intubation was done. Considering the angiomatous nature of the lesion in maxilla, prior to Maxillectomy, the ECA was exposed and held for immediate ligation in case of untoward hemorrhage. The surgery was done as two stages: (1) neck and (2) maxilla.\nSkin incision was placed on Resting Skin Tension Line on the left side of the neck, followed by layer-by-layer dissection. Weber-Ferguson incision was placed on the left side and layer-by-layer dissection done to locate the left maxillary buttress region. Osteotomy was done at Lefort I level from left pyriform aperture to maxillary tuberosity region. After complete excision of the lesion with adequate clearance, an obturator was placed over a Bismuth Iodide Paraffin Paste pack. The resected tumor was sent for histopathological examination.\nHistopathological examination of the soft tissues revealed an encapsulated mass comprising stratified squamous epithelium and underlying richly cellular connective tissue stroma, containing plenty of multinucleated giant cells and dilated blood capillaries. H&E 40x view showed multinucleated giant cells with agglomeration of around 20–40 hyperchromatic nuclei in the center surrounded by clear cytoplasm and pleomorphic proliferating stromal cells. Some of the sections showed the increased vascularity with extravasation of red blood cells. Cellular pleomorphism and mitotic figures with an average of 4 per high power view were also seen which indicates local aggressiveness of this lesion (Figures , , , and ). The level of serum alkaline phosphate was highly increased (320 U/L) (normal level: 45–129 U/L). A final diagnosis of giant cell tumor was given based on these characteristic findings: the characteristic appearance of proliferating stromal cells, presence of multinucleated giant cells, occurrence of cellular atypia and mitotic activity, CT, and laboratory findings. There was no evidence of recurrence in eleven months of follow-up. |
A 26-year-old Caucasian man with end-stage renal disease received kidney transplantation from a deceased donor in October 2013 after having performed chronic peritoneal dialysis and hemodialysis for seven years. He had suffered from juvenile rheumatoid arthritis since early childhood. Because the patient had initially been admitted with end-stage renal disease, his primary renal diagnosis was unknown, but secondary amyloidosis due to rheumatic disease was suspected.\nThe patient received induction therapy with basiliximab followed by an immunosuppressive regimen consisting of tacrolimus, mycophenolate mofetil (MMF), and prednisolone. Because of CMV mismatch he also received valganciclovir prophylaxis. He was discharged ten days after transplantation with serum creatinine of 83 μmol/L. On day 11 after transplantation pronounced proteinuria with a protein-creatinine ratio of 3.34 g/g was noted. An allograft biopsy showed ten normal glomeruli with negative immunohistology, and early recurrent primary FSGS was deemed to be the most likely diagnosis. Despite nine PE sessions with exchange of one plasma volume each (5% human albumin as substitution fluid) over a period of three weeks, proteinuria remained significantly elevated after an initial decline from 5.5 g/g to around 3.5 g/g. Treatment with rituximab was not considered a therapeutic option because of severe immunoglobulin deficiency (IgG 370 mg/dL). Instead, after extensive discussion with the patient, a single dose of abatacept 10 mg per kg body weight was given. In order to avoid overimmunosuppression the MMF dose was reduced from 1000 mg to 500 mg daily. Within the next three weeks, proteinuria decreased to 1.5 g/g creatinine but over another three weeks began to rise again until it peaked at 4.5 g/g. The patient's serum creatinine increased to 151 μmol/L. A second allograft biopsy showed progressive disease with diffuse mesangial expansion ().\nElectron microscopy revealed dystrophic podocytes with flattened foot processes ().\nThe patient was treated with further eight PEs, which reduced proteinuria to around 3.0 g/g creatinine with a tendency to increase. We therefore decided to give a second dose of abatacept. This was followed by a rapid decline in proteinuria to 1.0 g/g and a further decline to 0.15 g/g in the following months. The time course of treatment with PE and abatacept, serum creatinine, and proteinuria is shown in .\nTwo weeks after the second dose of abatacept BK viremia was detected. BK viremia increased to 200.000 copies/mL and JC viremia (6800 copies/mL) was also detected. We discontinued MMF and reduced the tacrolimus dose, aiming for trough levels between 3 and 5 ng/mL, and prednisolone to 5 mg daily. JC viremia subsided after five months, whereas mild BK viremia (10.000 copies/mL) persisted. As the patient had negative EBV serology, regular EBV DNA monitoring was performed, which remained negative throughout the disease course. Over the next year, the patient remained in complete remission without proteinuria and with serum creatinine stable at 125 μmol/L. |
A 42 year-old woman presented with intermittent dry cough of ten months duration. No fever, chest tightness, bone pain, anorexia, dyspnea on exertion or body weight loss was noted. She took some medicines at a local clinic to control her cough. However, blood-tinged sputum appeared one month prior to hospital admission. No bone pain or lymphadenopathy was noted on examination. Her breathing was slightly coarse without rales or rhonchi on auscultation. A chest X-ray (Figure ) and computed tomography (CT) (Figure ) revealed a right anterior mediastinal shadow with multiple pulmonary nodular lesions.\nTracing back her history, she was a non-smoker, without occupational or environmental exposure to air pollutants or micro-organisms. No family members had any similar clinical manifestations nor had any died of cancer before. No related travel history was noted during this time.\nAfter admission, a bronchoscopic examination revealed no endobronchial lesion or abnormal secretions. A pulmonary function test showed a mild restrictive ventilatory defect. The whole body bone scan was negative for tumor involvement. A CT-guided biopsy was recommended but the patient refused to undergo this procedure. The hemogram, leukocyte differentiation count, and coagulating profile were all within normal ranges.\nA biopsy through video-assisted thoracoscopic surgery (VATS) was then indicated to confirm the diagnosis. A 3 cm working incision and a 1 cm scope port were designed for performing this procedure. Removing a wedge of lung tissues including the masses from two separate sites were performed smoothly and the microscopic examination showed a solid mass made up mostly of plasma cells (Figure ). These tumor cells stained positively for kappa light chains (Figure ), but negatively for lambda chains.\nAfter confirming the diagnosis, the patient received a series of related evaluations such as serum calcium, urine Bence-Jones protein and plasma electrophoresis for M protein detection. However, all of the above examinations were negative. The skull, spine and pelvis X-ray revealed no osteolytic lesions. The bone marrow biopsy revealed normal patterns of cell distribution.\nShe received adjuvant chemotherapy, as originally planned, including mephalan and steroids, and her symptoms improved after two months of treatment. |
A 63-year-old female with no comorbidities presented with gradually progressive, painless, upper abdominal distension and loss of appetite for one year. Physical examination revealed a distended abdomen and epigastrium, as well as right and left hypochondrium fullness extending to the umbilicus. A nontender mass of about 15 cm × 12 cm in size was palpable in the epigastrium, the right and the left hypochondrium, and the umbilical regions. The mass was firm, had a smooth surface, moved from side to side, and fell forward when the patient was in the knee-elbow position. The findings of abdominopelvic ultrasound showed a 12.5 cm × 9.6 cm uniformly echogenic lesion with unclear margins in the midline; the lesion occupied the epigastric and umbilical regions and pushed bowel loops to the periphery.\nContrast enhanced computed tomography (CECT) of the abdomen and the pelvis showed a space-occupying lesion in the epigastric/umbilical region measuring 16 cm × 16 cm × 10 cm. The lesion was a nonhomogenous mass with irregular enhancement and displaced the bowel loops (straddling sign, ). Bowel loops were located between the tumor and the anterior abdominal wall, suggesting a probable mesenteric origin []. No evidence of a retroperitoneal lymphadenopathy was found, and the lesion was reported as a large teratoma occupying the anterior epigastric/umbilical regions with a possible mesenteric origin.\nThe patient underwent surgery. A midline laparotomy was done, and the peritoneal cavity was explored. A huge lipomatous mass measuring 23 cm × 16 cm × 12 cm was found in the lesser sac over the transverse mesocolon. The lesser sac was opened, and the mass was found to be well defined without any major vascular invasion. The tumor was excised in toto with macroscopically negative margins. On further exploration, multiple such separated masses with sizes ranging from 4 to 7 cm were found to be attached to the transverse mesocolon ( and ), and each was excised with clear margins. Postoperative period was uneventful. Histopathology showed similar features suggestive of an atypical lipoma or a well-differentiated liposarcoma in all of the excised lesions, suggesting possible multicentricity or the presence of satellite nodules. |
