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A 28-year-old Caucasian male (80 kg, 178 cm) was admitted to the emergency room with acute urinary retention and lower abdominal pain. The patient had consumed 1 l of wine and several beers within the last few hours. Otherwise, he was in a good general condition and had not complained about any urinary symptoms beforehand. The patient did not have any pre-existing conditions and his family history was negative for genitourinary disease including renal calculi. He admitted to smoking cigarettes and drinking alcohol regularly for more than 10 years. He did not take any illicit drugs or prescription medication. He had no known allergies. Physical examination revealed a distended lower abdomen. Digital rectal examination and all other physical findings did not show any abnormalities. Neurological examination was non-contributory. Ultrasound showed normal kidneys and a full bladder without any wall irregularities. The prostate was unremarkable and its size was not documented. Hence, the first working diagnosis was alcohol-induced urinary retention. Consequently, a Foley catheter was placed without any problems. It drained 1 l of clear urine. The complaints of the patient diminished gradually. Urinalysis showed normal values. A therapy trial with the nonsteroidal anti-inflammatory drug diclofenac (75 mg, twice daily) was initiated. The patient came back 2 days later for a trial without catheter (TWOC). Hereafter, he was able to urinate sufficiently. A month later, the patient suffered acute urinary retention again, this time without being under the influence of alcohol or other drugs. Physical examination and abdominal ultrasound now both revealed an enlarged prostate measuring approximately 50 ml. Laboratory tests did not show any abnormal values, including those of prostate-specific antigen (PSA) and lactate dehydrogenase (LDH). The working diagnosis was changed to urinary retention caused by benign prostatic hyperplasia. Alpha-blocker therapy with tamsulosin (0.4 mg, once daily) was started. Three days later another TWOC failed and a new Foley catheter was inserted. During the following weeks intermittent vesical tenesmus developed. Symptomatic treatment with the muscarinic antagonist trospium chloride (15 mg, twice daily) was started. Cystoscopy revealed diffusely erythematous and villous epithelium of the prostatic urethra up to the bladder neck and large obstructive lobes. An appointment was arranged for a transurethral resection of the prostate and bladder in a month’s time. In the meantime, the patient developed persistent right-sided flank pain. Urinalysis showed leukocyturia and right-sided pyelonephritis was diagnosed. Antibiotic treatment with ciprofloxacin (500 mg, twice daily) was started by his general practitioner. Two days later the patient presented with hematuria and right-sided 2nd degree hydronephrosis in the emergency room. Right-sided renal colic and hemorrhagic cystitis was suspected and the patient referred to the Department of Urology. Spasmoanalgesic therapy with metamizole (1 g, four times daily) was initiated and antibiotic treatment continued. A low-dose CT scan did not show urolithiasis but a tumor of the prostate. Cystoscopy revealed obstructive lobes of the prostate and an erythematous epithelium of the prostatic urethra and bladder neck again. The new working diagnosis was an unnoticed passing of the ureteral calculus alongside cystitis and benign prostatic hyperplasia, albeit persisting right-sided hydronephrosis. Flank pain and hematuria receded under analgesic therapy and Foley catheter placement. Transurethral biopsy of the prostate was scheduled. Until then left-sided flank pain and concomitant 2nd degree hydronephrosis developed while 2nd degree hydronephrosis on the right side persisted. Creatinine levels were elevated slightly and LDH levels were still within normal range. Cystoscopy now revealed a partly villous and solid tumor formation of the prostatic urethra which was infiltrating the trigone of the bladder including the ureteric orifices. Biopsies were taken endoscopically and ureteral stents were inserted bilaterally.\nThe histologic specimen provided evidence for a highly malignant Burkitt’s lymphoma and proved the diagnosis of benign prostatic hyperplasia wrong (Fig. ). It showed diffuse infiltration of the prostatic parenchyma by sheets of monomorphic medium-sized lymphoid cells with basophilic nucleoli. They were highly proliferative, indicated by many mitotic figures and also had a high number of apoptotic cells. Scattered among them were numerous tingible body macrophages. Due to their pale cytoplasm with incorporated apoptotic bodies they created a so called starry sky appearance, which is characteristic for Burkitt’s lymphoma. Immunohistochemistry confirmed this diagnosis and showed the typical reactivity for CD20, a co-expression for CD10 and a strong expression for Ki-67 of nearly all tumor cells. Untypical for Burkitt’s lymphoma was the relatively strong Bcl-2 expression of B-cells, which is only expressed by about 10% of Burkitt’s lymphomas []. The Ebstein-Barr virus-encoded small RNAs (EBER) in situ hybridization stain was negative.\nDiagnosis was further confirmed by fluorescence in situ hybridization that gave normal results for the BCL6 probe, but showed the typical translocation t(8;14) with probes for MYC and t (8;14) (Fig. ). This translocation induces the fusion of MYC and IGH gene loci and thereby leads to the dysregulation of the protooncogene MYC [].\nThe patient was referred to the Department of Oncology and was scheduled for staging CT scan in preparation for chemotherapy 1 week later. His general condition was progressively declining as he experienced night sweats, melena and constipation. Laboratory results showed anemia (8.2 g/dl), increased C-reactive protein (CRP) levels and normal LDH levels. Contrast-enhanced staging CT of thorax and abdomen revealed an 85 × 65 × 44 mm tumor of the prostate with seminal vesicle and bladder invasion. Also, retroperitoneal and iliac chain lymphadenopathy (up to 17 × 13 mm in size) and gastric wall thickening were present (Fig. ).\nGastroscopy ruled out upper gastrointestinal bleeding and confirmed diagnosis of gastric involvement of Burkitt’s lymphoma via biopsy. Bone marrow biopsy and lumbar puncture excluded involvement of bone marrow and liquor concluding a stage IV Burkitt’s lymphoma according to the Lugano staging system []. Before chemotherapy was initiated, the patient performed sperm cryopreservation.\nUltimately, the diagnosis of Burkitt’s lymphoma was made 4 months after initial presentation and only 6 days later systemic chemotherapy according to the GMALL (German Multicenter Study Group for Adult Acute Lymphoblastic Leukemia) B-ALL/NHL 2002 protocol was started. It consisted of a total of 6 cycles of 3 different drug regimen including dexamethasone, cyclophosphamide, rituximab, dexamethasone, vincristine, ifosfamide, etoposide, cytarabine and high-dose methotrexate. Concurrent medication was composed of acyclovir, trimethoprim/sulfamethoxazole, ciprofloxacin, pantoprazole, G-CSF, amphotericin B mouth rinse and Glandomed® mouthrinse. The first cycle was given as a milder regimen with dexamethasone and cyclophosphamide together with hydration, allopurinol and urinary alkalization in order to prevent tumor lysis syndrome. Nonetheless, the patient developed neutropenic fever and grade 4 mucositis and required extensive analgesic and antibiotic treatment. Due to increasing abdominal pain a CT scan was done which excluded a tumor lysis with gastric perforation. It rather showed a response to treatment according to Lugano treatment response criteria (Fig. ) [].\nDuring the following cycles there was one more episode of neutropenic fever. Mucositis persisted only on a lower level. The Foley catheter was removed 2 months after initiation of chemotherapy. Hereafter satisfactory voiding with insignificant post-void residual volume was established. After finishing chemotherapy, a CT scan showed complete response. Following this, both ureteral stents were removed. Subsequent sonography of the kidneys could exclude persisting hydronephrosis. Another CT scan 3 months later confirmed complete remission of Burkitt’s lymphoma (Fig. c). The patient recovered completely and is now participating in follow-up care. |
The patient was a 55-year-old male, a parent of one of our youth outreach patients. He reported being First Nations, living in a single-room-occupancy hotel, and supported by income assistance. He had a long history of opioid and stimulant use disorders. Given the incredible urgency and need for flexibly service delivery in the context of OAT, he was taken as a patient in our youth outreach program to help family members in innovative ways. His presentation was complicated by an evolving left leg cellulitis, untreated hepatitis C, and a history of gout. At the time of initial assessment, he was not taking any medications. Our team was consulted to see him for buprenorphine/naloxone microdosing induction in his residence. Visiting the patient in his residence was used as a measure to lower the threshold for access to care and improve his engagement with the treatment.\nOn initial assessment, the patient self-reported injecting 200 mg of heroin daily 200 mg of crystal methamphetamine every 3 days. The actual amounts used was difficult to measure given the variability in chemical make-up and potency of heroin and other street drugs in Vancouver, including their adulteration with fentanyl and other contaminants [, ].\nThe patient had previously trialed methadone but had relapsed. He had multiple trials of traditional buprenorphine/naloxone inductions but was unable to complete them due to his intolerance of withdrawal symptoms. He had experienced at least one overdose requiring resuscitation with naloxone.\nOutreach visits to this patient began February 28, 2018 and he was seen four times (out of five attempted visits) over 3 weeks at his residence. The patient was prescribed a buprenorphine/naloxone microdosing regimen (see Fig. ). The BUP/NLX tablets were split by the local pharmacy for off-label administration of small doses in microdosing protocol. Since the smallest available dose in Canada is 2 mg, it was more practical to split the tablets into 0.25 mg as opposed to 0.2 mg, which was prescribed in Bernese Method. The patient was instructed to use decreasing doses of heroin as buprenorphine/naloxone doses increased, then to stop heroin once the buprenorphine dose reached 12 mg. He completed the microdosing regimen and over the course of 7 days his dose was titrated to 12 mg daily. On day 8 the dose was increased to 16 mg daily and the patient abstained from illicit drugs. Despite the team offering to deliver the medication to the patient’s home, he chose to pick them up himself daily from the pharmacy. The outreach team supported him with reminders to pick up his medications regularly. Due to a prescription error, he missed 3 days of his buprenorphine/naloxone (March 20–23, 2018), and a relapse of heroin and crystal methamphetamine ensued. A subsequent retrial of buprenorphine/naloxone microdosing induction was prescribed, but the patient did not pick up his medications, and was difficult to find for follow up.\nThe patient was seen again on outreach visits to his residence and neighbourhood starting on May 14, 2018. He was contemplative about quitting heroin. He wanted to retrial the microdosing induction method. We conducted a medical history, screening for major health concerns, liver failure, other medications and allergies. Blood work from December 2017 showed normal complete blood count, electrolytes, blood urea nitrogen, and creatinine. December 2016 liver function tests were normal. A microdosing buprenorphine/naloxone regimen was re-prescribed, along with a blood requisition for updated liver function tests, human immunodeficiency virus, treponema pallidum, hepatitis B virus, complete blood count, electrolytes, blood urea nitrogen, and creatinine. Medication was dispensed daily to the patient at a pharmacy situated across the street from his residence. The prescriber and the pharmacy stayed in close phone and in-person connection regarding dosage adjustments and medication adherence.\nThe patient picked up and took all but two doses in the first week.\nThere were no symptoms of withdrawal throughout the induction. This was documented via the patients self-report and the clinicians’ overall assessment of the patient’s withdrawal symptoms as the use of standardized tools was neither feasible nor necessary given that withdrawal prior to induction is not a requirement in microdosing technique. The patient reduced illicit heroin use to 200 mg every 2 days during the week of the microdosing. The patient had mild cravings when he reached 12 mg, though decreased his use to 100 mg every three days. Follow up was challenging because the patient had difficulty keeping appointments, and we were not always able to locate him on outreach. The team looked for the patient in his home and in the neighborhood, and after several attempts assessed him on May 28, 2018. At the time, he was on 12 mg daily. He continued to self-report using heroin 500 mg every 3 days and experiencing cravings, so his dose was increased to 16 mg. He continued to stay on buprenorphine/naloxone 16 mg daily, with no further missed doses.\nHe was assessed again on June 11, 2018. Notably, a painful left leg cellulitis persisted, and he continued to use heroin 500 mg every 3 days when he felt leg pain. While he reported mild cravings, he reported using heroin to manage pain. The team connected him to a nearby primary care clinic for wound care and antibiotics. The dose of buprenorphine/naloxone was increased to 20 mg daily. He adhered to his agonist medication till August 27, 2018 and reported using 50 mg approximately every 4 days, which he was willing to taper. Interestingly, he reported no longer using any other illicit substances. The outreach team supported him to connect with an adult-oriented primary care and OAT clinic, and his care was transferred accordingly. |
A 27-year-old woman at 37 weeks and two days of gestation got admitted to the labor room with pain abdomen and bleeding per vaginum. She was a second gravida with no living issue. Before one and a half years back, she had a preterm twin vaginal delivery at 36 weeks of gestation with early neonatal death of both babies.\nIn the current pregnancy, a prior obstetric ultrasound done at 20 weeks showed a myoma measuring 11.5 cm x 8.5 cm in the cervical region and the placenta occupying the right lateral position of the uterus. The patient was not under regular antenatal check-ups. She had no other associated comorbidities.\nOn general examination, she was pale, but her vitals were stable. Obstetrical examination revealed a uterus of term size with mild contractions, and there was moderate bleeding per vaginum. An ultrasound examination was done, which showed a central placenta previa and a central cervical fibroid of size measuring 10 cm x 8 cm (Figures , ). The fetus was in cephalic presentation with an estimated weight of 3.1 kg. The amniotic fluid was of adequate amount. The blood investigations showed anemia with hemoglobin of 7.8 gm%, and liver enzymes were deranged suggestive of obstetric cholestasis. The rest of the blood investigations were within normal limits.\nShe was planned for an emergency cesarean section because of central placenta praevia with antepartum hemorrhage. Blood components were cross-matched before surgery. Given the possible need for a cesarean hysterectomy in case of uncontrolled intraoperative bleeding, informed consent was obtained.\nDuring the surgery, the abdomen was opened by Pfannenstiel incision. Then uterine incision was given carefully in the lower segment above the fibroid after separating the uterovesical fold of the peritoneum. A term live baby was delivered by cutting through the placenta. The baby cried immediately at birth with an APGAR (Appearance, Pulse, Grimace, Activity, and Respiration) score of 9/10. The placenta and the membranes were separated easily. A central cervical fibroid measuring approximately 10 cm x 8 cm was observed that was extending posterolaterally. After placental separation, profuse bleeding from the placental bed was noticed, for which hemostatic sutures were applied, and bilateral uterine arteries were ligated. In addition to that uterine tamponade was done using two Foley catheter bulbs of volume 50 mL each. Uterotonics were given, and bleeding was controlled. The uterus was closed in two layers. A pelvic drain was put, and the abdomen was closed in layers. Intraoperatively, two packed RBCs were transfused, and postoperatively the patient was managed in the high dependency unit. The rest of the postoperative course was uneventful. The patient was discharged on the fifth postoperative day with advice for follow-up of the cervical fibroid in the postpartum period after six weeks. |
We describe a case of a 36-year-old black African woman with two previous live births by cesarean section and two previous miscarriages who was referred in her fifth pregnancy after 6 weeks of amenorrhea. Her serum quantitative β-human chorionic gonadotropin (bHCG) was 16,124 mIU/ml. However, an intrauterine or extrauterine pregnancy could not be located on a transabdominal ultrasound scan.\nA copper intrauterine contraceptive device had been removed 2 months prior to her last menstrual period. She reported using one cycle of clomiphene 50 mg with the hope of achieving a twin pregnancy.\nShe had delivered twice by cesarean section for failure to progress. Her last two pregnancies had been first-trimester miscarriages; one was managed expectantly, and the other was surgically evacuated, though the actual procedure was unknown to the patient. She did not have any chronic medical illness and was not receiving any medication prior to this presentation. She stayed in a city suburb that was well serviced. She was a school principal in her second marriage with no children in the current relationship. She did not smoke or drink alcohol.\nOn examination, she had a normal blood pressure of 113/70 mmHg and a pulse rate of 98 beats/min. Her body temperature was 37.5 °C. On examination, her cardiorespiratory and neurological systems were normal. Her abdomen was soft and not tender. The result of her pelvic examination was normal. TVUS showed a gestational sac of 13 mm with irregular margins and a visible yolk sac located on the anterior isthmic portion of the uterus, raising suspicion of a cesarean section scar ectopic pregnancy. She declined a Doppler ultrasound evaluation scheduled for the next day. She was scheduled to have serial bHCG evaluations every 48 h.\nA repeat serum quantitative bHCG done 48 h after the initial test revealed a level of 21,521 mIU/ml, a 33% rise. She defaulted follow-up until 1 week later, when she presented with pelvic pain of increasing intensity for 5 days. An urgent transvaginal scan was performed. A fetal pole with active cardiac activity (crown-rump length 0.9 cm) in a gestational sac was located in the anterior low myometrium. The sac traversed the full width of the anterior myometrium, with the posterior margin of the sac abutting the anterior margin of endometrium and the anterior margin of the sac extending to a subserosal location in a fairly exophytic fashion. There was evidence of trophoblastic circulation on Doppler examination. There was no endometrial fluid or free pelvic fluid (Fig. ).\nShe was immediately admitted for a diagnostic laparoscopy/hysteroscopy and possible excision of the scar pregnancy if confirmed. A preoperative complete blood count showed hemoglobin 13.4 g/dl, white blood cells 7.2 × 103/μl, and platelet count 243 × 103/μl. The patient’s kidney function was normal with sodium 135 mmol/L, potassium 4.9 mmol/L, urea 3.5 mmol/L, and creatinine 65 μmol/L. The patient’s liver function test results were also normal. She had a negative result in a blood test for human immunodeficiency virus. Urinalysis did not show abnormalities. The patient’s random blood sugar was 5.6 mmol at admission. At laparoscopy, the bladder was adherent high on the anterior uterine wall, and the ectopic pregnancy was not visualized (Fig. ). At hysteroscopy, there were extensive adhesions within the lower endometrial cavity, which obscured visibility. There was no active intracavitary bleeding ruling out a threatened or inevitable miscarriage. We could not visualize any obvious bulge in the cervical canal suggestive of a cervical ectopic pregnancy. Because of the uncertainty of the location of the pregnancy due to adhesions, excision was postponed. Postoperatively, the patient became unstable with low blood pressure, systolic pressure range of 82 to 95 mmHg and diastolic pressure range of 40 to 55 mmHg, and a pulse rate range of 64 to 73 beats/min, but without active vaginal bleeding or use of medications inducing persistent hypotension. Anesthesia had been induced with etomidate 16 mg and suxamethonium 100 mg, and maintenance was initiated with isoflurane 0.8–1.5%. Intra- and perioperative analgesia was induced with fentanyl 200 mg intravenously (IV), indomethacin 100 mg rectally, and paracetamol 1 g IV. Antibiotic prophylaxis was with ceftriaxone 1 g IV and metronidazole 500 mg IV. This prompted us to order an urgent MRI scan to map the location of the pregnancy in the immediate postoperative period. MRI confirmed the TVUS findings of a cesarean section ectopic scar extending to the serosa (Fig. ).\nAn emergency laparotomy was then performed on the same day. The abdomen was entered through a Pfannenstiel incision along the old skin scar. A transverse incision was made in the upper uterine segment just above the adherent bladder. The products of conception were removed with forceps, and the gap in the anterior myometrium at the old scar was seen and felt. There was massive bleeding from the implantation site. Twenty milliliters of vasopressin (20 U diluted in 100 ml) in normal saline was administered into the bleeding myometrium edges. The edges were apposed in layers with VICRYL suture (Ethicon, Somerville, NJ, USA) to repair the defect. Estimated blood loss was 2000 ml. The patient was transfused with 1 U of packed cells intraoperatively. She was continued on the same intravenous antibiotics and analgesia that had been commenced after the laparoscopy. Her hemoglobin count on day 1 postoperatively was 8 g/dl, and she declined any further transfusion. Oral iron and folic acid supplementation was commenced.\nThe patient’s postoperative recovery was uneventful, and she was discharged on day 4 after surgery. Histology confirmed the presence of decidua and chorionic villi. The patient wanted a child because she was in a new relationship, but she was no longer sure of her future fertility plans after the ectopic pregnancy. A levonorgestrel implant was inserted 2 weeks postoperatively. The patient last attended physical review at 6 weeks, and she was well with no problems related to the surgery at a telephone review at 3 months postoperatively. |
A four-year-old boy was scheduled for cleft palate repair, as the third patient on the list. After managing two adult patients earlier, the corrugated tubing of the circle breathing system was changed to the pediatric type and tested for leaks. Anesthesia was induced with sevoflurane using JRMATP. After tracheal intubation, the circle breathing system was used with pressure-controlled ventilation (PCV). After a few breaths, FiCO2 increased to 9 mmHg []. Soda lime was only partly discolored. The flow rate setting in the ventilator (i.e., the flow rate used to achieve the set inspiratory pressure, an equivalent of Pramp or ‘rise time’) was increased and FiCO2 became zero. With manual ventilation, FiCO2 increased whenever the tidal volume was delivered slowly and decreased with rapid delivery of tidal volume. We noted that the leaflet of the expiratory UDV was deformed and part of it remained open during inspiration []. The valve was closing fully with rapid delivery of tidal volume (or higher flow rate). The flow waveform on the ventilator showed minimal flow during inspiration []. The flow sensor was not direction sensitive, and was showing both the normal expiratory flow and the abnormal reverse flow on the same side of the Y-axis (the ventilator was not equipped with any alarm to indicate reverse flow). Later, the expiratory UDV was tested and found to be incompetent. The machine was used in subsequent patients after replacing the defective valve leaflet. The problem recurred after a few days and the new leaflet was also found to be deformed.\nOn checking all anesthesia machines with similar UDVs, the inspiratory UDVs were found to be deformed and incompetent in some machines, although no problem was reported during clinical use. The matter was reported to the manufacturer and they concluded that the problem was caused by the thinness of the valve leaflet (0.3 mm). All the leaflets in UDVs were replaced with thicker ones (0.5 mm). No similar problems have been reported since then. |
A 9-year-old female pupil referred from a primary health-care center on account of back pain and swelling of 9 days duration. The patient was beaten with a broom on the back by her older female cousin as a punishment for an offense. The broom purportedly swiped a metallic object (needle) from the hand of another person standing nearby and drove it into the patient's back. This was suspected as the metal was nowhere to be found and patient began complaining of excruciating pain at the affected site. Attempts were made to remove the needle after a chest radiograph showed the needle in the subcutaneous region. She was subsequently referred to the hospital after attempts made at removing the needle proved abortive.\nThe pain was exacerbated by lying on the affected side. There was no history of cough, hemoptysis, and fever or dyspnea at rest or during exertion; there was no bleeding at the site of the trauma.\nOn examination, she was not pale, anicteric, cyanosed, and no respiratory distress. Scarification marks were however noted over the body, more pronounced in the trunk. Normal weight and height for age were seen.\nEssential findings were in the musculoskeletal system. There was a thin plaster over the right paravertebral region. Mild swelling was seen at the site, with an incision marks over the swelling. The site was mildly tender with no differential warmth or pointing sign.\nChest radiograph revealed a sharp linear metallic density structure (the needle) in the chest at the level of the 9th posterior rib extending from the subcutaneous tissues into the adjacent lung [Figure and ].\nSoft-tissue ultrasound of the affected region was essentially normal. No foreign body was seen within the soft tissues.\nChest computed tomography also demonstrated the linear metallic density structure in the right chest within the lung fields, with no hydropneumothorax or reactive pneumonia. Mild pleural thickening was however noted at the site. The needle had been displaced further into the lung fields [].\nSince the patient was asymptomatic at the time of presentation (no pneumothorax or pleural effusion), the surgeons hence decided to practice “watchful waiting.” Both parents were advised of the need for video-assisted thoracoscopic surgery. She is presently being followed up at the clinic. |
A 37-year old female patient was admitted to the emergency room 24 h after binge eating. She had no relevant medical history but described a loss of 10 kg in 1997. At that time, she was 62kg and wanted to lose weight. She started vomiting. She also reported a stressful relationship with her partner at this period. BN remained undiagnosed during 15 years.\nshows the timeline of the most relevant events of this clinical case report, and the evolution of weight and body mass index (BMI).\nIn the emergency room, in July 2012, the patient reported chronic abdominal pain. Physical examination at admission revealed hemodynamic stability, sepsis syndrome, and distended abdomen with defense. Laboratory test results showed high lipasemia and acute renal failure. Abdominal Computed Tomography (CT) imaging showed major gastric distension reaching the pelvis and compressing the digestive organs () with no signs of pneumoperitoneum. Conservative gastric decompression was started first using a nasogastric tube aspiration that discharged almost 6.5 litres. Twenty-four hours after hospitalization, the patient exhibited tachycardia and hypotension. Increased doses of Noradrenaline were prescribed unsuccessfully. The patient was prepared for urgent laparotomy showing a large gastric distention associated with necrosis. Total gastrectomy and jejunostomy were performed. The patient was discharged 35 days after the surgical intervention. She was referred to the Department of Clinical Nutrition (Rouen, France) for the nutrition rehabilitation. She weighed 40 kg at this time. Enteral nutrition was administrated through the jejunostomy (1500 kcal/day) and well tolerated. Enteral nutrition was progressively relayed with oral nutritional supplements associated with oral food intakes. The patient reported reduction in bulimic symptoms, structured meals () and weight stabilisation around 48 kg (body mass index=17.6kg/m²). At the time of first referral in our institution, the patient did not declare any fear for gaining weight or any body shape concern. She was happy with gaining weight thanks to jejunal tube feeding. On following consultations, eating disorders were routinely screened with by the self-administered French version of the SCOFF questionnaire (SCOFF-F) (). This validated test is routinely used, composed of five dichotomous questions. One point is given for each “yes” answer. At least two positive answers indicate a positive SCOFF score with a sensitivity of 88.2% and a specificity of 92.5%. This screening consistently resulted in 5 negative answers. One year after total gastrectomy, jejunostomy was finally removed and the patient returned to work. She was admitted to the Digestive surgery department for occlusive syndrome, 5 years after gastrectomy. Laparoscopic viscerolysis has been done, with no complications. The patient has been discharged and maintained sufficient oral food intake. Today, she is still followed-up in the Nutrition unit at least once a year. She still has a negative SCOFF-F score, with abstinence of binge eating and compensatory behaviors (self-induced vomiting, laxative use, diuretics, compensatory exercise, fasting), structured meals and biological markers in normal range (). |
A 71-year-old female presented to an outside hospital with a 1-month history of progressive lower left extremity weakness and a left foot drop with resulting difficult ambulating. Over the days prior to admission, she also developed difficulty in holding objects with her left hand. A cranial computed tomography (CT) showed an approximately 3.5 cm nodular right-sided thalamic lesion with heterogeneous enhancement, a central hypodensity, and mild midline shift [].\nShe was transferred to our tertiary care center for further evaluation. On admission, the patient showed persistent left hemiparesis. Based on the initial CT results, the differential diagnosis of the lesion included primary brain neoplasms, metastatic brain disease, lymphoma, and less likely abscess or inflammatory or demyelinating disease. Magnetic resonance imaging (MRI) with contrast was obtained, again demonstrating a poorly demarcated mass in the right thalamic area with plentiful irregular blood products. The lesion showed avid heterogeneous contrast enhancement with highly prominent vasculature consisting of multiple feeders and enlarged venous channels [Figure –]. This added to the differential diagnosis entities such as an AVM in combination with a malignant glioma, a hypervascular glioma, or atypical AVM, respectively.\nTo further assess the vasculature, a computed tomography angiography (CTA) was obtained. Here, the mass was characterized by numerous dense calcifications, as well as by intrinsic hemorrhage posteriorly []. Other significant findings included marked dilatation of clustered vessels along the medial, posterior, and superior margins of this lesion [Figure and ]. No definite aneurysms were detected but could not be ruled out with certainty. The working diagnosis at this point included a large and partially thrombosed AVM, resulting in high flow enlarged draining veins and hemorrhage versus an aggressive glioma with neovascularization []. A cerebral digital subtraction angiography (DSA) confirmed extensive hypervascularity and was interpreted as displaying AVM-typical morphology [Figure and ].\nMR-perfusion was performed and showed high perfusion and markedly increased blood volume within the right thalamic lesion maintaining the concern for an AVM, whereas MR multivoxel spectroscopy indicated an increase in creatinine/choline peak ratio [], substantiating the working diagnosis of an underlying glioma.\nBecause the prognosis and treatment algorithms for an AVM and a malignant glioma differ substantially, a definite tissue-based diagnosis was required, however, it was only hesitantly considered given the high risk of procedural bleeding. A stereotactic biopsy was finally performed under general anesthesia using a CRW frame (Radionics Inc., Burlington, MA). Preoperative T1 gadolinium MR as well as CTA-images were fused to the intraoperative frame-based CT using the StereoCalc® and NeuroSight® Arc software (Radionics Inc., Burlington MA) allowing three-dimensional planning of coordinates. Meticulous attention was paid to the delineation of a suitable target area for the biopsy at the inferior margin of the hypervascular zone but avoiding any prominent vessels [Figure and ].\nMultiple serial stereotactic biopsy specimen were ascertained for histopathological analysis using a set of unique microforceps with a cubic bite size of 1 mm3 (Medical High Tech Inc., Emmendingen, Germany), as described by Warnke et al.[] A total of 7 specimens were obtained in 1 mm steps (−3 mm to +3 mm from the target point) without hemorrhagic complications.\nIntraoperative fresh frozen analysis showed malignant glioma. Final histopathological analysis revealed a densely cellular, astrocytic glial fibrillary acidic protein positive tumor with necrosis, thrombosed vessels, and scattered mitosis among irregular hyperchromatic nuclei. The proliferative index (MIB-1) was above 10%. Thus, final histopathologic diagnosis confirmed GBM with profound hypervascularity.\nAfter an uneventful biopsy and discharge home, subsequent treatment consisted of involved field radiation and standard chemotherapy with Temozolomide, as per Stupp et al.[] Given the poor prognosis of GBM at this age, no additional treatment was recommended for the vascular malformation possibly associated with this lesion since this was considered not to impact her remaining lifetime. As expected, our patient did well for several months before the tumor progressed at which point she decided not to pursue further treatment. She expired 6 months after her initial presentation. |
A 31-year-old female patient with a height of 164 cm and weight of 60 kg was admitted to undergo ethanol sclerotherapy for a large painful vascular malformation in her left leg. Magnetic resonance imaging revealed an AVM measuring 28 × 7 × 4 cm in her left calf (). All preoperative examinations, including laboratory tests, electrocardiography, and chest X-ray, were normal. Vital signs before general anesthesia were stable: her blood pressure was 130/80 mmHg and her heart rate was 82 beats/min. Following the application of routine hemodynamic monitoring (3-lead electrocardiogram, noninvasive blood pressure, and pulse oximetry), anesthesia was induced using a bolus intravenous injection of 120 mg propofol and 40 mg rocuronium. A 20-gauge catheter was inserted into the radial artery to monitor her arterial blood pressure continuously. The target controlled infusion of 2% propofol and remifentanil (2-3 µg/ml and 2-5 ng/ml of the effect-site concentrations, respectively) was applied to maintain anesthesia. Immediately following the injection of ethanol, the patient's blood pressure and heart rate rose slightly to 160/100 mmHg and 100 beats/min, respectively. Thereafter, the patient's blood pressure and heart rate were stable throughout the procedure while she was in the prone position. A total dose of 50 ml of 99% ethanol was injected into the lesion over a period of 30 minutes without the use of a tourniquet. As soon as the patient was turned from prone to supine after finishing the procedure, an abrupt drop in her arterial blood pressure occurred, 40-50 mmHg systolic and 10-15 mmHg diastolic blood pressure with a simultaneous increase in her heart rate to 100-110 beats/min. To restore blood pressure, 20 mg of ephedrine was immediately injected, but the patient's heart rate rather decreased abruptly to < 30 beats/min. Chest compressions were performed along with the simultaneous injection of 50 µg of epinephrine. A few minutes later, the patient's blood pressure and heart rate recovered to 180/100 mmHg and 100-110 beats/min. At that moment, the arterial blood gas analysis showed metabolic acidosis with otherwise normal findings (pH 7.29, PaCO2 28 mmHg, PaO2 466 mmHg, SaO2 100%, base excess -11.6, Hematocrit 38%, Na+ 137 mEq/l, K+ 3.7 mEq/l, and Ca2+ 1.11 mmol/l). The patient was transferred to the intensive care unit with the endotracheal tube in place. Transthoracic echocardiograph (TTE) and embolism computed tomography (CT) were performed to confirm pulmonary hypertension or pulmonary embolism. There was no evidence of pulmonary embolism on CT. In addition, the peak tricuspid regurgitation jet velocity on TTE was 2.3 m/s, indicating no evidence of pulmonary hypertension at that moment (). However, TTE revealed dilated right ventricle () and severely decreased right ventricular function.\nOn postoperative day 1, the patient regained her consciousness and was discharged five days after the operation without any complications. |
A 35-year-old female living in a rural area came to our department with more than 3 years history of chronic diarrhea, rickets, high serum value of immunoglobulin A protein, and anemia. She had been diagnosed with suspected celiac disease and chronic bacterial intestinal infection in other hospitals and then been treated with gluten-free diet for more than 6 months, some antibiotics such as tetracycline, metronidazole, and ampicillin for more than 1 month, but with no effect. On physical examination, the patient was pale and nervous, and the body mass index (BMI) was about 15.6 kg/m2.\nInitial laboratory investigations manifested that she was suffering with severe electrolytic disorder, anemia, but with normal liver and renal functions, no allergic food ingredient, no pathogenic bacteria in stool cultures and parasitological examination of stools, negative human immunodeficiency virus (HIV) and autoimmune antibodies, and light chains including kappa and lambda levels in serum and urine were normal. The Helicobacter pylori (HP) hydrogen breath test was negative. But her immunoglobulin A titer in serum was about 22,900 mg/L (the normal value is below 3820 mg/L in our hospital). Because of her high level of immunoglobulin A protein, we completed the bone marrow aspiration and found no evidences to diagnose the patient with lymphoma, plasmoma, multiple myeloma, and so on. The percentage of mature and immature plasma cells in the smear of bone marrow were about 3% and 2.5% (normal ratio) and a lot of megacaryocytes with many small round nuclei were found simultaneously (Fig. ) which suggested that the patient was in a sideropenic and megalobastic anemia stage. Protein electrophoresis and immunofixation electrophoresis showed that there were no definitive monoclonal immunoglobulin protein bands in her serum.\nGastrointestinal endoscopy was completed then. Duodenal and ileac biopsies revealed atrophy and blunting villi (Fig. ). The bowel lamina propria was infiltrated with slightly increased intraepithelial lymphocytes and mainly with diffuse plasma cells (Fig. A). The following enzyme labeling immunohistochemistry results were strongly positive to alpha-heavy-chain (Fig. B), but negative to lambda and kappa light chain. Computed tomography (CT) manifested she had no hepatosplenomegaly and no lymphadenopathy but had diffuse thickening of small intestine wall (Fig. ).\nWe diagnosed this patient with an immunoproliferative small intestinal disease based on upper investigation. She was started on vitamin D supplements, calcium, magnesium, potassium, iron, folic acid, and mecobalamin replacements. At the same time, we treated her with microflora probiotics additionally. One month later, her frequency of water diarrhea alleviated slightly, but her weight loss, anxiety neurosis, and other disorders were still severe. She refused to be treated with anthracycline chemotherapy regimens, and agreed to be treated with prednisone (40 mg per day, and gradually reduced to the lowest dose). Another 1 month later, she was feeling better, with solid stool (once or twice per day) and had obvious weight restoration (BMI 19.5 kg/m2). The laboratory investigation manifested that the value of immunoglobulin A protein titer in serum decreased to 16,700 mg/L. She would be in a closely outpatient follow-up procedure in our department. |
A 20-year-old girl was brought to the emergency department with a history of sudden onset convulsions. There was no history of fever, drug usage, trauma, or any past history of convulsions before the onset of convulsions. The patient was given a single dose of intravenous (IV) diazepam 10 mg for control of generalized tonic-clonic convulsion but since control over the convulsions was achieved, a loading dose of phenytoin 1 g was given. As the convulsions persisted, propofol 50 mg IV was given, which controlled the convulsions. Computed tomography (CT) scan head revealed no abnormality. Electroencephalograpy could not be done due to lack of facilities. Blood sugar, electrolytes, and arterial blood gas analysis showed no deviation from normal values. Noninvasive blood pressure, electrocardiography, and oxygen saturation monitoring was done. The patient was then shifted to the intensive care unit (ICU) and IV infusion of midazolam 0.1 mg/kg/h was initiated and phenytoin 100 mg given 8 hourly. There was no episode of convulsions thereafter. The patient developed hypotension for which inotropic support was started. A central venous cannulation was done to guide the fluid therapy and titrate the dose of inotrope. Breathing was normal and there was no need to mechanically ventilate the patient.\nOn arrival in the ICU, the relatives accompanying gave some history of domestic dispute and doubt of ingestion of some substance. A Ryle's tube was inserted and gastric lavage given. The patient had complaint of nausea, vomiting, and abdominal discomfort for which symptomatic treatment was given. The patient's sensorium improved the next day and she gave the history of ingestion of contents of two bottles of mosquito repellent available in the house commercially marketed as All-Out (prallethrin 1.6% w/w liquid, 35 mL in each bottle, that is, total dose of 1120 mg). The patient had excessive secretions so atropine 0.6 mg IV was started at 4 hourly intervals, in addition to antiemetics and proton-pump inhibitors. The condition of the patient improved gradually and by 5th day the patient was shifted to the ward from where she was discharged on the 7th day. |
History and physical
A 67-year-old male former smoker with a history of prior occupational asbestos exposure and recurrent bronchitis presented with progressive dyspnea and thoracic pain to the point that he could not lie down in bed. A computed tomography (CT) scan of the chest was performed, which was interpreted as right-sided pneumonia with right parapneumonic effusion. He was sent to his local emergency department, where he was admitted for antibiotics and thoracentesis, the latter which demonstrated the presence of atypical mesothelial cells with inflammatory cells. He was readmitted two weeks later for progressive thoracic pain, was found to have a recurrent right-sided pleural effusion, and was managed with partial right pleurectomy with pleural biopsy, and talc pleurodesis. Right pleural pathology demonstrated atypical mesothelial proliferation at the pleural surface, without true invasion or definitive pathologic evidence of malignancy. Following surgery, he felt substantially better, such that he could sleep in the bed again, and he was able to return to his baseline activity levels. He underwent repeat chest CT five months later, which showed right pleural thickening and a small loculated pleural effusion, favored to represent a combination of calcification, pleurodesis, and atelectasis. He remained clinically well for another five months until he presented with cough and sinus congestion unrelieved by guaifenesin, dextromethorphan, and antibiotics. He underwent repeat chest CT that showed extensive mass-like pleural thickening completely encasing the right lung, with prominent involvement of the mediastinal pleura, and probable mediastinal extension into the right paratracheal and precarinal space, with pericardial effusion and probable pericardial metastases. There was no definite invasion into the right chest wall and no evidence of disease outside of the thorax.