A 58-year-old African American male was seen in hepatology clinic for a surveillance liver ultrasound following a diagnosis of hepatitis C. The patient had experienced mild intermittent upper abdominal pain consistent with a history of gastroesophageal reflux disease. He had no weight loss, change in appetite or bowel habits, jaundice, or general malaise. Past medical history included hyperlipidemia, peptic ulcer disease, and hepatitis C (antibody positive, PCR negative). The patient's physical examination and laboratory studies were otherwise normal. A surveillance abdominal ultrasound demonstrated a well circumscribed mass measuring 3.4 × 2.9 cm within the head of the pancreas. A computed tomography (CT scan) further confirmed an enhancing mass involving the head of the pancreas (Figure ). No evidence of extra-pancreatic disease was found on further evaluation. Although tumor-associated antigen levels (CEA, CA 19-9, and CA-125) were normal our differential diagnosis still included adenocarcinoma of the pancreas. Endoscopic ultrasound (EUS) guided needle biopsy was then performed but the pathological findings were non-diagnostic. Our concern for possible undetected malignancy remained and a diagnostic laparoscopy was performed followed by PD. The patient recovered well following the procedure and was discharged home on the 15th postoperative day. He continues to receive close outpatient surveillance without additional treatment.\nWithin the PD specimen, a protuberant 4 × 3 × 3 cm circumferential mass at the superior border of the pancreatic head was appreciated (Figure ). The external surface of the mass was tan/gray, firm, and smooth. Cut sections of the mass also revealed a partially encapsulated large nodule with tan/gray fleshy to firm parenchyma infiltrating the pancreas at its distal portion. Surgical margins were clear (Figure ). Regional lymph nodes were identified near the mass; microscopic examination revealed that all were hyperplastic lymph nodes. The remaining surgical margins were negative.\nThis lymphoid lesion within the pancreatic head showed marked vascular proliferation and hyalinization of germinal centers. Involuted germinal centers were surrounded by concentric rings of small lymphocytes. i.e. "onion-skinning" penetrated by hyalinized vessels (Figure ). Moreover, the interfollicular zones were rich in plasma cells and hyalinized vasculature. These features are consistent with localized hyaline-vascular type CD. Distribution of T and B cell markers (CD20, CD3, CD45) was normal and markers indicative of lymphoid malignancies (i.e. CD30, CD15, CD5, CD10, BCL-2) did not show evidence of such entities. Furthermore, PCR analysis of paraffin-embedded tissue revealed no clonal rearrangement of the immunoglobulin heavy chain or T-cell receptor gamma and beta chain genes. MALT1 (18q21) clonal rearrangement by FISH was negative. Together, these morphologic and immunohistochemical findings are most consistent with a diagnosis of CD. |
A 31-year-old woman, gravida 2 para 1, presented for a prenatal ultrasonographic examination at 36 gestational weeks owing to a suspicion of a fetal thoracic wall defect. Her personal history revealed a spontaneous abortion and no consanguinity. She underwent routine ultrasonographic examinations at 13, 22, and 30 gestational weeks at a regional hospital; however, at 35 gestational weeks, ultrasonography revealed an abnormal fetal thoracic wall.\nPrenatal ultrasonography revealed a fetal thoracoabdominal wall defect with partial displacement of the left ventricle and the liver associated with rotation and elongation of the heart and a high index of clinical suspicion for intracardiac malformations such as tricuspid atresia, a ventricular septal defect, and pulmonary artery hypoplasia (Figures –).\nBased on the aforementioned findings, she was admitted to the Obstetrics and Gynecology Clinic in Târgu Mure at 39 gestational weeks, where she underwent a cesarean section. The male newborn weighed 3,100 g with an APGAR score of 7. Clinically, he demonstrated a superior abdominal wall defect, a partial extrathoracic displacement of the heart, and a partially herniated liver (these structures being covered by a very thin skin layer), and also a diastasis of the sagittal suture (Figure ). The newborn was intubated, and we applied a saline-soaked gauze pad on the thoracoabdominal and cranial defects to maintain humidity.\nPostnatal echocardiography confirmed the prenatal diagnosis and also showed a partial extrathoracic and extra-abdominal displacement of the heart and liver, a large ventricular septal defect, great arteries originating from the left ventricle with the aorta situated anteriorly, a posterior deviation of the outlet septum causing severe subpulmonary stenosis, hypoplasia of the pulmonary artery, and a large hourglass-shaped left ventricle secondary to narrowing of the heart at the level of its extrathoracic displacement.\nWe also performed thoracoabdominal CT-angiography, which showed complex cardiac malformations consisting of large ventricular and atrial septal defects, an increased left ventricular volume, with apical extrathoracic aneurysmal dilatation below the xiphoid process at the level of the abdominal midline, hypoplasia of the right ventricle, and a reduced caliber of the pulmonary trunk artery. Abdominal CT revealed partial transparietal herniation of the left hepatic lobe adjacent to a left ventricular diverticulum, and an increase in the size of the right adrenal gland with hyperdense contents suggesting an adrenal hematoma. Cranial CT revealed a diastasis of the sagittal suture causing subcutaneous herniation of the venous sagittal sinus.\nFollowing admission to the Neonatal Intensive Care Unit, the newborn was administered ampicillin and amikacin, fluconazole, prostaglandin E, and phenobarbital (because he presented with multiple seizures), and also received daily dressing changes. During the first week of life, he showed multiple episodes of bradycardia and low oxygen saturation despite undergoing orotracheal intubation; therefore, surgical intervention was postponed until he was hemodynamically stable. He underwent surgical intervention at 14 days of age, consisting in the replacement of the heart inside the thorax via a systemico-pulmonary shunt procedure, with vascular prosthesis, the ligature of both persistent arterial canal and pulmonary artery trunk, and repair of the diaphragm defect. The abdominal wall defect was also sutured, but the thorax remained open. The surgical procedure was performed in extracorporeal circulation, and lasted 4 h and 15 min.\nPostoperatively, the newborn developed multiple episodes of tachyarrhythmia and low cardiac output suggesting an inability of the heart to adjust to the intrathoracic pressure. Unfortunately, the newborn died 5 h postoperatively secondary to progressive hemodynamic deterioration, metabolic acidosis, and hypoxia. |
A 51-year-old previously healthy male was brought by his family members following sudden loss of consciousness. On admission his Glasgow coma scale (GCS) was 7/15 (eye 1, verbal 2, and motor 4). Neurological examination revealed bilateral symmetrical sluggish pupils of 3 mm. We could do only a limited neurological examination due to low GCS. He moved all four limbs to a painful stimulus and the deep tendon reflexes were normal. Bilateral flexor plantar response was present. With the suspicion of any drug overdose, a urine sample for toxins was sent urgently, but all the tested toxins including opioids, benzodiazepines and amphetamines were negative. An urgent NCCT brain was done within two hours of symptom onset to exclude intracranial hemorrhage and it turned out to be normal.\nAfter four hours of admission, his GCS improved to 11/15 (E3V2M6). However he was drowsy and there was bilateral asymmetrical ptosis, right more than left. Third nerve palsy without pupillary involvement was evident on the right side. Right eye medial and downward gaze were impaired. He also had bilateral upward gaze palsy, but had no nystagmus (Fig. ). These findings suggested a right-sided nuclear third nerve palsy. He did not have pyramidal or cerebellar signs and the visual fields were normal.\nAs the NCCT brain was normal, we proceeded with magnetic resonance imaging (MRI) and magnetic resonance angiogram (MRA) brain. It was reported as acute infarction in bilateral paramedian thalami and medial rostral mid brain, suggesting AOP territory infarction. MRA showed hypoplastic right vertebral artery. Apparent diffusion coefficient (ADC) images and diffusion weighted images (DWI) showed the paramedian thalamic infarctions in the index case due to AOP involvement. Here the rostral mid brain involvement is asymmetrical as was suggested by the clinical findings as well (Fig. ).\nOver a week, his hypersomnolance gradually improved but his diplopia persisted. After the diagnosis of ischemic stroke, the underlying risk factor assessment was performed. He had hypertension, but no diabetes mellitus. Lipid profile was deranged with a low density lipoprotein of 140 mg/dL. Electrocardiogram did not show any evidence of arrhythmia or past cardiac ischemic events. Transthoracic echocardiogram was normal except for the left ventricular hypertrophy. He did not have polycythemia or thrombocytosis. As he had conventional risk factors for a stroke, we did not further evaluate for other thrombophilic conditions. He was discharged after 1 week of hospitalization with out-patient speech and physiotherapy. We started aspirin 75 mg, clopidogrel 75 mg and atorvastatin 40 mg daily. At the clinic visit scheduled 3 weeks after the discharge, clopidogrel was withheld and aspirin 75 mg and atorvastatin 40 mg were continued. By the time of clinic visit he had a marked recovery and could engage in his day-to-day activities. However he could not resume his work as a taxi driver even 3 months after the initial stroke. |
In 1988, a 4 cm benign ganglioneuroblastoma [] was surgically removed from the left posterior rib cage, adjacent to the thoracic spine, of a 3-year-old girl. Post-surgical radiographs showed a straight spine. Five years later, when the patient was 8 years old, radiographs showed left 4th and 5th rib fusion. In 1999, at the age of 11, the patient was diagnosed with adolescent idiopathic scoliosis (AIS) by her family physician and was managed with the “wait and see” observation method at the children’s hospital in Toronto, Canada, until the age of 17. Radiographs taken between the ages of 8 and 17 were no longer available at the time of this case presentation, but the patient’s medical record did mention a diagnosis of scoliosis at age 8, without scoliosis follow-up. The patient was only monitored for recurrence of the ganglioneuroblastoma. At the age of 15 and again at the age of 17, spinal fusion surgery was recommended to the patient but the patient refused.\nIn November 2008, at the age of 23 years, the patient presented for an initial physiotherapy evaluation, complaining of a 2-year history of severe (7/10 on the pain scale) intermittent episodes of low back pain and shortness of breath while going up stairs. Her pain and shortness of breath had progressively worsened to the point where she had to discontinue all physical activities, including scuba diving because her rapid respiratory rate forced her to use up her oxygen supply in half the amount of time. At times, her back pain was so severe that she was unable to get out of bed.\nA prior radiograph from 2006 showed a 68-degree Cobb angle thoracic curve and a 47-degree Cobb angle lumbar curve. Upon initial physiotherapy assessment, there was visible severe spinal deformity (Fig. ). The thoracic angle of trunk rotation (ATR) was 19° and chest expansion was 1.5 cm at the nipple line. Chest auscultation revealed absent breath sounds over the lower lobe of the left lung. Inspection of the chest wall showed a 10 cm long scar resembling a lobectomy. Concern for more serious underlying lung pathology warranted that repeat radiographs be obtained. The radiographs taken in November 2008 shortly after initial physiotherapy assessment revealed a 70-degree Cobb angle thoracic curve and a 48-degree Cobb angle lumbar curve (Fig. ), reflective of the average 1° annual scoliosis curve progression observed in adults. In addition to curve progression, the follow-up radiograph showed fused 4th and 5th left ribs, a new finding for the patient at the time. In December 2008, the patient began Schroth physiotherapy, focusing on directional breathing and scoliosis-specific exercises for a 3C curve type [] (according to Schroth scoliosis curve classification). |
A 73-year-old Asian woman with an underlying anxiety disorder, functional headache, and hypertension was prescribed escitalopram and lorazepam when she presented with progressively worsening headaches to her primary care doctor. Her symptoms did not improve with the medications, and she was unable to eat well and required bed rest. She was transported to our hospital 4 days later after developing chest and back pain with altered consciousness. She was a housekeeper, had no allergies, and had no alcohol or tobacco smoking history. On arrival, her Glasgow Coma Scale score was 3/15 (E1V1M1); both pupils were approximately 4 mm in diameter and reactive. Her blood pressure was too low to be measured, her carotid artery pulse was palpable, her heart rate was 112 beats/minute, and her respiratory rate was 30 breaths/minute. Her conjunctiva was pale. An auscultation of breath sounds did not reveal upper and lower airway obstructions and was within normal limits. Her abdomen was soft and flat without tenderness. She had no skin abnormalities (such as rash). Both legs had no edema. Echocardiography on arrival was performed as point of care ultrasound and revealed a hypercontractile left ventricle with an eliminated left ventricular cavity and a collapsed inferior vena cava without right ventricular dilation. There was no pericardial effusion or obvious large regurgitant jet observed on color Doppler. In response, we immediately inserted a peripheral venous catheter and began introducing fluid resuscitation; however, she developed PEA. Conventional CPR according to the adult advanced cardiovascular life support guidelines (including adrenaline) was initiated and a return of spontaneous circulation (ROSC) occurred. However, her blood pressure was unstable and PEA returned, prompting repeated CPR with immediate administration of fluids and three adrenaline injections. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) was initiated for refractory PEA. Whole-body contrast-enhanced computed tomography was unremarkable, and the admission laboratory results were also unremarkable, except for anemia (Table ). Her hemoglobin level decreased from 7.1 g/dL to 3.5 g/dL 1 hour later without obvious signs of gastrointestinal hemorrhage. Therefore, 8 units of packed red blood cells were transfused for 1 day, after which her hemodynamic status stabilized. She was in a coma without sedatives; thus, targeted temperature management at 34 °C was initiated on admission to an intensive care unit. Echocardiography in the intensive care unit showed a thickened interventricular septum (which was 12.8 mm), prolonged anterior mitral valve, and contact between the bodies of the anterior and posterior mitral valves, suggesting that the left ventricular obstruction could have potentially occurred through this redundant anterior mitral valve. VA-ECMO was terminated on day 3, and after stabilizing her hemodynamics, transthoracic echocardiography showed a sigmoid septum with normal left ventricular function (ejection fraction, 75%) (Fig. ). On day 26, dobutamine-infused (30 μg/kg per minute) Doppler echocardiography revealed a significant outflow gradient (236 mmHg) accompanied with chest pain (Fig. ) and intermittent systolic anterior motion (SAM) of the mitral valve; continuous monitoring during Doppler echocardiography showed a Brockenbrough–Braunwald sign (Fig. ), which is a fall of arterial blood pressure after premature ventricular contraction; these findings confirmed a diagnosis of latent LVOTO due to a sigmoid septum. The significant LVOTO was not dependent on SAM but might have occurred due to the greatly thickened interventricular septum. As a result, carvedilol was initiated with gradual increment up to 10 mg/day on day 35. In addition, verapamil (120 mg/day) was administered on day 29. A follow-up dobutamine-infused Doppler echocardiography on day 40 showed a reduction of the outflow gradient to 14 mmHg, indicating a successful medical therapy.\nThe worsening anemia that was identified at admission was suspected to be hemolytic anemia (HA) based on results of the blood test. The results of the laboratory examination, including total bilirubin, direct bilirubin, lactate dehydrogenase, reticulocyte count, and haptoglobin, and a direct Coombs test on day 1 are presented in Table . Her drug lymphocyte stimulation test was positive, and agglutination occurred when her serum reacted with lorazepam. Therefore, lorazepam-induced immunological HA was diagnosed. Her anemia improved following discontinuation of lorazepam. Her hemoglobin level was 11.9 g/dL on day 64, and she was eventually discharged on day 68 without any sequelae. |