He then established care at our institution’s mesothelioma and pleural disease multi-disciplinary program. Pathology review of the previously biopsied pleural tumor revealed that the pleural tumor cells were positive for Wilms' tumor-1 and calretinin, and negative |
A 32-year-old, hearing and speech impaired third gravida, presented at 35 weeks of gestation, for lower segment cesarean section (CS), with non-progress of labor. We elicited her history by communicating with the patient's relatives, who in turn used sign language to communicate with her. Diagnosed with subaortic ventricular septal defect with a left-to-right shunt at 10 years of age, she had a spontaneous abortion 5 years ago and an emergency CS under general anesthesia for fetal distress 3 years ago. There was no history of cardiac failure, thrombotic events, or ES. She remained asymptomatic for the first two trimesters of this pregnancy but now complained of dyspnoea on exertion grade III. On examination, she had grade II clubbing, pulse rate was 110 beats per minute, regular; blood pressure was 110/54 mm of Hg (right arm in lateral position), and her room air saturation was 94%. There was a loud P2 with pansystolic murmur over the left sternal border associated with thrill. She showed no signs of cardiac failure. Her electrocardiogram showed right ventricular hypertrophy. Echocardiography showed a large (24 mm), nonrestrictive, malaligned inlet VSD with bidirectional shunt, severe pulmonary stenosis (peak pressure 76 mm of Hg) with an ejection fraction of 65%. Her laboratory investigations were normal, with a hemoglobin of 11 g%. Her preoperative arterial blood gas showed a pH of 7.34, PaO2 60 mm of Hg, PaCO2 34 mm of Hg, and SaO2 92%.\nWith the use of sign language interpretation and pictures, we explained to both, our patient and her relatives, the prognosis and risks involved. The patient was offered a choice of anesthesia after explaining the risks and benefits of regional and general anesthesia. She chose to undergo epidural anesthesia. At the start of the procedure, our patient was anxious but cooperative. Hudson's mask was used to supplement oxygen at 6 L/min. Intravenous access was established with wide-bore peripheral cannulae on either hand and 14G peripherally inserted central catheter, in the right cubital vein. 500 ml Ringer's lactate was administered, slowly through the duration of the surgery. Care was taken to avoid accidental intravenous injection of air bubbles and prevent paradoxical air embolism. After establishing electrocardiography, noninvasive blood pressure monitoring, and pulsoximetry, a 16 G epidural catheter was inserted at T10-11 level, in sitting position. 8cc of 2% injection lignocaine and 8cc of 0.5% injection bupivacaine with 25 μg injection fentanyl were given in graded aliquots to achieve a level of T6 over 20 min, keeping a strict watch over her hemodynamics. Aortocaval compression was avoided by placing a wedge under the right buttock. The patient delivered a male child, who cried at birth and had no congenital anomalies. Slow intravenous injection oxytocin 10U infusion was given. After baby delivery, the mean arterial pressures transiently dropped to 50 mm of Hg, responded to an intravenous bolus of 100 μg phenylephrine. There were no episodes of desaturation. After her surgery, the patient was shifted to the intensive care unit for further monitoring. Postoperative epidural analgesia was achieved with 8 hourly top-ups of 6 cc of 0.125% bupivacaine, administered slowly. She was discharged on postoperative day 7. |
Our patient is a 67-year-old male with a history of hypertension, type 2 diabetes, and hyperlipidemia, who went through the Inova Movement Disorders Program due to a history of fluctuating, progressing uncontrollable movements of the left side of his body. He reports these movements had been present for around 10 years, worsening over time.\nAt baseline, he always felt that his left arm and leg were moving, at times exhibiting large amplitude movements which could knock things down or affect his walking, making him fall. He described it as abnormal, uncontrollable movements that made his arm ‘reach around or fling out’ His leg was also described to ‘dance on its own.’ While at baseline, these were present but minimal stress or anxiety would exacerbate the movements. There were no movement issues on his right side, nor any facial movements noted.\nHis exam showed episodic, mild with some interspersed moderate-amplitude quick movements of the left hand, forearm, proximal arm muscles, as well as movement of the left leg. These were not suppressible, brought out more by distraction or with anxiety, such as discussing an MRI scan as he was claustrophobic. He also demonstrated some lateralizing movements of the head to the left, but no facial or tongue movements. His right side showed no such signs, and his neurological exam was otherwise unremarkable.\nHe was sent for a CT scan of the head initially due to a fear of MRI scanners, and was found to have asymmetric calcification of the right caudate nucleus and anterior putamen (Figure ). The calcification spared the anterior limb of the internal capsule. Laboratory work for parenchymal calcification was then sent and was normal. The MRI of the brain with contrast demonstrated both hypointense gradient echo (GRE) signal in the right caudate nucleus and putamen, as well as an underlying DVA (Figures -).\nGiven the lateralizing of his deficits and correlation with opposite hemispheric parenchymal calcification, he was diagnosed with unilateral hyperkinetic movements as a product of putamen and caudate calcification from an underlying DVA. Consideration was made for focal hemorrhage, but presentation and imaging were not thought to reflect this pathology. He was then treated with 0.25 mg clonazepam 2x daily with significant improvement in his hyperkinetic movements. |
A 68-year-old man visited our outpatient clinic with aggravated both leg pain caused by exercise but relieved by rest for several days. Two years previously, he had been diagnosed lumbar spinal stenosis for similar symptom. Taking a pain killer medication, his symptom has been tolerable with visual analogue scale (VAS) score about 2 or 3. Suddenly, the pain was aggravated to VAS score 8 several days ago and he visited our neurosurgical outpatient clinic. On the neurological examination, he complained of symmetric radiating pain on exercise which was relieved remarkably by rest. He had a left hemiparesis 4/5 due to the sequelae of cerebral lacunar infarction occurred 15 years ago, but no recent developing weakness was checked. Straight leg raising test (SLRT) was unremarkable and deep tendon reflexes (DTR) of lower extremities were symmetric.\nUnder the impression of lumbar spinal stenosis, routine lumbar MRI was checked and it revealed mild spinal canal stenosis caused by hypertrophy of ligamentum flavum on L4-5 (). Not only spinal lesion, it also revealed the huge sized AAA with luminal dilatation and inhomogeneous density inside which correlated to internal rupture of aneurysm. He referred to emergency room and notified to department of vascular surgery. To complete the imaging study, the vascular surgeon performed the abdominal computed tomography (CT) scan, which revealed a 70-mm-wide infra-renal aortic aneurysm(length approximately 20 cm, from the origin of left renal artery to aorto-iliac bifurcation carrefour), with a signs of internal bleeding (). A final diagnosis of abdominal infra-renal aneurysm with internal bleeding was thus formulated. After preoperative evaluation including an ankle-brachial index (ABI) which decreased to 0.81 on right side and 0.88 on left side, he was sent to surgery (AAA repair with tube graft). His postoperative course was excellent and he is alive in good condition after 9 months. Moreover, the symptom, claudication on both leg, was improved to tolerable state (VAS 3), and ABI was improved to 1.10 on right side and 1.06 on left side postoperatively. |
A 52-year-old male patient with abdominal pain was admitted to the Wonju Severance Christian Hospital. His medical history was unremarkable, except for pulmonary tuberculosis, which had been cured with medication 6 months earlier. An intraperitoneal mass was noted on abdominopelvic CT conducted by an external facility ().\nThe patient had mild abdominal pain in the middle of his abdomen that was not associated with food intake or positional change. Upon system review, there were no specific findings such as weight loss. Findings of the physical examination were unremarkable and did not reveal a palpable mass or abdominal tenderness. Laboratory analysis revealed a CEA level of 29.3 ng/mL (standard value: <5 ng/mL), and a markedly increased level of CA 19-9 of up to 4881 U/mL (standard value: <5 U/mL). Other laboratory results were within normal ranges.\nChest CT revealed improved pulmonary tuberculosis and an approximately 4.3-cm-sized cystic mass in the sub-gastric area; the internal contents of the mass showed an enhanced density greater than that of the surrounding fluid. The mass contained a partially calcified region and was not attached to an adjacent organ such as the stomach or small intestine.\nThe endoscopy finding from an external facility indicated only chronic superficial gastritis. There was no visible mass or mass effect lesion. Endoscopic ultrasound (EUS) was available for further evaluation of the mass because it was located near the lower border of the stomach. On EUS, the mass was found to be in the extra-gastric area and presented as a cystic feature with an internally mixed echoic pattern, and the cystic mass wall had both an inner smooth hyperechoic mucosal layer (, arrow a) and an outer hypoechoic muscular layer (, arrow b).\nOn the basis of these findings, we diagnosed this case as a malignant gastrointestinal tumor or malignant lymph node and performed surgery. The entire cyst was excised without disturbing the normal bowel or mesenteric anatomy. The mass featured a well-encapsulated oval shape (). During surgery, we found that the mass was located in the peritoneum, was not attached to the small bowel or stomach, and had a separate feeding vessel. Upon a gross sectional inspection, the mass was a 4×3×3-cm unilocular cyst filled with dark brown necrotic material that appeared to comprise hemorrhagic contents. The cyst wall was evenly thin with focally located, ill-defined yellowish-brown mural nodules (). Upon microscopic examination, the cyst wall was found to be composed of an inner columnar epithelial lining, 2 smooth muscle layers, and serosa, thus mimicking the intestine. However, the epithelial lining of the entire cyst consisted of gland-forming neoplastic columnar epithelium, which was characterized by a loss of nuclear polarity, a high nuclear/cytoplasmic ratio, and hyperchromasia, without spared non-neoplastic epithelium (). Focal areas of invasion into the smooth muscle were observed, and the cyst wall contained multifocal cholesterol granulomas that matched the yellowish nodules observed during the gross examination (). The immunohistochemical stains were positive for cytokeratin 20 and negative for cytokeratin 7 in the neoplastic epithelial lining of the cyst (). These findings were consistent with an adenocarcinoma that had arisen from the intestinal duplication cyst.\nThere were no metastases or local cancer invasion, according to 18F-fluorodeoxyglucose PET-CT. No postoperative complications occurred, the patient remains well, and the tumor marker levels had decreased to within normal ranges at follow-up. |
A 61 year old man presented with increasing dyspnea on exertion, ascites, and lower extremity edema. His medical history was remarkable for FAP which initially manifested ten years earlier as nausea and vomiting due to gastroparesis. In addition, he developed a painful peripheral neuropathy approximately two years later. Due to his progressive symptoms, he underwent OLT at another institution in August, 2006. Prior to his transplant, he had no cardiovascular complaints. His preoperative evaluation included a 2-dimensional echocardiogram. He did not have a cardiac catheterization. Approximately 2 months after his transplant, he began feeling dyspneic with mild to moderate activity. Shortly thereafter, he began to develop increasing abdominal girth and lower extremity edema. He presented to our institution in February, 2007 with further progression of these symptoms. His current medications included prednisone, tacrolimus, gabapentin, clotrimazole, valcyclovir, and pentamadine. His family history was significant for a father dying in his 50s due to gastrointestinal complications from FAP. On physical examination his heart rate was 100 with a blood pressure of 110/60 mmHg. He had an oxygen saturation of 97% while receiving oxygen at 4 L/min by nasal canula. He had no evidence of jaundice. He had crackles at the bases of his lungs bilaterally. His cardiovascular exam was remarkable for 10 cm of jugular venous distension, a regular rhythm with no murmurs, and an S4 gallop at the apex. His abdomen was mildly distended with shifting dullness to percussion and a liver edge 4 cm below the right costal margin. He had 2 + bilateral lower extremity edema. The ECG demonstrated sinus tachycardia with normal voltage, right axis deviation, and a left posterior divisional block. The Chest XRAY demonstrated mild cardiomegaly, patchy infiltration with associated atelectasis at the lung bases and a small right sided pleural effusion. The echocardiogram demonstrated an increase in left atrial volume, but was otherwise unchanged from his preoperative echocardiogram. (Table ) This prompted an inferiorvenocavogram to evaluate for possible stenosis at the level of the anastomosis. The pressure gradient across the inferior vena cava anastomosis was unremarkable at 1–2 mmHg. There was no evidence of portal hypertension. However, the overall central venous pressure was elevated at 18–19 mmHg. It was decided at this time to perform a right and left heart catheterization. The coronary arteries were angiographically normal. The right and left heart pressures were elevated. (Table ) His pressure tracings demonstrated no evidence of intrathoracic-intracardiac pressure dissociation or ventricular interdependence. Overall, this pattern was most consistent with restrictive physiology. An endomyocardial biopsy was performed which was consistent with cardiac amyloidosis. (Figure ) Subsequently, it was learned that the patient's mutation was on the transthyretin (TTR) gene, Threonine60Alanine. He was started on an aggressive diuretic regimen and began to feel moderate relief. He is currently being closely followed as an out-patient and will be considered for cardiac transplantation if his symptoms cannot be controlled with medical management. |
A 70-year-old female presented with a history of painless swelling of the left lower eyelid for one year (). She had no systemic complaints. On examination, her visual acuity was 20 / 20 (left eye), and a slitlamp examination of the left eye revealed no abnormalities except for corneal granular dystrophy. The lower eyelid of the left eye was diffusely swollen without any palpable mass. The left upper eyelid showed a 3-mm ptosis with 10-mm levator function, without swelling, and the ptosis resolved after administration of 2.5% phenylephrine in the left eye. No exophthalmos was present, and the extraocular movements were normal. A fundus examination revealed no abnormalities. A computerized tomography (CT) scan of the orbit was unremarkable ().\nThe patient's white blood cell count, chemistry panel, and thyroid function tests were normal. The erythrocyte sedimentation rate was 31 mm in the first hour, and serum angiotensin converting enzyme (ACE) levels were 58 units (normal, 8 to 52 units). A chest radiograph revealed an old tuberculous lesion without hilar lymphadenopathy (), and a Mycobacterium tuberculosis antigen-specific interferon gamma assay was negative. As these results were inconclusive, surgical debulking of the swollen lower eyelid via an inferior conjunctival fornical approach for diagnosis and treatment was performed, as well as a conjunctivo-mullerectomy for ptosis correction. The operative findings showed stiff and coarse orbital fat particles, and the histopathologic examination demonstrated non-caseating granulomas consisting of epitheloid histiocytes in the orbital fat, most likely caused by sarcoidosis (). Special stains for acid-fast bacilli and fungi were negative.\nThe patient was further reviewed by a pulmonologist who noted hilar lymphadenopathy on the chest CT. The patient was diagnosed with orbital sarcoidosis, and started on prednisolone (10 mg/day) in consideration of the significant improvement in eyelid swelling after surgical debulking (). The symptoms completely resolved within one month, and she has been followed for 12 months without a recurrence. |
A 1-day-old male newborn (34 weeks of gestation) delivered by cesarean section with a birth weight of 2280 g had APGAR scores of 8 and 9 at 1 and 5 min, respectively. The infant was admitted to the NICU with respiratory distress syndrome and was managed by noninvasive positive pressure ventilation (NIPPV). The chest X-ray showed severe perihilar infiltrates bilaterally with a fine granular pattern [Fig. ]. The respiratory status of the infant deteriorated on his second day of life; he required intubation and received surfactant therapy. After surfactant administration, the condition of the infant showed no improvement. A second chest X-ray was requested, which showed severe lung disease with no evidence of pneumothorax or pneumomediastineum. Free intraabdominal air was noted [Fig. ]. The abdomen was mildly distended and soft to palpation. Bowel sounds were normal. The temperature, blood pressure, and capillary refill time of the infant were also normal. The laboratory results were all within the normal range except for a low serum calcium level.\nUrgent laparotomy was done. There was no gastrointestinal perforation, and the bowels were grossly healthy. A few hours post surgery, the infant developed metabolic acidosis, followed by hypotension and delayed capillary refill. He was managed with inotropes and intravenous hydrocortisone. A head ultrasound was done, which showed bilateral grade 3 intraventricular hemorrhage. The infant had a severe drop in hemoglobin level that necessitated blood transfusion. This was later complicated by posthemorrhagic hydrocephalus that needed VP shunt insertion. The infant also developed a large ductus arteriosus, which responded well to fluid restriction and a 3-day course of oral paracetamol.\nA follow-up abdominal X-ray showed no free air in the abdomen. The results of the abdominal examination remained normal, and the infant passed meconium on the second postoperative day. After he was taken off the inotropes, gradual enteral feeding was started, which the infant tolerated very well. |
A 65-year-old man of Asian origin underwent LT at our institution on June 2002, for decompensated Hepatitis B related cirrhosis. The patient did not have a history of abnormal calcium metabolism, or hyperparathyroidism. Upon listing his serum ionized calcium was 1.07 mmol/L. The donor was a previously healthy, 42-year-old male, who suffered intracranial bleeding following a motor vehicle crash. He did not suffer any period of hypoxia but he was hypotensive (SBP = 90 mm Hg) prior to procurement. His serum liver function tests were normal prior to harvesting (AST 32 U/L, ALT 40 U/L). The organ was procured by our institution's transplant team. During procurement the liver was found to be well perfused with no focal injuries and no macroscopic evidence of steatosis.\nThe recipient underwent an uncomplicated conventional LT without the use of a veno-venous bypass. There were no periods of hypoxia or severe hypotension during transplantation. The cold ischemic time of the graft was 8 hours while the warm ischemic time was 45 minutes. The graft reperfused well and no biopsies were taken. Intravenous methylprednisolone (500 mg) was administered intraoperatively, and postoperatively the patient received induction with Antithymocyte Globulin, which is the protocol followed at our institution. During transplantation the patient received a total of 4 units of packed red blood cells (PRBCs) and 6 units of fresh frozen plasma (FFP).\nAt the time of LT, the international normalization ratio (INR) was 1.53, while serum total bilirubin was 103 μmol/L (Figure ). On postoperative day 2 the patient had a peak of his serum AST (3469 U/L) and at this point he had further biochemical evidence of primary graft dysfunction, with inability to normalize his INR (Figure ), and with progressive elevation of his total serum bilirubin (Figure ). Repeated ultrasonographic examination revealed a patent hepatic artery and portal vein, as well as patent hepatic veins. At this point, a liver biopsy demonstrated severe reperfusion injury with several apoptotic bodies, several dystrophic calcifications (Figure ), and no evidence of acute cellular rejection. His clinical status deteriorated, he developed multiorgan system failure and died 12 days after his transplantation. No septic focus was identified. Both kidneys harvested from the same donor did not present any signs of delayed graft function after transplantation. |
An 80-year-old woman presented with a history of intermittent, severe epigastric pain. Over the preceding 5 months, she had less severe and self-resolving epigastric pain 15–30 min after every meal. The full blood count, serum biochemistry values, and C-reactive protein level were within normal ranges. Additionally, arterial gas analysis was normal. Abdominal plain radiography showed free air between the liver and diaphragm, and PI (Fig. ). Abdominal computed tomography showed severe calcification of the SMA origin; SMA peripheral flow was reserved (Fig. ). Computed tomography (CT) revealed bubble-like intramural gas of the small bowel with the contrasted wall pneumoperitoneum (Fig. ).\nSince the patient did not have peritonitis, a conservative approach was performed. She was managed in the condition of intensive care unit, due to fears of the potential for acute mesenteric ischemia due to mesenteric artery occlusion or non-occlusive mesenteric ischemia. She was managed for bowel obstruction, which included fasting and intravenous fluid administration. She received heparin infusion to prevent SMA occlusion and maintain collateral flow. During admission, she reported abdominal pain relief. Seven days after admission, abdominal plain radiography showed improvement in PI and pneumoperitoneum; therefore, she was permitted to begin drinking fluids. There was no evidence of recurrent abdominal pain after the fluid consumption, so she was allowed to eat solid food.\nAngiography was planned to relieve the postprandial abdominal pain. The findings showed severe stenosis of the SMA origin with calcification, and the SMA had replaced the common hepatic artery (Fig. , ). ET, namely stenting to the SMA and common hepatic artery, was performed. The SMA trunk was engaged with a 6F Parent Plus 60 guiding catheter (Medikit, Tokyo, Japan) from the left brachial artery. Initially, 8000 units of heparin was infused, and additional heparin was added to keep the activated whole blood clotting time over 200 s. The SMA occlusion was traversed using a 0.014” NEO EVT Guide Wire Cruise (ASAHI INTECC J-sales, Tokyo, Japan). The SMA trunk to the hepatic artery was traversed using a 0.014” NEO EVT Guide Wire Cruise, which was engaged with a 6F SheathLess PV (Cardian Health, Ohio, USA) from the right brachial artery. Intravascular ultrasonography (IVUS) (Navifocus WR, TERUMO, Tokyo, Japan) revealed severe stenosis of the SMA trunk with calcification. Balloon dilation was performed with the kissing ballon technique using a 4-mm Coyote ES (Boston Scientific, Marlborough, MA, USA) to the SMA and 5-mm SHIDEN RX (KANEKA MEDICAL, Osaka, Japan) to the common hepatic artery. After dilation, two balloon-expandable stents (5-mm PALMAZ Genesis (Cardian Health, Ohio, USA) to the SMA and 6-mm PALMAZ Genesis to the common hepatic artery) were implanted with the kissing stent technique from the common ostium (Fig. ). The sizes of the stents were based on the IVUS measurements. IVUS showed good orifice expansion. Final angiography showed satisfactory circulation to the terminal organ from the orifice (Fig. ).\nEndovascular therapy relieved the patient’s symptoms. She required dual anti-platelet therapy (DAPT) with aspirin and clopidogrel post-intervention. Follow-up computed tomography showed patency of the orifice of the common hepatic artery and SMA, and there was no evidence of PI or pneumoperitoneum. No recurrent postprandial abdominal pain occurred for two years after the ET. |
A 54-year-old female with a past medical history of hypertension, heart failure with reduced ejection fraction (HFrEF, EF <35%), left ventricular thrombus (not compliant with warfarin), obesity, hypercholesterolemia, chronic obstructive pulmonary disease (COPD), schizophrenia presented to the emergency room with a chief complaint of shortness of breath and a sensation of indigestion for three days. Shortness of breath was intermittent, aggravated by moderate exertion or lying down and relieved by rest or sitting upright. The patient has had a five-pillow orthopnea for many years and it worsened over the past three days prompting her to the emergency department (ED). Upon review of systems, the patient claimed that she had a bloating sensation with mild epigastric pain which radiated to the back intermittently and was relieved after one episode of vomiting. Numbness in bilateral lower extremities was present which has been ongoing for several months. The patient denied fever, cough, dizziness, syncope, numbness in the upper extremities. The patient claimed that she was not compliant with her medications as well.\nUpon examination, the patient was in mild distress due to tachypnea at 25 breaths per minute and hypoxic requiring supplemental oxygen (oxygen saturation of 95% on 4 liters of nasal cannula), pulse rate at 90 bpm, and regular and equal on both hands. Blood pressure measured automatically on the left arm was 195/129 mmHg and right arm was 163/90 mmHg and blood pressure measured manually on the left arm was 180/100 mmHg and the right arm was 170/110 mmHg. Cardiovascular examination revealed jugular venous distension, intact bilateral carotid pulses, normally first and second heart sounds with no gallop, rub or murmur. Pulmonary examination showed wheezing all over the bilateral lung fields along with bilateral basilar crackles, therefore, with signs of fluid overload. Lower extremities exam showed bilateral mild pedal edema with unequal dorsalis pedis pulsation (less on right but intact on left lower extremities). Initial chest x-ray showed severe cardiomegaly with prominent pulmonary vasculature. There was suspicion of borderline widening of the mediastinum measuring 8.2 cm. The basic metabolic panel showed acute renal failure with a blood urea nitrogen (BUN) of 42mg/dl and Creatinine of 1.7mg/dl. Due to a history of vague epigastric pain, unequal distal peripheral pulses in lower extremities, along with unequal blood pressure on upper extremities as well as suspicion of mediastinum widening, a computed tomography angiogram (CTA) of chest, abdomen, and pelvis was performed. It showed dissecting aneurysms of the thoracic and abdominal aorta (Figure ) beginning just distal to the origin of the left subclavian artery measuring up to 4.7 cm in diameter, with extension into the right common iliac, external iliac (Figure ), and common femoral arteries (Figure ).\nLabetalol drip was started for tight blood pressure control with goal systolic blood pressure of less than 110 mmHg and heart rate less than 60 bpm. The patient was admitted to the coronary care unit (CCU) for close monitoring. The patient was transferred to the tertiary center for an escalated level of care and surgical intervention.\nBlood pressure was tightly controlled over seven days of admission to surgical ICU and endovascular intervention was later performed with TEVAR (Thoracic Endovascular Aortic Repair) and stenting in the thoracic and suprarenal abdominal aorta. The patient was discharged with a plan of following up in a vascular clinic for serial imaging follow-up with CTA at three, six, 12 months, and annually thereafter and heart failure clinic for optimization of core measures and follow-up for blood pressure control. |
A previously healthy 50-year-old male was referred to a dermatologist for assessment of a pruritic plaque on his left hand. This started as a small, erythematous papule with central vesiculation. The papule slowly increased in size over the course of several months to become a larger, scaly plaque. In addition to pruritus, the patient began to experience a tingling sensation within the affected skin. A variety of topical steroids had been previously prescribed by his family physician, as well as by another dermatologist. These brought temporary relief of his pruritus and paresthesia; however, the plaque did not resolve.\nOn examination of the dorsal aspect of the patient’s left fifth digit, a 2 cm × 2.5 cm, well-circumscribed, erythematous to slightly violaceous plaque was noted with a small amount of adherent white scale. His surrounding skin was entirely normal. Due to its unusual morphology, a biopsy was performed. The pathology report revealed spongiotic dermatitis compatible with nummular dermatitis. Based on this report, a more potent topical steroid was prescribed. However, as before, this brought only temporary flattening of the plaque.\nSeveral months later, the patient returned with a significant change: ulceration had occurred within the centre of the plaque (). In addition, approximately half a dozen tender, subcutaneous nodules were noted, extending from his left fifth digit superiorly to his elbow in a sporotrichoid pattern (). His symptoms of pruritus and paresthesia were persistent. At this time, further history was obtained, and it was determined that the patient had a salt-water fish tank containing tropical fish. Furthermore, he reported that many of his fish had died several weeks after the onset of his symptoms. Unfortunately, the deceased fish were not available for necropsy.\nBased on this history, two additional punch biopsies of the ulcerated plaque were obtained; one biopsy was sent for culture and the other biopsy was sent for histopathological examination. The biopsy sent for culture grew M. marinum, confirming the diagnosis of fish-tank granuloma. Ultimately, the biopsy sent for histopathology revealed a dermal granulomatous infiltrate supporting this diagnosis ().\nAntimicrobial susceptibility testing for the cultured strain of M. marinum showed sensitivity to amikacin, clarithromycin, doxycycline, ethambutol, moxifloxacin, rifabutin, and rifampin. This particular isolate of M. marinum was resistant to ciprofloxacin and trimethoprim-sulfamethoxazole. Based on these susceptibility results, the patient was started on doxycycline at a dose of 100 mg twice daily in combination with azithromycin at a dose of 250 mg once daily. However, despite immunocompetence, after 3 months of continuous therapy, the patient’s ulcerated plaque and sporotrichoid nodules remained unchanged. At this time, the patient’s antibiotic therapy was changed to moxifloxacin at a dose of 400 mg once daily in combination with clarithromycin at a dose of 1000 mg once daily. In addition, the patient began applying local heat to the affected skin several hours per day using a reusable heat pack. The patient was also referred to a plastic surgeon for excision of the subcutaneous nodules on his hand, wrist, and arm. After excision of these nodules, and further 3 months of the new antibiotic regime, the patient’s ulcerated plaque healed, and the excised subcutaneous nodules did not recur. Clinical resolution of the patient’s disease was confirmed 17 months after the initial appearance of the papule overlying his left fifth digit. |
A 64-year-old man with hypothyroidism, on a daily dose of levothyroxine 100 mcg, presented to our emergency department with the complaint of difficulty in breathing of gradual onset and progressive in nature for the past 5 days associated with chest tightness. He denied the presence of any other symptoms. He had a history of coming into contact with COVID-19 patients and a history of domestic travel within the country in the last two weeks.\nThe only abnormal findings on physical examination were oxygen saturations of 79% on room air and tachypnea of 26 breaths/min. Blood pressure and heart rate on arrival were 124/71 mm Hg and 101 beats per minute, respectively. Chest auscultation revealed anterior bilateral basal crackles, and the entirety of the posterior, superior, and inferior aspects had crackles. It was noted that the patient experienced fatiguability on moderate exertion, however, had no difficulty in completing sentences.\nChest X-ray (PA and lateral views) was performed and revealed bilateral peripheral and central alveolar infiltrates suggestive of atypical pneumonia (Figure ). Nasal swab PCR test for COVID-19 was positive. A series of blood investigations were performed on days 1, 3, 5, and 7 of hospital stay (Table ).\nIt was exceptional and noteworthy that throughout his hospital stay, the patient was never supplemented with any oxygen but continued with pharmacological therapies. The patient was on intravenous ceftriaxone 2 g once a day, methylprednisolone 125 mg, oral azithromycin 500 mg, and colchicine 1 mg once a day. He was also on oral multivitamin and mineral supplements throughout his hospital stay and continued after discharge. He was initiated on a heparin infusion of 2,000 IU/h for 5 days, along with warfarin 5 mg once a day. He also received a stat dose of tocilizumab 600 mg on day 3 of his admission. Chest physiotherapy using a spirometer was encouraged and along with awake prone positioning 6–12 h.\nFrom day 5 onward, the trend of results showed an improvement in numbers and likewise clinical improvement was noted in the patient along with the subsiding of chest tightness and fatiguability. Tachypnea subsided over time returning the patient to an almost normal respiratory rate, and room air oxygen saturations showed some improvement, with the highest levels recorded being 92%, constant up to the point of discharge and for isolation at home on day 8 since admission. |
A 45-year-old female who was diagnosed with lung metastases of breast cancer and received combination therapy with Bevacizumab and chemotherapy developed dyspnea.\nThe case is a 45-year-old female. She visited our hospital because of an abnormality in her mammography and was diagnosed with left breast cancer (cStageT3N1M0). Pathological analysis of a core needle biopsy sample indicated a triple negative subtype (estrogen receptor-negative, progesterone receptor-negative and human epidermal growth factor receptor 2-negative). We planned 4 cycles of 5-fluorouracil + epirubicin + cyclophosphamide (FEC) followed by weekly paclitaxel (PTX) given 12 times as neoadjuvant chemotherapy. However neoadjuvant chemotherapy was stopped because of progression during weekly PTX; a left mastectomy and axillary lymph node dissection was then performed. The patient received postoperative chemotherapy (2 cycles of FEC followed by 8 cycles of capecitabine), but bilateral lung metastases were detected after the 5th cycle of capecitabine following a chest computed tomography (CT) that was performed due to her complaint of a persistent cough.\nSome masses were on the pleura and one of the masses developed surrounding a bronchus at the right lower lobe (Figure and ). We made a diagnosis of multiple lung metastases of breast cancer. BV + PTX therapy was started due to its expected high antitumor effect. One cycle was 28 days, and BV was administered at 10 mg/kg on days 1 and 8 and PTX was administered at 80 mg/m2 on days 1, 8 and 15. The patient noticed dyspnea before cycle 3, day 1.\nShe was tachypnea. The oxygen saturation at atmospheric pressure was 95%.\nThere were no noticeable abnormalities.\nA right pneumothorax was diagnosed following a chest X-ray. The coronal plane CT revealed one solid mass replaced by a cavity that passed through the bronchus in the right lower lobe (Figure ). The cavity eventually ruptured the pleura and made the bronchopleural fistula that led to this pneumothorax (Figure ). |
The patient in this study was a 39-year-old Chinese woman who presented with a painful right breast lump on April 5, 2008. She had a history of schizophrenia for over 20 years and had been receiving risperidone (2 mg bid) for more than 3 years. She had developed a lump in her right breast a month before her visit and had noted a progressive worsening tenderness with erythematous changes around the lesion. She had no systemic symptoms nor did she have any known previous fungus or tuberculosis exposure. The patient had a gestational history of G0P0 and had amenorrhea for approximately a year. She had no history of any other breast disease or receiving any breast surgery, and her medical history was otherwise unremarkable. She had never consumed tobacco, alcohol, oral contraceptive pills nor did she have any family history of breast cancer. On physical examination, a 9-cm × 6-cm breast lump with localized redness over the right breast lateral aspect with an ill-defined margin was noted with no palpable lymphadenopathy at the axilla. There was no splenomegaly or hepatomegaly. She did not have fever, joint pain, airway or urinary tract bleeding. No other skin lesion was found. The patient's blood count was normal. Breast ultrasound images revealed a large ill-defined area with heterogeneous echoes in the right upper and lower outer quadrants, associated with increased vascularity. Micro-calcification and tissue edema were noted. No enlarged lymph node was noted in the right axilla, and there was no dilatation of the lactiferous duct. CXR didn't show any lung lesions. Mammography showed asymmetry with increasing radiodensity at the outer upper quadrant of the right breast with nipple retraction. Mastitis with edema and micro-abscess in the right upper and lower quadrants was suspected. However, inflammatory carcinoma could not be ruled out. All other laboratory and other radiological studies including C-reactive protein (0.8 mg/L) were normal except that the prolactin level was 84.5 ng/ml (normal, < 20 ng/ml); FSH, 4.6 mIU/ml; and LH, 6.0 mIU/ml. Although malignancy had not been excluded, the patient received a presumptive diagnosis of infectious mastitis and was treated with a 14-day course of keflex (first-generation cephalosporin), but there was no improvement. Therefore, core breast biopsy was performed, which revealed an adipose tissue with acute and chronic inflammation. Aspiration culture showed no evidence of bacterial or mycobacterial growth. In order to rule out previously missed diagnosis, another incision biopsy was performed 1 week later, and the pathology showed chronic inflammation with focal fibrotic changes in the fat tissues. Due to persistent drainage with erythematous swelling and in consideration of malignancy, the patient then was scheduled for simple mastectomy. Macroscopic appearance demonstrated a huge mass with an ill-defined margin of inflammatory tissue at the center with peripheral fatty necrosis and hematoma (Figure ). Microscopic examination revealed chronic inflammation and macrophage, giant histiocyte, and epithelioid-like cellular infiltration, with cytologic features suggestive of a granulomatous process. The noncaseous granulomatous lesions were centered at the breast lobules. The lesions were characteristic with an empty space in the center and surrounded by microgranulomas and microabscesses. Some granulomatous lesions composed of confluent epithelioid cells and huge abscess were also seen (Figure ). Based on the histological features, the differential diagnosis included autoimmune response, undetected organisms, systemic granulomatous disease with breast involvement, granulomatous reaction in a carcinoma and foreign body reaction. There was no evidence of carcinoma or specific organism can be found. All cultures, Ziehl-Neelsen staining, PAS staining and GMS staining yielded negative results. Considering the patient's clinical history and all the laboratory findings, the diagnosis was interpreted as IGM. In suspect of the dopaminergic effect of risperidone to cause hyperprolactinemia and IGM, the prescription was shifted to prolactin-sparing second line agent (clozapine). The residual breast lesions resolved completely 3 months later and no recurrence was noted since then. |
A 29-year-old male with a history of right tibial shaft and lateral malleolus fractures following a fall injury underwent open reduction and internal fixation with a locking plate for the fibula fracture and minimally invasive percutaneous osteosynthesis for the tibial shaft fracture at another hospital. Postoperatively, the patient was followed up at our hospital for 1 year due to residential issues, and the removal of the plate was scheduled after confirmation of bone union. According to the previous hospital’s medical records, a 9-hole locking plate (APIS®, Gwangju, Republic of Korea) was used for the distal tibia, with three 5.0-mm locking screws for proximal fixation and six 3.5-mm locking screws for distal fixation. Under spinal anesthesia, a plate removal surgery was performed, following which the fibular plate was easily removed. However, five of the six distal locking screws were damaged, and they could not be removed with a screwdriver. This situation was explained to the patient during the operation, and we could confirm that the patient wanted to remove the plate irrespective of the amount of time required. At the time, a tourniquet was used for approximately 1 h, and screw removal was attempted using a conical extraction screw, but only one of the six screws could be removed. Previously proposed techniques requiring additional skin incisions were not considered because there were multiple damaged screws that could not be removed, these techniques would require extended amount of time for removal, and the surrounding soft tissues could be damaged. Hence, we decided to use a screw extractor (IRWIN®, Huntersville, NC, USA), a non-medical instrument, following sterilization with ethylene oxide. In brief, 6-mm drill bit of the extractor was used, four screw heads were drilled, the locking screws and locking plate were separated, and the plate was removed. Of the remaining four screws, one was removed using a vice grip; the remaining three screws could not be removed with the vice grip owing to a short extruded portion. A hollow reamer could not be used because the three screws were adjacent to each other and the reamer could cause a large bone defect; hence, we created sufficient space around the screws by forming small holes around them with a 1.8-mm K-wire, following which all screws could be removed using the vice grip. After saline irrigation, debridement of the soft tissues contaminated with metal debris was performed. The sizes of the metal debris were found to be relatively large, which facilitated easier debris removal. With 2-h use of a tourniquet, all implants could be removed and the skin could be closed (). Postoperatively, no complications were noted during physical and radiological examinations. Remarkably, no problems were noted at the final outpatient visit at 3 months after discharge; therefore, follow-up was terminated. The patient had not visited an outpatient clinic for more than a year since the last follow-up, thus we thought that he had no specific complications including infection and re-fracture. |