A 42-year-old man presented with a 4-month history of hoarse voice and a 12-month history of intermittent high dysphagia and odynophagia. His symptoms started immediately after he accidentally swallowed an acrylic dental plate (Figure ) whilst eating. He presented to the emergency department of his local hospital: plain x-rays were performed and reported as normal. He was reassured and discharged. Over the next 6 months, his symptoms of high dysphagia and odynophagia persisted. His general practitioner referred him to the local ENT department. Flexible laryngoscopy was normal on two occasions. A barium swallow was also reported as normal. Two months later, after swallowing a piece of meat, he had an episode of complete dysphagia followed by severe odynophagia and a hoarse voice. Repeat flexible laryngoscopy demonstrated left recurrent laryngeal nerve palsy and he was referred for urgent computed tomography of the neck and chest. This showed thickening in the mid-oesophagus and a possible intraluminal foreign body (Figure ). At oesophagoscopy, the dental plate was found to be impacted in the oesophagus at the level of the aortic arch (25 cm from the incisor teeth; Figure ) and could not be safely removed. He was referred to a specialist oesophagogastric unit for surgical intervention. Due to its location in the thoracic oesophagus, it was elected to approach the foreign body via a right posterolateral thoracotomy through the fourth intercostal space. At operation, the mid-oesophagus appeared thickened, and there was marked peri-oesophageal inflammation and fibrosis. There was no evidence of perforation or fistula within the right pleural cavity. After dividing the azygos vein, the dental plate was removed via a longitudinal oesophagotomy (Figure ), which was closed in two layers with an absorbable 2/0 suture. He made an uneventful postoperative recovery. A postoperative water-soluble contrast study was normal, and he was discharged on day 8. At follow up, his swallowing had returned to normal, but he had a persistent hoarse voice due to recurrent laryngeal nerve palsy, for which he has been referred for injection laryngoplasty. |
We report the case of a 74-year-old white woman who was hit as a pedestrian by a car moving at approximately 40 to 50 km/hour. The initial emergency physician started treatment according to standard protocols. Her cervical spine was immobilized with a stiff collar and a pelvic compression belt was applied anticipating a pelvic injury. With a Glasgow Coma Scale of 14 at that time, there was no need for an emergency intubation. Her vital signs were documented; she had an arterial blood pressure of 160/100 mmHg and a heart rate of 86 beats per minute, indicating a negative shock index.\nDuring transport she received 2×500 ml of crystalloid fluids (Sterofundin; B. Braun Melsungen, Germany). At our emergency department a multidisciplinary team of trauma surgeons, anesthesiologists, a neurosurgeon, a visceral surgeon, and a radiologist was awaiting the patient. At the time of her arrival, she was still awake and responsive with a Glasgow Coma Scale of 14. A physical examination according to the guidelines of Advanced Trauma Life Support showed no signs for an airway, breathing, or circulation problem (ABC-problem). Her thorax was stable and there were no indications for deterioration in gas-exchange. The pelvic splint applied by the emergency physician was left in place. An abdominal examination by a trained visceral surgeon showed no signs of tension or muscular defense. There were no visible abdominal contusion marks. Numerous fractures of her extremities were suspected, which were immediately repositioned and splinted. A focused assessment with sonography for trauma indicated no pleural effusion or pericardial effusion, and no free abdominal fluid, air, or hints for organ lacerations. A thoracic X-ray showed no signs of a pneumothorax or rib fractures. After a pelvic X-ray, non-dislocated fractures of the left ramus superior and inferior of her os pubis were suspected. The first blood gas analysis in our emergency room revealed a hemoglobin (Hb) level of 10.6 g/dl and a lactate level in physiological range (1.4 mmol/l). After initial assessment of our patient, a full body trauma computed tomography (CT) scan with the application of a radiocontrast agent was performed and the following diagnoses were stated using fracture classifications according to the Association for the Study of Internal Fixation (AO):Traumatic subarachnoid hemorrhage Tripod fracture on the right side with orbital floor affection Three-part fracture of her proximal humerus on the left side (AO 11 C3) Two-part fracture of her proximal humerus on the right side (AO 11 C2) Small intracapsular hematoma of her spleen Pelvic ring fracture (AO type B)○ Fracture of the left ramus superior and inferior of her os pubis ○ Longitudinal fracture of her right sacrum Pertrochanteric femur fracture right (AO 32 A1) Fracture of her left proximal tibia (AO 42-C3)\nThere were no signs for free abdominal fluid or air, no signs for the extravasation of the radiocontrast agent in her thoracic, abdominal, or pelvic cavity (indicating a vascular injury), and no signs for a greater hematoma in the small pelvis. Her medical history revealed several internal comorbidities but no signs of coagulopathy or anticoagulant medication.\nDuring the diagnostic phase she remained awake and neurologically adequate with stable hemodynamics. According to the concept of damage control the indication for external fixation of her pelvis and her lower extremity was made and she was transferred to our intensive care unit (ICU) before early operative treatment. In the course of the preoperative preparations she presented signs of respiratory exhaustion with increasing hypoxia and had to be intubated. During the intubation her clinical condition worsened dramatically due to aspiration and hemodynamic decompensation. Stabilization was only achieved after a period of approximately 5 minutes of mechanic cardiopulmonary resuscitation (CPR) and continuous infusions of catecholamines. A physical examination of her abdomen showed no signs of tension. An immediate blood gas analysis revealed an Hb level of 5.7 g/dl; her lactate level was still in physiological range (0.5 mmol/l). An additional CT scan of her thorax, abdomen, and pelvis was performed due to the suspected diagnosis of anemic shock in order to rule out new sources of bleeding after the CPR. However, the second CT scan showed no new findings; explicitly, no free abdominal fluid or air, no signs of extravasation of the radiocontrast agent in her thoracic, abdominal, or pelvic cavity, and no signs for a progressing hematoma of her spleen or in her small pelvis (Fig. ).\nShe had received 5×500 ml of crystalloid fluid (2.5 liters) and 2×500 ml of colloidal fluid (1 liter) so far. According to clinical transfusion protocols, from this point of time on she received packed red blood cells (PRBCs), platelets, and fresh frozen plasma (FFPs) to compensate for the sudden drop in her Hb level. Within 25 minutes the first six PRBCs were transfused. In addition, she required high doses of catecholamines (Fig. ).\nAfter the second CT scan, she was brought immediately to our operating room to stabilize her pelvic ring fracture. At the start of the operation, her ventilation situation worsened dramatically, with hypercapnia, hypoxia, and an increasing peak airway pressure of up to 60 cmH2O (Fig. ). In parallel, she presented an upper venous congestion with subsequent hemodynamic decompensation, necessitating mechanic CPR. Her abdomen appeared increasingly rigid and tense. Her lactate level was now increasing to 6.8 mmol/l (Fig. ). Bilateral chest drainages were applied to rule out a hemopneumothorax, but pulmonary ventilation became almost impossible. At the same time, a cardial tamponade was excluded by transesophageal echocardiography, and bronchoscopic evaluation of her airways showed no pathological findings. Under ongoing CPR, a pelvic clamp was applied to her dorsal pelvic ring for mechanical stabilization before performing a decompressive laparotomy. After opening her peritoneum, 2 liters of clear ascites-like fluid could be removed. After opening her abdomen widely, her ventilation situation immediately improved and her peak airway pressure was lowered to 35 cmH2O. After a total of 12 minutes of CPR a sinus rhythm and hemodynamic stabilization could be retained again. Catecholamine doses could be reduced extensively. She received 12 PRBCs, eight FFPs, and four platelets prior to the decompressive laparotomy. Combined with crystalloids and colloids, this equals a total volume of 10.5 liters of resuscitation fluids.\nA detailed inspection of her abdominal cavity by a specialized visceral surgeon revealed no major bleeding or organ injury. Her spleen showed a small intracapsular hematoma, but no signs of greater injury. Her liver appeared to be congested. Her bowel looked edematous and showed signs of the beginning of ischemia. An inspection of her small pelvis showed no signs of bleeding. As a precaution, her pelvis was packed and the laparotomy was temporarily closed with a low-adherent wound contact layer, consisting of a flexible polyamide net coated with soft silicone.\nIn the postoperative course of treatment, additional operations followed. Her abdomen was closed 23 days after the decompressive laparotomy. She was transferred from our ICU to our intermediate care unit 40 days after trauma. She was transferred to a neurological rehabilitation center 8 weeks after trauma. |