A 23-year-old man was admitted with the chief complaint of chest pain associated with cough for approximately 3 days. He denied a history of tuberculosis and had no history of surgery or trauma. His medical history was unremarkable.\nPhysical examination showed an approximate 7 cm diameter mass that could be palpated in the right paravertebral area of the thoracolumbar spine; the mass was slightly hard, immobile, and obviously tender. The mass margin was not clear and was adherent to the adjacent tissue. No local superficial venous distention was observed around the thoracic vertebra. His vital signs were normal, with oxygen saturation 99%.\nComputed tomography (CT) (Fig. –) demonstrated an osteolytic, expansive, and eccentric lesion on the vertebral bodies and right accessory processes, with spinal cord compression at the T9/10 level, with right rib also having bone destruction. The bone destruction penetrated the local cortical bone with a large mass around it. The mass of density was inhomogeneous. The CT value was decreased from 40 to 20 Hu. A thin and discrete rim of bone was seen around the mass. In addition, the adjacent rib showed osteolytic and expansive destruction, and the cortical bone of the adjacent rib was thin. The contrast-enhanced CT showed obvious inhomogeneous enhancement of the lesions.\nMagnetic resonance imaging (MRI) (Fig. –) found that the lesion had inhomogeneous signals and low signal intensity in T1-weighted MR images, and relatively mix signal intensity in T2-weighted images. The margin between the lesion and the preserved bone was clear. Multiple fluid–fluid levels could also be seen; fluid–fluid levels were observed in more than one-third of the lesions. Some pleural effusion was seen in the right thoracic cavity.\nDue to bone destruction on the vertebral bodies and right accessory processes and multiple cysts in the lesion, the diagnosis based on CT and MRI findings was malignant tumor with aneurysmal bone cyst (ABC). Wide spondylectomy of T9/10 was performed to resect the paravertebral tumor, with reconstruction using an endoprosthesis (Fig. ). The surgical findings were as follows: The mass, which grew into the thoracic cavity, was located in the T9/10 thoracic vertebrae and paravertebral structures. It infringed on the 9th and 10th ribs and invaded the partial diaphragm. The mass invaded the spinal canal, resulting in cord compression.\nThe gross pathology (Fig. ) findings were as follows: The tumor with its complete capsule was resected integrally. It measured 9 cm × 8 cm × 6 cm. On the cut surface, the mass was grey-white. Some residual blood was seen in the cystic area. The solid mass was soft on palpation and grey-pink.\nThe microscopic findings (Fig. ) were as follows: A large number of nuclear giant cells and powder stained bone matrix were observed. Immunohistochemical examination showed positive staining for CD163, CD68, SMA, and Ki-67, and negative staining for S-100 and P53. The presence of osteoid matrix in partial areas, cells with mild-to-moderate atypia, multiple nerve invasion, and vascular tumor thrombus were observed, suggesting malignant transformation. On the basis of the above findings, PMGCTB with ABC was the pathological diagnosis.\nAfter surgery, adjuvant chemotherapy based on the protocol used for osteosarcoma was administered. After 4 years of follow-up, the patient has no clinical or radiological evidence of recurrence.\nThe patient provided written informed consent. In this case, the patient accepted standard, proven diagnosis and therapy in the Clinical Department of Bone and Soft Tissue Tumor Department, so ethical approval was not necessary. |
A 33-year-old Caucasian female at 32 weeks 1 day gestation, presented in hemorrhagic shock after a 2 h history of severe abdominal pain without trauma or vaginal bleeding. She was a high-risk pregnancy with an outpatient diagnosis of placenta accreta by ultrasound, tobacco use, history of pre-eclampsia and late presentation to prenatal care. Blood pressure was 54/31 and heart rate was 97. Fetal monitoring showed a category 2 tracing, with minimal variability, no accelerations or decelerations. Due to presence of shock, patient was emergently taken to the operating room with a suspicion of uterine rupture for delivery and total hysterectomy assisted by REBOA.\nREBOA was introduced to zone 3 with a 7 Fr sheath into the right common femoral artery. Uterine rupture with an adherent placenta was found. A bookwalter was placed and the fetus delivered. Following delivery of the fetus, the REBOA was inflated with 22 ml of fluid and SBP rose from 70 to 170. Total balloon inflation time was 23 min during total hysterectomy (Fig. ). Estimated total blood loss was 4500 ml. The patient received 4 l of crystalloid, 8 packed red blood cells, 10 fresh frozen plasma and 2 platelets intraoperatively. Prophylactic enoxaparin 30 mg twice daily and sequential compression devices were initiated in the ICU. Approximately 9 h post-operatively, the 7 Fr sheath was removed once rotational thromboelastometry (ROTEM) normalized per protocol and manual pressure was held to the right groin for 30 min. Patient remained hemodynamically stable with lower extremities neurovascularly intact and progressed to full ambulation by postoperative Day 2.\nOn the following day routine ankle/brachial index (ABI) and arterial duplex showed the ankle brachial index on the right was 0.71, left 1.22 and an occlusion of the right external iliac and proximal common femoral artery. The patient had no evidence of right groin hematoma and no resting leg pain. She was placed on heparin drip and computed tomography angiography confirmed complete occlusion of the right external iliac artery beginning 3 cm distal to its origin (Fig. ). The patient was taken back to the operating room for right external iliac artery thrombectomy by vascular surgery (Fig. ). Aortogram showed occlusion of the right external iliac artery with reconstitution distally in the common femoral artery. She was discharged home 5 days later on aspirin 81 mg daily for 6 months. |
A 35 years old (height, 159 cm; weight, 85 kg; gravida 3, para 1) pregnant woman with SAS presented to our hospital and was scheduled to undergo elective CS under epidural anesthesia at 36 weeks of gestation. An echocardiographic examination at 34 weeks of gestation revealed that the diameter of the beginning portion of the aorta (under the aortic valve) was narrowed by about 17 mm. The diameter of the ascending aorta was normal, the average pressure gradient was 40 mmHg, and the ejection fraction was 67%.\nIn the operation room, her baseline vital signs were as follows: heart rate (HR), 82 bpm with normal sinus rhythm; noninvasive blood pressure (BP), 130/76 mmHg; and oxygen saturation (SpO2), 96%. Supplemental oxygen was administrated by a face mask at the rate of 3 L/min. Traditional epidural puncture was performed at the L1–2 and L3–4 interspaces in the left lateral position, and a catheter was inserted to a depth of 4 cm into the epidural space at the cranial and caudal ends of the surgical field. The patient was then placed supine in a left-tilt position; 3 mL of 2% lidocaine was injected as a test dose, and another 7 mL was injected 5 min later through the cranial catheter. Additionally, 3 mL of 1.5% lidocaine was injected through the caudal catheter. At 20 min after the lidocaine injection, the sensory block had reached T6. The patient had no discomfort, and her hemodynamic parameters were stable. The operating bed was adjusted from the left-tilt to horizontal position, and the operation was allowed. Two minutes later, the patient reported chest distress and difficulty breathing (HR, 110 bpm; BP, 80/69 mmHg; SpO2, 96%), and 3 mg of intravenous ephedrine was promptly administered. The patient immediately lost consciousness with no response (HR and BP were depressed to 40 bpm and 53/15 mmHg, respectively). The lowest BP at the time was not measured because the noninvasive BP measurement interval was set to 1 min. The obstetricians sped up the surgery, and in another 2 min, a 2750-g male newborn was delivered with an Apgar score of 10 at both 1 and 5 min. Spontaneous circulation of the patient was returned immediately after delivery, and stable vital signs returned with no other treatment. The postoperative course was uneventful. The mother and neonate recovered uneventfully and were discharged 4 days later. |
A 51-year-old Asian Indian man noted 1 month of blurred vision with photopsia and floaters. There was no other significant medical or family history. External examination showed the presence of periocular melanocytosis []. The visual acuity was 20/40 in the right eye and 20/20 in the left eye. Intraocular pressure was 13 mmHg in both eyes. The anterior segment was unremarkable in the left eye, while the right eye revealed sectoral scleral melanocytosis []. There was no temporal fossa, palate, or iris melanocytosis.\nFunduscopically, the left eye was normal. Evaluation of the right eye fundus revealed two separate pigmented choroidal tumors [Figs. and ]. The smaller tumor was juxtapapillary and temporal in location and measured 4 × 4 mm in basal diameter and 2.8 mm in thickness. The larger inferior tumor measured 13 × 11 mm in base and 9.2 mm in thickness. Both tumors demonstrated signs of tumor activity including prominent overlying orange pigment and subretinal fluid. Though quite subtle, trace sectoral choroidal melanocytosis was noted surrounding the two tumors. B-scan ultrasonography documented both tumors with acoustic hollowness and without extrascleral extension.\nThorough systemic evaluation with imaging of the chest and abdomen and blood tests revealed no evidence of any other primary or secondary malignancy. The findings were consistent with unilateral primary multifocal choroidal melanomas arising in sectoral choroidal melanocytosis of the right eye. The treatment options included enucleation or plaque radiotherapy. After appropriate informed consent, the patient underwent enucleation with a primary silicone orbital implant.\nGross examination of the enucleated eye revealed patchy grey pigmentation of the episclera. On histopathology, the choroid temporal to the optic disk [] and in the inferior quadrant was thickened by a dense population of darkly pigmented plump and benign dendritic melanocytes, characteristic of choroidal melanocytosis []. There was no evidence of melanocytosis in the remainder of the choroid, iris, and ciliary body stroma. There were two elevated choroidal tumors [Fig. and ], both of which were composed of pigmented epithelioid melanoma cells and neither tumor showed scleral or retinal invasion. On careful serial sections, both tumors were confirmed to be within the area of flat choroidal melanocytosis with a steep indentation and base continuity in the transition between the two []. |
A 35-year-old male patient reported to the Department of Prosthodontics, Post Graduate Institute of Dental Sciences, Rohtak for the replacement of his missing tooth []. After a thorough history taking, clinical and radiographic examination, the treatment options were explained to the patient, and it was decided to place an implant with respect to missing 16. Since the available bone height in this region was only 5 mm, hence, it was decided to perform an indirect sinus lift and bone grafting with simultaneous implant placement.\nThe crestal approach sinus kit (CAS-KIT, Hiossen) was used in this case to provide a convenient sinus grafting surgery and to enhance maximum safety while easily providing the lift of the membrane in a controlled manner for the maxillary sinus [] After anesthetizing the patient, the crestal incision was given [] and flap was reflected The CAS-drills were used in a sequential manner to prepare the osteotomy site. Due to the inverse conical drill design of the CAS-KIT, the conical bone chips in between the cutting blades create an auto-lifting function to elevate the membrane safely. The atraumatic design of the drill tip allows the user to perform sinus surgery even if the sinus floor is flat, incline, or septum. The unique stopper system also prevents membrane perforation and excessive drill penetration []. Thus, progression of 1.0 mm increments could be accomplished until penetration was achieved in a controlled manner The hydraulic lift system was used to elevate the sinus membrane by hydraulic pressure of saline injected through a syringe [Figures and ] The bone carrier was used to deliver bone graft material (Ostofom) [] into the space created by sinus lift [Figures and ]. Graft material required is in direct proportion to the amount of fluid used to inflate the balloon: 1cc of fluid will require 1cc of graft material After delivering the bone graft material in the osteotomy site, the bone condenser [] was used to compact the bone material. Using a slow speed (50 rpm), the bone spreader was used to evenly spread the material within the cavity. This step was repeated until the desired height and volume of the sinus cavity was filled and was ready for implant insertion. This was followed by placement of Implant (3.75 mm × 10 mm, Osstem) and the site was sutured []. |
An 84-year-old man presented to our department with general weakness, weight loss, shortness of breath, and a two-month history of chest pain. Preceding these symptoms, two months previously he had suffered a myocardial infarction with ST elevation, entailing emergent percutaneous coronary intervention and stent insertion to the left anterior descending artery.\nHis past medical history included hypertension, dyslipidemia, chronic renal failure, and valvular heart disease; 12 years prior to this hospitalization he underwent aortic valve replacement to a biological valve because of severe aortic stenosis. In addition, 4 months prior to the current admission the patient was evaluated by a hematologist for newly diagnosed anemia and thrombocytopenia with splenomegaly and was awaiting a bone marrow biopsy.\nIt is worth mentioning that the patient had sought medical attention 1 month prior to admission, when he had complained of general weakness, weight loss, decreased appetite and abdominal pain. A CT scan performed in the emergency department at the time was negative and he was discharged home for further investigation by a gastroenterologist.\nOn admission to our facility, the patient was stable and vital signs were normal without fever. Physical examination was significant for high jugular venous pressure (JVP) and peripheral pitting edema. A chest X-ray showed signs of pulmonary congestion and a new right pleural effusion without signs of pneumonia. Blood tests showed a mild known anemia, Hb 11.5 g, platelets 85,000, creatinine 2.5 (baseline around 1.5 mg/dL), alkaline phosphatase 300 U/L, GGT 455 U/L, CRP 1.5 mg/dL, and NT-proBNP >35,000. Having assumed the patient had acute decompensated heart failure, he was treated with an escalated dose of diuretics with minimal improvement in his general condition.\nOn day 2 of admission, the patient had a transthoracic echocardiogram which revealed a mobile bioprosthetic aortic valve mass with moderate aortic regurgitation and moderately reduced left ventricular function. A transesophageal echocardiogram the subsequent day revealed the same finding with measured dimensions of 1 × 0.4 cm ( and ). This finding was suspected to be a vegetation.\nAt this point, the patient had no fever, vital signs were stable, and blood cultures were still without growth. Serological tests were sent for Q fever, Brucella and Bartonella. Because the patient had one major criterion (a vegetation) and one minor criterion (predisposition), according to Duke criteria, antibiotic treatment was not initiated and evaluation for marantic endocarditis was carried out. Serology for antiphospholipid antibody syndrome was checked and returned negative; the rheumatological panel was negative; a total body CT was negative; and neoplastic markers were negative.\nOn day 14, serological tests for Bartonella henselae returned positive; both IgM and IgG were positive. A polymerase chain reaction (PCR) for Bartonella returned negative. Antibiotic treatment with rifampin and doxycycline was initiated; however, an aminoglycoside was not incorporated into the treatment regimen because of the patient’s borderline kidney function. At this point, the patient recalled being scratched by a cat several months prior to his current admission. Subsequently, his condition acutely deteriorated, his creatinine elevated to 4 mg/dL and he became anuric, necessitating hemodialysis being performed multiple times. His liver enzymes were significantly elevated on day 16: AST was 604, ALT 204, direct bilirubin 6.5 mg/L, total bilirubin 9 mg/dL. Accordingly, treatment with rifampin was replaced with gentamicin. Despite treatment, there was no improvement and the patient was not considered for surgical treatment because of the high surgical risk. Hence, antibiotic coverage was continued solely without any significant improvement. On day 24, the patient was in asystole, and considering his very poor prognosis and because of his family request and his own preferences, resuscitation was not performed. He died the same day. |
A 33-year-old male patient presented with the complaint of an asymptomatic growth of 5-month duration on the lingual gingiva in the left lower premolar region. On intraoral examination, an exophytic, sessile, oval-shaped mass measuring 2 cm × 1 cm was noticed in the lingual gingiva in relation to 33 and 34 []. The lesion was normal in color with white papillary projections on the surface. On palpation, it was firm in consistency, was slightly tender and fixed to the underlying tissue. The intraoral periapical radiograph of the region showed cupping resorption of the bone []. Based on the clinical presentation of a soft-tissue lesion on the gingiva with numerous papillary projections on the surface, a provisional diagnosis of papilloma was given. Pyogenic granuloma, peripheral giant cell granuloma, peripheral ossifying fibroma and peripheral odontogenic neoplasms were considered as differential diagnosis. Excisional biopsy was done, and the tissue was sent for histopathological examination.\nMicroscopically, the lesional tissue showed a superficial hyperplastic stratified squamous epithelium with numerous papillary projections. At multiple areas, the epithelium showed ameloblastomatous change []. The proliferation of transformed epithelium with odontogenic islands budding off into the underlying connective tissue stroma [] was noticed. Within the connective tissue stroma, there were numerous ameloblastomatous islands lined peripherally by tall columnar cells showing reversal of polarity and central stellate reticulum-like cells []. Some of these islands showed acanthomatous change []. The superficial epithelium showed positivity for the odontogenic marker, cytokeratin-19 (CK-19), confirming its odontogenic transformation []. The ameloblastomatous islands in the connective tissue stroma were also positive for the marker, CK-19 []. Based on the clinical and histopathological presentation, the lesion was diagnosed as peripheral ameloblastoma. Since saucerization of the bone, adjacent to the tumor, was noticed, marginal osteotomy was carried out to evaluate any bone involvement. Multiple sections from the decalcified peripheral bone were studied. A few odontogenic islands were noticed in one of the sections studied [Figures and ], while all the other sections showed intact cortical bone. Although the presence of tumor islands within the adjacent bone made us consider the possibility of a peripheral intraosseous ameloblastoma, based on the presence of an extraosseous ameloblastomatous lesion showing a clear origin from the superficial epithelium which had undergone odontogenic transformation and the presence of few tumor islands within the adjacent bone suggestive of a neoplastic invasion, the lesion was diagnosed as of peripheral ameloblastoma with neoplastic osseous invasion.\nThe healing of the area was uneventful; the patient was on a regular follow-up for a period of 2 years, and there was no recurrence of the lesion. |
A 51-year-old man presented with a history of a progressive loss of his peripheral visual field in the right eye and photophobia in both eyes that was first noted in February 2003. He had been treated with two courses of 1000 mg intravenous methylprednisolone for 3 days by his previous physician. After those treatments, he was referred to our hospital in April 2005.\nOur initial examination in 2005 showed that he had no personal or family history of ocular or autoimmune diseases. His best-correlated visual acuity (BCVA) was 20/25 in the right eye and 20/16 in the left eye. A swelling of the optic disc was detected but only in the right eye. The diameter of the retinal vessels in the fundus photographs was narrower in the right eye than that of the fellow eye (Fig. a-b), and the optical coherence tomographic (OCT; Fig. c) images showed that the outer retinal bands in the right eye were not clear and edema was present in the macula. Fluorescein angiography (FA) demonstrated window defects corresponding to the site of the retinal pigment epithelial atrophy. FA also showed staining of the parafoveal tissue and leakage from the right optic disc (Fig. ). Electroretinograms (ERGs) were non-recordable from the right eye and normal in the left eye (Fig. ). Goldmann perimetry detected a peripheral visual field loss in the right eye (Fig. ). Immunoblot analyses detected no anti-retinal antibodies. During the entire course, no tumor lesions were found by systemic examinations including gastrointestinal endoscopy, computed tomography (CT), and positron emission tomography CT (PET-CT). Because the search for anti-retinal antibodies was negative, npAIR was suspected based on the clinical findings []. The response to steroid treatment was poor, and the vision in his right eye decreased to no light perception.\nAlthough the left eye had photophobia since the initial visit in 2005, no abnormal subjective or objective findings were observed until 2014. He noticed an upper visual field defect in the left eye in January 2014, ten years after the first onset in the right eye. The BCVA was 20/16 in the left eye at this time. Fundus photographs showed no obvious abnormal findings in the left eye (Fig. a). However, a reduction in the length of the ellipsoid zone (EZ) and a partial discontinuity of the interdigitation zone (IZ) were seen in the OCT images (Fig. b). In addition, OCT showed macular edema in the left eye in October 2014 (Fig. c). FA detected peripheral window defects in the left eye (Fig. ). No visual field abnormality was observed until 2013, however Humphrey field analyzer (HFA) 30–2 examination in January 2014 showed an upper visual field defect. The visual field defect progressed rapidly to a ring scotoma (Fig. ). Dark-adapted ERGs of the left eye were non-recordable. The amplitude of the light-adapted and flicker ERGs of the left eye were reduced with prolonged implicit times (Fig. ). Immunoblot analyses were positive for anti-α-enolase antibodies. A complete examination including gastrointestinal endoscopy, CT, and PET-CT was performed again, and no tumor lesions were detected. No inflammation was observed in the anterior segment or the vitreous at any time.\nTwo courses of 1000 mg intravenous methylprednisolone for 3 days was given based on a diagnosis of npAIR. After the steroid pulse therapy, oral administration of prednisolone of 30 mg/day and oral immunosuppressant were initiated and were continued. The symptoms gradually worsened in spite of these treatments. Therefore, six plasmapheresis treatments were administered from June 2016. However, the response to these treatments was poor, and the vision in his left eye eventually became no light perception. At that time, funduscopic examination of the fellow eye demonstrated attenuation of the retinal vessels, and fundus autofluorescence of the fellow eye demonstrated retinal pigment epithelial atrophy in the midperipheral area. |
A 43-year-old female patient suffered from head trauma resulting from a car accident. She was seated in the passenger seat of a minivan was stopped at a signal light behind a truck, and another truck collided with her car from behind. The minivan then repeatedly collided with the trucks in front and behind. During that collision, her head repeatedly struck the minivan seat resulting in whiplash injuries. The patient reported that she did not experience loss of consciousness or post-traumatic amnesia. The patient’s initial Glasgow Coma Scale score was 15. After the head trauma, she felt pain in both legs, mild motor weakness in all four extremities, and memory impairment. Although she visited several hospitals to determine the cause of her pain, she was unable to obtain a precise diagnosis for her pain because the conventional brain and whole spine MRIs did not show any abnormality. At eight years from onset, she was diagnosed as CRPS type 1 with the clinical features of hyperalgesia and mild edema and motor weakness of both legs; moreover, abnormality was not detected on plain radiography for hand and leg, three-phase bone scan, and thermography. Nine years after the head trauma, she visited the rehabilitation department of our university hospital complaining of pain in the right arm and both legs. The characteristics and severity of the pain were as follows: Constant tingling and burning sensation with allodynia and hyperalgesia (visual analog scale score: Right arm and leg, 9; left leg, 7) []. She also exhibited mild weakness of the four extremities (4−/4), mild edema of both legs, and memory impairment. However, trophic changes of the arm and legs, including skin and nails, were not observed. In addition, she mentioned that she did not experience the distal edemas on the right arm and both legs, which could observe during the acute stage of CRPS []. Electromyography for all extremities and trunk failed to detect any abnormality (A). The patient provided signed, informed consent, and the study protocol was approved by the institutional review board of our university hospital (YUMC 2019-06-032, approved on 21 June 2019). |
A nine-year-old boy who was previously healthy presented to the outpatient department complaining of double urinary stream that was initially noticed after a circumcision was performed a few weeks after birth. Other urinary symptoms, history of urethral discharge and urinary tract infections, and previous hospitalizations were not reported. A general examination showed the boy appeared healthy. Genital examination showed a circumcised penis with a mild degree of dorsal penile chordee. He had two urethral openings: one was normal in size and orthotopic in position at the glans, while the other was small in size and located on the dorsal part of the penis (epispadias) 1 cm proximal to the normal urethra ().\nA scrotal examination was normal. With regard to investigations, renal and bladder ultrasounds were normal, and bladder emptying was complete. A voiding cysto-urethrogram (VCUG) was conducted using an 8-Fr feeding tube. We cannulated the normal urethra and normal saline with iodinated contrast media (250 cc), which was infused slowly into the urinary bladder under gravity control, to obtain fluoroscopic images. The results showed the urethra was normal and the shape, size and capacity of the urinary bladder were normal with no vesico-ureteral reflux (VUR). Then, cannulation of the accessory urethra was performed using a 4-Fr feeding tube that was inserted just about1 cm from the accessory meatus. The minimal amount of contrast media was injected under fluoroscopic images (retrograde urethrogram). Antero-Posterior and oblique exposures were taken and it showed complete duplication of the urethra down to the bladder. The voiding phase showed two completely duplicated urethras that started from the bladder ad had two separate lumens and two separate openings distally ().\nAt the time of surgery, a cysto-urethroscopy was performed that showed a normal urethra, a normal intact external urethral sphincter, and a normal prostatic urethra; the bladder neck and bladder were observed and were also normal. The accessory urethra was cannulated using a hydrophilic small catheter (3-Fr) (0.35”/150 cm/RoadrunnerR UniGlide™/COOK MEDICAL). The catheter passed easily through the meatus towards the bladder and was easily seen to enter the bladder from a point anterior to the bladder neck position.\nAfter cystoscopy, cannulation of the accessory urethra was performed with a 3-Fr hydrophilic catheter and a Foley catheter (10 Fr) was inserted in normal urethra, the penile skin was completely degloved, and the accessory urethra excised down as deep as possible behind the symphysis pubis. The dissection was confined to the hydrophilic catheter and was close to the bladder wall but did not actually reach it, and involved the lining of the accessory urethra so as not to jeopardize the surrounding vessels, nerves, or the normal ventral urethra (,,,). During dissection, it was observed that tethering of the accessory urethra had induced a mild degree of dorsal chordee. Once this was excised, the traction disappeared, and the dorsal chordee corrected. Foley catheter was kept in place for 4 days (). The patient was discharged 1 day after the procedure.\nThe post-operative period was uneventful, and the patient was followed for 9 months, during which he had only one urinary stream and a straight penis. |
A 64-year-old male visited our institute in February 2018 for a cough productive of purulent sputum for one month. He had smoked half a pack of cigarettes daily for 30 years but had stopped 12 years previously. He was clinically diagnosed with pulmonary tuberculosis in 2011 based on radiologic findings in the upper lobes of both lungs with multiple small dominant lung nodules (Figures and B). After completing six months of anti-tuberculosis treatment, he did not visit our clinic again due to improved respiratory symptoms. He had been working in a gristmill for 25 years.\nHis current physical examination was unremarkable, and there were no abnormal sounds in either lung field. In sputum and bronchoalveolar lavage fluids at admission, tests for acid-fast bacilli and pyogenic cultures were negative. Mycobacterium tuberculosis-polymerase chain reaction and Xpert® MTB/RIF also did not detect tuberculosis in the specimens. His pulmonary function tests revealed a mild obstructive pattern [forced expiratory volume in 1 (FEV1): 75% predicted, FEV1 to forced vital capacity ratio: 0.66] with a positive bronchodilator response (ΔFEV1: 310 mL). On chest radiograph and computed tomography scans in 2018, the size and number of lung nodules significantly increased compared with those from 2011, mainly in both upper lobes (Figures and D). The patient was hospitalized for percutaneous needle lung biopsy to differentiate between cancer and connective tissue disease. The patient’s occupational history was investigated. He had worked in a stone processing factory for 17 yr between 1972 and 1992, except for three years of military service. He processed granite for 12 h a day, six days a week without protective equipment in a poorly ventilated tent. After he quit working in the stone processing factory in 1992, he operated his own gristmill and worked seven days a week grinding rice, beans, red beans, and peppers using a granite roller grinder in a poorly ventilated underground mall without protective equipment for 25 years. His gristmill was in a poorly-ventilated small room located underground. He poured the grains directly into the funnel-shaped grinder entrance, and then put the grained grains into the bag by connecting the grinder outlet.\nHistopathologic examination of the lung specimen showed diffuse infiltration of phagocytic macrophages with focal sclerosis (Figure ) and a few multi-nucleated giant cells (Figure ). Macrophages contained anthracotic pigment (Figure ), and lightly birefringent crystals were observed with polarized light microscopy (Figure ). Without detailed knowledge of the patient’s history, the initial histopathologic diagnosis was silicosis with rapid progression. |
A 70-year-old man was admitted to our clinic for progressive gait disturbance. He had undergone aortic valve replacement surgery 11 years ago, and had been taking warfarin ever since. Three years ago, he underwent a retromastoid suboccipital craniotomy to remove a right vestibular schwannoma. Unfortunately, an intracerebellar hemorrhage developed 2 months after the surgery, and posthemorrhagic hydrocephalus developed as a consequence. A VPS (Codmann Hakim Programmable Valve; Johnson & Johnson Co., Boston, MA, USA) was placed. All connection sites between the catheters and the reservoir was reinforced with a tie using 3-0 black silk. After the operation, he had been doing well for 18 months.\nHe complained of a recent tendency to trip that began 5 months before admission. At the outpatient clinic, we performed brain computed tomography (CT) first to look for any signs of shunt malfunction. Since CT showed no change in ventricle size, we decided to lower the shunt pressure setting and to observe any symptom improvement in the patients. However, the patient did not get better despite repeated subsequent adjustments of shunt pressure. While attempting to examine the cause of his gait disturbance, we became aware of the fact, by chance, that the distal catheter was not visible on an outside chest X-ray film ().\nAt the time of admission, the patient's vital signs were stable, and his body temperature was 36.3°C. He had not suffered any abdominal symptoms, and physical examination showed no tenderness or rebound tenderness in the abdomen. Upon neurological examination, his mental status was indicative of slight drowsiness, and his orientation was intact. Mild cognitive dysfunction was observed. However, there was little difficulty with communication. The motor power of both the upper and lower limbs was assessed as grade 4/5, and he could walk a short distance with assistance. According to the laboratory tests, the whole white blood cell count was 6.13×109 count/L (reference range, 4.0 to 10.0), and the CRP level was 0.2 mg/dL (reference range, <0.3). A set of plain X-rays revealed that the distal catheter was missing (). Additional abdominal CT was performed to locate the retained distal catheter in the abdominal cavity, but it failed to be found. No intraabdominal organ abnormalities associated with shunt catheter migration were observed.\nUnder the diagnosis of shunt malfunction secondary to disconnection and disappearance of the distal catheter, shunt revision surgery was performed. As we exposed the shunt valve, the proximal shunt system seemed to be working well. No CSF collection was observed at the disconnected site juxta of the shunt valve reservoir. A subcutaneous tract where the missed distal catheter previously located was observed, and it seemed that CSF coming from the valve was continuously drained through this tract. A new distal catheter was connected to the shunt valve, and its distal tip was inserted into the peritoneal cavity through a new subcutaneous tunnel. We did not explore the abdominal cavity to look for the hidden distal catheter.\nThe postoperative course was uneventful, and the patient was discharged 8 days after the surgery. During the 2-year follow-up, the patient was in good condition, and no complications related to the operation were observed. His gait had improved, and he could walk without assistance, although some difficulty remained. |
The patient is a 71-year-old white male who was found to have a 3.5 cm right kidney mass and had been followed by the urology team closely at VA Pittsburgh Healthcare System. Urine cytology was suspicious for malignant cells. He underwent a radical right nephrectomy on February 3, 2014. Pathology showed clear cell RCC. The tumor was located at the lower pole with a size of 4.5 cm (pT1b) and Fuhrman nuclear grade 2. All margins were not involved by carcinoma, and there was no vascular invasion. He had been followed with a regular CT scan every year. He was found to have small bilateral lung metastasis and lymphadenopathy in 2016. The PET scan on April 26, 2016, revealed FDG activity in the lung and hilar and mediastinal lymph nodes. He underwent endobronchial ultrasound biopsy of the mediastinal lymph node which confirmed to be metastatic from clear cell RCC. Due to his comorbidities and mild thrombocytopenia, we started him on lower dose sunitinib at 37.5 mg per oral daily ×4 weeks every 6 weeks in May 2016. In total, he received 7 cycles of sunitinib. He had been followed every 6 weeks in the clinic. He only developed fatigue due to mild hypothyroidism for which he received levothyroxine. During the follow-up, he was found to have worsening thrombocytopenia with platelet counts in the range of 60,000 to 90,000. A follow-up CT scan and PET scan in October 2016 showed improvement of the lung metastasis and lymphadenopathy. He was last seen in the clinic on March 13, 2017.\nHe was admitted on March 29, 2017, due to muscle weakness, fatigue, poor oral intake, and difficulty swallowing for 2 weeks. During admission, his platelet count was found to be 13,000, serum creatinine 2.3, total bilirubin 4, AST/ALT > 2000, INR 2.9, calcium 7.5, creatine phosphokinase (CPK) > 5000, and uric acid 12 (see ). Sunitinib was discontinued on the first day of admission. CT head revealed no evidence of metastatic disease. Chest X-ray did not show evidence of infiltration or effusion. Echocardiogram showed severe global hypokinesia with LVEF of 30–35%. His LVEF was 55% prior to starting on sunitinib. He quickly developed lactic acidosis and acute respiratory failure. In the intensive care unit, he received bicarbonate, high-dose oxygen, furosemide, and treatment for hyperkalemia. Despite all treatment support, he continued to decline. His family chose to deescalate care, and he died on April 1, 2017. |
A 72-year-old Caucasian female patient was referred to the respiratory clinic with a 6-month history of exertional breathlessness. She gave a history of yearly chest infections, particularly in winter months. She, however, had no history of childhood respiratory problems or any significant respiratory symptoms during her adult life. She had a 30-pack-year smoking history but she quit 25 years prior to presentation. She worked as a nursing auxillary at a local hospital and had no prior asbestosis. She was on a 1 mg maintenance dose of prednisolone for polymyalgia rheumatica. Otherwise, she had no significant medical condition and was not on any regular medications. Her exercise tolerance was unlimited and she enjoyed a good health.\nHer O2 saturation was 95% on room air and her spirometry showed obstructive pattern with FEV1 1.14L (55%), FVC 2.09L (83%) and FEV1/FVC ratio 54%.\nAn initial chest radiograph 2 years prior to presentation suggested an increased radiolucency of the left lung but this was not investigated further (). Serial CXRs 2 years later showed a relatively unchanged appearance of the left hemithorax with progressively increased opacification in the periphery of the middle and lower zones, and accompanying reduction in lung volume, on the right side ().\nThe patient underwent a contrast-enhanced CT of the chest in view of the repeated chest infections and lack of complete resolution to rule out the possibility of an adenocarcinoma in situ or endobronchial lesion, respectively. Cross-sectional imaging was also carried out to assess the possibility of an underlying bronchiectasis in the presence of the recurrent chest infections and to further assess the interstitial changes seen on the CXR on the right side.\nHer CT showed hypoplastic left pulmonary artery (; arrow), attenuated peripheral pulmonary artery branches on the left (: arrows), hyperinflated left lung with air trapping and cystic bronchiectasis of the lingua and left lower lobe (). Features were in keeping with the diagnosis of Swyer-James-MacLeod syndrome (SJMS). Interestingly, the right lung was small in size with evidence of subpleural reticulation, traction bronchiectasis and ground-glass opacification suggesting an underlying pulmonary fibrosis (). The oligaemia of the left lung is shown as reduced FDG uptake () on PET CT, which was done to investigate a different pathology.\nSputum culture grew pseudomonas species and bronchoalveolar lavage from the right lung showed mixed cellularity with 22% eosinophils and a 36% neutrophils. |