A 41-year-old pregnant woman, in her first gestation, sought medical care complaining of pain in her right shoulder for the last two months, associated with the impairment of moving her arm. She was 20 weeks pregnant and did not attend any prenatal consultation. Her medical history included smoking, correctly treated syphilis, and pulmonary thromboembolism. The patient did not have any family history of thyroid disease.\nRight arm radiography () demonstrated a lytic bone lesion on her shoulder, apparently involving the surrounding soft tissues. The initial working diagnosis was a soft tissue or bone tumor with differential secondary lesions, for example, metastatic carcinomas. The patient was submitted to a biopsy of the lesion (), which revealed a high-grade malignant neoplasm with an epithelioid aspect on the histological examination. The predominant pattern was solid with sheets of atypical cells and a sparse microfollicular-like arrangement. There were many mitoses and, occasionally, nuclear hyperchromasia. The morphological hypothesis was a metastatic carcinoma and, more remotely, primitive neuroectodermal neoplasms.\nThe immunohistochemistry (IHC) was diffuse and strongly positive for cytokeratin (AE1/AE3) and TTF1. CD45, CD99, chromogranin-A, and synaptophysin were negative. Since the morphology was favorable for thyroid differentiation, the IHC panel was extended to thyroglobulin, calcitonin, and surfactant with a positive result only for thyroglobulin. Thus, the diagnosis of metastatic thyroid carcinoma was made. Also, the thoracic computerized tomography (CT) failed to show any pulmonary lesion. The thyroid examination revealed an increased cervical volume, with some firm and painful areas, in the paramedian region. It is important to mention that until that moment the patient had no previous medical care. The cervical enlargement observed in the anterior portion of the neck was not reported by the patient. When asked, the patient reported that there might have been some increase in the region.\nThe ultrasonography (US) and magnetic resonance imaging (MRI) revealed a suspected nodule localized in the left lobe of the thyroid, measuring 4.0 cm in diameter (). An FNA was performed, and cytological evaluation was consistent with category IV of Bethesda Classification, suspected for follicular neoplasm ().\nAfter this diagnosis, the option for treatment was a total thyroidectomy. The macroscopic examination demonstrated a diffusely increased glandule where there was a nodule measuring 4.0 × 4.0 × 3.0 cm in the left thyroid lobe. It was an expansive tumor with macroscopic infiltration of the capsule ().\nThe histopathologic finding was similar to the bone/soft tissue lesion biopsy. Immunohistochemistry results were the same (). The final diagnosis was follicular carcinoma of the thyroid, with follicular (90% of the tumor) and solid (10% of the tumor) patterns accompanied by necrosis, vascular infiltration (at least six areas), and lymphatic infiltration. Mitosis number is two in ten high magnification microscopic fields. Near the thyroid, there were two lymph nodes without neoplasm. However, no specific resection of cervical lymph nodes was performed. The pathological stage was pT3a pN0 pM1 (AJCC, 2017). The tests performed were: IHC markers such as thyroglobulin and TTF1 with a diffuse pattern of immunostaining. The Ki-67 index was compatible with the mitosis number (8%), and the p53 marker was negative.\nA cesarean was performed at the same time, at 30 weeks of pregnancy. After three months, the patient underwent surgery of the right arm to install a prosthetic. She received therapeutic iodine treatment and had medullary compression syndrome, secondary to the bone and spinal involvement. There were no other sites of metastasis. The disease progressed, and the patient died one year after the diagnosis. The child survived without morbidities. |
A 70-year-old woman presented in November 2017 to the Emergency Department at Skåne University Hospital, Sweden, due to the rapid onset of fever, shivers, and a suspected skin infection. She had a previous medical history of left-sided ductal breast cancer with lymph node involvement in 1999, which was treated chronologically with neoadjuvant chemotherapy, partial mastectomy, axillary lymph node dissection, and radiation therapy. In addition, in 2001, a right-sided localised ductal breast cancer in situ was identified and was treated surgically with a partial mastectomy. Secondary to her lymph node dissection, she developed lymphoedema of her left arm, which had been continuously treated with compression stockings. The patient was on treatment with an ACE inhibitor and a beta-blocker due to hypertension, and in addition, she had a known systolic murmur, characterized as physiological, as transthoracic echocardiographs in 2011 and 2017 were normal. Since her surgery in 1999, on a total of six occasions prior to her last and seventh visit, of which the first episode occurred in 2008, she had been treated for erysipelas in her left upper arm. The presentation had always been sudden with spiking fever and erythema spreading in approximately the same localisation. Interestingly, on all three out of the three occasions where a blood culture has been drawn on presentation with erysipelas, the cultures have shown growth of a bacterium belonging to the S. mitis group. These first two isolates also had similar MIC values for penicillin of 0.064 and 0.125 mg/L, for vancomycin of 0.25 and 0.5 mg/L, and for gentamicin of 2 and 2 mg/L (). In addition, they were both sensitive to clindamycin.\nOn the present visit, she once again had a sharply demarcated, warm, swollen, and painful erythema measuring approximately 7 × 15 cm in the lymphoedematous area on her left upper arm. No local portal of bacterial entry was found. Vital parameters showed a temperature of 38.0°C, respiratory rate of 16 breaths/min, O2 saturation of 96% on room air, heart rate of 80 beats/min, and blood pressure of 120/70 mmHg. On physical examination, a grade II systolic murmur was heard with punctum maximum I2 dexter. She had no signs of septic emboli, oral examination showed no signs of infection, and examination of lymph nodes was normal. Possibly due to her quick presentation, that is, less than 6 hours from the onset of symptoms, her laboratory results were normal with a white blood cell count of 8.4 ∗ 109/L, platelets of 263 ∗ 109/L, and hemoglobin of 147 g/L. Her CRP was 12 mg/L. She was clinically diagnosed with erysipelas, and due to previous bacteraemia with the S. mitis group in relation to erysipelas and the presence of a systolic murmur, blood cultures were drawn and she was treated with one dose of intravenous penicillin (3g≈5 million IU) followed by an oral penicillin (1g≈1.6 million IU) three times daily, for seven days. Once again, now for the third time, the two blood cultures showed growth of a bacterium belonging to the S. mitis group. The MIC value for penicillin was 0.125 mg/L, for vancomycin 1 mg/L, and for gentamicin 16 mg/L (). Similar to the two previous isolates, it was also sensitive to clindamycin. Her treatment was prolonged for 10 days, and a follow-up visit was arranged. Repeat blood cultures were drawn 14 days after discontinuation of antibiotics and they were negative. To prevent further infections, she has once again been referred to the lymphoedema outpatient clinic as well as to the dentist office. On follow-up, thereafter, the patient had no sequelae to her infection, and she gave informed consent for this case report to be published.\nThe three blood isolates, one analysed in 2015 and two in 2017 (15 and 8 months apart), were initially subgrouped to S. mitis/S. oralis/S. pseudopneumoniae of the S. mitis group by combining the MALDI-TOF MS results (MALDI Biotyper, Bruker) with the information that the three stains were resistant to optochin. To allow a more detailed comparison, the three stored isolates were reanalysed and now ethanol/formic acid extractions were performed on the strains, and the updated and improved Bruker MALDI Biotyper database (DB-7311 MSP Library) was used for the MALDI Biotyper analysis. In addition to the standard log (score), weighted list (scores) was also calculated []. S. mitis was the best match for both the first and second isolates when both log (score) and list (score) were calculated. For the third isolate, the best match was S. oralis for both types of scores (). Next, the mass spectra of the three isolates were inspected manually. All three strains showed the specific peak 6839.1 m/z which is associated with S mitis and S. oralis strains, but only the third isolate showed the specific peak 5822.5 m/z which is associated with S. oralis () []. In addition, no peak profiles typical for S. pneumoniae and S. pseudopneumoniae could be detected in the three isolates [, ]. These results further support that the first two isolates are S. mitis and the third isolate is S. oralis. Many differences were seen in the mass spectra of the third isolate (S. oralis) compared to the first two (S. mitis). On the other hand, no clear differences in the spectra between the first and second isolate could be seen, and one can therefore not exclude that they belong to the same clone. |
A woman aged 42 years who had no relevant history and no history of cigarette smoking visited our hospital reporting of left inguinal pain for 1 month. Ultrasound examination revealed a left inguinal tumour of 60 mm in diameter. The pathological findings from the biopsy of the left inguinal lymph node showed poorly differentiated cancer. Metastasis from a gynaecological malignancy was suspected initially, but a gynaecological malignancy was not recognised.\nChest X-ray and CT scan of the chest revealed a 70 mm tumour in the lower lobe of the right lung (A, B). The pathological findings of the transbronchial lung biopsy showed poorly differentiated adenocarcinoma with positive thyroid transcription factor-1 (TTF-1), carcinoembryonic antigen (CEA) and ALK by immunohistochemistry (IHC) (A, B). In addition, ALK positivity was revealed by the fluorescence in situ hybridisation (FISH) method, which indicated that 28% of the tumour cells showed either split red and green signals or single red signals. Afterwards, the metastasis of the left inguinal lymph node also was found positive for TTF-1 and ALK by IHC. Positron emission tomography (PET) demonstrated fluorodeoxyglucose (FDG) accumulation in the lower lobe of the right lung, the right thyroid lobe, both adrenal glands and other areas (). The highest standardised uptake value (SUV) in the right lobe of the thyroid gland was 14.0. We suspected thyroid metastasis from lung cancer and performed a thyroid ultrasound examination, which showed a hypervascular tumour of 16 mm in diameter (). We performed FNAB of the right lobe of the thyroid gland tumour to evaluate whether this was a primary tumour or metastasis (). The thyroid tumour was determined to be a metastasis from the lung adenocarcinoma because of the positive finding of EML4-ALK (variant 3a/3b was amplified) obtained using the RT-PCR method. We could not evaluate the thyroid tumour by IHC and FISH methods due to the smaller sample volume of FNAB. Multiple brain metastases in the left cerebellum were also observed with brain contrast-enhanced MRI. Therefore, we diagnosed the patient as having EML4-ALK-positive lung adenocarcinoma with a TNM classification of T3N1M1b, Stage IV. Her laboratory data showed normal thyroid function and a high CEA level (16.5 ng/mL).\nThe multiple brain metastases in the left cerebellum were treated with stereotactic radiotherapy, because multiple brain metastases were at two places and tumours of 6 mm in maximum diameter. We initiated molecularly targeted drug therapy with crizotinib (500 mg/day). We judged a partial response 3 months after the beginning of treatment with crizotinib (A, B). The patient has been obtained effect under crizotinib treatment. |
The patient was a 30-year-old primipara with a twin pregnancy at 36 weeks. She had an accompanying premature rupture of the membrane and had planned to have a cesarean section. She did not have a past medical history and her height and weight was 164 cm and 69 kg, respectively. The physical examination, blood test, urine analysis, chest x-ray, and EKG were all within normal limits (). Combined spinal-epidural anesthesia was planned because the patient wanted regional anesthesia for the twin pregnancy and there were with no special contraindications.\nPreanesthesia medication was not used. EKG monitoring, non-invasive automatic blood pressure measuring, and pulse oximetry were set up after the patient arrived at the surgical theatre. The patient's blood pressure was 130/80 mmHg; heart rate was 90 beats per minute before induction. 3 L of oxygen was provided through nasal prong while observing the patient's vital signs. She was positioned left lateral decubitus for the regional anesthesia using an 18 G modified Tuohy (Espocan®, B. Brown, Germany) needle, and epidural access between the third and fourth lumbar vertebrae was attempted. Right after the puncture, bleeding was observed through the needle, and the puncture needle was immediately removed. Compression was attempted for bleeding control. Since there was no observed bleeding or hematoma in the puncture area, a second attempt at puncture was tried between the second and third lumbar vertebrae with a 27 G subarachnoid needle inserted in an 18 G epidural needle after examining the epidural area with the loss of resistance technique. After observing spontaneous flow of CSF fluid, 5 mg of 0.5% bupivacaine dextrose was injected, and then, the subarachnoid needle was removed. The subarachnoid needle bevel was toward the cephalic, with a 20 G epidural catheter (Perifix® soft tip, Braun, Germany) located 3 cm from the cephalic. Then, the patient was repositioned to the supine position. There were no difficulties during the procedure, and she did not complain of any dysesthesia, numbness, or pain. After no blood or CSF had been aspirated, a 3 ml test amount of epinephrine (1 : 200,000) was injected through the epidural catheter with a 3-minute observation period. The patient was stable with blood pressure at 130/80 mmHg and heart rate at 95 beats per minute. 10 minutes after intraspinal injection, the level of the sensory block was at the tenth thoracic spinal nerve segment. In order to increase the level of the sensory block, after checking again that no blood had been aspirated, 5 ml of 2% lidocaine mixed with epinephrine (1 : 200,000) was injected repeatedly with a total of 20 ml being injected. After 20 minutes, the level of the sensory block did not change remaining at the tenth thoracic spinal nerve segment. Therefore, it became necessary to switch to general anesthesia. 250 mg of Thiopental and 75 mg of succinylcholine were infused. After observed loss of consciousness and muscle relaxation, intubation was done. While the surgery was proceeding, the anesthesia was maintained using 2 L/min of O2, 2 L/min of N2O, 5 vol% of desflurane, and 4 mg of vecuronium until the babies were delivered. 4 minutes after induction, the baby boy was delivered through cesarean section, and the baby girl was delivered 1 minute after the birth of the boy. The babies weighed 2.4 kg and 2.6 kg with 5-minute-apgar scores of 7 and 8. After the placenta was expelled, 20 units of oxytocin mixed with 100 ml of normal saline were infused, but uterine contraction did not successfully proceed. Bleeding of more than 3,000 ml occurred in the 5 minutes after delivery. 0.2 mg of methylergonovine was injected to advance uterine contraction, but the bleeding continued. Her hemoglobin level dropped to 8.4 mg/dl. Since massive bleeding was not expected before operation, there was no blood prepared. One hour after the operation, 2 units of packed RBCs prepared under emergency conditions were transfused. Crystalloid solution and colloid solution was infused before the transfusion with repeated injections of ephedrine to maintain systolic blood pressure above 90 mmHg. After the operation, blood pressure was maintained above 90/40 mmHg without an inotropic or vasopressor agent. Total anesthetic time was 2 hours 10 minutes; the operation time was 1 hour 20 minutes. Total bleeding during anesthesia was approximately 4,000 ml; urine amount was approximately 200 ml, and 2 units of packed RBCs were transfused. 1,000 ml crystalloid and 2,000 ml colloid solutions were infused. In the recovery room, the patient received an extra 2 units of packed RBCs and 2 units of fresh frozen plasma were transfused.\nAfter arriving at the recovery room, the level of the sensory block was below T12. She was transferred to the ward after 1 hour after partial recovery of sensory and motor function was observed and the epidural catheter was removed. Right after arriving at the ward, a blood test was done; hemoglobin was 10.1 mg/dl with prolonged PT and aPTT each measured at 21.1 sec and 52.3 sec, respectively. Vaginal bleeding continued after arriving at the ward. Intrauterine compression was attempted by foley catheter insertion and 2 units of fresh frozen plasma were transfused. 4 hours after the operation, PT and aPTT were 19.3 sec and 46.4 sec. Hemoglobin level decreased to 7.1 mg/dl and platelet count decreased to 82,000/mm3. 3 units of packed RBCs and 10 units of platelet concentrate were transfused. The vaginal bleeding continued but decreased.\nAt the ward, the patient complained of abdominal pain and back pain but was able to get some sleep by controlling pain through patient-controlled-analgesia (PCA). Next morning, 10 hours after the operation, the back pain continued, and sensory and motor paralysis on both lower extremities were observed. Emergency magnetic resonance imaging (MRI) was done and a compressive mass was found at the posterior of the lumbar vertebrae (). Acute epidural hematoma was diagnosis, and an infusion of high dose steroid therapy was carried out. The patient was transferred to the surgical theatre 4 hours after symptoms developed. An emergency decompression laminectomy was done to get rid of the epidural hematoma at the L2/L3 segment of the lumbar vertebrae. The patient's coagulation process did not work well and more than 1,000 ml of bleeding occurred with 3 units of packed RBCs being transfused. After the operation, motor and sensory function of the lower extremities did not change but the pain in the back had mildly improved. However, 1 day after the hematoma was removed, the back pain had become aggravated. Another emergency MRI was done and the hematoma was found to have occurred again, and the operation was done again. She complained of excessive numbness in her lower extremities but muscle strength gradually recovered. One month after the operation, she was discharged after she was able to walk without any working aid with some restrictions. Five months after the operation, neurological symptoms were totally improved without any sequelae. |