The patient was a 3-year 7-month-old female referred by her speech-language pathologist, presenting with oromyofascial dysfunction characterized by speech sound production errors, difficulty swallowing, open mouth breathing, and noisy breathing during sleep. With respect to sleep, there were reports of difficulties going to sleep, waking up two to three times per night to drink water, getting up to go to the bathroom, open mouth breathing while asleep (), snoring during sleep, and sweating more than usual during sleep. She experienced wheezing that was associated with asthma, which was treated with an Albuterol sulfate inhaler. There were reports of difficulty with effective chewing. In addition, the patient would eat around 50% of her meals, before refusing the rest. She experienced chronic cough and recurrent upper respiratory tract infections.\nPhysical examination () of the patient showed her to be well developed, well nourished, and to appear the stated age. The patient was alert, oriented, able to communicate, and respond appropriately to questions. During the nasal examination, the nose had no external deformity. The nasal septum was straight, and the inferior turbinates were grade 2 bilaterally. There were class 3 dental occlusion and a class 3 facial-skeletal relationship characteristic of anterior-posterior maxillary deficiency. Oropharyngeal examination showed grade 3 modified Mallampati tongue position and grade 2 tonsils. The patient was found to have a restrictive class 2 upper labial frenulum with tethering of the upper lip () and restrictive grade 4 lingual frenulum [] ().\nBased on the patient history and the physical examination, the assessment revealed that the patient had functional and structural mouth breathing due to open mouth posture and low tongue posture in the setting of restrictive labial and lingual frenulum. The risks and benefits of lingual and labial frenuloplasty to facilitate lip closure and proper tongue resting posture were discussed with the parents and included, but were not limited to pain, inflammation, bleeding, scarring, need for revision surgery, and failure for significant improvement. An informed consent document was signed by the parents.\nThe maxillary labial frenuloplasty was performed under general anesthesia. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the maxillary labial frenulum via a 27-gauge needle. Pressure was applied lateral to the frenulum to locate the point of maximum tension. The maxillary labial frenulum was incised at the base of attachment with the use of sharp scissors. A V-to-Y lip closure was performed with a 4-0 chromic suture applied in a simple interrupted fashion ().\nThe lingual frenuloplasty procedure was then performed. A 2-0 silk suture was applied to the tip of the tongue as a retraction stitch. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the lingual frenulum via a 27-gauge needle. The tongue was retracted in the anteroposterior direction extending to the roof of the mouth and maxillary central incisors. Tension was applied to the floor of the mouth to protect the floor of mouth salivary glands, as well as Wharton's duct. A hemostat was used to clamp the restrictive lingual frenulum 5 mm above the attachments of the sublingual gland duct. The fibrous band was gently excised with the use of iris scissors. The underlying myofascial fibers of the genioglossus muscle were dissected further, with blunt cotton tips and sharp iris scissors being used to release the muscle from the overlying mucosa. The dissection was continued until there was adequate improvement to the tongue range of motion such that the tongue could extend up towards the maxillary central incisors in maximal mouth opening position. Simple interrupted sutures were used to close the diamond-shaped defect into a vertical line, as a means to lengthen the ventral tongue, with 4-0 chromic suture applied in a simple interrupted fashion. In total, the tongue was released from a grade 4 restricted range of motion to a grade 1 range of motion (). All wounds were hemostatic at the completion of the procedure. The patient was gently awoken from anesthesia and taken to recovery in stable condition.\nThe patient returned to the clinic four days after the procedure. The wound sites were healing appropriately, and there were no postoperative complications observed. The patient's mother reported that within the first day of returning home, the patient's issues with chewing had improved significantly, and she was more interested in eating different foods. In addition, her appetite appeared to have increased, and the patient would complete her entire meal before asking for more food (as compared to having only eaten around 50% before treatment). By the fourth day after surgery, the patient exhibited closed-mouth nasal breathing while asleep (). There were no longer any observed events of snoring and/or noisy breathing. The mother reported that the patient had remained compliant with myofunctional and speech therapy.\nThe patient returned for a 2-month postoperative visit. During the examination at this visit, no scar tissue was observed, and the wound sites had closed. Grade 1 tongue range of motion was observed ().\nThe patient's family wrote in a letter to the clinic approximately six months after the procedure, mentioning that the patient was doing very well with no complications. In addition, the patient had been reported to have completely stopped mouth breathing and snoring while asleep. However, the patient was reported to still have occasional episodes of cold and cough, as well as one episode of asthma exacerbation. Finally, the patient was reported by her family to have made progress with myofunctional and speech therapy, but the goals of eliminating the tongue thrust, achieving proper resting posture, and improving speech sound production errors were not met due to early discontinuation of treatment. |
A 60-year-old Caucasian male with medical history of psoriatic arthritis on adalimumab and methotrexate presented to his primary care physician with burning sensation in his feet and unintentional 14-pound weight loss over a period of 3 months. He did not have a history of diabetes mellitus or alcohol abuse. He had worked for a bank. His examination was notable for sensory impairment in the stocking distribution and diminished reflexes suggestive of peripheral neuropathy. He was referred to neurology for further evaluation of his neuropathy. As a workup for peripheral neuropathy, B12, thiamine level, serum protein electrophoresis, and thyroid function tests were checked and found to be negative. Nerve conduction studies and electromyography revealed sensorimotor polyneuropathy with axonal and demyelinating features.\nOne month later, he presented to the Emergency Department with episodic diplopia and ataxia. Episodes were characterized as brief (seconds to minutes) and happened multiple times a day, without any provoking factors. He denied any chewing or swallowing problems. He did endorse difficulty getting up from a chair as well as gait unsteadiness. He was alert and oriented with good fund of knowledge. Physical examination was significant for hypophonia, mild hoarseness, and dysarthria. His extraocular movements showed difficulty with vertical eye movements, worse with upward gaze with intact horizontal gaze. He was found to have dysmetria, which was noted in all extremities. His gait was wide-based and ataxic.\nGiven the patient's presentation of progressive neuropathy, ataxia and ophthalmoplegia, MFS variant of GBS was considered high in the list of differential diagnosis. Other differential diagnoses were CSF infection, given his relative immunosuppression with adalimumab and methotrexate, primary CNS neoplastic, and a paraneoplastic process. CJD was also considered, especially given the patient's family history. However, since the patient had no cognitive impairment, the possibility of CJD was deemed to be less likely.\nMRI brain with and without contrast [] was performed and read as unremarkable with no abnormalities on DWI. CSF analysis showed WBC of 0, RBC of 0, protein of 72 mg/dl (normal 15–45 mg/dl), and glucose of 65 mg/dl (normal 40–70 mg/dl). Serum and urine protein electrophoresis were normal.\nThe patient was started on plasmapheresis. In the interim, results of paraneoplastic antibodies, namely Anti-Hu, anti-neuronal nuclear autoantibody type 2 (ANNA-2), ANNA-3, amphiphysin antibody, Purkinje cell cytoplasmic antibody type-1 (PCA-1), PCA-2, collapsin response mediator protein-5, and anti-N-methyl-D-aspartate returned and were negative. Acetylcholine receptor antibodies, Lyme's titer, and Anti-GQ1b ganglioside antibodies were also negative.\nDuring his hospitalization, he had an episode of mild encephalopathy. Repeated EEG showed theta slowing of the background with no focal slowing or epileptiform activity. The patient's blood culture was found to be positive, and due to concern for infection, plasmapheresis was discontinued. The patient completed a total of four treatments with minimal improvement. He was discharged then to an inpatient rehabilitation (IPR).\nWhile at the IPR, his ophthalmoplegia worsened; he became severely dysarthric, and his mental status began to rapidly decline, which prompted readmission to the hospital. MRI was repeated and showed asymmetric restricted diffusion at the left caudate and anterior left putamen with mild cortical involvement in the left cerebral hemisphere (cortical ribboning pattern) []. These findings were highly suggestive of CJD.\nHis examination was significant for substantial cognitive impairment, severe dysarthria, and both vertical and horizontal gaze ophthalmoplegia. He was noted to have ataxia and diffuse rigidity in all extremities. Myoclonus was noted to be absent. Repeated CSF studies revealed positive 14-3-3 proteins, positive real-time quaking-induced conversion assay (RT-QuIC), and total-tau (t-tau) protein with a level of 5243 pg/ml (normal range 0–1149) []. Based on the results, the estimated probability of prion disease, CJD in this case, was deemed >98%. Follow-up EEG study revealed diffuse slowing with occasional triphasic waves. However, the characteristic periodic sharp discharges classically described in CJD were not present [].\nThe patent continued to decline. He had a repeated MRI, which showed the further progression of the restricted diffusion signal abnormality []. He passed away approximately 14 weeks after his initial presentation to the neurologist. Genetic testing and autopsy were declined by his family. |
A 64-year-old man who had no symptoms was diagnosed with thoracic superficial esophageal cancer that was detected by screening upper endoscopy. He had a history of hypertension. He had also been found to have a vascular abnormality (DAA) as an adult and was observed in an asymptomatic state.\nPhysical examinations showed no unusual findings, and the laboratory examination data, including tumor markers, such as squamous cell carcinoma-related antigen and carcinoembryonic antigen, were all within normal ranges. Chest X-ray demonstrated a widening in the upper mediastinal silhouette, reflecting the superior right aortic arch. An endoscopic examination revealed superficial esophageal cancer located in the left side of the wall in the upper thoracic esophagus and the invasion of the submucosa (Fig. ). A histological examination of biopsy specimens confirmed the presence of squamous cell carcinoma. Enhanced computed tomography showed a swollen lymph node in the right upper mediastinum, which was diagnosed as metastatic (Fig. ). No distant metastasis was detected. Computed tomography also confirmed the DAA. The right aortic arch was dominant, and the descending aorta was located at the right side of the post-mediastinum, as is common in cases of DAA (Fig. ). The patient was therefore diagnosed with upper thoracic esophageal cancer of cT1bN1M0 Stage IIB (UICC-TNM 7th) and a DAA.\nHe underwent neoadjuvant chemotherapy prior to sub-total esophagectomy with three-field lymphadenectomy. The neoadjuvant chemotherapy regimen was 2 courses of 5-FU (800 mg/m2) and cisplatin (80 mg/m2) every 3 weeks.\nWe planned to perform radical subtotal esophagectomy with three-field lymph node dissection after neoadjuvant chemotherapy. We first planned to perform cervical procedure in a supine position before the thoracic procedure in order to identify the bilateral inferior laryngeal nerves and avoid causing them injury or inducing palsy. We also planned to perform upper mediastinal lymph node dissection during this preceding procedure because the DAA was expected to interfere with upper mediastinal dissection attempted via either side of a transthoracic approach. We then planned to perform lymph node dissection via a left-thoracoscopic approach below the left aortic arch, as we worried that the right-sided descending aorta might interfere with a right-thoracic approach (Fig. ). The laparoscopic procedure was planned to be performed via an abdominal procedure in a supine position. Reconstruction would use the gastric tube pulled up via the retrosternal route with cervical esophago-gastric anastomosis.\nIn the preceding cervical procedure performed in a supine position, we identified the bilateral inferior laryngeal nerves, which were thought to be recurrent at each side of the aortic arch (Fig. ). After upper mediastinal dissection was performed, the left thoracoscopic procedure in a prone position was performed for middle and lower mediastinal lymph node dissection below the left aortic arch. We first confirmed that the right-sided aortic arch and descending aorta would interfere with the usual right thoracic approach (Fig. a). Upper mediastinum dissection was also deemed impossible via a bilateral thoracic approach because of the bilateral aortic arches and subclavian arteries, as expected preoperatively (Fig. a, b). Postmediastinal reconstruction also seemed impossible. The port position for the left thoracoscopic procedure was set symmetrically to our normal right thoracoscopic procedure for middle to lower mediastinal dissection, as shown in Fig. . No major anatomical findings other than those noted preoperatively were observed during the left thoracoscopic procedure. We were unable to identify where the thoracic duct ascended because of the preservation of the thoracic duct. We were also unable to confirm the details concerning both recurrent laryngeal nerves around each aortic arch.\nThe abdominal procedure in a supine position was performed laparoscopically with the simultaneous cervical procedure for bilateral supraclavicular lymph node dissection. Reconstruction was performed with cervical esophago-gastric tube anastomosis. The gastric tube was pulled up through a retrosternal route as planned. Three-field lymph node dissection and complete resection (R0) were achieved. The operative time was 8 h 9 min, and the total bleeding was 70 ml. No vocal cord palsy was observed on flexible laryngoscopy after the operation.\nThe patient’s postoperative course included minor leakage that was cured conservatively after 2 weeks, and he was discharged at postoperative day 29. The pathological diagnosis was ypT1bN0M0 Stage IA (UICC-TNM 7th edition). The patient was followed for 2 years with no signs of cancer recurrence. |
A 54-year-old male worker was brought to our emergency room. He had been knocked down by a falling object while working in a construction site. On arrival his Glasgow Coma Scale was 9/15 with blood pressure of 80/50 mmHg, pulse rate of 88/min, and respiration rate of 26/min. His physical examination disclosed diminished right breath sound. Laboratory examinations revealed gross hematuria and anemia (hemoglobin level: 8.3 g/dL). Because his peripheral oxygen saturation was 90%, he was immediately intubated.\nHis chest X-ray showed a massive right hemothorax, which was treated with tube thoracostomy. Multi-detector computed tomography (CT) of his chest and abdomen showed disruption of the right diaphragm, superior diaplacement of the liver into the thorax, active bleeding from the left kidney with perirenal hematoma, nonvisualization of the right kidney in its anatomical position with its dislodgement into the thoracic cavity, and right renal artery and vein obstruction with thrombus (). Left renal angiogram revealed hemorrhage from the upper polar branch of the left kidney, and the bleeding vessel was embolized with coil. Other associated injuries were multiple fractures of the ribs, the right humerus shaft, the right 4th and 5th metacarpal bone, and the spines.\nThrough right posterolateral thoracotomy, we identified the liver herniated into the thorax. The right diaphragm had a circumferential tear of approximately 25 cm in length along the whole periphery of diaphragm except the medial aspect. The right kidney was found lacerated in the upper thoracic cavity, separated from its vascualr pedicle and ureter (). Because the renal vasculoureteral stump could not be found in the retroperitoneal space and there was no evidence of active bleeding, ligation of the stump was not attempted. We restored the liver into the abdominal cavity and repaired the diaphragm using interrupted non-absorbable sutures.\nHe was extubated 4 days after surgery. A few days later, pneumonia developed. His antibiotics regimen was changed, and new antibiotics were administered for 2 weeks. The patient gradually recovered with no further events. |
A 75-year-old woman had a large blue patch on the right shin for 3 years. She underwent a left total knee replacement 18 years ago and a right total knee replacement 14 years ago due to severe osteoarthritis of both knees. Each of the operations was carried out successfully without any serious adverse events. Eleven years after the right total knee replacement, she noticed a discoloration of her right shin, and the lesion continued to enlarge slowly over time. She was referred to a dermatology clinic where a large, well-demarcated bluish-gray patch was noted on her right shin. The surface of the skin was smooth with no palpable infiltration. For 1 year, she experienced pain when walking on the right shin. She had a 10×15 cm well-demarcated bluish-gray patch on the extensor surface of her right shin (). Routine laboratory tests, including complete blood count, liver function tests and urine analysis, yielded normal findings. Histopathologic findings from the skin lesion showed perivascular, fine black particles in the dermis (). The black particles were stained positive with both melanin and demelanin stains. Polarizing microscopy of the skin specimen revealed refractile foreign materials in the perivascular area of the dermis (). Further, energy-dispersive X-ray spectrophotometry revealed the presence of titanium and aluminium (). Metallic discoloration was diagnosed on the basis of clinical, histopathological, polarizing microscopy and energy dispersive X-ray spectrophotometer findings. The radiographs revealed loosened and dislocated tibial components of the right knee prostheses. Preoperative and intraoperative cultures were negative for bacteria. During surgical revision, marked loosening of the tibial component and wearing of polyethylene were found. Additionally, an intense black staining of a substantial amount of wear debris adjacent to the tibial components and tibial bone defect was observed (). The patient was treated by debridement, and the right tibial component was replaced. After the revision procedure, metallic discoloration disappeared and has not reoccurred over the past 4 years. |
The patient is a 41-year-old female with a past medical history of a living-unrelated renal transplant for end-stage renal disease due to focal glomerulosclerosis. During evaluation for pyelonephritis, a CT scan found multiple non- cystic liver lesions in both lobes. Her medications were significant for maintenance immunosuppression, consisting of tacrolimus 1 mg, cellcept 500 mg, prednisone 5 mg. She denied any history of hepatitis, OCP use or diabetes. Her BMI was 26. She was referred to the hepatobiliary clinic where her initial work-up consisted of lab work, colonoscopy, ultrasound and MRI. Her AFP and CEA were normal at 2.3 and 0.8, respectively. Her colonoscopy was normal. An ultrasound revealed multiple echogenic round lesions in both lobes with no ductal dilatation. An MRI showed ~50 hyper-intense lesions measuring up to 1 cm in both right and left lobes (Fig. ). A percutaneous liver biopsy was found to be normal hepatic parenchyma. She was managed expectantly for a year until a follow-up MRI showed an increase in size and number of her liver lesions, some of which contained a fatty component.\nGiven the sheer number of her lesions, a multidisciplinary group at our institution decided that a tissue type and specifically a genetic type diagnosis was required to rule out her risk of malignant transformation. A laparoscopic left lateral wedge resection was thus performed in order to obtain adequate tissue for diagnosis. The patient tolerated the procedure well and was discharged from the hospital after a 3-day hospital stay.\nPathological analysis showed well circumscribed, but non-encapsulated liver lesions, comprised of cords of benign hepatocytes (Fig. a). The hepatocytes had regular, uniform nuclei and a low nuclear to cystoplasmic ratio. The lesions lacked portal structures and had evidence of unpaired arteries. Moderate microvesicular steatosis was noted within the lesions but none within the uninvolved liver parenchyma. Mitoses or pseudoacinar changes were not identified (Fig. b). Immunohistological analysis showed no liver fatty acid binding protein (LFABP) staining (Fig. c) and had no nuclear localization of β-catenin (Fig. d). These characteristics were consistent with hepatocellular adenoma, HNF1A type. |
An 18 year old male presented with pain in his right upper arm after trying to put out a fire in his hand in June 2005. On physical examination, tenderness, swelling, angulation, and crepitus on right upper arm were noticed. Radiographs of the arm revealed a humeral shaft fracture with intramedullary patchy signs of osteoporosis []. After correlating with history, physical examination and radiographs, a diagnosis of pathologic fracture shaft humerus was made. Bone scan and magnetic resonance imaging (MRI) of the right arm were then performed. Bone scan revealed mild nonspecific uptake intensity on shaft of humerus []. No other skeletal abnormalities were noticed on the scan. On MRI, T1-weighted image showed medium intensity at the bone marrow, and high signal intensity at the same area was shown on T2-weighted image. Findings also revealed cortical bone erosion and heterogeneity around fracture lesion with multiple bone absorption []. MRI findings suggested avascular tumor such as hemangioendothelioma or hemangioma. Blood parameters such as calcium, phosphate, alkaline phosphatase, osteocalcine, and calcitonin were all within normal range. Rheumatoid factor was negative. Patient then underwent the curettage with biopsy with open reduction and internal fixation using Limited Contact Dynamic Compression Plate (LC-DCP, Synthes©, Paoli, [PA], USA) on June 2005. Intra operative findings revealed a fibrotic mass located in the medullary cavity and signs of cortical thinning. Histopathological result was chronic inflammation with vascular proliferation. Therefore, he wasn’t diagnosed as Gorham's disease. 7 months postsurgery, the patient consulted the emergency room complaining of right upper arm pain that occurred while trying to stand up after leaning on his right side. Radiographs showed enlarged and new developed radiolucent areas suggesting a nonunion fracture of the humerus. A periprosthetic fracture on the distal plate was also noted []. On January 2006, the patient underwent removal of implant and resection of the osteolytic lesion. Dual onlay bone graft using autologous tibia and iliac cancellous bone was performed at the bone defect. And open reduction and internal fixation was performed using intramedullary nailing (AO-UHN, Synthes©, Paoli, [PA], USA) []. Histopathologic findings revealed numerous proliferating blood vessels with different capillary sizes anastomosed as hemangiomatosis and lymphangiomatosis are causing cortical bone destruction []. The patient's history, clinical symptoms, radiologic and histopathologic findings were all correlated and therefore, diagnosed as Gorham's disease. After 2 weeks postsurgery, intravenous zoledronic acid infusion was started once a month at a dose of 4 mg for a duration of 6 months. Followup radiographs were taken after 6 months postsurgery and showed massive osteolysis of humerus, and grafted bone resembling a “licked candy stick” deformity []. Wide resection and temporary cement material filling of the implant were done for the bone gap and subsequently functional range of motion started. Postoperatively, radiation therapy was started at a single dose of 2.0 Gy (20 cycles) for the duration of 4 weeks with a total dose of 40 Gy. After 1-year postsurgery, radiographs showed no evidence of osteolysis and bone resorption []. Removal of the intramedullary nail and cement material, and insertion of an autogenous vascularized fibular graft was performed on his fourth operation []. Postoperatively, zoledronic acid was started using the previously given regimens. Radiographs of the patient 7 years postsurgery and 10 years after the first surgery showed evidence of bone union and no signs of osteolysis []. |
This 54-year-old female was referred to the Neurofibromatosis outpatient clinic of the Oral and Craniomaxillofacial Surgery Department for treatment of numerous cutaneous tumors that preferentially covered her trunk and extremities. On admission, the patient described the tumors to be painless and disfiguring. Besides the cutaneous tumors the patient sought advice for treatment of a painful region of her left small finger (Figure 1 ). For some time the ulnar side of the distal phalanx of this finger had become very touch-sensitive. There was neither a tumor visible nor any other pathological alteration of the skin. However, the finger could not be examined by palpation. The suspected diagnosis was initially a glomus tumor [] or a non-palpable nodular PNF.\nMRI at 3T revealed numerous, popcorn-like, contrast-absorbing, hyperintense formations on T2-weighted images, up to 1.2 x 1.9 cm² in size located on the palmar side of the left hand (Figure 2 ). These small lesions mainly affected the metacarpal bones II and III. MRI also showed a sharply defined lesion of max. 1 cm in diameter in the subcutaneous layer at the level of the radiocarpal joint and distal to first digit that reached to the tendons of the extensor musculature. The bony structures showed a homogeneous signal. The findings were interpreted as disseminated subcutaneous and cutaneous neurofibromas of the entire left hand. Structures were found on the distal phalanx of the left small finger whose intensity pattern corresponded to that of the other lesions.\nDuring surgery for the excision and vaporization of numerous neurofibromas of the trunk and extremities, the pain sensitive finger region was also explored. After incision of the skin, no tumor was visible. Therefore, a circumscribed subcutaneous excision was taken as a tissue sample and examined histologically.\nThe tissue findings confirmed cutaneous neurofibromas for the numerous nodules excised from the trunk and extremities. Surprisingly, there was no evidence for a glomus tumor in the tissue sample of the finger. Smaller neurofibromas were differentiable in this tissue sample. Within the corium as well as in the subcutaneous adipose tissue there were some clustered Vater-Pacini (VP) corpuscles. Next to the corpuscles several small nerve fibers were present. Diagnosis of VP neuroma was made (Figure 3 ).\nThe nerve fibers were positive for S100 and neurofilament. S100 immunoreactivity was especially strong in the center of the corpuscle. The perineurium of the nerve fascicles and the lamellae of the corpuscles expressed epithelial membrane antigen (EMA).\nThe healing process of the wounds was inconspicuous. The patient noticed that the sensations of pain had diminished significantly in the area of the small finger soon after surgery. |
A 17-year-old Caucasian female presented to the emergency department with the complaint of left forearm pain. She initially noted paresthesia of the left hand and then left forearm pain. The pain had been progressive over the previous 10 hours and was exacerbated with flexion or extension of the wrist. She could not recall any injury or inciting event. She was otherwise healthy and without any significant past medical history. Her family history was unremarkable with the exception of her mother’s brother who died of Duchenne muscular dystrophy. Her mother had been tested for Duchenne carrier status and was negative. Her only medication was an oral contraceptive. She denied the use of tobacco, alcohol, and illicit drugs. Her initial vital signs were an oral temperature of 36.8°C, pulse of 76 beats per minute, blood pressure of 144/96 mm Hg, respiratory rate of 16 breaths per minute, and room air pulse oximetry of 100%. The physical examination of the left forearm revealed it to be firm and tender to palpation, and it measured 2cm larger in circumference than the right. Her radial pulse was palpable and her fingertip capillary refill was less than two seconds. The skin appeared normal without overlying erythema or calor. The patient experienced pain with flexion or extension of the digits and wrist. No sensory deficits of the hand or forearm were noted. Her initial laboratory evaluation showed the following: leukocytosis of 15,300 10(3)/mcL, blood glucose of 93mg/dl, elevated creatine phosphokinase of 15, 750U/L, normal erythrocyte sedimentation rate, and slightly elevated c-reactive protein at 1.27mg/L. No coagulation studies were obtained. A radiograph of the forearm was obtained and demonstrated only soft tissue swelling. Venous and arterial duplex studies of the extremity were performed. The arterial duplex demonstrated no evidence of arterial thrombosis, stenosis, or occlusion. The venous studied showed normal flow and compressibility, but the waveforms were continuous. Compartment pressures were measured in the volar and dorsal compartments of the forearm with a Stryker Needle™ (Stryker Intra-Compartmental Pressure Monitor, Stryker Corporation, USA) and found to be 86mmHg and 62mmHg, respectively. The patient was taken to the operating suite and underwent an emergent 10cm long fasciotomy of both the volar and dorsal compartments of her left upper extremity. Intraoperative cultures were obtained and showed no growth. Fortunately, the patient required minimal debridement. Total hospital course was four days. A precipitating cause could not be identified. The patient’s fasciotomy sites were surgically closed and she was discharged home with a diagnosis of idiopathic compartment syndrome. At 18-month follow up, the etiology of the compartment syndrome has not been identified, although she has been found to have a chronically elevated creatine phosphokinase (1,500U/L). |
A 53-year old female was admitted to the Intensive Care Unit of Kingston General Hospital in Kingston, Ontario, Canada with diabetic ketoacidosis (DKA). She had a past medical history of type 1 diabetes with recurrent DKA and end stage renal disease secondary to diabetic nephropathy on intermittent hemodialysis. Prior to admission, she was being dialyzed through a left arm arteriovenous fistula.\nShortly after admission, her left arm fistula thrombosed, and a right internal jugular (IJ) tunneled double-lumen catheter was inserted for dialysis along with a peripherally inserted central catheter (PICC) via right basilic vein for intravenous access. The patient had a previous failed right arm AV fistula so the left arm was avoided in hopes of future recovery of the left AV fistula.\nTwo weeks later during the course of her admission, she continued to have episodes of DKA, and inadvertently her PICC line was dislodged, requiring removal. The patient had poor peripheral intravenous (IV) access and multiple attempts to insert peripheral IVs under ultrasound guidance were unsuccessful. Ultrasound examination of the patient's left internal jugular showed significant narrowing. The patient declined an attempt femoral venous access because she had previous lower extremity venous grafting for her AV fistula and she was told to avoid cannulization of any of those vessels. A 7 French, 16cm triple-lumen CVC was placed into the right internal jugular vein with sonographic guidance without difficulty. The puncture site for the CVC insertion was significantly distal to the tunneled dialysis catheter. Post-procedure, all three lumens were able to draw blood and flush saline. A chest X-ray confirmed placement of the catheter adjacent to the tunneled dialysis catheter () close to the cavoatrial junction. The patient did not have any immediate complaints or discomfort. Approximately six hours later, the on-call resident was called by nursing staff to assess the patient for new neck discomfort and jaw pain which had begun two hours prior.\nAt the time of assessment, the patient's heart rate was 95 beats per minute and regular. Her blood pressure was 188/84, respiratory rate was 18 and oxygen saturation was 99% on room air. She was afebrile. On examination, the patient had distended neck veins, pronounced facial edema and plethora (). She also had bilateral upper-extremity edema primarily manifesting in her digits. The rest of her cardiorespiratory examination was unremarkable.\nAll three lumens of the CVC were withdrawing blood and flushing without difficulty.\nPoint of care ultrasound of the right internal jugular vein revealed no thrombus at the site of CVC entry or distal to it. The SVC was not visualized directly. The CVC was removed promptly. There was no visible clot on the catheter, and no clot was pulled along with the catheter. Within an hour, the patient's facial swelling and plethora began to resolve, and her neck pain began to improve. The next morning, pain and swelling had resolved completely ().\nA retrospective chart review revealed a fistulogram performed months prior that showed significant stenosis at the left subclavian vein and mild narrowing at the junction where the two brachiocephalic veins merge to form the SVC (). At that time, angiography was attempted to alleviate the stenosis. However, the patient did not tolerate this, and the procedure did not lead to a significant change in the degree of stenosis.\nThe events surrounding the SVC syndrome and the previously identified SVC stenosis were disclosed to the patient. In order to prevent a similar event from occurring in the future, a note was made in the chart that would be more visible should the need for further CVCs arise. |
A 52-year-old nondiabetic female presented with progressive swelling, pain, and limited movement in her left shoulder joint combined with asthenia of her left upper extremity for 3 months. Accordingly, she was hospitalized for diagnosis and treatment. The patient denied experiencing neck pain, steeping on cotton, unstable walking and muscle atrophy. A review of her personal and family medical history was unremarkable. In addition, it was noted that she was not taking any medication.\nA physical examination revealed that lateral flexion and extension of the cervical spine was slightly painful and limited at the end of movement. Her left shoulder abduction was recorded as 50°, flexion 90°, internal rotation 30°, and external rotation 40°, all of which were recorded as painful for the patient in all directions. There was swelling in the left shoulder joint, and the left upper extremity was swollen compared with the right. Interestingly, Spurling's test was negative. A magnetic resonance image (MRI) of the left shoulder showed left shoulder dislocation, downward displaced humeral head, and irregular glenoid shape. The left shoulder joint cavity contained corpus liberum and effusion (Fig. ).\nOn neurological examination, left and right upper extremities were observed to have a muscle strength of grades 4 and 5 according to the muscle strength testing scale, respectively. Tendon reflexes were hypoactive in the patient's left upper extremity. Moreover, the sensation of pain and temperature in the left hand, upper extremities, and shoulder were decreased. Sensation of deep pain was normal, and joint position sense was preserved in bilateral upper extremities. Pathologic reflex was absent. Complete blood count, biochemical analysis, C-reactive protein, rheumatoid factor, erythrocyte sedimentation rate, and tumor markers were normal.\nA MRI) of the cervical spine revealed a syringohydromyelia cavity extending from C2 to T4 vertebral levels (Fig. ). This odontoid tip is above the Chamberlain's line (a line traced from the posterior margin of the hard palate to the dorsal margin of the foramen magnum) in 6.0 mm (Fig. ), which the diagnosis of basilar impressions is clear.\nIn the light of the clinical, laboratory, and imaging findings, the case was diagnosed as the NA of the shoulder associated with syringomyelia and BI.\nAccordingly, a surgical treatment plan was formulated to solve the patients’ disease. The patient was not observed to have atlanto-occipital dislocation (AOD). As such, a posterior fossa decompression (PFD) was conducted. The patient was administered general anesthesia and placed in a prone position. Their skin, subcutaneous tissues, and occipital were dissected with a midline incision extending from the occipital protuberance to the C2 spinous process. The inferior part of the occipital bone and the posterior lamina of C1 were removed to achieve a bony decompression (approximately 3 cm × 2 cm). Next, a thick Atlas pillow fascia, compressed the dura and was then removed. Therefore, the dura mater of the AOD restored the pulsation. Finally, the outer layers were sutured step-by-step to achieve anatomical reduction.\nAfter operation, the pain of left shoulder was relieved, but the other symptoms have not improved. At the 6-month follow-up, the asthenia of her left upper extremity was also improved and the patient felt clinically well. The physical examination revealed that her left shoulder abduction was recorded as 90°, flexion 90°, internal rotation 50°, and external rotation 50°, all of which were improved postoperatively. MRI at this time showed adequate foramen magnum decompression and reduction of the size of syrinx (Fig. ). |
The patient was a 23-year-old woman charged with attempted manslaughter. She was ordered by the court to undergo a pre-trial forensic psychiatric examination which took place in a maximum security state mental hospital. The trial records and all available background information were reviewed. Both of her parents were alcoholics and she was taken to protective custody under the age of one year. Her grandmother suffered from schizophrenia and her mother had been in a psychiatric hospital after a suicide attempt. The patient used to shoplift before the age of 15 and later she stole money from time to time. She also worked as a prostitute to earn money. She started to abuse alcohol at the age of 15. She had several boyfriends but has never married and has no children. Her personality was noticed to change before the age of 17. She started to have obsessive-compulsive behaviour, paranoid thoughts as well as depressive symptoms. She tried to kill herself by hanging and by several drug intoxications. She brutally killed her own pet. She was never hospitalized before the forensic mental examination but she irregularly visited an outpatient clinic. Despite psychiatric treatment, she impulsively tried to kill her male friend with whom she was drinking. During the psychiatric examination the diagnoses – schizophrenia paranoid type, alcohol dependence and features of antisocial personality disorder – were made by a senior forensic psychiatrist using the structured clinical interview for DSM-IV, SCID I and II [,]. Neither waking EEG nor brain MRI (1.5T) disclosed any abnormality. She had no somatic disorders. She had finished high school and vocational school, and was within average intelligence (WAIS-IQ total 109). She was not sentenced, but was ordered by the Finnish National Board of Medico-Legal Affairs to stay in the state mental hospital as a criminally insane patient. The sleep recordings were performed during the psychiatric examination period and the patient was completely medication-free and had abstained from alcohol and drugs for six months.\nThe control group consisted of three 23-year-old female students without criminal records or a history of physical violence. They were healthy with no signs of somatic, psychiatric, or neurological disorders. As part of a psychiatric interview, the SCID-non-patient version [] was filled in. To exclude general diseases that could affect sleep, blood tests (including serum prolactin, thyroid function, kidney and liver function) and electrocardiograms were taken and they were within normal range, both in the patient and in controls. No history of alcohol abuse or dependence was detected in controls. The controls were asked to avoid alcohol, drugs or medication two weeks prior to the sleep examinations. Caffeine and nicotine consumption was neither restricted nor recorded.\nWritten consent was obtained from all participants after the study procedure had been fully explained to them. The study was approved by the local human ethics committee.\nPolysomnography (PSG) was recorded over two consecutive nights but only the second night was considered for the study. The study patient slept in a single room within the department, while controls slept in the hospital guest room. All participants were allowed to sleep as long as they wanted to. Recordings took place on an ambulatory basis; the participant had a portable recording device (Embla, Flaga hf, Reykjavik, Iceland) that was connected to the recording electrodes. The recordings were performed using the standard Rechtschaffen-Kales method []. The high-pass filter was 0.5 Hz and the low-pass filter 45 Hz, with a sampling rate of 100 Hz. Commercial software (Somnologica, version 2.0, Flaga hf, Reykjavik, Iceland) was used for scoring and calculation of sleep parameters. Sleep onset was defined as the first occurrence of three consecutive epochs (90 sec) of stage 1 (S1) or other sleep stages. The following parameters were calculated: time in bed, sleep latency, sleep period (time in bed – sleep latency), wake after sleep onset, total sleep time (sleep period – wake after sleep onset), sleep efficiency (total sleep time/sleep period), number of awakenings, REM latency and percentage amounts of different sleep stages (S1–S4 %, REM %). All data for the analysis were scored by the same scorer (NL), not blinded to the patient group.\nA sleep diary was used for one week during the study period to ensure a normal sleep-wake rhythm and to exclude the effects of daytime naps. The participant filled in the time of retiring to bed, estimated time of falling asleep and time of awakening in the morning for each consecutive night as well as daytime naps. |
A male, 4-year-old, was admitted to our hospital with persistent respiratory symptoms for three consecutive years. On his physical examinations during three consecutive years, the patient's SpO2 was 98% in the first checkup; after that, his SpO2 gradually reduced to 90% in room air, and clubbing of the fingers has gradually occurred during the same time. His vital findings of blood pressure, heart rate, and respiratory rate were normal, both lungs were equally ventilated, and mild wheezing existed in the lungs. Chest X-ray pointed out bilateral infiltrations in the lungs; a prominent hilum was discovered. There was no evidence suggestive of a focal air space disease. Transthoracic echocardiogram (TTE) demonstrated that the left atrium was expanded and had the fibromuscular membrane, which divided the left atrium into two chambers (). The interatrial septum was intact. RV systolic pressure was measured to be 50 mmHg on echocardiography. Transesophageal echocardiography (TEE) was performed to have a better view of the membrane. TEE revealed cor triatriatum had two fenestrations: one with a width of 2 mm and the other with 3 mm diameter. A feneration had a maximum pressure gradient by continuous-wave Doppler of 20 mmHg (). To be able to evaluate the patient's pulmonary vascular bed, a heart catheterization was performed. Right atrial pressure was a mean of 6 mmHg. The pulmonary artery pressure was 60/24, with a mean of 38 mmHg. Right ventricular pressure was 60/6 mmHg.\nFurthermore, a selective pulmonary angiography was performed in addition to cardiac catheterization to evaluate the patient's pulmonary venous drainage and the pulmonary bed. The results were extremely intriguing. The angiography showed the late opacification of drainage veins connecting to the other pulmonary vein from the different segments of the left lung (). The angiography disclosed the mimicking of pulmonary venous stenosis/obstruction due to the existence of restrictive cor triatriatum sinister. There was a diffuse PAVM in the upper part of the left lung as well. Contrast echocardiology (CE) was performed with a manual injection of 10 mL of agitated saline. Pulmonary AVM diagnosis was confirmed by the appearance of bubbles in the left atrium after more than three cardiac cycles after the first bubble was detected in the right atrium. Also, the patient's thorax CT was evaluated as PAVM after the cardiac catheterization.\nAlso, the patient continued to have pulmonary venous hypertension and cor triatriatum sinister with restrictive fenestration. The patient's dilemma was discussed in a council. The council was composed of pediatric cardiologists, pediatric cardiovascular surgeons, and thoracic surgeons. The final decision was to perform a surgical membrane resection to follow PAVM. After the surgery, on his first postoperative follow-up time, pulmonary artery pressure was reasonable and PAVM completely resolved. |