A 59-year-old female was admitted with a fractured neck of the right femur after a trivial fall at home. The patient had no history of any other associated injury. She was suffering from multiple comorbidities like hypertension, type II diabetes mellitus, bronchial asthma, major depression, and vertigo. She also had a history of stroke 15 years back. Preoperatively, she was on nifedipine 10 mg twice daily, acarbose 50 mg twice daily, aspirin 75 mg once daily, and atorvastatin 20 mg once daily. She was using inhalational bronchodilators on and off for asthmatic exacerbations. She was also being treated for depression and was on dosulepin 75 mg once daily and quetiapine 25 mg once daily. For vertigo, she was on betahistine 20 mg twice daily, along with flunarizine 10 mg once daily. On reviewing her medical chart, she was found to be suffering from restless leg syndrome, for which she was taking pramipexole 0.25 mg twice daily. There was no history of any drug allergies, and she denied any addiction or drug abuse. There was no previous anesthesia exposure.\nShe was scheduled for a modular bipolar hemiarthroplasty surgery. The preoperative blood investigations, chest X-ray, and electrocardiogram (ECG) were within normal limits, except for anemia with hemoglobin of 7.6 g/dl. The patient was classified as an American Society of Anesthesiologists (ASA) physical status classification III, and the surgery was planned under subarachnoid block. Preoperatively, she continued all her medications as per schedule till the morning of surgery, except the oral antidiabetic drugs. She received pantoprazole 40 mg and metoclopramide 10 mg orally two hours before surgery as per standard premedication protocol. Intraoperatively, she received a subarachnoid block with 3 ml of 0.5% hyperbaric bupivacaine. The anesthesia block produced a sensory deficit up to the T10 dermatome. There was a transient fall in blood pressure to a mean arterial pressure of 60 mmHg, which was managed with intravenous fluids and mephentermine injection (6 mg). Throughout the surgery, her vital signs remained stable. She was not administered any sedatives in the intraoperative period. She was fully conscious and oriented throughout the surgery.\nAfter an uneventful intraoperative course, when she was being transported to the post-anesthesia care unit (PACU), she suddenly started extending her neck and back, clenched her teeth, and her eyeballs rolled upward. There was paradoxical respiration implying upper airway obstruction. She remained unresponsive to verbal commands as well. Her blood pressure increased to 180/90 mmHg, her heart rate was 112 beats per minute, and her oxygen saturation was 94% on room air. The airway obstruction was managed with difficulty by applying jaw thrust and insertion of the nasopharyngeal airway. She was provided supplemental oxygen by a Bain circuit. Midazolam 2 mg was immediately administered intravenously due to a suspicion of convulsion followed by phenytoin infusion. The patient’s symptoms and signs resolved after five minutes. She became fully conscious, but she was amnesic to the previous events. Her overall vitals were stable, and she was transferred to PACU. She again had a similar episode after about 15 minutes. However, this time it was mild and involved the eyes only, and lasted for one to two minutes without any signs of airway obstruction. The second episode resolved spontaneously without any further sequelae. Based on the typical symptomatology, a diagnosis of an extrapyramidal reaction (acute dystonia) triggered by metoclopramide was established. The phenytoin infusion was stopped as epilepsy was ruled out. A normal arterial blood gas ruled out any metabolic derangement. The patient did not have any further episodes of extrapyramidal reaction in the PACU, and she was shifted to a high dependence unit after four hours of observation. Postoperatively, a neurologist reviewed the case and corroborated our diagnosis based on the history and presentation. They augmented the dose of pramipexole she was taking earlier and advised her to follow up in the neurology outpatient department after discharge. The patient remained asymptomatic during the rest of her hospital stay. |
An 88-year-old man presented to the hospital with a chief complaint of nausea and vomiting. He had a past medical history significant for recurrent esophageal adenocarcinoma status post-chemotherapy and radiation.\nFifteen months before the current presentation, following hospitalization for fatigue and melena, esophagogastroduodenoscopy (EGD) had been performed and noted mucosal changes consistent with long-segment Barrett’s esophagus associated with an esophageal nodule (). The nodule was confirmed to be an adenocarcinoma on biopsy and was staged as T3 N1 M0 through endoscopic ultrasonography. Positron emission tomography (PET) scan revealed marked fluorodeoxyglucose (FDG) uptake in the mid-distal esophagus and indeterminate mild activity in prominent mediastinal lymph nodes.\nHe received chemotherapy and radiation. Repeat endoscopy four months later demonstrated two linear esophageal ulcers and pathology reported no evidence of malignancy. Eight months later, repeat endoscopy revealed esophageal mucosal changes consistent with long-segment Barrett’s esophagus present in the distal esophagus, which was confirmed by pathology.\nThree months after the last endoscopy, at the current presentation, the patient was admitted for nausea and vomiting. At this time, endoscopy revealed a single 6 mm mucosal nodule in the lower third of the esophagus along with mucosal changes compatible with the established long-segment Barrett’s disease. Histopathology of the nodule was reported as moderately differentiated adenocarcinoma. PET scan indicated focal increased FDG metabolism in the distal esophagus which was worrisome for recurrent malignancy. Furthermore, FDG metabolism was noted at the left supraclavicular, left superior mediastinal, peritracheal, right hilar, and para esophageal nodes suspicious for metastatic disease. A moderately FDG avid nodule in the left upper abdominal quadrant was also noted. This could signal mesenteric adenopathy or non-opacified small bowel. The patient was then started on oral capecitabine treatment. He had noted constipation with his last bowel movement occurring three days prior. A computerized abdominal tomography (CT) showed concerns for small bowel obstruction and small mesenteric mass ().\nThe primary differential at this point was possible chemotherapy-induced enteritis causing ileus or obstruction. Conservative measures with bowel rest and nasogastric (NG) tube decompression failed to alleviate symptoms. The obstruction persisted, and the patient was initiated on total parenteral nutrition (TPN). Surgical consultation was subsequently obtained, and an exploratory laparotomy was performed, revealing a malignant small bowel obstruction. Small bowel resection was performed with end to end anastomosis, and pathology revealed multiple deposits of moderately differentiated adenocarcinoma consistent with esophageal metastasis (). Nausea and vomiting eventually resolved. His constipation and rectal pain worsened, and the rectal exam revealed perianal skin induration. The perianal biopsy was compatible with dermal tissues completely replaced by adenocarcinoma and foci of lymphovascular invasion similar in histology to the known primary cancer. It was, therefore, determined to be a metastatic adenocarcinoma from the known esophageal primary neoplasm (). The patient was transferred to hospice care and passed away twelve days after the diagnose of perianal skin metastasis. |
A 73-year-old Asian woman with an underlying anxiety disorder, functional headache, and hypertension was prescribed escitalopram and lorazepam when she presented with progressively worsening headaches to her primary care doctor. Her symptoms did not improve with the medications, and she was unable to eat well and required bed rest. She was transported to our hospital 4 days later after developing chest and back pain with altered consciousness. She was a housekeeper, had no allergies, and had no alcohol or tobacco smoking history. On arrival, her Glasgow Coma Scale score was 3/15 (E1V1M1); both pupils were approximately 4 mm in diameter and reactive. Her blood pressure was too low to be measured, her carotid artery pulse was palpable, her heart rate was 112 beats/minute, and her respiratory rate was 30 breaths/minute. Her conjunctiva was pale. An auscultation of breath sounds did not reveal upper and lower airway obstructions and was within normal limits. Her abdomen was soft and flat without tenderness. She had no skin abnormalities (such as rash). Both legs had no edema. Echocardiography on arrival was performed as point of care ultrasound and revealed a hypercontractile left ventricle with an eliminated left ventricular cavity and a collapsed inferior vena cava without right ventricular dilation. There was no pericardial effusion or obvious large regurgitant jet observed on color Doppler. In response, we immediately inserted a peripheral venous catheter and began introducing fluid resuscitation; however, she developed PEA. Conventional CPR according to the adult advanced cardiovascular life support guidelines (including adrenaline) was initiated and a return of spontaneous circulation (ROSC) occurred. However, her blood pressure was unstable and PEA returned, prompting repeated CPR with immediate administration of fluids and three adrenaline injections. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) was initiated for refractory PEA. Whole-body contrast-enhanced computed tomography was unremarkable, and the admission laboratory results were also unremarkable, except for anemia (Table ). Her hemoglobin level decreased from 7.1 g/dL to 3.5 g/dL 1 hour later without obvious signs of gastrointestinal hemorrhage. Therefore, 8 units of packed red blood cells were transfused for 1 day, after which her hemodynamic status stabilized. She was in a coma without sedatives; thus, targeted temperature management at 34 °C was initiated on admission to an intensive care unit. Echocardiography in the intensive care unit showed a thickened interventricular septum (which was 12.8 mm), prolonged anterior mitral valve, and contact between the bodies of the anterior and posterior mitral valves, suggesting that the left ventricular obstruction could have potentially occurred through this redundant anterior mitral valve. VA-ECMO was terminated on day 3, and after stabilizing her hemodynamics, transthoracic echocardiography showed a sigmoid septum with normal left ventricular function (ejection fraction, 75%) (Fig. ). On day 26, dobutamine-infused (30 μg/kg per minute) Doppler echocardiography revealed a significant outflow gradient (236 mmHg) accompanied with chest pain (Fig. ) and intermittent systolic anterior motion (SAM) of the mitral valve; continuous monitoring during Doppler echocardiography showed a Brockenbrough–Braunwald sign (Fig. ), which is a fall of arterial blood pressure after premature ventricular contraction; these findings confirmed a diagnosis of latent LVOTO due to a sigmoid septum. The significant LVOTO was not dependent on SAM but might have occurred due to the greatly thickened interventricular septum. As a result, carvedilol was initiated with gradual increment up to 10 mg/day on day 35. In addition, verapamil (120 mg/day) was administered on day 29. A follow-up dobutamine-infused Doppler echocardiography on day 40 showed a reduction of the outflow gradient to 14 mmHg, indicating a successful medical therapy.\nThe worsening anemia that was identified at admission was suspected to be hemolytic anemia (HA) based on results of the blood test. The results of the laboratory examination, including total bilirubin, direct bilirubin, lactate dehydrogenase, reticulocyte count, and haptoglobin, and a direct Coombs test on day 1 are presented in Table . Her drug lymphocyte stimulation test was positive, and agglutination occurred when her serum reacted with lorazepam. Therefore, lorazepam-induced immunological HA was diagnosed. Her anemia improved following discontinuation of lorazepam. Her hemoglobin level was 11.9 g/dL on day 64, and she was eventually discharged on day 68 without any sequelae. |
A male patient, aged 68 years, was referred to us in October 2004 with a corneal herpetic ulcer in his right eye. The ulcer had appeared after an influenza vaccination. The anamnesis showed that the patient was HIV-positive without signs of AIDS and with a CD4 count of 496/mm3. Furthermore he was not on Highly Active Anti-Retroviral Treatment (HAART). We treated the patient with oral acyclovir, 800 mg five times daily, and topical acyclovir ointment four times daily. After 10 days of treatment no clinical improvement was observed (Fig. ). We collected a sample of corneal cells that was found to be positive for HSV-1. The sensitivity of the isolated HSV-1 to acyclovir was tested using a dye uptake assay that measured the quantitative cytopathic effect [] and we found that HSV-1 isolates were resistant to acyclovir.\nOn the basis of the persistence of the corneal herpetic ulcer we decided to discontinue the topical and systemic acyclovir therapies and to trial topical treatment with NGF eye drops. Before treatment the patient was informed of the procedures and the aim of the therapy and he signed a written consent form. The institutional ethics committee of the S. Orsola-Malpighi Hospital also approved the study.\nTreatment consisted of murine NGF purified from sub maxillary glands and produced in the laboratories of the Institute of Neurobiology & Molecular Medicine (CNR-EBR, Roma, Italy).\nWe administered NGF eye drops (10 μg NGF dissolved in 50 μL saline solution and 0.9% sodium chloride) every two hours in the inferior conjunctival fornix until the ulcer was healed, which occurred after 23 days of treatment (Fig. ). After healing, we continued treatment for another 15 days, reducing the administration of eye drops to just 4 times daily. A year after the treatment with NGF eye drops, the patient was reviewed and there was no sign of recurrent keratitis |
A 35-year-old man presented at a local hospital with epigastric pain. Esophagogastroduodenoscopy (EGD) showed that an IVC filter strut had penetrated the third portion of the duodenum (arrow, Fig. ), and this was confirmed by computed tomography (CT) (arrow, Fig. ). In order to retrieve the IVC filter, the patient was referred to our department. He had a history of testicular cancer with para-aorta lymph node metastasis. Left renal vein thrombosis developed because of neoadjuvant chemotherapy before RPLND, and anticoagulants were administered before RPLND. Three years previously, he had undergone left orchiectomy, retro-mediastinal lymph node dissection, and RPLND at the previous hospital. The left common iliac vein was intraoperatively damaged during RPLND. Because the previous surgeon was worried about the high incidence of postoperative DVT and PTE, anticoagulant therapy was continued after RPLND. However, because DVT developed in the left common iliac vein after the initial surgery, a retrievable IVC filter (ALN, France) was placed in the IVC caudal to the renal vein to prevent PTE, and the patient had been receiving anticoagulant therapy. Because follow-up CT after IVC filter placement showed that DVT persisted at the left common iliac vein despite anticoagulant therapy, the IVC filter could not be retrieved at the previous hospital.\nEnhanced CT also revealed that DVT remained in the left common iliac vein. Because ultrasound examination showed organized DVT, an IVC filter was considered unnecessary. An endovascular approach was considered unfeasible for retrieval because two of the filter struts had penetrated the duodenal wall. An extensive discussion with an internal medicine specialist was performed. Because DVT remained with no remarkable changes in CT images for 3 years and the incidence of PTE caused by DVT in the left common iliac vein would be low, we chose surgical treatment for this patient in order to prevent bleeding at the duodenum.\nThe IVC filter was retrieved through cavotomy, and the duodenal penetration site was repaired using intraoperative EGD clipping. The operation lasted 5 h and 54 min, and the intraoperative bleeding volume was 1172 mL. Because it was not possible to mobilize the duodenum due to adhesions resulting from the previous surgery, the IVC at the sites caudal to the renal vein could not be explored. However, a mesenteric incision caudal to the third portion of the duodenum enabled encircling and taping of the IVC (Fig. ). After clamping the IVC cranial and caudal to the duodenum, a 5-cm vertical incision was made on the IVC cranial to the duodenum and the IVC filter was retrieved (Fig. a). Although the head of the IVC filter had penetrated into the IVC intima, we were able to bluntly peel the filter head from the intima. The IVC incision was closed using a continuous 5-0 Prolene suture (arrow, Fig. b). The IVC clamping time was 22 min. Intraoperative EGD revealed no bleeding at the duodenal penetration site (Fig. ). To prevent duodenal bleeding or perforation, the penetration site was repaired by EGD clipping. Retrieved IVC filter was presented as (Fig. ). The patient did not develop any postoperative complications and was discharged on postoperative day 16. |
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