A 46-year-old woman (gravida 2, para 2) was referred to our hospital complaining of a lower abdominal mass and pain. Her medical history was unremarkable. She was initially diagnosed with a uterine leiomyoma by transcervical needle biopsy. CT revealed a large heterogeneous tumor occupying the pelvic cavity and an intravascular tumor within the dilated left internal iliac and ovarian veins (Figures and ). Her preoperative cervical cytology results were negative for intraepithelial lesions and malignancy. The endometrial cytology and needle biopsy results were also negative. Thus, the preoperative diagnosis was IVL, with extension of the tumor into the left internal iliac and ovarian veins.\nIntraoperatively, multiple myomas were found within the uterine corpus and cervix, and the tumor extended to the parametrium and paracolpium. Detachment of the tumor from the left ureter and vaginal wall was very difficult. Intravenous tumors in the left internal iliac and ovarian veins could be palpated. The left internal iliac vein forming the common iliac vein was transected at the bifurcation region. In addition, TAH and bilateral salpingo-oophorectomy (BSO) were performed, resulting in the complete surgical resection of the tumor (operative time, 11 hours; blood loss, 8462 g). The resected uterus and adnexa weighed 897 g (Figures and ). There was no residual tumor detected in the venous resection stump.\nThe nodule resected from the uterus and the internal iliac and ovarian veins consisted of a proliferation of spindle cells. There was no nuclear atypia and the mitotic index was low. In addition, vessel endothelium cells and a vascular smooth muscle layer covered the IVL (Figures and ). The tumor cells stained positive for Alcian blue (pH = 2.5) and the staining disappeared after hyaluronidase digestion. However, compared to that in Case 1, the intensity of the staining was weaker and less diffuse (Figures and ). Similar findings for hyaluronan expression were obtained using the sample retrieved from the preoperative needle biopsy.\nThe histopathological diagnosis of the uterine and intravascular tumors was IVL. There has been no evidence of IVL recurrence, with the most recent follow-up at 38 months postoperatively. |
An 82-year-old elderly female with a history of severe right-sided TN was previously treated unsuccessfully with numerous medications and percutaneous rhizotomy. She was referred to the radiation oncology clinic for consideration of SRS after neurosurgical evaluation.\nThe patient’s right-sided facial pain began initially in the 1980s, but she reported a long period of disease quiescence until symptoms recurred in 2007, one year after suffering a right cerebral hemispheric embolic stroke. At that time, her TN was treated with carbamazepine with a good initial response, but in 2010 this was discontinued due to an increasing dose requirement associated with the development of intolerable side effects. Multiple alternative medications were prescribed including gabapentin, Lyrica, fentanyl transdermal patches, and sumatriptan, without significant improvement of her pain.\nIn December 2014 and January 2015, she underwent percutaneous rhizotomy with radiofrequency ablation to her right trigeminal nerve, but despite these interventions, her facial pain persisted. In February 2015, she underwent brain magnetic resonance angiography/magnetic resonance imaging (MRA)/(MRI) to include her right internal auditory canal. Imaging demonstrated a right superior cerebellar artery (SCA) loop abutting CN V, causing a mass effect on the nerve near the root entry zone (REZ). The patient was subsequently referred to neurosurgery for possible surgical intervention. After neurosurgical evaluation, due to her age and multiple medical comorbidities, she was not considered an ideal surgical candidate for MVD and was referred to radiation oncology for consideration of SRS.\nThe patient was initially seen by Radiation Oncology in March 2015. During consultation, she described her facial pain as unilateral located on her right side and spanning from her lip to her temple region near the auricle. The pain episodes were characterized as sharp, intermittent, unpredictable, and severe. On average, her pain was 6/10 with breakthrough periods of 8/10. The pain was associated with chewing/biting, swallowing, light touch, and palpation of her right eyelid or nose. She reported that her symptoms were significantly affecting her quality of life. Her medication regimen consisted of Lyrica and Fentanyl patches, with hydromorphone for severe discomfort. On physical examination, sensory abnormalities were noted, specifically tenderness to light palpation on the right side of her face. After her assessment, she was considered to be a good candidate for SRS, especially given her history of classic TN refractory to medical therapy and her comorbidities.\nIn July 2015, the patient was simulated for treatment. Computed tomography (CT) was performed using .75 mm thick slices. At that time, a custom fitted SRS immobilization mask was created (Figure ). Conical collimator planning was utilized using Brainlab iPlan RT planning software, which utilizes a pencil beam algorithm. An MRI was also performed prior to treatment and fused with the CT scan.\nIn August 2015, she underwent frameless cone collimator radiosurgery targeting the right trigeminal nerve, specifically the dorsal root entry zone (DREZ) (Figure ).\nPrior to receiving SRS, she was premedicated with 6 mg of oral Decadron. She was set up on the treatment table in the supine position, and her custom immobilization mask was applied (Figure ). She subsequently received 88 Gy prescribed to a single point dose with the 30% isodose line (IDL) abutting the brainstem (Figure ). A volume of .019 cc of the nerve received at least 54 Gy.\nDelivery of her radiation was completed using 6 MV photons on Varian’s TrueBeam, a D-LINAC treatment system. Seven arcs (SAD setup) were used during treatment totaling ~21,000 MUs at a 1,200 MU/min rate (couch angles: 0, 25, 50, 75, 280, 310, and 335 degrees). A 4 mm cone shaped the beam leaving the accelerator. Image guidance was performed using the Brainlab Novalis ExacTrac® system, which utilizes orthogonal x-rays to verify positioning at each treatment position. Her treatment was delivered over a period of one hour. Overall, the patient tolerated the procedure well without complaints. No anxiolytics were required during treatment.\nAt her two-month follow-up appointment, she reported that her facial pain had remitted within one week of treatment. She denied any facial pain, dry or painful eyes, and no sensation changes in the CN V region. The physical examination was normal with no CN deficits noted. She continues to report no facial pain without the need for pain medication six months after her treatment. A follow-up brain MRI is pending. |
A 36-year-old man who denied previous systemic disease had a history of drug abuse with ketamine for 6 to 7 years (at a frequency of 2–3 times per week, by nasal inhalation, and hence the dosage could not be measured), and had then ceased use for approximately 4 years.\nHe had suffered from dysuria, bladder pain, and a mild burning sensation during urination, especially over the urethral meatus and the perineal region, for approximately 1 month prior to admission. He ignored these symptoms initially, but the burning pain worsened, with concomitant urinary frequency and urgency. He visited another hospital for help, at which routine urine analysis revealed pyuria. Under the impression of acute prostatitis, oral antibiotic treatment with ciprofloxacin was initiated during an outpatient visit; however, his symptoms remained, with no improvement. Two days before admission to our hospital, the symptoms worsened, with a newly-developed decreased voiding amount (approximately 50 mL per void) and urgency with urge incontinence, accompanied by painful hematuria and blood clot formation, especially at the first urine void of the morning. The patient then presented to our Emergency Department. Urine analysis showed pyuria, over 100 white blood cells (WBCs)/high-power field (HPF), and significant tenderness and swelling of the prostate was noted upon digital rectal examination, but no pus-like urethral discharge was seen. Under the impression of acute prostatitis, for which oral antibiotic treatment had failed, the patient was then advised to undergo hospital admission for advanced antibiotic treatment.\nAfter admission, we consulted an infectious disease specialist for evaluation, and antibiotic treatment with ceftriaxone was started immediately. A blood test revealed WBC 4870/μL without predominance of neutrophils or eosinophils. No marked elevation of serum C-reactive protein (CRP) (0.25 mg/dL) was noted. The patient's temperature after admission had remained within the normal range, and there were no accompanying signs or symptoms of toxicity. Several blood and urine cultures were performed, including tuberculosis, but all results were negative. The symptoms of pyuria (which remained over 100 WBCs/HPF), urgency, and painful hematuria persisted with no improvement after one week of intravenous antibiotic treatment. However, a sonogram performed upon admission revealed suspected bladder wall thickening. Due to the persistent symptoms that failed to respond to advanced intravenous antibiotic treatment for 1 week, abdominal computed tomography (CT) with contrast was then arranged. The CT scan showed asymmetrical wall thickening (thickness of up to 1.2 cm) of the anterior aspect of the urinary bladder with a mural nodule, and mucosal enhancement with perivesical fatty stranding (Fig. ). According to the above findings and the clinical symptoms, bladder cancer was highly suspected, and we discussed cystoscopy with bladder biopsy with the patient and his wife, which was then performed the next day. Prior to hydrodistension, the bladder mucosa presented with hypervascularity, but there was no tumor over the anterior wall of the bladder as seen on the CT scan. The bladder mucosa of the anterior wall was erythematous, with multiple hump-like changes, and several biopsies were performed. After hydrodistension at a pressure of 90 cm H2O for 8 minutes, the bladder capacity was approximately 150 mL, and bleeding over multiple aspects of the bladder was seen, with glomerulation and ulcerative changes (Fig. A and B). Pathologic analysis of the bladder biopsies showed erosive cystitis, characterized by denuded urothelial cells, with prominent infiltration by eosinophils, lymphocytes, neutrophils, and plasma cells over the mucosa and submucosal layer. In addition, hypervascularity and submucosal granulation formation with fibrosis were observed (Fig. A and B).\nAfter the operation, the bladder capacity increased a little, to approximately 70 to 80 mL per urination, but the urgency, frequency, nocturia, and hematuria still persisted. We also prescribed an anticholinergic agent, a beta-3 agonist and a nonsteroidal anti-inflammatory drug (NSAID), and the patient was then discharged. During 2 weeks of outpatient treatment, his symptoms did not improve with medication. Thus, we discontinued medical treatment and performed hyaluronic acid (HA) instillation, once a week for a total of 10 times. After the treatment, the symptoms of urgency, frequency and nocturia improved, and the bladder capacity increased to 350 mL per urination according to the patient's own voiding diary; in addition, no morning hematuria or hematuria after holding back urine occurred. After the patient's symptoms had improved, we arranged follow-up MRI of the bladder and cystoscopy; on the images, no thickening of the bladder wall nor nodules were observed (Fig. ). Cystoscopy showed marked improvement of the previously-noted erythematous bladder mucosa, and there was neither active bleeding nor glomerulation seen during the whole procedure. Bladder biopsy near the previous biopsy site was performed, and the final pathologic analysis showed decreased inflammatory cell infiltration, regeneration of the urothelium, and less vascularity (Fig. A and B).\nWritten informed consent to publish this case report was provided by the patient, and the consent procedure was approved by the Ethics Committee of Tri-Service General Hospital. |
A 78-year-old male with multiple comorbidities, including hypertension, hyperlipidemia, and type II diabetes mellitus, presented to our emergency setting with complaints of recurrent bouts of abdominal pain and fluctuating fevers for the previous two weeks. The patient reported that the pain is a new manifestation of a previously dull aching pain that had waxed and waned over the last decade. His description alluded to a pain that was sharp and intermittent with localization in the right upper quadrant. He could not attribute the intermittent nature of his predicament to any aggravating or relieving influences. The pain was associated with fluctuating low-grade fevers (99°F-100°F), anorexia, and an associated 13-pound weight loss, which culminated in a visit to our clinical setup.\nFurther interrogation disclosed that the patient underwent a laparoscopic cholecystectomy in 2003. The ensuing year was relatively pain-free but was followed by recurrent bouts of right upper quadrant pain, albeit less upsetting than his current presentation. He was subsequently diagnosed in 2005 with gallstone spillage. The patient chose conservative treatment for his abdominal pain, rather than invasive interventions, which included the administration of acetaminophen and non-steroidal anti-inflammatory drugs (NSAIDs). This treatment modality was sufficient for the duration of a decade. He presented to another medical facility with similar complaints of fever and abdominal pain in 2016. A computed tomography (CT) scan of his abdomen disclosed the presence of a necrotic phlegmon, which was subjected to aspiration. Its composition included a combination of fibrous material, granulation tissue, and inflammatory infiltrate. The aspiration provided considerable relief of symptoms and he was discharged on a gabapentin prescription that was well-tolerated and produced sustained amelioration of his pain, with only occasional wavering with respect to his baseline.\nThe initial assessment showed an elderly gentleman, who was alert and well-orientated but under considerable distress due to the abdominal pain and accompanying chills. He had a fever of 104°F, a heart rate of 120 beats per minute, a blood pressure of 95/70 mm Hg, and a respiratory rate of 19 per minute. An abdominal exam revealed a non-protuberant, soft, and tender abdomen with marked sensitivity in the right upper quadrant. He had perceptible bowel sounds, an absence of dullness on percussion, and a clear rectal vault. Pertinent initial laboratory investigations included an elevated white blood cell (WBC) count of 16,900/µL, C-reactive protein (CRP) of 6.5 mg/dL, and a random blood glucose (RBG) of 280 mg/dL. The patient subsequently underwent a whole body CT scan, which revealed a large 19-cm sub-diaphragmatic, right retroperitoneal abscess, which was inferior and posterior to the right hepatic lobe (Figure ).\nIn lieu of these findings, the patient was admitted and started on a combination of intravenous fluids, intravenous (IV) vancomycin and piperacillin-tazobactam due to an underlying suspicion of sepsis secondary to a hepatic and/or perihepatic liver abscess caused by a lack of gallstone retrieval following his cholecystectomy. He was subsequently subjected to a percutaneous CT-guided drain placement, which allowed for the evacuation of approximately 700 mL of grossly purulent material and provided prompt pain relief. The fluid sample was sent for a gram stain, culture, and a bilirubin assay. The gram stain of the abscess fluid revealed branching gram-positive rods concerning for Actinomyces, Nocardia, and Streptomyces; therefore, the patient remained on IV vancomycin and piperacillin-tazobactam, with the addition of trimethoprim-sulfamethoxazole (TMP-SMX) to the antibiotic regimen. Remarkably, the culture growth was positive for Propionibacterium and ampicillin-sulbactam was added to the list of antibiotics. Two days later, the patient was shifted to the intensive care unit (ICU) following unrelenting fever and persistent tachycardia, even after his abdominal drain placement was supplemented with vigorous antibiotic and antipyretic therapy. His ICU stay was complicated by episodes of dyspnea, tachypnea, and pleuritic chest pain, while a chest auscultation unveiled a decrease in breath sounds at the right basal region. A repeat CT scan confirmed the reduction in the size of the abscess and showed a new right-sided pleural effusion, which explained the patient’s breathing difficulties (Figure ).\nThe patient underwent an ultrasound-guided chest tube placement, which evacuated 10 mL of purulent fluid, followed by two doses of lytic therapy with tissue plasminogen activator (tPA) via the catheter. The following morning, he described an aggravation in his pleuritic chest pain, and an episode of shortness of breath, whereby, the oxygen saturation dipped to 90%. A new chest CT scan was ordered, which showed an interval increase in the right basilar opacity with a moderately sized pleural effusion (Figure ).\nHe was subsequently upgraded to a larger catheter via a CT-guided approach that evacuated a total of 900 mL of purulosanguinos fluid. The patient tolerated subsequent lytic therapies well and showed vast clinical improvement following this procedure, with a reduction in dyspnea, pleuritic chest pain, and fever, as well as a down-trending of his inflammatory markers.\nA repeat CT scan was performed on the 10th day of admission, which revealed a near-complete resolution of his right-sided pleural effusion, and a decrease in his right-sided retrohepatic intrabdominal abscess (Figure ).\nThe chest drain was removed and the patient was subsequently discharged with a transhepatic drainage catheter, daily intravenous ampicillin-sulbactam, and acetaminophen, as needed for pain control. The patient was shifted to oral amoxicillin-clavulanate a month after the resolution of his pain, fever, and stabilization of inflammatory markers. The transhepatic percutaneous drain was left in place in order to allow for the formation of a chronic drainage tract, with hopes of localizing the perpetrating gallstones at its base.\nTwo months after discharge, the patient underwent a procedure for upsizing the drain in order to accommodate for a choledochoscope into the abscess cavity and the maturing sinus tract. He subsequently underwent an abscess cavity endoscopy, whereby a catheter and scope were advanced into the cavity for exploration. An abscess catheter study was performed through the existing catheter and showed no filling defects, after which the catheter was removed and an endoscope was inserted for direct visualization of the main cavity, as well as the two “fingers” branching out of the abscess cavity. Two small stones were expelled after flushing the main cavity multiple times. Following the expulsion of the gallstones, a replacement catheter was advanced into the cavity and a second catheter study was performed to rule out the possibility of any other stones. The patient tolerated this intervention well and had no further drainage from the intra-abdominal drain.\nFollowing a lack of drain output and resolution of symptoms after his last endoscopic procedure a month ago, the patient's intra-abdominal drain was removed. The patient is currently stable and is being followed as an outpatient to date. |
A 31-year-old male of Eastern European origin, who was referred to our clinic after a 3-month history of unilateral nasal obstruction, swelling and sporadic, short lasting episodes of epistaxis. He had noticed a swelling of his right lateral nasal wall. On examination there appeared to be a large, right-sided nasal polypoidal mass, which seemed to be occluding most of the right nasal airway. Mucosa was smooth, and appeared healthy; however, the nasal septum was deviated significantly to the left side of the nose. Endoscopic examination of his pharynx, larynx and contra-lateral side of his nose was otherwise normal. There was no palpbable lymphadenopathy, and the rest of the ear nose and throat and physical examinations were normal.\nNon-contrast CT assessment of the sinonasal region was performed which revealed a soft tissue lesion arising from the right side of the nasal septum (Fig. ). At the initial discussion at the local head and neck multidisciplinary team meeting, radiological opinion was that the lesion may possibly represent olfactory neuroblastoma, inverted papilloma or pleomorphic adenoma of the septum and that MRI would be beneficial in diagnosis. MRI imaging of the lesion showed general increased T2 signal but with heterogeneous nodular T2 heterogeneity (Fig. a and b). On the unenhanced axial T1-weighted sequence a small focus of T1 shortening was noted in keeping with some low volume focal cystic degeneration within the tumour (Fig. c).\nUrgent biopsy of the nasal lesion was arranged and tissue on initial histologic examination revealed mixed Antoni A and B substructure and when stained with S100, had a moderate uptake, confirming a diagnosis of schwannoma of the nasal septum. Later this patient underwent a mid-facial de-gloving approach for excisional biopsy of the entire tumour which was performed as an elective, non-urgent admission. Excisional biopsy histopathological staining was consistent with that of the biopsy, reaffirming the diagnosis of septal schwannoma. Deep margins of the specimen contained schwannoma tissue.\nPost-operatively the patient continues to be well and has had no complications after removal of the lesion. There was no sign of septal perforation on any review and the area of the initial lesion had re-epithelialized. No sign of deep margin recurrence has been found. |
A 25-years-old female patient reported to our unit with pain in the right lower back tooth region for 1 month. Pain was sudden in onset, throbbing in nature, and continuous in character. There was no history of fever, swelling, or paraesthesia associated with the pain.\nOn intraoral examination, pain seemed to be because of irreversible pulpitis with respect to 46. There was no sinus opening or tooth mobility present with respect to the same tooth. Electric pulp vitality test was negative. Medical history was noncontributory. IOPA was obtained and periapical radiolucency of 4 cm × 4 cm in size was seen with respect to the same tooth. Thereafter, an orthopantamogram (OPG) was recommended. The OPG revealed well-defined multilocular lesions [], around 4 cm × 4 cm in size associated with respect to the right mandibular first and third molars and a well-defined unilocular radioluceny of about 12 cm × 5 cm size, extending from the mandibular canine to the third molar on the left side of the mandible. Another well-defined unilocular lesion of 10 cm × 10 cm in size was present with respect to the mandibular anterior region. There was no associated resorption of roots or pathological displacement of tooth.\nA cone-beam computed tomography image was acquired to establish the position of the inferior alveolar neurovascular bundle and to determine the status of bone []. The findings revealed no buccal and lingual expansion of bone. The inferior alveolar canal was severely displaced downward. On the basis of radiological and clinical findings, our differential diagnosis of the individual lesions consisted of SBC (traumatic bone cyst [TBC]), keratocystic odontogenic tumor (KCOT), and central giant-cell granuloma. Multiple KCOTs are seen in basal cell nevus syndrome. However, on clinical examination, no evidence was found for the same. We subsequently proceeded with the diagnosis of multiple TBC.\nAspiration was negative and an incisional biopsy specimen was obtained from the periapical region of the right second molar under local anesthesia. No tissue lining was obtained, so a second incisional biopsy was performed on the opposite side with respect to the left mandibular molars. Very scant tissue was obtained, which was sent for histopathological examination, which showed a thin band of vascular fibrous connective tissue with scant liquid content without any evidence of epithelial lining. These features were suggestive of a TBC.\nUnder general anesthesia, enucleation and curettage of the lesion were done, followed by bone graft placement. The lesion was approached by buccal crevicular incision. Full-thickness mucoperiosteal flap was raised. Surgical exploration revealed empty cavities with scarce amounts of tissue [Figures and ]. A diagnosis of multiple SBC was surgically confirmed. Thorough curettage of the internal walls of the cavities was carried out and bleeding was induced. The cavity was packed with chips of Perioglas allogenic bone graft and closure was done using 3–0 vicryl sutures [].\nHistopathological examination of the excised specimen confirmed the diagnosis.\nPostoperatively, the patient had mild diffuse swelling for 3–4 days, which subsided eventually. The patient has been followed up for 9 months and is asymptomatic with no positive findings of pain, swelling, and paraesthesia. Follow-up panoramic radiograph showed satisfactory bone healing without any evidence of enlargement of lesion and recurrence. |
We describe the case of a 32-year-old female with a past medical history of anemia who was transferred to our center for further assessment and management of multiple medical problems including anemia, GIB, hemoptysis, and reportedly lung and liver lesions. She reported to the outside center with a 7-month history of progressive fatigue, hemoptysis, splinter hemorrhages and diffuse joint pain that involved both knees, hips, and left wrist. At the outside hospital her severe anemia persisted despite 9 units of PRBC transfusions. She underwent extensive assessment including esophagogastroduodenoscopy (EGD) and colonoscopy. EGD revealed gastric lesions consistent with AVMs while colonoscopy was negative. A bronchoscopy was negative but CT imaging showed liver and lung lesions of unknown etiology. On presentation to our center, vitals and physical examination were unremarkable. Initial blood work was remarkable for anemia, thrombocytopenia and hypokalemia. Repeat EGD and capsule endoscopy revealed active bleeding in the duodenum secondary to diffuse mucosal vascular lesions. Due to persistent bleeding she was transferred to the intermediate care unit (IMC) for closer monitoring. She continued to receive transfusions of PRBCs, FFP, cryoprecipitate, and platelets on a daily basis. During her hospitalization, she received a total of 4 EGD, 2 capsules and a push enteroscopy with similar findings of blood in the stomach, duodenum and small bowel with scattered AVMs, which were repeatedly cauterized. A bone marrow biopsy with pathology was inconclusive but concerning for possible angiosarcoma. A PET CT skull base to mid thigh did not show any evidence of malignancy but revealed diffuse low-level activity through out the axial skeleton that was deemed secondary to increased hematopoiesis. About 2 weeks following admission to our center, the patient became hypotensive from worsening hematemesis. She was transferred to the medical ICU. In the ICU, she continued to require daily transfusions of blood products with persisting GI bleed. She was started on estrogen/progesterone therapy, Avastin and aminocaproic acid for suspected hereditary hemorrhagic telangiectasia (HHT). Subsequently, she developed fulminant DIC and was rapidly transfused blood, factors, and platelets with improvement. A few days later, she developed respiratory failure requiring intubation and mechanical ventilation in addition to hemorrhagic shock requiring pressors and continued transfusions. Given her extremely poor prognosis, and through multiple discussions, the family decided against further escalation of care. Autopsy revealed angiosarcoma most likely arising from the liver and spreading to the lungs, spleen, mediastinal lymph node, gastrointestinal tract, skin and bone marrow. The liver appeared to be the primary site due to the size of the lesion involving approximately 75% of liver compared to the diffuse patchy involvement of lungs and spleen. Immunohistochemistry with CD31 and CD34 which are markers specific for endothelial cells and vascular tumors such as Angiosarcoma positively stained the tumor (, ). The immunohistochemical stain human herpes virus 8 (HHV) did not stain the tumor making a diagnosis of Kaposi’s sarcoma unlikely. Gene sequence of ACVRL1, ENG and SMAD4 with deletion/duplication analyses of ACVRL1 and ENG specific for HHT did not detect any abnormality in an ante mortem whole blood sample and the vascular lesions evaluated were not AVMs. |
A 70-year-old African American male was seen in the emergency department for acute anxiety and paranoia. He reported that his son gave him melatonin to help him sleep, but he felt the medication was poisonous. He also reported that he was struggling with the death of his partner of 40 years and was feeling anxious. The patient was prescribed risperidone and lorazepam and was discharged shortly thereafter. Twenty-four hours later, he was seen again in the emergency department for worsening anxiety, psychosis, suicidal ideation, and command hallucinations. The patient's son reported that his father “had not slept in weeks.” The son reported progressive agitation, paranoia, and bizarre behavior. Due to the worsening psychosis, he was admitted to the medical floor for further work-up. He reported feeling as though people were watching him and that someone was going to harm him. When questioned about his reported suicidal ideation, he blamed that thought on his post-traumatic stress disorder; but would not elaborate on the event. The patient was given a one-to-one sitter due to his suicidal ideation.\nThis patient lives with his family of seven children. He has a history of prolonged incarceration. His past medical history is significant for hypertension and negative for seizure disorders. The patient had never been hospitalized for psychiatric issues prior to this visit. He denied past suicidal behavior and any history of physically or sexually aggressive behavior. The patient reported a history of excessive alcohol abuse for more than a year following the death of his partner. He reported that he stopped drinking “cold turkey” five months prior to this visit. The patient's family history is significant for a son with an anxiety disorder. He denied a history of physical or sexual abuse in the past.\nThe patient's mental status exam was significant for an anxious affect with referential and paranoid ideations. He denied any thought broadcasting, insertion, or withdrawal. He had some paranoid and persecutory delusions. Insight, judgment, and impulse control were poor.\nThe patient's initial physical exam revealed an inability to ambulate and overall weakness. Further neurological examination revealed mental disorientation with bilateral muscle wasting, sensory deficit, and hyporeflexive ankles. Additionally, the patient exhibited stance and gait abnormalities. A later eye exam revealed bilateral light-near dissociation with accommodation but no reaction to light. Otherwise, all other neurological components were intact and within normal limits.\nInitial routine testing was positive for the Treponema pallidum antibody, suggesting prior infection, but the distinction between treated and untreated syphilis cannot be made as the Treponemal-specific immunoglobulin G (IgG) may remain elevated throughout life. An RPR was then ordered to help distinguish between acute or chronic infection and a Treponema pallidum particle agglutination test was ordered to distinguish between syphilis infection and a false positive screening test. The RPR returned nonreactive but the Treponema pallidum particle agglutination returned reactive, indicating a prior infection of Treponema pallidum. The initial differential diagnosis workup included testing to exclude thiamine, folate and B12 deficiency, insomnia, hypocalcemia, hypothyroidism/hyperthyroidism, HIV encephalopathy, dementia, stroke, drug or alcohol intoxication, and normal pressure hydrocephalus.\nFurther labs showed white blood cell count, sodium, potassium, calcium, anion gap, creatinine, glomerular filtration rate, glucose, mean corpuscular volume, and thyroid stimulating hormone within normal limits. Urinalysis, toxicology screening, alcohol levels, and HIV antibody were also negative. Imaging included a head computed tomography (CT), which showed no abnormalities, a brain magnetic resonance imaging (MRI) that showed cortical atrophy, and an electroencephalogram within normal limits. |
In September 2004, a 24-year-old male presented with progressive dyspnea on exertion, paroxysmal atrial fibrillation, malaise, and peripheral eosinophilia. Initial evaluation showed a left atrial mass and a separate mass adherent to the mitral valve for which the patient underwent surgical resection and mechanical mitral valve replacement. The diagnosis of noninfectious hypereosinophilic granulomatous disease was made by histopathologic evaluation. The patient underwent treatment with imatinib mesylate and a 5-month steroid course, which eventually led to complete resolution of eosinophilia, residual masses, and symptoms.\nIn December of 2009, the patient presented to an outside facility with fevers, night sweats, and weight loss and was diagnosed with subacute mechanical valve endocarditis from Streptococcus viridians. TTE noted multiple large vegetations at the four and five o'clock positions of the mechanical valve. The patient underwent a second mitral valve replacement using a 29 mm St. Jude mechanical valve, with a cross-clamp time of 85 minutes and total bypass time of 132 minutes. Intra-operative TEE was performed without mention of aortic valve injury or regurgitation. Pathology review of the prosthetic valve vegetations showed focal necrosis with fibrinous exudates without prominent eosinophilia. A two-day postoperative TTE report noted new mild to moderate aortic insufficiency. His postoperative course was significant for atrial flutter and mild congestive heart failure symptoms. He was discharged on appropriate heart failure therapy and neurohormonal blockade.\nThe patient's condition continued to deteriorate, and he presented to our institution 2 months after the second operation with NYHA Class III decompensated heart failure. Repeat echocardiography revealed an overall preserved ejection fraction of 45%–55%; however, a new finding of severe aortic regurgitation was noted ().\nTEE also demonstrated a large regurgitant jet originating from the non-coronary and left coronary cusps concerning for valve perforation ().\nFor better visualization, a cardiac gated CT scan was done, which confirmed a 4-5 mm perforation of the aortic valve non-coronary cusp ().\nGiven extensive scarring from his recent surgical procedures, the patient underwent a trial of optimal medical management for the ensuing 4 months. Despite these efforts, he did not recover and was referred for surgical correction requiring a third sternotomy. Operative evaluation revealed a misplaced prolene suture used in closure of the transseptal surgical approach which perforated the non-coronary cusp. The defect was repaired with a pledgeted subannular stitch to reapproximate the leaflet. Intra-operative TEE demonstrated aortic and tricuspid valve competence, without stenosis or insufficiency. Postoperatively the patient made an excellent recovery. |
A previously well 66-year-old male presented with one episode of haemoptysis and intermittent chest discomfort. He was a lifelong non-smoker and worked as a professional cycling tour guide. Eastern cooperative oncology group (ECOG) performance status at presentation was zero. He was diagnosed with a stage IIIb, pT4b N2 M0 moderately differentiated squamous cell carcinoma of the right upper lobe (Fig. ). Immunohistochemistry (IHC) was positive for CK7, CK5/6 and P63. He initially was treated with Cisplatin and Vinorelbine chemotherapy but discontinued after cycle 2 due to grade 3 toxicity. On recovery, he completed 70 gray in 35 fractions with a good partial response seen on a restating scan (Fig. ).\nTumour molecular profiling identified an activating EGFR mutation in exon 21, L858R, and at that stage he commenced Gefitinib. He achieved a 3.5-year period of disease stability on this therapy. On progression he was found to have a solitary brain lesion and a single liver lesion (Figs and ).\nThe brain metastasis was excised and he received 10 fractions of whole brain radiotherapy. Surprisingly, the pathology results from his brain lesion returned as metastatic adenocarcinoma likely of a lung origin. IHC was positive for CK7 and TTF-1. This suggested that his primary lung tumour was likely a mixed adeno-squamous lung cancer. The regional hepatobiliary tumour board recommended against local therapy to the liver metastasis. It was decided to continue EGFR directed therapy beyond progression in the form of Afatinib given his low disease burden. With this approach his liver disease remained stable for a further 1 year. At the time of his second progression of disease he presented with fatigue, weight loss, right wall chest swelling, pain and prominent chest wall vasculature. Imaging revealed a new lesion involving the right chest wall with an increase in size of the liver metastasis (Figs and ).\nA right chest wall biopsy was performed to evaluate for the presence of a T790M mutation. On this occasion the histological features were consistent with small cell carcinoma. IHC was positive for synaptophysin and TTF-1. T790M testing was negative. It was concluded his cancer had dedifferentiated into small cell carcinoma post prolonged EGFR inhibition. He commenced Carboplatin and Etoposide combination chemotherapy for treatment of extensive stage small cell carcinoma. He achieved an initial partial response clinically to chemotherapy with evidence of shrinkage of the chest wall lesion. Within a month of completing four cycles of Carboplatin and Etoposide he deteriorated rapidly and passed away due to rapidly progressive disease. |
Our patient is a 32-year-old otherwise well man who was initially diagnosed with melanoma following excision of a 5 mm naevus from his back in June 2012. The primary pathology showed it to have a Breslow thickness of 5 mm, a Clark level of 4, no evidence of ulceration, and a mitotic count of 7/mm2. Subsequent gene testing demonstrated the tumour to be BRAF positive. On the basis of this poor pathology, a Wide Local Excision and sentinel node biopsy were performed. The positive nodal biopsy resulted in the patient having an axillary clearance (5 out of 30 nodes positive). He had adjuvant radiotherapy (48 Gy to the axilla) and he was enrolled in a clinical trial which involved the administration of two immunomodulatory drugs: Nivolumab and Ipilimumab.\nOn a routine staging CT-abdomen/pelvis in December 2014, the patient was first noted to have a mild left-sided hydroureter. Neither the CT scan nor ultrasound performed soon after demonstrated a clear cause. A repeat CT-abdomen/pelvis in March 2015 showed the development of a 6 mm nodule in the mid left ureter at approximately the level of L4 vertebrae. A PET Scan performed at the time did not suggest it to be a metastatic deposit. A further repeat CT was performed in May 2015 by which time the nodule had progressed to being 10 × 9 mm and the hydroureter had progressed significantly. Throughout this period, serial measurements of renal function did not demonstrate any abnormalities, with a stable Creatinine between 90 and 100 μmol/L. The patient denied haematuria, and urinary cytology was negative. At this time, another metastatic deposit was found (a metastatic deposit in the left parafalcine region of the brain, subsequently treated with stereotactic radiotherapy) in addition to apparent lymphadenopathy.\nOn the basis of evidence of the progression of the nodule and increasing hydroureter, the patient was referred to the Uro-Oncology Clinic at the Crown Princess Mary Cancer Centre, Westmead. In light of the position of the nodule in the mid ureter, the decision was made to attempt an endoscopic resection.\nThe patient underwent ureteroscopy at Blacktown Hospital in July 2015. The retrograde pyelogram confirmed the presence of the lesion, with evidence of obstruction in the mid ureter above the lesion (). Direct visualisation demonstrated a large ovoid mass attached by a thin stalk to the ureteric wall. This stalk was lasered, and the mass retrieved in three pieces by a basket through a sheath in order to minimise the risk of seeding into the lower tract. Since the depth of the lesion was unknown and the stork had a narrow base, the lesion was lasered full thickness in order to ensure maximal clearance. Low pressure irrigation was used throughout. Macroscopic inspection did not suggest involved margins, and completion ureteroscopy and pyeloscopy did not demonstrate any further suspicious lesions. In order to avoid the risk of causing a postoperative stricture, a ureteric stent was placed at the end of the case.\nHistopathology of the retrieved fragments confirmed the diagnosis of the mass being a metastatic melanoma.\nFollowing removal of the stent, further ureteroscopy was performed in October 2015. The retrograde pyelogram did not demonstrate any filling defects or any evidence of the hydroureter (). Direct inspection did not demonstrate any evidence of the metastatic deposit or its stalk or any further metastatic deposits.\nAt patient's most recent follow-up in late July 2016, there is no evidence of local disease recurrence. His serial staging CT-abdomen/pelvis remains clear of any hydroureter, the apparent lymphadenopathy has resolved, and his renal function remains stable (Creatinine 100 μmol/L). He remains clinically well. |
An 11-year-old male Rwandan, a known patient of multiple hereditary exostoses (MHE), presented to our hospital with a 6-month history of unrelenting bone pain despite treatment with NSAIDs; he later developed (two weeks prior to admission) a dull persistent aching pain and swelling of his left lower thigh that was worse at night and unresponsive to morphine. His parents noticed that he had also lost weight and appetite. There was no history of trauma or a fall. He was diagnosed with MHE at age 3 and had been living a relatively normal life and attending school. None of his siblings or any other member of his family had MHE. His past surgical history was unremarkable. On general examination, he was well nourished, had mild pallor of the mucus membranes, no jaundice, lymphadenopathy, or skin rash. All the other parameters were normal. Examination of the musculoskeletal system revealed normal stature except for the curving deformity of the left leg. There were multiple palpable bony swellings bilaterally on the upper humeri and lower femurs. The lesion on the left distal femur was markedly enlarged and tender, with induration, reddening, and limited range of motion of his knee joint (). Repeat X-rays confirmed the presence of bony outgrowths (exostoses) on the medial and lateral aspects of the distal femurs bilaterally and left metaphyseal widening common in this condition as had been previously identified when he was diagnosed at age 3, and further investigations of the left distal femur swelling () with magnetic resonance imaging (MRI) revealed a distinct enhancing lesion in the distal aspect of the left thigh at the site of intense swelling and pain. MRI showed a lesion with hypointense signals on T1WI sequences and has heterogeneous signal intensities with moderate and heterogeneous enhancement on T1WI postcontrast study and on T2WI sequences; the lesion had heterogeneous signal intensities (Figures –). At surgery, there was a necrotic lesion, and excision biopsy at the site of the left distal femur exostosis was taken (Figures and ). Hematological evaluation was normal except for a mild leukocytosis. The results of the serum biochemical tests were also normal. We did not do genotyping for EXT-1 and EXT-2 due to lack of facilities.\nHistopathological examination revealed a characteristic fibrous cartilaginous cap with a broad base (1.293 mm thick), covering a layer of normal appearing marrow and bone below which was a tumor-forming osteoid, an osteoblastic tumor as evidenced by presence of numerous bone spicules of varying maturity. There was also marked cellular atypia, grossly pleomorphic osteoblasts in the marrow with frequent mitoses. These features were those of a high-grade osteoblastic osteosarcoma. In conclusion, histopathological revealed an osteochondroma with an underlying high-grade osteoblastic osteosarcoma involving the marrow cavity (Figures –).\nFor staging purposes, CT scans of the chest, abdomen, and pelvis were done to investigate any presence of metastatic lesions (Figures and ). There were no metastatic deposits in the lungs, abdomen, and pelvis, and this was confirmed with PET scan in India where the patient was referred for specialized bone tumor treatment including limb salvage therapy. He was reevaluated, and the diagnosis of MHE and osteoblastic osteosarcoma confirmed. Whole body PET scan showed metabolically active disease in the distal left femur 8.6 × 8.1 × 16 cms in dimension with features consistent with osteosarcoma, multiple hereditary exostoses with evidence of skip lesions, loco-regional lymph node involvement, and no distant metastases. Histopathological review confirmed the earlier diagnosis of osteochondroma with osteoblastic osteosarcoma. The conclusion was that the patient had clinically localized disease. The following treatment plan was proposed and instituted: initiation with neoadjuvant chemotherapy followed by limp salvaging surgery and finally adjuvant chemotherapy. He received doxorubicin 35.5 mgs/m2 per day (day 1 and day 2), cisplatin 60 mgs/m2 for 7 days (days 1 and 2), methotrexate 12 gms/m2 per day, etoposide 100 mgs/m2/day for 5 days, and ifosfamide 2.8 gms/m2/day for 5 days followed by Ifosfamide 3 gms/m2/day for 3 days and subsequently pegylated Interferon-α2b 0.5 mcg/kg - 1mcg/kg. He completed a total of six cycles of chemoimmunotherapy.\nOur patient is still alive and back at school with no evidence of disease after 11 months of treatment and follow-up and continues to be followed up by the oncologist. |
A 12-year-old female patient with non-contributory medical history sought dental evaluation due to painless swelling on the right mandibular molar-ramus region. The patient described initial observation of the swelling approximately 2 months prior to presentation. Clinical examination revealed an expansible lesion in the right mandibular third molar region and blue colored swelling distal to first molar []. Panoramic radiograph disclosed a unilocular radiolucent lesion (approximately 7 cm × 4 cm) with well-corticated borders involving right body, angle, and ramus of mandible. The impacted second and third molar was displaced up to the inferior border of the body and the coronoid process of mandible, respectively []. On aspiration of cyst cavity, golden yellow colored fluid was noticed. The CT scan confirmed the buccal and lingual cortical expansion with perforation of lingual cortex of the mandible. The lesion was difficult to diagnose on the basis of clinical and radiographic findings. Therefore, UA, dentigerous cyst, odontogenic keratocyst were considered in the differential diagnosis. Patient's parents were informed regarding the complexity of diagnosis, different treatment options, and recurrence. Taking into consideration the age and complexity of diagnosis surgical enucleation followed by Carnoy's solution application and extraction of closely related teeth was planned. Under local anesthesia (Lox 2%, Mumbai), crevicular incision was given from permanent mandibular right canine to permanent mandibular first molar. The incision extended distally over anterior border of ramus of mandible followed by mesial releasing incision from permanent mandibular right canine. Mucoperiosteal flap was reflected and underlying bone was exposed followed by decortication of the buccal cortex with bone rongeur []. The lesion was enucleated along with impacted teeth and erupted first permanent mandibular molar []. Copious irrigation of bony cavity was done with betadine solution and normal saline. Carnoy's solution was applied in the bone cavity for 3 min with cotton applicators. The bony cavity was rinsed with normal saline and packed with glycerin and betadine-soaked ribbon gauze. Then horizontal matrix sutures with mersilk (Ethicon, Johnson & Johnson) were given. The excised lesion was sent for histopathological examination and gauze pack was removed 2 days postoperatively. Histological analysis of surgical specimen showed a cystic lesion surrounded by a fibrous tissue capsule lined by odontogenic epithelium of variable thickness proliferating into the lumen in a plexiform pattern. The lesion was diagnosed as unicystic ameloblastoma with intralumenal proliferations. In the post-operative days, the patient was assessed for parasthesia of lip or cheek. As the diagnosis carries a risk of recurrence, a long term follow-up period was planned. |
A 62 year old hypertensive non diabetic female presented with pain, swelling in the left knee and difficulty in walking in the left knee for the last 6 weeks. A TKA of the left knee was done 2 years ago. She had an uneventful period after the TKA.\nOn examination, a 15 cm vertical midline surgical scar healed by primary intention of the initial TKA was present on anterior aspect of knee. The local temperature was raised. The range of motion was terminally restricted in flexion and was associated with pain. No deformity was noted, however, mediolatera instability was present. Patient was neurologically intact and distal pulsations were well felt.\nRadiologically, there was osteolysis over the anterior femoral cortex, posterior femoral condyles and under the tibial base plate []. Complete hematological study and urine microscopy revealed no significant abnormality besides elevated erythrocyte sedimentation rate (ESR) (75 mm at 1 hour) and C-reactive protein (CRP) (84 mg/l).\nKnee joint aspiration was carried out under sterile conditions. The fluid obtained was sent for analysis. Characteristics of the aspirate were 5 ml fluid, cloudy with low viscosity, white blood cell count was 15,000/cubic mm, with 68% polymorphonuclear leucocytes. Gram staining was negative, but 10% KOH mount, done to rule out fungal infection as a standard hospital protocol, was positive suggesting a fungal infection.\nA decision to perform 1st stage of revision TKA was taken after consultation with the patient and her family. In consultation with infectious disease specialist, it was decided to culture and identify the organism on intraoperative samples. The previous vertical incision was used to approach the joint. Per-operatively there was turbid synovial fluid, granulation tissue, implant loosening and adherent cement Synovial fluid and tissue was sent for culture and sensitivity studies.\nImplant removal, debridement and antibiotic (vancomycin) plus antifungal (amphotericin B) impregnated cement spacer insertion was done []. Post-operative period was uneventful []. Medical therapy included intravenous fluconazole 400 mg OD for 6 weeks followed by oral fluconazole 400 mg BD for 12 weeks. Bacterial culture was negative. Fungal culture was positive for Candida tropicalis. Sabouraud dextrose agar and the rapid API 20 microtube system were used for identification of species. Antifungal sensitivity was performed on the isolate.\nPatient was followed up at weekly intervals and after 20 weeks, the ESR (23 mm at 1 hour) and CRP (6.3 mg/l) were within normal limits. Repeat aspiration was sterile. During the 2nd stage of revision, TKA quadriceps snip technique was used. Spacer was removed; the defect was non contained involving cortical and metaphyseal bone. TC3 Depuy system and fluted stems were implanted (Depuy, India). Posterior femoral augmentation and reconstruction with allograft was done. Femoral head allograft was used as bone graft to fill up the defect. There was no maltracking of patella []. Tissue was sent for microbial analysis, which turned negative. Postoperative period was uneventful and patient was discharged on oral fluconazole 400 mg BD for 10 weeks.\nThe surgical wound healed adequately and after physiotherapy, a painless range of motion of 10-90° with a stable knee was obtained. We did not use any scoring system pre or postoperatively. Patient maintained status quo until last followup 24 months after 2nd stage of revision surgery []. |
A 39-year-old woman, gravida 1, para 0, was diagnosed with invasive squamous cell carcinoma of the cervix following conization. Pathological findings showed carcinoma consistent with FIGO stage IA1 with lymphovascular invasion. She was referred to Keio University Hospital. The patient and her husband were informed of the treatment options, including AmRT and pelvic lymphadenectomy. The patient was told that the outcome of this procedure could not be guaranteed because an insufficient number of these procedures have been performed worldwide to yield reliable conclusions. The patient wished to preserve fertility, and she and her husband signed a written consent form agreeing to this treatment. Pathological findings after AmRT and pelvic lymphadenectomy showed no residual tumor and no lymph node metastasis. There was no finding of an ovarian tumor before surgery. A left ovarian cyst of 4 cm was identified during postoperative follow-up.\nAt 3 years and 6 months after surgery, the patient underwent IUI and then had fever and pain in her left lower abdomen 10 days later. At her first visit, her temperature was mildly elevated to 37.5°C. The patient's pregnancy was denied because a qualitative urine human chorionic gonadotropin (hCG) test was negative. A tumor with tenderness was palpated in the left adnexal area. A cystic tumor of 64x 41 mm was found by transvaginal ultrasonography (). Blood tests showed increases in white blood cell (WBC) count to 11900/μL and C-reactive protein (CRP) to 22.80 mg/dL. The patient was diagnosed with PID with ovarian cyst infection and hospitalized for treatment. Conservative treatment with antibiotics was initially used, but her symptoms did not improve. On hospital day 8, blood tests showed a further increase in WBC count to 23900/μL and CRP to 28.17 mg/dL, and pelvic CT showed that the ovarian cyst had grown to 10 cm in size ().\nWe decided to perform laparoscopic left ovarian cystectomy on day 8. Since the patient had a history of open surgery, adhesion was likely in the abdominal cavity. Before surgery, we asked the urologist to insert bilateral 6 Fr ureteral catheters because of possible difficulty identifying the ureters. The catheters were fixed to the thigh with tape (). The uterine manipulator was not inserted before surgery to avoid the risk of uterine perforation.\nCO2 pneumoperitoneum was established at 10 mmHg. Laparoscopic left ovarian cystectomy was performed using typical trocar placement. The left ovary was swollen to 10 cm and the fluid contents were purulent (). The left adnexa, posterior uterine wall, and retroperitoneum were firmly adhered (). The bilateral fallopian tubes were firmly adhered to the surrounding tissue and were unable to be identified. During surgery, by moving the catheter manually back and forth from outside the body, we were able to identify the ureters visually (). The 7 cm uterine manipulator was inserted during surgery under a laparoscopic view. The left ovarian cyst was excised, leaving the normal part of the ovary (). The operation time was 2 h and 58 min, and blood loss was 550 mL. No complications occurred during or after surgery. After the operation, symptoms improved rapidly and the patient was discharged 8 days after surgery. The excised specimen was pathologically an endometriotic cyst. A bacterial culture of the cyst fluid was positive for Prevotella bivia, Prevotella species, and Finegoldia magna. |
A 65-year-old female presented to our institution with a chief complaint of syncope and chest pain. She had a past medical history of untreated hypertension and recently diagnosed peptic disease with noncompliance to proton pump therapy. Additionally she reported a 30-pack year tobacco history with continued use. In the afternoon of her admission, she was walking from her house to her mailbox, when she developed sudden onset of chest pressure and tightness. She subsequently lost consciousness and was awoken by emergency medical personnel. The duration of loss of consciousness is unclear, and there was no rhythm strip reported by the EMS staff. She denied any prodromal symptoms of lightheadedness, nausea, and palpitations prior to the syncopal event. Furthermore, there were no signs or evidence of a post-ictal state. She was immediately transferred to our facilities emergency room where she was fully examined and reported no symptoms on initial triage. Her presenting electrocardiogram was largely nonspecific with a normal sinus rhythm and no significant ST or T wave abnormalities suggestive of an ischemic process (ECG #1) ().\nAn initial troponin I level of 0.2 prompted treatment for a non-ST elevation myocardial infarction. She was given aspirin 325 mg, plavix 600 mg, and an appropriate weight-based dose of lovenox. She continued to be symptom free for three hours while being in our emergency room. Approximately three and half hours after her arrival to our institution, she developed sudden onset of chest pressure and pain, with a rapid deterioration in her clinical status. She was bradycardic with heart rate 30–35, and blood pressure readings of 60s systolic. She appeared pale, diaphoretic, and in severe distress. A repeat ECG at the time was significant for 2 : 1 sinus bradycardia with a prolonged PR interval, as well as significant ST elevations (5-6 mm) in the inferior leads with ST depressions (2-3 mm) anteriorly (ECG #2) ().\nThis clinical status persisted for approximately ten minutes when suddenly the heart rate increased to 90, and systolic blood pressure increased to 130s. Her symptoms had completely resolved and a repeat ECG demonstrated complete resolutions of the ST segment elevations (ECG#3) ().\nAt this time cardiology was notified, and the patient was taken immediately for coronary angiography under the presumption for an acute coronary syndrome. In transport to the catheterization lab, the patient developed profuse melanotic stools mixed with evidence of bright red blood.\nIn the catheterization lab, her radial artery was canalized with a 6 French sheath for coronary access. Multiple views of a diagnostic coronary angiogram failed to reveal any culprit lesion and all three major vessel beds demonstrated nonobstructive disease. The angiographic findings in conjunction with the clinical course suggested severe coronary vasospasm as the etiology for the patient's condition. Intra-coronary provocative testing was not undertaken given the risk associated with the current acute situation. The patient was subsequently transferred to coronary care unit overnight for closer monitoring. There was no significant elevation in cardiac biomarkers. Throughout the night she developed multiple bouts of hematochezia with a decline in hemoglobin level to 5.4 from the initial 10.6 level on admission. The patient was treated for hypovolemic shock secondary to gastrointestinal hemorrhage and urgent endoscopy was performed revealing multiple duodenal ulcers that were cauterized. The following morning the patient developed another episode of coronary vasospasm with the ECG changes identical to the initial episode. Despite endoscopic therapy, the gastrointestinal hemorrhage did not cease. The vasospasms continued to occur intermittently over a 48-hour period with a frequency of 10–15 minutes culminating in complete heart block and hemodynamic instability. Nitroglycerin was attempted temporarily for relief of the vasospasms, but the intermittent hypotension with the vasospasms precluded its clinical effectiveness. Placement of a transvenous pacer wire was attempted unsuccessfully. Unfortunately the patient expired approximately 72 hours after her arrival to our institution. Just prior to her cardiac arrest, a troponin I level of 140 was noted confirming our suspicion that the coronary spasms had lead to myocardial infarction. |
A 32-year-old gravida 4, para 1, live 1, abortion 2 presented with a history of amenorrhoea for one and half months in the outpatient department with urine pregnancy test positive. She presented with complaints of lower abdominal pain for three days and vaginal bleeding. The patient’s gestational age was seven weeks and two days according to her last menstrual period. Prior to her presentation, she had normal regular menstrual cycles. She underwent one lower segment C-section with the outcome of a live female child. Then she had one spontaneous abortion. She had a history of previous ruptured ectopic pregnancy in the left fallopian tube for which she was operated and left-sided salpingectomy was done. She was a non-smoker and had no history of consumption of alcohol. She had no other co-morbidities like diabetes mellitus, hypertension, or tuberculosis. On general examination, the patient was afebrile with vitals stable with a pulse rate of 82 beats per min and blood pressure of 110/70 millimeters of mercury. Her complete blood picture, liver function test, kidney function test, and blood glucose were normal. Serum beta-human chorionic gonadotropin hormone was raised with a value of 10,000 mIU/mL (normal range in the seventh week is 7,650-229,000 mIU/mL). Per abdominal examination revealed uterus of normal size and tenderness was elicited in the right iliac fossa. On per speculum examination, the vagina and cervix were normal with the cervical opening closed and per vaginum examination revealed uterus of multiparous size with cervical motion tenderness and pain elicited in the right fornix. Transvaginal ultrasonography was done, which revealed an anteverted uterus within normal dimensions with no signs of intrauterine pregnancy. Bilateral ovaries were normal and mass of 6 cm with twin ectopic pregnancy with cardiac activity seen in both embryos (Figure ). Color Doppler and M mode ultrasound (Figure ) confirmed the presence of cardiac activity in both embryonic poles. The crown-rump length (CRL) of the first twin was 18 mm with the cardiac activity of 122 beats/min, and the CRL of the second twin was 20 mm with a cardiac activity of 128 beats/min, hence, in correspondence to eight weeks and four days of gestational age by Hadlock scale. There was no evidence of free fluid in the pouch of Douglas during the scan. The ultrasound findings suggested live monochorionic diamniotic twin ectopic pregnancy. As the patient had a history of left-sided ruptured ectopic pregnancy and had already undergone left salpingectomy for same, in order to conserve her fertility, the patient was managed conservatively by daycare procedure and underwent ultrasound-guided fetal reduction. In this, with the help of a 23 gauge needle, the yolk sac was punctured percutaneously and 0.5 ml methotrexate was instilled in both sacs. Immediately, there was an absence of cardiac activity in both embryos, and the sacs appeared to be irregular with evidence of hemorrhage within the sacs, thus concluding the procedure. |
This 52-year-old man presented at age 46 years with left-sided weakness, 2 days after a boating accident. He had a background history of hypertension and dyslipidemia. A right middle cerebral artery infarct was diagnosed. He was treated with thrombolysis and made a good recovery. An echocardiogram at the time showed a thickened interventricular septum (1.3 cm), a mildly dilated left ventricle, and impaired systolic function with an estimated ejection fraction of 40–50 %.\nHe had a normal birth and childhood. There were no other specific medical issues. The family is non-consanguineous of English background. He has three siblings—an elder brother in his 50s with type 2 diabetes and two younger sisters, in their 40s. None of his siblings have known cardiac disease. A paternal grandfather died from a myocardial infarction aged 63 years. A paternal aunt had a valve replacement. There is no other cardiac history within the family.\nHe remained well until aged 50 years when he developed palpitations, sweatiness and chest pain whilst driving. He was found to have ventricular tachycardia with presyncope and ECG showed sinus rhythm with markedly poor lateral R wave progression (Fig. ). An echocardiogram showed severe left ventricular dilatation. Systolic function was severely impaired with an estimated ejection fraction of 30–35 %. There was akinesis of the inferolateral wall and apical lateral wall and hypokinesis of the anterolateral and anterior wall. There was severe left ventricular diastolic dysfunction and moderate left atrial dilatation with mild mitral regurgitation. Cardiac MRI (Fig. ) confirmed that the left ventricle was severely dilated with a large area of thinning and akinesis affecting the entire lateral wall from base to apex (anterolateral, anterior and inferolateral walls). Other regions were hypertrophied with preserved systolic function. Late enhancement following gadolinium contrast was seen in the entire thinned region.\nOn examination, his weight was 81.2 kg. He did not have palpable hepatomegaly. Skeletal muscle strength was normal. An EMG was also normal. Blood glucose and lactate levels were normal. Blood film showed no vacuolation or increase in lymphocyte glycogen content. Red blood cell glycogen level was also normal at 22ug/g Hb (RR: 10–120). An acylcarnitine profile was normal. Creatine kinase was 88 IU/L (RR: 38–204). No urinary glucose tetrasaccharide was detected.\nThe patient remained symptomatic with shortness of breath on exertion. An ICD was inserted aged 49 years and a left ventricular assist device aged 51 years. Orthotopic cardiac transplant was performed aged 52 years. Initial post-operative course was complicated by renal impairment requiring haemofiltration. He is now well 6 months post-transplant. |
A 45-year-old male patient reported to our Department of Oral Medicine and Radiology, Vasantdada Patil Dental College and Hospital, Kavlapur, Tal-Miraj, Dist. Sangli, Maharashtra, India, with the chief complaint of facial asymmetry and missing teeth in the upper right posterior region.\nThe patient had noticed shrinkage of the right side of the face when he was 20 years old. The patient had visited a local physician, who suggested cosmetic correction for the same, but could not carry out the treatment because of financial reasons. The patient's past dental, medical and family history were non-contributory.\nOn inspection facial asymmetry was detected on right side []. The face on the affected side appeared smaller. Eyes appeared normal and of the same size. The pinna of the ear on the right side was pulled towards the front giving it a bat ear appearance. Ala of nose on right side was small as compared to normal side [].\nThe zygomatic bone appeared more prominent on right side due to depression in the cheek region.\nOn inspection the tongue was normal but on protrusion it deviated slightly towards the affected right side. The corner of mouth on right side was retracted. When patient was asked to clench, the prominence of the masseter muscle could not be palpated on right side.\nOn palpation, the ramus area and the depression below the zygomatic bone felt bony hard as there was absence of muscles. The skin on right side of the face was taut and not of the same texture when compared with the other side.\nLocal examination, measurements were taken from the nasion to the tragus, nasion to angle of mandible, and mid chin to tragus of both right and left side []. The measurements showed hemifacial atrophy of the right side.\nThe intraoral examination revealed edentulous area in first quadrant []. The ridge in first quadrant was thin. Patient also gave history of absence of deciduous and permanent teeth in the first quadrant. Generalized periodontitis was seen with the remaining teeth. Patient also gives history of exfoliation of lower molars on right side 10 years back.\nBased on the clinical features and facial measurements, a diagnosis of Parry-Romberg syndrome was made. Routine blood investigations were carried out which revealed all values within normal limits.\nAs a part of radiographic investigations an orthopantomogram, posterior-anterior cephalogram, transcranial, posterior-anterior chest X-ray and CT and MRI was made.\nThe orthopantomogram revealed that the changes in the mandible were more apparent than in maxilla. There was absence of maxillary teeth on right side. The affected right side of the jaw was small in overall dimension than the opposite side.\nThe condyle and coronoid process of mandible were normal in shape but smaller in size on right side. The body of the mandible was reduced in size on the affected side. Prominent antegonial notch on the affected side was evident []. The midline of the jaw was deviated towards the affected side. Transcranial radiograph and chest X-ray did not show any abnormality.\nComputerized tomogram (CT) of head and neck showed, hypoplastic right mandible and its condyle, absence of right masseter muscle as well as absence of right parotid and submandibular salivary glands [Figures and ].\nMRI also confirmed the absence of right masseter muscle and right parotid and submandibular salivary glands []. |
A 59-year-old female with a past medical history of breast cancer (status after radiation therapy (RT) and a subsequent mastectomy for tumor recurrence), osteoporosis, and hypercalcemia was referred for elective thyroid and parathyroidectomy. The patient had a thyroid lump for months which on imaging (PET CT scan) and ultrasound guided biopsy was found to be a papillary cancer with no distant metastasis (two weeks prior to the presentation). A parathyroid localization scan one day prior to the presentation was suggestive of parathyroid adenomas. At the time of presentation, vital signs, physical examination, and basic diagnostic lab test including complete blood count, comprehensive metabolic panel, and thyroid function tests were within normal limits except for a serum calcium level of 10.8 mg/dL (8.4–10.5 mg/dL). On December 20, 2007, the patient underwent a radicle surgical procedure where total thyroidectomy, parathyroidectomy and thymectomy were done. Muscular layer of esophagus was resected to ensure complete removal of the tumor. Esophageal repair and lymph nodes' dissection were performed and laryngeal nerves were preserved. Diagnosis of parathyroid hyperplasia and poorly differentiated ATC () with few nests of well-differentiated papillary cancer in a background of multinodular goiter with no involvements of regional lymph nodes was made. Histological examination of the biopsy specimen revealed that ATC approached within 1 mm of resection margins at several locations and was poorly differentiated with prominent eosinophilic cytoplasm of neoplastic cells, irregular nuclei with coarse chromatin, and prominent nucleoli. The biopsy specimen stained positive for cytokeratin AE1/AE3, cytokeratin CK5/6, and was negative for thyroid transcription factor-1, thyroglobulin, vimentin, smooth muscle actin, leucocyte common antigen, and carcinoembryonic antigen. During postoperative course, the patient did well and was discharged with instructions to continue with levothyroxine and to follow up in an outpatient oncology clinic. Subsequently, the patient was started on carboplatin, paclitaxel with concomitant RT. The patient tolerated the RT well except for some odynophagia necessitating a break of one week during the course of RT treatment. RT was delivered in daily fractions for a total of 36 days using three-dimensional conformal radiation therapy (3D-CRT) followed by intensity-modulated radiation therapy (IMRT) (total dose, 60 Gray). Chemotherapy was given in seven cycles for four months. The patient was closely monitored with frequent PET/CT scans, thyroid function tests, thyroglobulin, and thyroglobulin antibodies levels. In May 2010, PET/CT scan identified a hot 1.1 cm nodule in the right pulmonary apex which on wedge biopsy was found to be a metastatic ATC. The metastatic focus of the lung also stained positive for cytokeratin AE1/AE3, cytokeratin CK5/6, and was negative for thyroid transcription factor-1, thyroglobulin, vimentin, smooth muscle actin, leucocyte common antigen, and carcinoembryonic antigen, same as the original ATC tumor. The patient was given another course of RT for 46 consecutive days using 3D-CRT (total dose, 66 Gray), which patient tolerated well. Since then, the patient has been in a good state of health and has been regularly following in our oncology clinic without any evidence of tumor recurrence. |
A 10 year-old girl presented with the complaint of palpitation to a cardiologist. She had normal physical examination and laboratory tests, except tachycardia (heart rate = 130 per minute) and low TSH levels (0.005) with normal T3 (9.46) and T4 (145). She was referred to endocrinologist for possible hyperthyroidism evaluation. The thyroid gland was normal size, with no nodularity. She was diagnosed with possible thyrotoxicosis, but due to the normal physical examination, she underwent thyroid scan to rule out possible thyroiditis, which did not show any uptake in the thyroid gland, while there was an increased uptake in the right ovary (). Pelvic trans-abdominal sonography showed a heterogeneous complex solid mass of 113 × 112 × 100 mm with volume of 670 cc in the right ovary with no ascites. The patient had no complaint of abdominal pain or pelvic pain or abnormal uterine bleeding.\nShe was treated with methimazole 10 mg daily and propranolol 40 mg daily and were candidate for surgery after being euthyroid. The patient was referred to a gynecologist with the possible diagnosis of struma ovarii for further evaluation. She underwent right oophorectomy with the presumption of teratoma combined with thyroid-stimulating hormone (TSH)-suppressive therapy following treatment with I131. Total thyroidectomy was performed to permit evaluation for metastatic disease and monitoring for recurrence by thyroglobulin levels. The pathology report of the ovary mass indicated teratocarcinoma with 60% well-differentiated follicular thyroid carcinoma and 40% well differentiated follicular-variant with tumor necrosis, microscopic capsular invasion and peritumoral lymphovascular invasion, considering stage IC of PTC () and the thyroid gland did not show pathologic features of PTC.\nFurther evaluation with whole body scan with Iodine 123 (I123) showed metastasis to lymph nodes. She had high levels of thyroglobulin and received iodine therapy (150 mCi) twice. In the follow-up whole body scan, there was no trace of iodine uptake and the patient was symptom free.\nThe patient is now under treatment with levothyroxine 0.1 mg daily. Following 8 months after surgery and iodine therapy, she is totally symptom free. |
A nine-month-old boy was admitted to Vilnius University Children’s Hospital, Lithuania, due to a swelling mass of the anterior chest wall, which had appeared a week prior to hospitalization. He had fever, which had lasted several days a month before the appearance of the mass, but the boy was otherwise healthy and well-developed exhibiting sufficient weight gain. He received his BCG vaccination two days after his birth with no significant side effects after vaccination. At the age of three months he experienced a mild fall with no serious injuries. At the age of four months he had pneumonia, which was treated with a course of oral cefuroxime followed by a daily vibrant massage for five days.\nSwelling of approximately 2×3 cm diameter appeared in the upper sternal area at the age of nine months. The mass was hard, fixed and sensitive to palpation with no local skin hyperemia. No other signs of infection (e.g., fever) were present. Routine laboratory tests were within normal range. Frontal chest X-rays revealed a round mass anterior to the sternum, suggesting a diagnosis of osteomyelitis.\nAfter performing surgical resection of the affected sternum, a histopathologic examination of the mass tissue showed active granulomatous inflammation, the granulomas contains macrophages, epithelioid cells and multinucleated giant cells (Langhans giant cells), with subchondral bone destruction and necrotic areas suggesting Mycobacterium tuberculosis etiology. However, no acid-resistant bacilli were found. A diagnosis of chronic osteomyelitis was defined with reference to the histopathologic findings.\nAfter surgery the patient was consulted by many specialists and specific tests were performed for further elaboration of the diagnosis. There was not substantiation for neoplastic processes, therefore an oncological diagnosis wasn’t performed. Normal immunologic tests (normal values of immunoglobulin A, M, G, Ag, normal phagocytic activity of neutrophils and nitro-blue tetrazolium test, a sufficient level of circulating immune complexes and negative immunofluorescence assay of Human Immunodeficiency Virus (HIV) ruled out a possible diagnosis of primary or secondary immunodeficiency, ruling out the possibility of hematological disease. Changes in the chest computer tomography (CT) scan revealed a round mass with central hypodense areas both with and without contrast (). Additional data was collected from the patient’s parents claiming that the boy’s grandmother was suspected of suffering from tuberculosis. A consequent sternal biopsy was performed and M. bovis BCG was identified by a positive growth culture in liquid MGIT 960 system using the GenoType method (BD Biosciences, Sparks, MD, USA). These findings confirmed the diagnosis of M. tuberculosis BCG osteomyelitis. Treatment with rifampicin, isoniazid, ethambutol and streptomycin was started. Control CT scans were performed every 3 months. Full recovery was achieved after six months of antibacterial therapy (). |
A 74-year-old man was admitted to the hospital with a 2-week history of cough, fever and progressive shortness of breath. He had a background of hypothyroidism, hypertension and benign prostate hypertrophy. His initial imaging and swab test confirmed COVID-19. The patient was placed on a trial of continuous positive airway pressure to support his deteriorating oxygen saturation. However, after a week of supportive therapy, he was transferred to the ICU and was supported with mechanical ventilation and proning. The proning protocol used involves patients being proned for up to 18 hours a day ().\nWhile in ICU, the patient’s recovery was impacted by a Klebsiella-positive pneumonia, and a subsequent CT scan showed multiple pulmonary emboli. This was treated with low molecular weight heparin. During the second week in ICU, the patient’s critical care team identified anisocoria. They reported a left relative afferent pupillary defect (RAPD) with no new neurology or squint on gross assessment. There was no significant ocular history prior to this, and the patient’s ICU team booked a CT scan of the head and accompanying venogram, which did not highlight any notable pathology and ruled out venous sinus thrombosis.\nAn ophthalmology review was requested: on bedside examination the patient appeared orthophoric and a ptosis assessment was difficult to assess as the patient was in a sedated state. Pupillary inspection revealed a notable anisocoria; the left pupil was measured at 6 mm and was fixed with no change in light or dark/dim lighting (). The right pupil was 3 mm in light and 4 mm in dark assessment. Assessment of the left pupillary reflex identified no direct or consensual response and an RAPD. The right pupillary reflex identified a normal direct response, no consensual response and no RAPD. A full ophthalmological examination revealed a normal anterior segment without any signs of inflammation. There was an early cataract in both eyes. There was no inflammation in the vitreous, with normal optic nerve heads, retina and macula. Digital palpation was normal. iCare intraocular pressure (IOP) readings for the right eye were 18 mmHg and in the left eye 22 mmHg, although this was difficult to record accurately due to patient positioning. As IOP readings were relatively normal, we did not think IOP could be a cause of the anisocoria.\nAround week 4, the patient underwent a tracheostomy and was eventually decannulated. As he was weaned off his sedation, a clearer assessment of his lids was made and no ptosis was identified. The patient’s anisocoria did not change over the course of his admission, and as he was weaned off ventilation he expressed blurred vision.\nHe was now able to be safely transferred for an MRI of the head, where the ophthalmic team specifically asked the radiologist to focus on the midbrain. There were scattered intracranial haemorrhages, with a particular concentration of microhaemorrhages in the midbrain.\nThe patient was brought to ophthalmology clinic when it was safe to do so. Visual acuity in the right eye was 6/6 and in the left eye was 6/30. IOP in the right eye was 13 mmHg and in the left eye was 44 mmHg. Ishihara scores in the right eye were 17/17 and in the left eye 3/17. Diluted pilocarpine or accommodation did not cause constriction of the left pupil. Gonioscopy showed closed angle in the right eye and between 180° and 270° angle closure in the left eye. There was no cupping of the optic disc. Visual fields were normal in the right eye and revealed a glaucomatous visual field defect in the left eye. A diagnosis of subacute closed angle glaucoma was made. |
The blind and painful right eye of a 38-year-old man was eviscerated in September 2016. The patient stated that his right eye had been blind since early childhood due to a unilateral congenital anomaly complicated by secondary glaucoma. He received the diagnosis of SO in January 2017 after he experienced visual loss in his only seeing (left) eye. At the time of diagnosis, the patient was admitted to the hospital and meticulously investigated for possible infectious and noninfectious causes to rule out other uveitic entities, but without any positive findings. At that time, his best-corrected visual acuity was 6/10. Slit-lamp examination yielded some vitreous cells in the left eye. Fundoscopy showed a few scattered pigmented chorioretinal scars and discrete yellowish round choroidal lesions throughout the left fundus (). Fluorescein angiogram delineated the active lesions as early hypofluorescent () with late staining. Left macular contour was normal on optical coherence tomography (OCT) examination (). He was started on oral prednisolone (64 mg) for 2 weeks with gradual tapering of 8 mg per week. Despite initial visual improvement, he experienced another episode of visual decline while taking 32 mg of prednisolone. His best-corrected visual acuity decreased to 2/10 and he had grade 4 vitreous haze according to the Miami grading. Fundus examination showed marked yellowish-white discoloration of the macula with some evidence of intraretinal hemorrhage ( and ). He was hospitalized and treated with pulse methylprednisolone 1 g (250 mg 4 times daily) for 3 days. Following pulse therapy, 64 mg oral prednisolone and 150 mg (50 mg 3 times daily) azathioprine were co-administered. Two weeks after the completion of pulse therapy, his visual acuity was still 2/10 despite a significant reduction in vitreous haze. Fluorescein angiogram and OCT demonstrated type 2 choroidal neovascularization (). Five intravitreal 2 mg aflibercept injections were given within a period of 8 months. His final visual acuity was 6/10 with a stable-looking macula ( and ) and he was continued on a treatment regimen of 150 mg azathioprine and 8 mg prednisolone daily. |
A 29-year-old woman Para 2 was admitted to the emergency department with increasing lower abdominal pain. The patient had no history of systemic disease or chronic abdominal tenderness. Her past obstetric history revealed a caesarean section performed at 38 weeks because of a breech presentation three years earlier.\nThe patient had undergone an uncomplicated elective repeat caesarean section 7 days before being admitted to the emergency department. Indication for CS was breech presentation at term. The operation was without complications except some heavy bleeding occurred after the incision of the lower uterine segment; the bleeding was controlled by means of haemostatic stitches. The postoperative course was uneventful and the patient was discharged in good clinical conditions four days after CS.\nThree days after CS she reported a significant decrease of postpartum discharge (lochia) as well as a sudden complete stop of vaginal bleeding and discharge on day 4 after SC.\nOn admission, patient was alert, conscious, and well-oriented. She complained of severe low abdominal pain. Blood pressure was 140/80 mm Hg, HR 105 b/min, and temp. 37.4. The pain started the day before the admission as an abdominal discomfort with a sudden increase during the following day. At the time of her admission the patient described a constant low abdominal/pelvic pain with colicky exacerbations. Examination at this stage found no vaginal bleeding. Palpation revealed a painful swelling on the right side just above the pelvis. There was no sign of peritoneal irritation or abdominal distension. The wound seemed to be healing well. Lab tests at admission: Hb 12,8 g/dL, Hct 35, PLT 280, and WBC 10,5 × 109/L.\nRoutine urine examination was normal. Ultrasound investigation showed a strongly involuted uterus (LD 7.5 cm, TD 4.2 cm, APD 3.7 cm) which was localized centrally in the pelvis and also an oval-shaped mass on the right side of the lower abdomen (25 cm × 8 cm), showing thickened walls with complex internal echo patterns apparently suggesting a organized haematoma. In the pouch of Douglas, there were no signs of active bleeding or clots. Despite of the administration of meperidine hydrochloride 100 mg IM, a reassessment after two hours showed increased symptoms with additional signs of peritoneal irritation and restlessness whereupon an explorative laparotomy was decided.\nAccess was gained through the previous Pfannenstiel incision. At the opening of the abdomen a large amount of coagulated, dark red blood was evident amongst the intestinal loops. A further exploration of the pelvis showed an involuted uterus without signs of recent hysterotomy. On the right side, at the level of right iliac fossa, there was an oval, reddish, soft structure with a maximum diameter 25 cm. Mobilisation and further inspection revealed an enlarged uterus situated on the right side in respect to the small one (uterus didelphys). This second uterus showed a torsion of 90° on its axis and had a congested appearance.\nThe uterine cavity was filled with blood (lochia) coming out the tubae by squeezing of the uterus. There were no signs of uterine weakening. During the inspection before detorsion it was noted a transverse hysterotomy suture on the right side of the uterus which was not bleeding. The other smaller uterus appeared firmly fixed to the anterior pelvic fascia and the lower segment of the left anterolateral wall of the other uterus (). Both adnexa were normal at inspection. All adhesions needed to be removed before attempting a complete emptying and detorsion of the organ (). Postoperative recovery was uneventful and the patient could be discharged 5 days after the operation in good clinical condition. She got prescribed enoxaparin 20 mg per day for 6 weeks to prevent thromboembolism. |
A 36-year-old Caucasian woman, gravida 3, para 2, presented to our antenatal outpatient clinic in the 10th week of gestation complaining of uterine prolapse and amenorrhea. Five years earlier, at the age of 31 years, she had her first spontaneous vaginal delivery, after 39 weeks of clinically unremarkable gestation and after a seven-hour labor. A living male baby weighing 2950 g, with Apgar scores of 10/10, was delivered. After that, a total uterine prolapse (POP-Q IV) was observed and, therefore, a pelvic reconstruction operation was scheduled. However, she missed the appointment and she was lost to follow-up.\nFour years later, at the age of 35 years, the patient had her first pregnancy in a prolapsed uterus and the delivery was performed by an elective caesarean section after 38 weeks of gestation. During this second pregnancy follow-up she experienced symptoms of heaviness, but no pelvic pain or urinary incontinence. Pelvic examination showed that the uterus persisted in the pelvis because of increased volume. The cervical os was closed, while the entire cervix was lying outside the vulva during the first three months and after week 18 it appeared completely inside. When the cervix was outside the vulva, it appeared enlarged and edematous with marked ectropion but it was not ulcerated. A live male baby weighing 3150 g, with Apgar scores of 10/10, was delivered with elective caesarian section. After that, a total uterine prolapse persisted but she refused any procedure for pelvic reconstruction; neither was a vaginal pessary used.\nOne year later, at the age of 36 years, she presented again in our clinic with a 10-week pregnancy in a prolapsed uterus. A vaginal pessary was applied to keep the uterus inside the pelvis after manual reposition. The pessary was removed at the 24th week. The gravid uterus persisted in the abdominal cavity because it was increased in volume (Figure ). She did not show any symptoms of heaviness or urinary incontinence. The cervix was lying at the os of the vulva (POP-Q II) without signs of dessication or ulceration. It was enlarged and edematous but showed no evidence of cervical incompetence.\nSerial transabdominal ultrasonograpic examinations showed a normally developing fetus in longitudinal position in the uterine cavity. Elective caesarean section was performed at the 38th week. A living, healthy female baby weighing 3030 g, with Apgar scores of 10/10, was delivered.\nThe postnatal period was uneventful and she was discharged home four days later in good health. Normal postpartum uterine involution was observed. After that, a total uterine prolapse (POP-Q IV) was still observed (Figure ).\nShe is scheduled for follow-up examination and pelvic reconstruction surgery. |
The patient is a 71-year-old white male who was found to have a 3.5 cm right kidney mass and had been followed by the urology team closely at VA Pittsburgh Healthcare System. Urine cytology was suspicious for malignant cells. He underwent a radical right nephrectomy on February 3, 2014. Pathology showed clear cell RCC. The tumor was located at the lower pole with a size of 4.5 cm (pT1b) and Fuhrman nuclear grade 2. All margins were not involved by carcinoma, and there was no vascular invasion. He had been followed with a regular CT scan every year. He was found to have small bilateral lung metastasis and lymphadenopathy in 2016. The PET scan on April 26, 2016, revealed FDG activity in the lung and hilar and mediastinal lymph nodes. He underwent endobronchial ultrasound biopsy of the mediastinal lymph node which confirmed to be metastatic from clear cell RCC. Due to his comorbidities and mild thrombocytopenia, we started him on lower dose sunitinib at 37.5 mg per oral daily ×4 weeks every 6 weeks in May 2016. In total, he received 7 cycles of sunitinib. He had been followed every 6 weeks in the clinic. He only developed fatigue due to mild hypothyroidism for which he received levothyroxine. During the follow-up, he was found to have worsening thrombocytopenia with platelet counts in the range of 60,000 to 90,000. A follow-up CT scan and PET scan in October 2016 showed improvement of the lung metastasis and lymphadenopathy. He was last seen in the clinic on March 13, 2017.\nHe was admitted on March 29, 2017, due to muscle weakness, fatigue, poor oral intake, and difficulty swallowing for 2 weeks. During admission, his platelet count was found to be 13,000, serum creatinine 2.3, total bilirubin 4, AST/ALT > 2000, INR 2.9, calcium 7.5, creatine phosphokinase (CPK) > 5000, and uric acid 12 (see ). Sunitinib was discontinued on the first day of admission. CT head revealed no evidence of metastatic disease. Chest X-ray did not show evidence of infiltration or effusion. Echocardiogram showed severe global hypokinesia with LVEF of 30–35%. His LVEF was 55% prior to starting on sunitinib. He quickly developed lactic acidosis and acute respiratory failure. In the intensive care unit, he received bicarbonate, high-dose oxygen, furosemide, and treatment for hyperkalemia. Despite all treatment support, he continued to decline. His family chose to deescalate care, and he died on April 1, 2017. |
A 19-year-old male of Caucasian origin was admitted to our center as a polytrauma after a road traffic accident. He was previously fit and well, a nonsmoker with an alcohol intake of approximately 10 units per month. The accident, in which his motorcycle collided with an oncoming vehicle, caused him to sustain multiple significant injuries including unstable pelvic fractures and femoral fractures. He had bilateral pneumothoraces, extensive pulmonary contusion, and a splenic hemorrhage. He presented in extremis with signs of hypovolemic shock. He was intubated and resuscitated using local major hemorrhage protocols to achieve a blood pressure of 159/93, receiving ten units of packed red cells and four units of fresh frozen plasma in the emergency department.\nHe underwent an emergency laparotomy and splenectomy and was subsequently transferred to the intensive care unit, where he became increasingly hypoxic with features of adult respiratory distress syndrome (ARDS). This culminated in him receiving veno-venous extracorporeal membrane oxygenation (ECMO) from day 15 of his admission for 21 days. He returned to theater on day 21 for a massive haemothorax which required an emergency thoracotomy. After being decannulated from the ECMO circuit, he was stepped down to the general intensive care unit on day 36 and was transferred to the ward on day 55 before being discharged after a 4-month admission including a prolonged rehabilitation and recovery period.\nAfter presentation and commencement of ECMO, there was a relatively modest change in liver function tests. Alkaline phosphatase (ALP) increased from 55 to 143 IU/L between day 1 and day 6 of hospital admission and no persistent alanine transaminase (ALT) rise until after decannulation. Proceeding decannulation, there was a sequential increase in ALP peaking at 2335 IU/L on day 113. ALT rose to a lesser extent, peaking at 781 IU/L on day 52. The bilirubin did not rise above 57 μmol/L. The pattern of liver function tests is summarized in Figure . Autoantibody screen, immunoglobulins, and viral hepatitis serology were negative.\nSerial ultrasound, computerized tomography (CT), and magnetic resonance imaging excluded biliary stones and sludge. The liver on CT at day 1 showed normal liver and biliary structure (Figure ), and it was not until 10 months after the admission, the repeat magnetic resonance cholangiopancreatography (MRCP) demonstrated a multistenotic pattern of disease within the intrahepatic ducts (Figure ).\nIn the absence of significant casts within the biliary tree and no evidence to suggest biliary sepsis, endoscopic retrograde cholangiopancreatography (ERCP) was felt not to be helpful. A conservative management approach was taken, and the patient was instigated on ursodeoxycholic acid to help improve cholestasis. With bilirubin improving, the patient was closely monitored as an outpatient upon discharge for progressive liver disease and dysfunction. Repeat imaging and noninvasive fibrosis assessments were undertaken. Despite having evidence of SSC, the synthetic liver function has remained excellent, cholestasis markers have improved, and there have been no episodes of cholangitis or biliary sepsis for over 1 year. |
A 79-year-old male with a medical history of cholelithiasis, for which he underwent cholecystectomy 11 years ago, hyperlipidemia, essential hypertension, and paroxysmal atrial fibrillation was admitted for mild acute pancreatitis. His presentation included severe epigastric pain radiating to the back, lipase of 1840 u/L, and no organ failure. He denied fever, chills, or body aches. The patient denied alcohol consumption and reported to have quit smoking 41 years ago. Liver chemistries, serum bilirubin, serum triglycerides, and immunoglobulin subclasses were within normal limits (). Abdominal CT scan demonstrated peripancreatic haziness consistent with noncomplicated acute pancreatitis (). Home medications included atorvastatin for several years and the addition of 160 mg fenofibrate six months prior to admission. These medications were held upon admission but resumed at discharge. Full medication lists on admission and at discharge are shown in (). The patient was managed with fluid resuscitation and pain control. The patient recovered well and was able to tolerate regular diet without any pain or nausea. The patient was discharged in a stable condition after three hospital admission days, and lipase level at the day of discharge was 307 u/l.\nThree days later, the patient returned with similar symptoms. Lipase levels were >30,000 u/l (), and magnetic resonance cholangiopancreatography showed acute interstitial edematous pancreatitis (). The patient was treated conservatively with intravenous fluid resuscitation, pain control, and nothing per mouth until his symptoms resolved. Four days after his second admission, the patient recovered well and was discharged home in a stable condition. Fenofibrate and atorvastatin were discontinued ().\nTwo months later, the patient remained asymptomatic and returned for further workup to rule out an alternative cause to fenofibrate-induced pancreatitis. Endoscopic ultrasound (EUS) was done, which showed a 12 mm x 20 mm pancreatic head mass without pancreatic duct stenosis, strictures, or dilation. There were no common bile duct abnormalities. Fine needle aspiration was performed, and cytology was negative for malignant cells. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to place a prophylactic pancreatic duct stent. Cholangiogram revealed a normal biliary system without dilation or strictures. The biliary tree was swept, and nothing was found. A prophylactic pancreatic duct stent was placed and a prophylactic sphincterotomy was performed. No recurrence of pancreatitis has occurred as of 6-month follow-up.\nGiven the course of the patient's illnesses in relation to fenofibrate usage, the timespan of drug initiation, and the fact that he had been taking atorvastatin for many years without previous signs or symptoms of acute pancreatitis, we hypothesize that his recurrent disease is probably due to the use of fenofibrate. |
An 11-year-old boy, educated up to class 3, belonging to a rural family of lower socioeconomic status was brought with complaints of academic decline since 2 years, repetition of acts, irritability since a year, and adoption of abnormal postures since 6 months. He was born out of a nonconsanguineous marriage, uneventful birth, and pregnancy. He was third in birth order and achieved developmental milestones at an appropriate age. Since 2 years, he would not attend to his studies, had poor attention, and difficult memorization. He attributed it to lack of friends at school and asked for school change. There was no history of low mood, depressive cognitions, conduct problems, or bullying and he performed his daily routine like his premorbid self at that time. Since a year, he was observed to repeat certain acts such as pacing in the room from one end to another, continuously for up to 1–2 h, with intermittent stops and often insisted his mother to follow the suit, stand nearby him, or else he would clang on her. He prohibited other family members except his mother near him and would accept his meals only from her. He repeatedly sought assurance of his mother if he had spoken everything right. He also washed his hands repeatedly, up to 10–20 times at one time, and was unable to elaborate reason for the same. His mood during that period was largely irritable with no sadness or fearfulness. He mostly wore the same set of clothes, would be forced to take bath or get nails/hair trimmed, and efforts to these were often met with aggression from the patient. Eventually, he stopped going to school and his family sought faith healing. Within the next 5–6 months, his illness worsened. Fixed gaze, reduced eye blinking, smiling out of context, diminished speech, and refusal to eat food were the reasons for which he was brought to us. His physical examination was unremarkable and his mental state examination using the Kirby's method showed an untidy and ill-kempt child, with infrequent spontaneous acts, and occasional resentment for examination. He had an expressionless face, with occasional smiling to self, negativism, and mutism. No rigidity in any of the limb was observed. He was diagnosed with catatonic schizophrenia and probable obsessive compulsive disorder (vs mannerisms). We performed a battery of physical investigation to rule out organic psychosis []. He responded to injection lorazepam with which catatonia melted away. He was also prescribed olanzapine up to 15 mg/day, fluoxetine 20 mg/day, and dietary modification and lactulose for constipation. The family left against medical advice with 50%–60% clinical improvement [rating on Bush Francis Catatonia Rating scale (BFCRS) reduced from 10 to 4]. He relapsed within a month of discharge, initially with predominance of the probable obsessive compulsive symptoms. Fluoxetine was further increased to up to 60 mg/day. But within the next 2 months, the catatonic symptoms reappeared and he was readmitted. He had received olanzapine up to 25 mg/day, which was replaced with risperidone. In view of nonresponse to intravenous lorazepam, we administered him five sessions of modified bilateral Electro-convulsive therapy (ECT) (rating on BFCRS reduced from 8 to 0). The family was psychoeducated about the child's illness and the need for continuous treatment was emphasized. He was discharged with up to 80%–90% improvement. At follow-ups, he started participating at farm work of the family, took care of self, with some repetition of acts such as washing of hands, and denied any associated anxiety symptoms. However, efforts to re-enroll in school had been futile as the child did not agree for it. He has been maintaining at the same level since 6 months of discharge. |
A 72-year-old Japanese woman was referred to our institution for treatment of congestive heart failure with dyspnea on exertion. At 57 years of age she had experienced Stanford type A acute aortic dissection and underwent replacement of the ascending aorta under deep hypothermia and retrograde cerebral perfusion using a 30-mm vascular prosthesis without malperfusion. At 66 years of age she was diagnosed with severe aortic valve stenosis with a narrow aortic annulus and underwent aortic valve replacement with a mechanical valve (ATS Open Pivot® AP™ 18 mm; ATS Medical Inc., Minneapolis, MN, USA) accompanied by enlargement of the aortic annulus (Nicks procedure\n[]). To enlarge the aortic annulus, a glutaraldehyde-fixed equine pericardial patch was used to form the aortic root from the ascending aortic vascular prosthesis to the non-coronary cusp of the aortic valve. The patient had received warfarin therapy for atrial fibrillation after aortic valve replacement. Six years after the aortic valve replacement with aortic annulus enlargement, she presented with congestive heart failure.\nChest x-ray showed cardiomegaly with pleural effusion and pulmonary congestion. Computed tomography (CT) revealed a huge mass consistent with pseudoaneurysm in front of the right atrium and aortic root (Figure \n). Magnetic resonance angiography showed a pseudoaneurysm with blood flow from the aortic root near the sinotubular junction (Figure \n). Echocardiography revealed a pseudoaneurysm with continuous flow from the aortic root, moderate tricuspid regurgitation, and moderate mitral regurgitation. Peak velocity in the aortic mechanical valve was 2.7 m/sec (peak pressure gradient: 27 mmHg), with normal ejection fraction and no left ventricular dilatation. No inflammatory or infectious response was noted.\nThese findings suggested that the patient’s congestive heart failure was caused by cardiac compression from the pseudoaneurysm.\nRepeat median sternotomy risked cardiac injury or rupture of the pseudoaneurysm. Therefore, we attempted median sternotomy under cardiopulmonary bypass with moderate hypothermia. Additionally, an aortic occlusion balloon was inserted into the ascending aorta through the right femoral artery to prevent bleeding from the pseudoaneurysm. After systemic heparinization, cardiopulmonary bypass was established with an arterial cannula to the left femoral and right axillary arteries and a venous cannula to the right atrium through the left femoral vein. We then performed a repeat median sternotomy with the patient under moderate hypothermia of 28°C. Because of dense adhesions around the superior vena cava and ascending aorta caused by the pseudoaneurysm, lysis of adhesions and clamping of the ascending aorta were not possible. After circulatory arrest, we excised the pseudoaneurysm. We identified a longitudinal tear in the equine pericardial patch near the ascending aortic vascular prosthesis and an intact suture line (Figure \n). The ascending aorta was balloon-occluded and circulation was quickly reestablished. Excision of the pseudoaneurysm with mural thrombus created a defect in the right atrial wall. We removed the equine pericardial patch around the tear and performed patch repair of the aortic root using a double-layered patch consisting of an inner bovine pericardial patch layer and an outer Hemashield patch layer (Hemashield Double Velour Fabric; MAQUET Holding B.V. & Co. KG, Rastatt, Germany) with a felt-reinforced suture line. We then performed tricuspid annuloplasty and repair of the right atrial wall using an equine pericardial patch.\nAfter the patient was weaned uneventfully from cardiopulmonary bypass, transesophageal echocardiography showed an improvement in mitral valve regurgitation from moderate to mild. The patient was extubated 20 hours after surgery and discharged on postoperative day 20 with improving congestive heart failure. A year after the operation, no pseudoaneurysm-related change was detected. |
A 39-year-old male was transferred to our university hospital from a rural general hospital with generalized tonic-clonic seizures. The patient presented high fever, severe headache, and purulent nasal secretions over the last three days but his vital parameters were normal. Apart from his statement that he was a tobacco smoker, no previous medical conditions were reported.\nInitially, one week prior to hospitalization, he was diagnosed with inflammation of the paranasal sinusitis by a private otorhinolaryngologist who had prescribed a combined antibacterial medical treatment of amoxicillin/clavulanate potassium and clindamycin. However, five days later, he was referred to the emergency department (ED) of the rural hospital with epileptic seizure. Regarding his medical history, the patient had suffered from sinusitis five years ago but no history of epilepsy was mentioned, not even in his family history.\nThe CT scan that was performed revealed findings only in the right maxillary, ethmoid, and frontal sinuses but not in the parenchymal brain (Figures , ). Concerning the laboratory results, it was found a leukocytosis of over 20,000 per cubic millimeter of blood, elevated levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), and mild electrolyte disturbances. Because of high suspicion for meningitis or encephalitis, the patient was transported by ambulance to our university hospital for further investigation and treatment.\nFirstly in the neurology department, an intravenous combination of ceftriaxone, clindamycin, and acyclovir was given along with antiepileptics. However, for the following three days, there was no response to the antibiotics. On the contrary, the patient displayed dyspnoea and bradycardia but echocardiography did not demonstrate any pathology. For the dyspnoea, a CT scan of the chest was executed, which revealed pleural effusion and therefore it was decided to modify the antibiotic medication with vancomycin and meropenem.\nAfter 48 hours of treatment, the patient presented disorientation and abnormal behavior. The MRI of the brain that was performed, indicated the presence of a cerebral abscess in the right frontal lobe surrounded by oedema, which caused a slight displacement of the midline (Figure ). In a new CT scan, shadows in the right frontal sinus accompanied by frontal bone thinning in the lower-posterior wall were observed.\nA new evaluation was made by the otorhinolaryngologists who decided to proceed operatively. Endoscopically and under general anaesthesia, nasal polyps were detected in the nasal cavity, which were removed with the use of a flexible rhino endoscope. Then anterior ethmoidectomy and middle maxillary antrostomy were done along with drainage of pus from right sinuses. Ten days later, the fever was lower but still high and the leukocytosis was remaining. The patient started having disorientation while his headache was getting worse over days.\nDue to the unimproved clinical condition, neurosurgeons considered to proceed more drastically. Specifically, under general anaesthesia, a pterional craniotomy was performed and an open approach of the frontal and right temporal lobe was achieved (Figure ). Thereafter, the brain abscess was found in the right frontal lobe and anterior part of the right temporal lobe. The purulent material and the surrounding abscess capsule were completely removed, with intend to eradicate the infectious sources and to decompress the brain parenchyma by lessening intracranial pressure. In addition, samples were taken from the abscess and sent for microbiological study for antibiotic sensitivity. Pus culture was consistent with alpha-hemolytic streptococcus that was sensitive to the antibiotics.\nSubsequently, the patient was referred to the intensive care unit for constant care and close supervision for five days continuing intravenous antibiotics. The fever subsided within 72 hours. Neurological symptoms started resolving after 15 days and the patient was discharged nearly one month after the last operation presenting mild irritability, which was decreased gradually over the next two months. Six months later, an MRI was implemented where no signs of brain abscess were observed (Figure ). At the time of the last follow-up, one year postoperatively, the patient was found to be totally asymptomatic. |
A 65-year-old Caucasian woman presented for evaluation after a 2-year history of left hip and knee pain. On physical examination, the left knee presented with a mild effusion, tenderness and with stiffness and decreased mobility. The hip showed a true capsular pattern of limitation and pain with internal rotation of the hip. Radiographically, the left knee showed degenerative osteophytic changes and osteophytes in all compartments of knees and mild to moderate narrowing of the joint space. In case of the left hip, radiographic evaluation showed also advanced osteoarthritic changes.\nSince the physical examination and radiographic findings were compatible with the diagnosis of an end-stage hip osteoarthritis and knee osteoarthritis, the patient was first listed for a cementless total hip arthroplasty (THA) (Fig. ), followed by a cemented total knee arthroplasty (TKA) (Fig. ).\nDuring surgical dissection it was noticed that the articular capsule and the cartilage of the femoral head were black (Figs and ). Histological sections of bone and soft tissue demonstrated classic findings of ochronosis, including multiple pigmented areas by deposition of ochre-coloured granules, reactive giant cells, hyaline cartilage degeneration and a thickened and inflamed synovium. The case was discussed intraoperatively with the Senior Surgeon: the macroscopic evaluation of bone quality seemed reasonable and the OA diagnostic was the most consensual between the team. We maintained our initial plan and finished the cementless THA.\nAfter surgery she confirmed that was never diagnosed with alkaptonuria in her past. She was re-examined and we found black ochronotic pigmentation in sclera and ear cartilage (Fig. ), as well as dark urine. A high level of HA was also found in the urine. She was evaluated by a senior rheumatologist and progressed favourably without any complications.\nEight months later she was treated for her left end-stage knee osteoarthritis and we noticed again the presence of black cartilages (Fig. ). On follow-up examination (36 months) the patient progressed well, with a good range of motion and no reported hip or knee pain. |
49-year-old gentleman presented to the outpatient department of Head and Neck Oncology services of our institution with complaints of a large lesion in the oral cavity for a duration of one month. The patient also complained of dyspepsia, generalized weakness, malaise and loss of appetite. On examination, the general condition of the patient was poor in view of cancer related cachexia. He had a soft tissue lesion of the upper alveolus extending from the right upper canine to the right superior retro-molar trigone involving the entire upper alveolus and extending into the upper gingivobuccal sulcus (). The mass bled to touch but was non tender. Medially, it extended onto the hard palate for about half a centimetre. On abdominal examination, a mass was palpable in the right hypochondrium extending to the epigastrium. Rest of the abdomen was soft and non-tender. On further evaluation with a contrast enhanced computed tomography of the thorax, abdomen and pelvis, a 7×7×7 cm hypodense mass was noted in the segment VII and VIII of liver, which showed enhancement on arterial phase with washout on venous phase (). The mass was infiltrating the hepatic capsule with a peritoneal deposit abutting the diaphragm and muscles of the chest wall. Enhancing tumour thrombus was noted in the distended main portal vein and its branches. On upper gastrointestinal endoscopy, no varices were noted. Serum alpha fetoprotein was 2440 ng/ml. Liver function tests were normal. He was detected to be positive for Hepatitis B surface antigen. Blocks of the alveolar growth biopsy done elsewhere were reviewed at our hospital and was found to be a metastatic deposit of hepatocellular carcinoma positive for HepPar 1, arginase and focally positive for Glypican 3 whereas negative for CK7, p63, and DOG1 on immunohistochemistry (IHC) (). An ultrasound guided biopsy of the liver lesion was suggestive of hepatocellular carcinoma, positive for Glypican 3 on IHC ().\nIn view of advanced metastatic disease and poor performance status the patient was declared to receive the best possible supportive care. Episodes of bleeding from the alveolar lesion were managed conservatively. |
Miss AB is a healthy 24-year-old female who developed progressively worsening right hip and buttock pain after cessation of the oral contraceptive pill. Initially, this pain occurred only during menstruation and ovulation; however, it became more constant over the next 6 months with severe exacerbation during menstruation rendering her housebound for 3 days of each month. She had no dysmenorrhea or pelvic pain to suggest endometriosis. Sitting or lying for prolonged periods exacerbated the buttock pain and was associated with paresthesia over the posterior aspect of her right thigh, calf, heel, and dorsum of her right foot in addition to right gluteal muscle atrophy. The pain persisted despite strong analgesia and recommencing the oral contraceptive pill. She trialed physiotherapy and acupuncture without success. Investigations including ultrasound of her pelvis and right hip, as well as X-rays and computed tomography scans of her right hip and lumbosacral spine failed to identify any pathology. Finally, magnetic resonance imaging (MRI) scans of her pelvis revealed a 3.5 cm endometriotic lesion over the pelvic segment of her right sciatic nerve, before exiting the great sciatic foramen. MRI also identified atrophy of the right gluteus medius and minimus muscles, indicating the involvement of superior gluteal nerve [].\nMiss AB was then referred to our unit, and following detailed gynecological consultation, she was also referred for a comprehensive neurosurgical review. Other than limited right straight leg raise, the remainder of her lower limb neurological examination was normal. Electromyography nerve conduction study demonstrated evidence of L5 radiculopathy. Following a multidisciplinary discussion with a neurosurgeon, an orthopedic surgeon, and a gynecological oncologist, the patient underwent laparoscopic excision of endometriosis.\nPreoperatively, the patient underwent bowel preparation, with 24 hours of clear fluid and a single dose of PicoPrep (sodium picosulfate, magnesium oxide, and citric acid). The patient was placed in the standard dorsal lithotomy position. Primary trocar was inserted with open Hasson technique followed by three 5 mm accessory ports under direct vision in lower abdomen. Laparoscopic bipolar forceps and scissors without monopolar energy were used when operating close to the nerves to minimize injury due to lateral thermal spread from monopolar currents. Following thorough inspection of abdominal and pelvic cavities, noting no evidence of pelvic endometriosis, the endopelvic portion of the right sciatic nerve was approached through lumbosacral space as described by Possover et al.[] A peritoneal incision parallel to the lateral aspect of external iliac artery was made, and the underlying vessels were carefully retracting medially, separating them from the adjacent psoas muscles. Dissection was carried out caudally to identify the proximal segment of the obturator nerve typically surrounded by adipose tissue at the level of pelvic brim in an oblique orientation toward the obturator foramen. The lumbosacral trunk was then identified lying caudally to the proximal obturator nerve, traveling toward the greater sciatic foramen, also surrounded by adipose tissue. The lumbosacral trunk at the pelvic brim, consisting primarily of L5 and S1 nerve roots, joins sacral nerve roots from the sacral foramina to form the endopelvic portion of sciatic nerve. By extending the dissection along the lumbosacral trunk, toward the greater sciatic notch, a large area of fibrosis was encountered encapsulating the structures ventral to the sciatic nerve. The fibrosis extended dorsally to the level of the iliolumbar vessels as they cross laterally to the psoas muscle; laterally over the obturator internus muscle fascia but not involving the underlying muscle; medially through the parametrium encasing distal branches of anterior division of internal iliac artery including uterine artery, superior vesical as well as obturator artery; and ventrally along the obturator nerve, reaching 1 cm short of obturator foramen. Within this area of fibrosis, the obturator nerve was also encased with fibrotic tissue and densely adherent to the obturator internus muscle fascia laterally [Figures and ]. Vessels that had to be divided in the process of excising this diseased fibrotic area included uterine artery, superior vesical, obliterated umbilical artery, and obturator artery as per Possover et al.[] The fascia over the obturator internus muscle was involved with disease and was removed creating a disease-free interface at the lateral boundary. Disease over the sciatic nerve was removed piecemeal to minimize harm to the underlying nerve []. Significant fibrosis was present in the ventral-caudal border of the sciatic nerve where the pudendal nerve would be expected to exit the pelvis. As there were no symptoms of pudendal nerve involvement, further dissection was not carried out. The superior gluteal nerve could also not be clearly identified. Minimal residual disease and fibrosis were left to minimize potential serious neurovascular injury. Fibrosis and distortion of the anatomy made identification of anatomical landmark and course of vessels very difficult. A Mirena was fitted at the end of the procedure.\nMiss AB recovered well from her surgery with significant early improvement of pain and lower limb paresthesia over the first few days. It is not uncommon for patients to experience increased pain due to irritation of the sciatic nerve immediately after surgery. Postoperatively, she received 6 monthly injections of Zoladex (goserelin acetate), as has been used previously,[] then continued on Primolut N (Norethisterone) 5 mg BD. She successfully conceived with in vitro fertilization 3 years after her surgery, following a failed course of Clomid (Clomiphene citrate) for anovulatory dysfunction. At the time of writing this article, she is 26-week pregnant. |
A 26-year-old female was admitted to our department for elbow trauma following an accidental fall on her outstretched right hand with her elbow extended and supinated. She presented with pain and swelling in her right elbow. On examination, the right elbow was swollen with tenderness over the anteromedial aspect of the distal humerus. The elbow range was restricted and too painful. Neurovascular examination was unremarkable. Lateral radiograph () showed an intra-articular half-moon-shaped fragment lying proximal and anterior to the distal humerus simulating a capitellar fracture without associated elbow dislocation. But, on anteroposterior view, the fracture appeared to involve the trochlea showing irregularity of the medial joint space. There was a comminuted articular fracture of the distal radius with an anterior displacement ().\nA computed tomography (CT) scan () allowed better analysis of the fracture. It showed an isolated shear fracture of the trochlea without any bony associated lesion of the elbow. The patient was surgically managed. Open reduction and internal fixation were planned for our patient. The fracture site was initially exposed through a medial approach. The ulnar nerve was identified and protected, the common flexors were detached from the medial epicondyle, and the capsule was incised. An osteochondral fragment was displaced proximally. Anatomical reduction was achieved and the fracture was fixed with one Kirschner wire which was directed perpendicular to the fracture line. The reduction of fracture was confirmed preoperatively by direct visualization and fluoroscopic examination (). Then, stability and range of movements were checked after the fixation. Common flexors were reattached to the medial epicondyle using nonabsorbable sutures. Subsequently, the distal radial fracture was treated by open reduction and plate fixation through an anterior approach. Postoperatively the limb was immobilized in 90° flexion with an above-elbow back splint for two weeks in order to allow soft tissues healing. Gradual mobilization was started after its removal. The follow-up period was 2 years. At the last control, she was symptoms-free. She turned back to her initial employment. We report excellent functional outcomes according to Dash score which was 83,25/100 (). No signs of osteonecrosis or arthritis were found on follow-up radiographs (). |
A 46-year-old woman (gravida 2, para 2) was referred to our hospital complaining of a lower abdominal mass and pain. Her medical history was unremarkable. She was initially diagnosed with a uterine leiomyoma by transcervical needle biopsy. CT revealed a large heterogeneous tumor occupying the pelvic cavity and an intravascular tumor within the dilated left internal iliac and ovarian veins (Figures and ). Her preoperative cervical cytology results were negative for intraepithelial lesions and malignancy. The endometrial cytology and needle biopsy results were also negative. Thus, the preoperative diagnosis was IVL, with extension of the tumor into the left internal iliac and ovarian veins.\nIntraoperatively, multiple myomas were found within the uterine corpus and cervix, and the tumor extended to the parametrium and paracolpium. Detachment of the tumor from the left ureter and vaginal wall was very difficult. Intravenous tumors in the left internal iliac and ovarian veins could be palpated. The left internal iliac vein forming the common iliac vein was transected at the bifurcation region. In addition, TAH and bilateral salpingo-oophorectomy (BSO) were performed, resulting in the complete surgical resection of the tumor (operative time, 11 hours; blood loss, 8462 g). The resected uterus and adnexa weighed 897 g (Figures and ). There was no residual tumor detected in the venous resection stump.\nThe nodule resected from the uterus and the internal iliac and ovarian veins consisted of a proliferation of spindle cells. There was no nuclear atypia and the mitotic index was low. In addition, vessel endothelium cells and a vascular smooth muscle layer covered the IVL (Figures and ). The tumor cells stained positive for Alcian blue (pH = 2.5) and the staining disappeared after hyaluronidase digestion. However, compared to that in Case 1, the intensity of the staining was weaker and less diffuse (Figures and ). Similar findings for hyaluronan expression were obtained using the sample retrieved from the preoperative needle biopsy.\nThe histopathological diagnosis of the uterine and intravascular tumors was IVL. There has been no evidence of IVL recurrence, with the most recent follow-up at 38 months postoperatively. |
A 38-year-old previously healthy male () presented with 2 months of MRC grade 3 dyspnea and 9 kg weight loss with no fever or night sweats. On arrival to the emergency room, he was requiring 6 L/min oxygen via nasal prongs to maintain saturations greater than 90%. Chest X-ray and chest computed tomography (CT) () suggested possible pneumonia overlying interstitial lung disease and pulmonary hypertension. CT and ultrasound showed small left axillary lymph nodes unamenable to biopsy. Abdominal ultrasound was grossly normal. Thus, the patient was diagnosed with presumed pneumonia and started on antibiotics, antiviral agents, inhaled corticosteroid, and bronchodilator therapy, with enoxaparin for venous thromboembolism prophylaxis.\nOver the next 5 days, the patient's oxygen saturation was kept above 90% with 8–10 L/min oxygen via nonrebreather mask. Barriers to diagnosis included inability to produce sputum despite induction, high oxygen demands contraindicating bronchoscopy, small lymph nodes unamenable to biopsy, and intolerance of supine position needed for perfusion scans. The patient was transferred to the Thoracic Surgery service for a surgical lung biopsy on day 6. However, the patient experienced right-sided chest pain, diaphoresis, and tachycardia of 160 beats per minute, followed by a convulsive episode with no postictal symptoms. After transfer to the intensive care unit, mean systemic arterial pressures were found to be low, with transient reduction to as low as 40 mmHg when the patient coughed or spoke. Transthoracic echocardiography () showed a dilated right ventricle with globally reduced systolic function. Right ventricular systolic pressures were shown to be 170 mmHg. Milrinone and epinephrine were started for hemodynamic support.\nDue to progressive symptomatic and hemodynamic deterioration, he was transferred to the Cardiovascular Intensive Care Unit under the cardiac surgery service at another hospital to facilitate Novalung insertion via central cannulation. The goal was to decompress the right ventricle in order to stabilize the patient's hemodynamics, as well as bridge to diagnosis and possibly lung transplant assessment.\nIn the operating room, the patient arrested when anesthetic induction was attempted. Thus, he was bridged to cardiopulmonary bypass via cardiac massage. Venous and arterial cannulation of the Novalung were, respectively, achieved via the pulmonary artery and left atrium through Sondergaard's groove, with simultaneous surgical lung biopsy. Novalung was started on 2 L sweep gas at 2 L flow. After sternal closure, the patient became increasingly hypotensive with transesophageal echocardiography showing right ventricular dysfunction. Thus, the patient was reopened and cardiopulmonary bypass was reinitiated. With extensive vasoplegia and increased demand for vasoconstrictors, cardiopulmonary bypass was converted to venoarterial ECMO to maintain mechanical support. At the end of the procedure, the sternum was left open with slight retraction provided by a converted 20 mL syringe, and the skin was closed.\nPostoperatively, the patient was maintained on VA-ECMO and Novalung for 110 h (). Flows were maintained at 2.5–3 L/min and 2.5–3.5 L/min for VA-ECMO and Novalung, respectively. On postoperative day 2, the surgical lung biopsy demonstrated signet-ring cell pulmonary tumor embolism and lymphangitic carcinomatosis. Figures and depict histological sections demonstrating lymphangitic carcinomatosis of the lung and tumor thrombus in both arterial and venous pulmonary vasculatures. demonstrates a thrombosed pulmonary artery, explaining the subacute pulmonary hypertension. Given the extent of metastatic infiltration and the need for mechanical support, palliative chemotherapy was contraindicated. After discussion with the family, all modes of support were withdrawn in appropriate order and the patient passed away.\nAutopsy results supported the diagnosis of lymphangitic carcinomatosis with signet-ring cell morphology. Vascular features suggested grades 3 and 4 pulmonary hypertension and multiple tumor thrombi. Right ventricle of the heart was moderately dilated. Examination of the stomach revealed a nonperforated 3.3 × 2 cm ulcer with involvement of poorly differentiated signet-ring cell adenocarcinoma. All sampled perigastric, peripancreatic, mesenteric, and omental nodules were positive for metastatic adenocarcinoma. |
Twenty four days old male neonate was referred to Shahid Sadoughi general hospital, Yazd, Iran, from Bandar Abbas on April 2008. He had a red mass on his left arm which was noticed at birth as a congenital lesion measuring 3 × 3 centimeters and occupying nearly 15-20% of his left arm length without any other problem. After two weeks, the lesion had become larger measuring 8 × 7 cm associated with anemia (hemoglobin = 9 mg/dl) and thrombocytopenia ( platelet count of 96 × 103 /μl). The platelet count decreased day by day and reached to 50 × 103 /μl and the mass occupied about 60% of arm length and circumference (). Magnetic resonance imaging (MRI) was not considered for the neonate because no MRI was available in the medical center and the patient's condition did not allow him to be referred to another center for MRI. He was transferred to ICU receiving oral prednisolone 5 mg /kg /day, but the platelet count didn’t rise and fell below 30 × 103 /μl. He then received 15 cc/kg platelet and intravenous methyl prednisolone (60mg/50cc DW, during three hours for five days). No rising in platelet count occurred. Meanwhile, the hemoglobin dropped to 4gr/dl. Despite platelet and packed red blood cell transfusions, no improvement occurred. So, subcutaneous injection of alpha interferon (5U/kg every other day) was considered as the next step. However, it was discontinued because of lack of effectiveness. In addition, the lesion was aspirated and the bulky mass was reduced in size but after 24 hours, it returned to its previous dimensions. The aspirated fluid was bloody and the pathology report was negative for malignancy. The patient became candidate of receiving vincristine, but his parents did not permit this treatment. However, the persistence of life-threatening thrombocytopenia needed further management. The alternative opinion was operation. Finally surgery was done with meticulous gentle dissection of tissues preserving intact blood vessels and nerves. The skin overlying the mass was tried to be preserved as much as possible. The mass was successfully excised and removed. Following surgery, platelet count quickly raised to 150 × 103 /μl. On macroscopic examination, there was reddish blue skin with underlying subcutaneous tissue measuring 8 × 6 × 2 centimeters. Microscopic examination revealed dermal tumoral lesion with subcutaneous component composed of ill-defined nodular lesion containing a mixture of small capillary-sized vessels which blended with slit-like spaces and glomeruloid nests of rounded and epithelioid endothelial cells with abundant eosinophilic cytoplasm (). Red blood cell fragments were identified between these cells (). Atypia and mitotic figures were minimal and inconspicuous. Diagnosis was confirmed by immunohistochemical staining (IHC). The neoplastic cells strongly expressed vascular endothelial growth factor receptor-3 (VEGFR-3) and were weakly positive for von-Willebrand factor (factor VIII-associated antigen). Most of the neoplastic cells expressed CD34 ().\nThe infant was discharged with good general condition two weeks after surgery (following forty five days of hospitalization). Six and twelve months follow up showed no evidence of recurrence or complications related to surgery. |
A 73-year-old Caucasian female patient was referred to our department for further work-up of painful cutaneous lesions (Fig. ) and several masses within her liver.\nEight weeks prior, she had observed an erythematous nodule on her right chest. Subsequently, similar cutaneous lesions had developed on her arms and legs, and later also on her buttocks and back. She did not report any abdominal complaints. Outpatient treatment with topical and systemic steroids based on a suspicion of erythema nodosum (EN) did not yield substantial effect.\nFour weeks prior, several liver lesions had been detected by ultrasound and were interpreted as metastases of a previously treated breast cancer. Additional imaging with computed tomography (CT) and magnetic resonance imaging (MRI) had been carried out (Fig. ) and confirmed the liver lesions.\nAs the nodules on her skin continued to spread and became increasingly painful, she was presented to the Department of Dermatology in our clinic. There, another attempt of steroids and an intensified local therapy resulted in no improvement of her clinical condition. Due to raising inflammatory parameters a work-up for possible infectious causes and an antibiotic therapy with piperacillin/tazobactam, and later with meropenem were initiated. A colonoscopy revealed two small polyps, which were completely removed. Pancreatic enzymes were markedly elevated. A punch biopsy of one of the skin lesions was obtained showing a lobular necrotizing panniculitis with “ghost cells” compatible with pancreatic panniculitis (Fig. ). CT, MRI and repeated ultrasound examinations (Fig. ) did not reveal any pathological findings in the pancreas. In contrast enhanced CT multiple sharply-bounded liver lesions were visualized in both liver lobes. Compared with the CT obtained during outpatient care, the lesions had progressed in size and measured from 1 cm to 6 cm. The perfusion pattern was non-hypervascular and the density was hypointense, partly comparable with the density of water. No necrotic areas were described within the lesions.\nBecause of a progressive worsening of her clinical condition and increasing laboratory markers of inflammation, the patient was referred to our Department of Internal Medicine. She complained about intensive pain all over her skin and required increasing dose rates of opioid analgetics. She did not report any weight loss, night sweats, fever, nausea or vomiting, abdominal pain or problems with food intake. Her past medical history was remarkable for invasive ductal breast cancer diagnosed in 1982 with local recurrences in 1990 and 2008. Moreover, a superficial spreading malignant melanoma had been treated in 2011 and a coronary artery disease with percutaneous coronary intervention in 2008 was reported. Family history was significant for malignant melanomas in all siblings and her mother. Continuous medication included acetyl salicylic acid, lercanidipine, metoprolol, enalapril and pravastatin with no recent change. No allergic condition was known.\nOn examination she was in poor general condition (ECOG performance status 4), tachycardic (102 bpm), slightly tachypnoeic (22/min) and normotensive (128/78 mmHg). Her temperature was 36.9 °C. Subcutaneous erythematous and painful nodules of 2–5 cm size were noticed throughout her integument. Some of them were spontaneously draining a brownish oily fluid. Moreover, more than 200 melanocytic nevi were observed on her skin. Examination of the head, especially focusing on the salivary glands was unremarkable. There was no pain on abdominal palpation, the liver was palpable 2 cm under the right costal arch and bowel sounds were normal. There was a positive tap sign on both patellae.\nLaboratory results of interest were: leukocyte count 21.5 * 10^3/μl (ref. 4–10 * 10^3/μl), hemoglobin 10.0 g/dl (ref. 12–16 g/dl), ASAT 52 U/l (ref. < 35U/l), GGT 235 U/l (ref. <40 U/l), AP 186 U/l (ref. 35–105 U/l), lipase 14747 U/l (ref. < 60 U/l) and CRP 237 mg/l (ref. < 5 mg/l). Alpha-Amylase, uric acid, ACE, CEA, CA19-9 and AFP were within normal range. Serology for Yersinia enterocolitica and pseudotuberculosis was negative, as well as testing for Mycobacterium tuberculosis and atypical mycobacteria. Rheumatologic testing including ANAs and ANCAs was unremarkable.\nScreening for possible infectious foci did not reveal any other source explaining the elevated CRP. Therefore, it was attributed to the skin lesions, which displayed clinical signs of inflammation and were partly draining pus in the further course. However, as microbiological evaluation was not able to prove any causative organism and inflammation markers were not substantially declining despite escalation of antibiotic treatment with additional vancomycin, skin lesions were classified as sterile. Leukocytosis was explained by concomitant steroid therapy.\nUltrasound displayed several liver lesions in both lobes with a maximum size of 53 mm. The pancreas was homogeneous and free of focal lesions. The pancreatic duct was not dilated and no avascular areas could be detected upon administration of ultrasound contrast agent. Ultrasound-guided puncture of one of the liver masses was performed leading to the histopathological diagnosis of a pancreatic-type ACC.\nUnfortunately, the condition of the patient had severely deteriorated in the meantime with further exacerbation of pain, increasing tachycardia and hypotension. Therefore, no tumor-specific treatment could be initiated. The patient died ten days after admission to our ward.\nHistopathological analysis of the core biopsy obtained from the liver mass revealed a cellular epithelial neoplasm composed of monomorphic polygonal or rounded cells arranged in compact acinar and trabecular structures (Fig. ). Immunohistochemical study revealed strong expression of pancytokeratin (KL-1) with variable expression of CK7 and diffuse strong cytoplasmic expression of trypsin (Fig. ), but lipase and amylase were negative. All other markers in the differential diagnosis were negative (CK5, CK20, HepPar-1, Synaptophysin, Chromogranin A, NSE, CD56, TTF1, ER, PR, protein S100, GATA3 and PAX8). These findings including in particular the strong and specific expression of trypsin confirmed the diagnosis of pancreatic-type ACC in the liver.\nAutopsy confirmed several liver masses measuring up to six centimeters in size. There was no evidence of a salivary gland tumor or a primary pancreatic tumor. Additionally, review of the slides from the patient’s previous breast cancer confirmed a breast cancer of no special type and excluded the possibility of acinar-like differentiation. Thus, the previous breast cancer was also unrelated to the patient's ACC. Cause of her death was attributed to multiorgan failure due to severe systemic inflammatory response syndrome.\nFinal diagnosis was pancreatic panniculitis due to primary pancreatic-type acinar cell carcinoma of the liver.\nTaking into account the conspicuous accumulation of malignancies in our patient and her family, genetic analysis for familial atypical multiple mole-melanoma (FAMMM) syndrome was recommended to her relatives.\nIn addition to the presented case, 130 reports on pancreatic panniculitis were identified in the English literature between January 1994 and November 2014 by using the search terms “pancreatic panniculitis”, “subcutaneous fat necrosis AND pancreas” and “lipase hypersecretion syndrome” in PubMed and by checking results for appropriate cross-references.\nIncluding the above case, all 131 cases (Table ) were analyzed in respect to available data on age and gender of the patients, the underlying condition, additional symptoms, the sequence of the appearance of panniculitis and the diagnosis of the underlying disease, laboratory values and the outcome. The stated percentages refer to the respective number of cases including data on the analyzed parameter. Statistical analysis was performed with IBM SPSS Statistics (IBM, Armonk, NY, USA) using Student’s T-test or Fisher’s exact test where applicable. p < 0.05 was considered significant. Graphs were generated with SigmaPlot (Systat, San Jose, CA, USA).\nOverall, 65 cases (49.6 %) were due to acute or chronic pancreatitis and 60 cases (45.8 %) had an underlying neoplastic condition. In six cases (4.6 %) other reasons were present, e.g. pancreas transplant rejection or pancreaticovascular fistula (Table ).\nPatients with pancreatic panniculitis had a mean age of 54.8 years. Yet, patients with neoplastic causes were significantly older than individuals with pancreatitis (Fig. ). 57.4 % of the patients were male with no difference in sex distribution between underlying pancreatitis and malignancy.\nIn 48.9 %, cutaneous lesions were noted prior to the diagnosis of the underlying disease. The mean duration from appearance of the first lesion to diagnosis was 85 days +/− 110 days (range: 2–540 days; median 42 days). This period was significantly longer when pancreatic panniculitis was due to a neoplasm than when a pancreatitis was present (Fig. ). Moreover, the portion of patients developing panniculitis before the diagnosis of the underlying condition was by trend higher in patients with neoplastic disease (66.7 %) than in patients with pancreatitis (48.3 %; p = 0.06).\nA PPP syndrome with additional signs of arthritis was present in 49 cases (37.4 %).\nOne hundred twelve case reports (85.5 %) contained information on the serum levels of at least one pancreatic enzyme. In all but two of these reports (1.8 %) either amylase or lipase were elevated – in one of these two cases only amylase had been measured. The mean level of lipase was 11560 U/l +/− 19010 U/l (range 7–89700 U/l, median 3942.5 U/l). Again, patients with pancreatitis and neoplastic conditions differed markedly with tumor patients having significantly higher lipase levels (Fig. ). ROC analysis identified a lipase level of 4414 U/l as best cut-off value with higher values having a sensitivity of 73.0 % and a specificity of 82.1 % for the diagnosis of a neoplastic cause (AUC = 0.785, 95 % CI 0.68 to 0.89).\nOnly limited data was available concerning survival and follow-up. 12 patients with pancreatitis (21.4 %) died from complications. For underlying malignancy, follow-up data was available for 29 patients. A Kaplan-Meier plot of survival was computed, yielding a median survival of 4.75 months after appearance of the first skin lesion (Fig. ).\nPanniculitis is a clinical finding, which can be caused by various etiologic factors including infectious, immunologic and neoplastic conditions [–].\nIn our case, numerous causes could be excluded, while others were very unlikely: No infectious organism could be detected directly or indirectly. Continuous medication was unchanged and unsuspicious for causing erythema nodosum. Imaging had not yielded any evidence of malignancy other than the finally diagnosed ACC. Rheumatologic disease was judged unlikely based on consultation with a rheumatologist.\nTherefore, regarding laboratory data and histologic results pancreatic panniculitis was the only possible diagnosis.\nOur case of pancreatic panniculitis is noteworthy for two reasons: The absence of pancreatic disease and the extrapancreatic manifestation of pancreatic-type ACC. The combination of both has not been previously described in the literature. Pancreatic panniculitis without definite proof of pancreatic disease is found in four cases in the literature: Beltraminelli et al. [] report a case of acinar cell cystadenocarcinoma of presumably pancreatic origin metastatic to the liver. However, clear evidence of a pancreatic primary tumor was absent on imaging. Freireich-Astmann et al. [] describe the history of a patient with hepatic metastases of an adenocarcinoma of unknown primary. CT did not show any pancreatic lesion and immunohistochemistry was negative for CA19-9 and CK19. Aznar-Oroval et al. present a case of gastric adenocarcinoma with hepatic metastases in association with pancreatic panniculitis, but without clinical or radiologic findings of pancreatic disease []. And finally, Corazza et al. [] report about a patient with multifocal hepatocellular carcinoma (HCC) and missing pancreatic lesions in CT.\nHowever, in all cases, no autopsy for definite verification of the absence of pancreatic disease was performed. Amylase or lipase were elevated in each of the cases, but could not be explained by clinical, radiologic or histological findings in all but Beltraminelli et al.’s case. While existence of a primary hepatic acinar cell cystadenocarcinoma should have been discussed in this case, findings are inconclusive in the other three.\nThe HCC described by Corazza showed “trabecular structures and acinar aspects”, features suggestive of or consistent with ACC []. As immunohistochemistry is not reported, the possibility of a pancreatic-type ACC of the liver cannot be fully excluded in that case.\nPrimary extrapancreatic ACC is extremely rare and only six cases of ACC originating in the liver have been described to date [, , ]. Diagnosis of pancreatic-type ACC originating from the liver requires exclusion not only of an occult pancreatic primary, but also of primaries at other possible sites, such as breast [] or salivary glands []. In our case, neither clinical nor radiological evidence for another primary was present, which was finally verified by autopsy findings. Moreover, re-analysis of the samples of the previously treated breast cancer excluded a hitherto undiscovered acinar cell carcinoma of the breast.\nBecause of the rarity of primary ACC of the liver, no typical pattern can be specified in the different imaging modalities up to now. So far, most of the cases described were initially misclassified as one of the most common primary liver malignancies, such as HCC or cholangiocellular carcinoma (CCC), due to their imaging appearance. Moreover, a recent study on imaging findings in pancreatic ACC also reported a high variability in several parameters analyzed []. Thus, a thorough histological work-up of specimens after a resection or core biopsy is required to ensure the correct diagnosis [, , ].\nWhat could be objected to the diagnosis of an ACC of the liver in our case is the multifocality of the liver lesions, which is suggestive for metastatic disease. However, despite thorough work-up no other primary was found. Furthermore, it is worth noting that ACC is normally relatively large in size by the time of diagnosis [], which makes an occult primary rather unlikely. In addition, multifocal growth of primary liver tumors is not unusual, e.g. in intrahepatic CCC [, ] and HCC [, ]. Indeed, primary hepatic ACC might originate from acinar trans-differentiation of biliary progenitor cells, thus representing the acinar counterpart of hepatic cholangiocarcinoma [].\nIn an analysis of more than 130 cases of pancreatic panniculitis described in the last 20 years, we could show that nearly half of the cases are associated with an internal malignancy. Current concepts of the pathogenesis of pancreatic panniculitis suggest a role of pancreatic enzymes produced or released by these tumors [, ]. Therefore – though only rarely so named [] – pancreatic panniculitis should be regarded as facultative paraneoplastic condition [] and a tumor screening, especially for pancreatic tumors, should always be included in the diagnostic work-up.\nThe analysis of different parameters of these cases revealed significant differences between patients with pancreatic panniculitis and associated neoplasm or pancreatitis. On average, patients with a tumor are older and have higher lipase levels. Moreover, it takes longer until a diagnosis is made in these cases. A lipase cut-off value of 4414 U/l is able to differentiate between underlying pancreatitis and neoplasm with a sensitivity and specificity comparable with CA 19–9 in ductal adenocarcinoma vs. benign pancreatic disease [].\nRegarding the epidemiology and the natural course of malignancy and pancreatitis these results are not very surprising. However, these items can provide a first orientation, which etiology has to be primarily suspected. Like this, they may trigger a particularly intensive search for tumors in older patients with high lipase levels and a long-lasting history of panniculitis.\nThis is even more important as pancreatic panniculitis seems to be a hallmark of poor prognosis in tumor patients. Median survival in the cases with underlying malignancy and included follow-up data was 4.75 months after appearance of the first skin lesion.\nOf course, this retrospective analysis has significant limitations as it is exclusively based on case reports. Though, it is the first systematic evaluation of survival in pancreatic panniculitis and poor outcome is remarkable, because over 50 % of the included cases were ACC patients, which otherwise have considerably better survival [, ].\nDue to the rarity of the disease, clear therapeutic algorithms for ACC are missing. Since most of the cases present with distant metastases only a subset of patients qualifies for resection []. Therefore, cancer therapy is often limited to palliative approaches like chemotherapy or ablative treatment. As in our case, patients often suffer heavily from the pain caused by their skin lesions and analgetic therapy is frequently not sufficiently able to reduce pain [, , , , ]. Thus, palliative treatment strategies are very important for symptom control as well.\nOctreotide has been reported to alleviate symptoms in some cases [–]. Chemotherapeutic agents reported to be used in patients with pancreatic panniculitis and underlying ACC include gemcitabine and the FOLFIRI regime [, ]. Furthermore, one case with resolution of panniculitis following metastasectomy [] and one case with marked symptom reduction after transarterial chemoembolization (TACE) of hepatic metastases [] are described in literature. Some success in the treatment of pancreatic ACC has been reported with the use of FOLFOX [], FOLFIRINOX [], cisplatin/etoposide [] and gemcitabine in various combinations including erlotinib []. |
A 75-year-old man was admitted to Accident and Emergency, drowsy, hypoglycaemic and unable to move his legs. He was found early in the morning by a family member; the last thing he remembered was watching television while sitting in a chair the night before. He was previously fully mobile, with a past medical history including insulin-dependent type 2 diabetes mellitus, hypercholesterolemia, hypertension and an alcohol drinking history of 30 units per week for 30 years. He reported no symptoms suggestive of either gastro-oesophageal or peptic ulcer disease and had no medical history of gastrointestinal disease.\nOn examination of his neurological system his cranial nerves were intact, upper limb power, reflexes, coordination and sensation were all normal. However on examination of his lower limbs, he had marked weakness predominantly affecting his proximal muscles, particularly on the right side (R > L 3/5 / 2/5). He had absent knee and ankles jerks, and plantar reflexes were up-going bilaterally. On sensory examination the spinothalamic tract was absent below the level of T6–T7 but the dorsal columns were unaffected. He was incontinent in both bladder and bowel function.\nAs spinal cord pathology of acute onset was suspected, urgent magnetic resonance imaging (MRI) of the spine was performed on day 1 (Figure ). This demonstrated a T5–T8 anterior infarction of his spinal cord; no compressive or haemorrhagic lesions were identified. Urgent neurosurgical and neurological advice was sought following which medical management with Aspirin, blood pressure lowering medication and prophylaxtic dose low molecular weight heparin was commenced on day 2. During the following days he underwent early neurorehabilitation with physiotherapy.\nOn day 4 he was awoken with nausea and an episode of dark brown vomit. On examination the abdomen was soft with mild distension. In addition he had absent bowel sounds and bilateral shoulder tip pain. Of note there was no guarding or rebound tenderness.\nInitial impression was an upper GI bleed and an emergency endoscopy was booked as well as an abdominal X-ray and erect chest X-ray. The chest X-ray showed bilateral signs of air under the diaphragm and the patient was urgently referred to the general surgeons with a suspected gastrointestinal perforation (Figure ).\nConfirmation of the perforation was sought by way of a computerized tomography (CT) scan of the abdomen. Urgent laparoscopic surgical management of the perforated viscus was subsequently organized.\nPostoperatively his rehabilitation course was slow. Over the following 4 weeks he regained some function in his legs with the proximal muscles regaining more than distal groups (R > L 4/5 / 3/5) though he remained incontinent of urine and faeces. He was discharged to a rehabilitation centre with a long-term catheter. |
A 2-months-old boy presented to the pediatrician for routine vaccination. Seizing the opportunity of this appointment, the parents mentioned their worries about constipation and failure to thrive. Poor height and weight gain compared to the child’s age was observed falling from the 50th centile for height and weight to the 10th centile. Physical examination revealed abdominal distension in the right flank and hypogastrium and diminished bowel sounds. On rectal examination the ampulla was empty, while the anal sphincter had normal tone.\nInitially a plain radiograph and an ultrasound exam of the abdomen were performed. A dilated bowel segment was imaged, in the right flank, full of feces with a peripheral intraluminal slit of air displacing other bowel loops to the left. Absence of air in the rectum was also observed (). The ultrasound exam revealed a hyperechoic «line» with posterior acoustic shadow, consistent with presence of air (). Subsequently, 48 h after rectal examination a fluoroscopic barium enema was obtained. The barium sulfate (BaSO) relative high-density suspension was preferred over water soluble contrast because of its ability for fine-detailed delineation of a possible transition zone. The preparation with laxatives was omitted. The barium was diluted with saline instead of water, to avoid increased water absorption by the bowel surface and subsequently was heated up to body temperature. A markedly dilated rectum and a somewhat normal caliber anorectal ring with presence of a not so discernible transition zone was demonstrated ( and C). Reviewing the abdominal X-ray, a senior radiologist, raised the suspicion of vertebral abnormalities at the level of lumbar spine (). These findings previously have been understated due to infant’s wrong position and the full of fecal content rectum. Specifically, a hemivertebra was noticed respectively to the first lumbar vertebra (L1) and a synostosis of L3 with L4 vertebrae. Further evaluation was completed with a Magnetic Resonance Imaging (MRI) ( and 3) revealing a syrinx (expanding cyst like lesion) in the medullary cone and multiple vertebral abnormalities. The brain MRI did not demonstrate Chiari malformation. The diagnosis of Hirschsprung’s Disease passed away and depression of autonomic nervous system activity due to syrinx was suggested. |
A 70-year-old woman with severe abdominal pain and vomiting of 24-hour duration was admitted to our hospital. She had history of scleroderma and rheumatoid arthritis and had been taking steroids for 5 years. In addition, she received operations of thymoma with myasthenia gravis 31 years ago and stomach cancer (pT1AN0M0) 3 years ago. Her gynecological history was unremarkable, and there was no history of postmenopausal bleeding or vaginal discharge. On the physical examination, her abdomen was very tender and was distended and showed muscle rigidity. Rebound tenderness was absent. Bowel sounds were hypoactive. There was no palpable mass. Her blood pressure was 81/43 mmHg and pulse rate was 110 beats/min. Laboratory studies demonstrated a white blood cell count of 22,350 × 106/L and C-reactive protein at 32.39 mg/dL (normal range <0.3). Urgent computed tomography (CT) of the abdomen reported the presence of fluid and free intraperitoneal air mostly in the upper abdomen (), in addition to dilatation of upper small intestine. It was not possible to confirm the specific site of perforation accurately but it was suggestive of perforation of GI tract.\nFollowing prompt resuscitation and intravenous antibiotics (flomoxef, 2 g/day, daily administration), laparotomy was performed, which confirmed the presence of free air and pus as well as an inflamed small intestine. Two thousand mL of pus was found to be present in peritoneal cavity. The rest of alimentary tract, gall bladder, and liver were normal. During peritoneal lavage, we found a perforation with a diameter of approximately 5 mm over fundus of the uterus. Total abdominal hysterectomy, copious saline lavage, and tube drainage were performed as a joint procedure. No colonic resection was deemed necessary. The uterus was soft and slightly enlarged. Retrospective review of the CT reported, in addition to the previously documented findings, a fluid-filled uterus with free air in the anterior wall (). Histological examination confirmed pyometra with no evidence of malignancy or cervical stenosis. Culture of the pus grew Bacteroides fragilis, Escherichia coli, and Eubacterium.\nPostoperatively, intravenous antibiotics (flomoxef, 2 g/day) and sepsis treatment were continued and the patient was admitted to the intensive care unit with strict management of respiration and circulation. Her condition improved over time and she was transferred to the gynecological unit on postoperative day (POD) 4. However, she broke into decrease in blood pressure and saturation, pyrexia, and abdominal distension on POD 11. Although hepatic and renal dysfunctions were not revealed, sepsis and multiple organ failure were suspected. A contrast-enhanced CT showed the increased abdominal pus, and then vancomycin (1 g/day) and meropenem (1 g/day) were added to antibiotic therapy. Despite exhaustive clinical efforts, sepsis grew progressively worse and the patient died on POD 16. |
A 61 year old man presented to emergency room with history of flank pain on right side, dysuria, urgency and frequency with occasional hematuria for 3 days associated with fever, chills and rigors. 3 weeks before this presentation he was admitted for renal colic and was found to have a new staghorn calculus in the kidney which was managed conservatively with 7 days of oral antibiotics. Review of systems noted a history of 40 pounds weight loss over 3 months, drenching night sweats and occasional low grade fevers for last 3 months. Past medical history was significant for multiple episodes of renal colic secondary to nephrolithiasis treated with lithotripsy several years ago. Social history was significant for 30 pack year history of smoking, occasional alcohol consumption and no substance abuse or high risk behavior. Family history and medication history were not contributory. At the time of presentation patient was noted to be hypotensive with a blood pressure (BP) of 78/49 mmHg and mean arterial pressure (MAP) of 59 mmHg. The hypotension was new compared with recent admission 3 weeks prior, where the BP readings were consistently above a MAP of 80. The hypotension did not correct with bolus of 3 liters of 0.9% normal Saline (NS) and in view of his history of dysuria and intermittent hematuria and recent diagnosis of staghorn calculus he was diagnosed with urinary tract infection (UTI) leading to urosepsis and septic shock. He was admitted to medical intensive care unit (MICU) where he was started on pressor support with norepinephrine and broad spectrum antibiotic coverage with vancomycin and piperacillin-tazobactam. Vitals recorded at presentation were temperature of 97.3°F, BP of 78/49 mmHg, MAP of 59 mmHg, heart rate 80/min, respiratory rate 18/min, SpO2 of 98-99% on room air. General exam was significant for an averaged sized man in mild distress with mild pallor, no icterus, cyanosis or edema. Systemic exam was significant for mild right costovertebral angle tenderness. Labs were significant for hemoglobin (Hb) of 9.2 g/dL, mean corpuscular volume (MCV) of 77.6 fL and leukocyte count (WBC) of 5.1 k/µL with differential of 77% neutrophils. Liver enzymes showed alkaline phosphatase of 212 U/L, Alanine transaminase (ALT) 80 U/L, Aspartate tranaminase (AST) 96 U/L, Total protein 3.6 g/dL and albumin 1.5 g/dL. Urine dipstick was positive for blood (1+), proteins (30) and glucose (50) and urine microscopy showed 109 RBC and 12 WBC. Rest of the lab results are shown in . Cortisol level at admission was 182.6 mg/dL which ruled out adrenal insufficiency. Two sets of blood culture and urine culture were done prior to starting antibiotics which showed no growth after 5 days of incubation. CT scan abdomen done at presentation showed numerous ill defined lesions in the liver which were new from the CT scan done 3 weeks before for evaluation of renal colic (). The study redemonstrated the staghorn calculus with no evidence of obstruction, no radiographic evidence of pyelonephritis or renal abscess. Patient received 2 days of pressor support with Norepinephrine drip, following which his blood pressure improved to a MAP over 70 mmHg, however he continued to have intermittent episodes of hypotension which were managed with frequent boluses of 1000 to 500 mL of 0.9% NS. Interestingly, on Day 5 of admission, patient developed increased shortness of breath and became hypoxic. Trans-thoracic echocardiogram done at bedside showed normal ejection fraction and normal inferior vena cava. Patient was diagnosed with fluid overload secondary to frequent fluid boluses and was given one dose of 20 mg i.v. lasix which led to resolution of shortness of breath. During this entire stay, he continued to have intermittent episodes of hypotension with mean arterial pressure dropping to low 60’s. Colonoscopy and esophago-gastro-duo-denoscopy (EGD) done as part of malignancy workup, showed 2 polyps which were diagnosed as tubular adenoma and thick gastric folds with chronic gastritis on histopathology respectively. A liver biopsy was planned after improvement in his overall condition however on day 9 of the hospitalization patient declined the procedure and requested a break from the hospital. Liver biopsy was deferred for a later date and patient was discharged in a stable condition. During this admission 4 blood cultures and 3 urine cultures did not show any growth after 5 days of incubation. He was discharged with oral levofloxacin to complete a course of 14 days of antibiotics for complicated UTI. Three days after being discharged from hospital, patient returned to emergency room with similar complaints of acute onset weakness and fatigue and a single episode of fever for which he received a single dose of Ibuprofen at home. Vitals at presentation showed rectal temperature of 94.1°F, BP of 84/49 mmHg, mean arterial pressure of 60 mmHg, breathing at rate of 18/min, heart rate 59/min and saturating 97% on room air. Examination this time was unremarkable. All labs at readmission are shown in . Patient was readmitted to MICU with a provisional diagnosis of urosepsis and was given vancomycin and piperacillin-tazobactam. Blood culture and urine culture done at this time again showed no growth which could support the diagnosis of sepsis. Biopsy of the liver lesions showed extensive lymphocytic and histiocytic infiltrates with abnormally large cells and positive stains for CD15 and CD30. Bone marrow biopsy also showed areas of residual trilineage hematopoesis with 40% cellularity alongwith several para-trabecular infiltrates composed of large atypical cells including Reed-Sternberg (RS) cells, in a mixed inflammatory background consisting of small lymphocytes, histiocytes, eosinophils and plasma cells (). The immuno-histochemical stains were positive for CD15 and CD30 and negative for CD45, CD3 and CD20. Bone marrow aspirate showed trilineage hematopoesis with orderly maturation. A 100 cell count showed granulocytes (56%), monocytes (1%), eosinophils (6%), erythroid precursors (34%), lymphocytes (2%) and plasma cells (1%). The presence of the characteristic RS cell in a mixed inflammatory background pointed towards a diagnosis of HL. The diagnosis was further confirmed by positive immune-histo-chemical (IHC) stain for CD15 and CD30 along-with negative IHC stain for CD45, CD3 and CD20. Since no pan-T antigens were missing, the possibility of a T-cell lymphoma was very low. A CT Chest for staging did not show any hilar lymphadenopathy. Soon after the diagnosis patient was started on chemotherapy with doxorubicin, dacarbazine, vinblastine. Bleomycin was initially withheld due to unknown pulmonary function in view of patient been active smoker and was added later after pulmonary function test turned out to be normal. After completion of the 1st cycle of chemotherapy the blood pressure started improving to a MAP of more than 80 mmHg (). At 6 month follow up the patient continues to be free of any episode of hypotension or hypothermia. |
A 20-year-old African American man was admitted to a psychiatric facility for psychosis. On initial presentation, the patient had an antalgic gait, which he attributed to his history of dopa-responsive dystonia. His mood was depressed and his affect was restricted. He had disorganized thought process and was slow to recall. He endorsed auditory hallucinations, paranoid delusions, depressive symptoms, frequent night awakenings, and persecutory nightmares. Per the ambulance report, the patient was wandering the streets in a confused state, so bystanders called 911. The patient stated that he had been homeless for the past 3 weeks. During this 3-week period, he admitted to not being complaint with his medications. Urine toxicology screen was negative.\nPer medical records, he was diagnosed with dopa-responsive dystonia at age 11 after a 2.5-year history of progressive abnormal gait. He was initially misdiagnosed with tight heel cords at age 10 and treated with serial casting that resulted in good improvement on the right but marginal improvement on the left. His toe walking became more pronounced overtime accompanied by worsening left calf pain and stiffness, increasingly frequent falls, and new onset of intermittent torticollis. These symptoms worsened over the course of the day. He was eventually taken to an urban teaching hospital, where he was diagnosed with dopa-responsive dystonia based on clinical presentation and marked improvement on a levodopa trial. Magnetic resonance imaging of the brain and spine was unremarkable at the time.\nAt age 15, he was diagnosed with schizoaffective disorder bipolar type. His psychiatric history is also significant for multiple psychiatric hospitalizations, history of previous suicide attempts with medication overdose, and history of trauma. He also endorsed marijuana use since age 15 and daily tobacco use since age 18. He denies using any other illicit drugs. Per collateral information from his mother, his schizoaffective disorder has never been well controlled given the conflicting effects of his medications. She also mentioned that he was placed in individualized education programs as a child due to learning disabilities. His family history is significant for bipolar disorder on his maternal side. His family history on his paternal side is unknown. In addition to carbidopa-levodopa, his outpatient medications included sertraline, divalproex sodium, aripiprazole, and benztropine.\nOn hospital day 1, he was started on carbidopa-levodopa 25/100 mg tablet three times daily for dopa-responsive dystonia. On day 2 of his hospital course, sertraline 50 mg once daily, benztropine 2 mg twice daily, divalproex sodium 500 mg twice daily, and risperidone 0.5 mg twice daily were added to his medication regimen. We started him on a low-dose risperidone to avoid exacerbating his dopa-responsive dystonia symptoms. Physical exams were also performed daily to assess for dystonia and parkinsonian symptoms. His initial physical exam revealed an antalgic gait secondary to left lower extremity dystonia, which improved by hospital day 2 and resolved by hospital day 3. On hospital day 3, he became agitated and aggressive with staff members, which led to intramuscular administrations of haloperidol 10 mg, diphenhydramine 50 mg, and lorazepam 2 mg. He continued to endorse auditory hallucinations, so risperidone was increased to 0.5 mg in the morning and 1 mg at bedtime. His auditory hallucinations resolved and then returned on day 6. He reported hearing “good” voices and “bad” voices. He also continued to endorse depressive symptoms, multiple night awakenings, and persecutory nightmares. As a result, his risperidone dosage was increased to 1 mg twice daily. On hospital day 7, the patient reported hearing “mumbling” voices only and improvement in his sleep and depressive symptoms. On hospital day 8, his auditory hallucinations fully abated. By hospital day 10, he slept throughout the night, no longer had depressive symptoms, and had normal spontaneous speech. His thought process was linear, logical, and goal-oriented. His mood and affect was euthymic and full range. No psychotic symptoms were noted. The patient was compliant with his medications throughout the whole hospital course and his daily physical exams were negative for dystonia or parkinsonian symptoms since day 3 of his hospitalization. He was subsequently discharged on hospital day 14 with appropriate outpatient follow-up. |
A 52-year-old man was referred to our dermatology department with painful, coalescent, erythematous, papulo-infiltrative, nodular plaques on his midface. He has a history of uncontrolled diabetes mellitus Type II and a history of CL that started 3 years ago as a slowly growing papule on his nose that evolved to a nodule. Direct smear was positive for leishmaniasis bodies. The patient was treated with systemic meglumine antimoniate (glucantime) for 20 days; the lesion was healed with a scar. Two years later, the patient had a recurrence of the lesion at the border of the scar; he sought folk medicine that resulted in severe ulcers and dissemination of the lesion, which led to deformity of the nose. Results of dermatologic examination revealed extensive erythematous papules and nodular plaques on his nose, upper lip and cheeks with telangiectasia on his cheeks and severe ulcers and scarring on the nose and upper lip (). The other parts of his face were spared. There was no evidence of systemic involvement. The results of the routine lab tests, including complete blood count (CBC) and serum biochemistry are listed in (). Smear was not made since the patient is known to have a history of leishmaniasis. Histological examination showed changes in the dermis with epidermal atrophy. There was a mixed inflammatory infiltrate composed of lymphocytes, histiocytes, plasma cells and multinucleated cells of Langhans (). Neither parasites within dermal macrophages (), nor tuberculoid granulomas with caseation necrosis were detectable. Diagnosis of LCL was established based on the clinical aspect, facial localization, chronic evolution, the history of CL and histopathologic findings even with the absence of intra-amastigote in the biopsy. Patient was admitted in the hospital and was started on systemic meglumine antimoniate (60 mg/kg/day) divided into two doses for 20 days in addition to ceftriaxone (500 mg twice a day) with application of topical cream diprogenta (betamethasone dipropionate and gentamicin sulfate). During the treatment, the patient was monitored daily for any clinical or laboratory signs of chemical pancreatitis, liver function tests, CBC in addition to a daily electrocardiography (ECG) test. On Day 10, the ECG showed a slight prolongation of QTc (QTc = 459 msec) for only 1 day. No other significant side effects were observed. At the end of the Day 20, improvement was noted and the patient was discharged and started on hydroxychloroquine (plaquenil) (200 mg twice a day) for a month. At the end of the month, he came for a follow-up and the lesion was completely healed (). |
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