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A 5-day-old male baby was referred to our hospital with a large oral swelling and difficulty in breastfeeding. On examination, there was a large tense, sublingual cystic swelling on the left side. It had lifted the tongue upward, backward, and to the right and produced a bulge in the submental and left submandibular region, which was confirmed on bimanual palpation. Another cystic swelling was present in the region of the left anterior triangle, which became more apparent when the child cried. Neither cross fluctuations nor any fluid shift could be elicited across the intraoral and the cervical swelling. The oral swelling was aspirated at birth in the previous center, but it had recurred rapidly to its present size. A possibility of plunging ranula/lymphangioma was kept.\nThe child was started on nasogastric feeds. His hematological and biochemical parameters were normal. Computed tomography (CT) scan revealed two large unilocular, homogenous cystic swellings. The oral swelling was noncapsulated, occupied most of the sublingual space with a retromandibular extension. The cervical swelling was in the subcutaneous plane, anterolateral, and to the left of larynx and trachea, capsulated and had no apparent communication with the oral swelling in either the sagittal or in axial view []. Considering the size and location of the two swellings, child's inability to breastfeed and history of recurrence following aspiration, excision was planned. Excision was commenced through the intraoral approach by incising the mucosa overlying the cyst. The cyst was carefully dissected from the mucosa and undersurface of the tongue avoiding the lingual vessels, nerves medially. The submandibular duct was cannulated at the outset with a 24 Gauge intravenous cannula as a safeguard against injury. The dissection was continued up to the level of floor of the mouth. Ipsilateral mylohyoid muscle was indistinct and stretched out by the cyst. To have a good access of superficial part of submandibular gland and in anticipation of any small communication between the two cysts that might have been missed at clinical examination and CT scan, it was decided thereupon to halt the oral dissection and commence dissection of the cervical cyst and traverse proximally to access the submandibular space. However, the cervical cyst was found to be independent of the oral swelling. The remaining oral dissection was then completed and the cyst excised. The use of magnification loupes was very helpful in delineating the small anatomical structures of neonate. We also used a muscle stimulator to protect the muscles of the tongue and floor of the mouth.\nGrossly, the cervical cyst was capsulated while the oral cyst was devoid of a capsule. Both cysts were filled with mucoid fluid. Postoperative period was uneventful. Breastfeeding was started on the 3rd postoperative day and he was discharged on day 8. Presently, he is thriving well. Histology revealed that both the cysts were lined by stratified squamous and columnar epithelium with an intact basement membrane. The subepithelial layers exhibited fibromuscular tissue, dense chronic inflammatory infiltrate, congested, and dilated blood vessels. In addition to the oral cyst, the cervical cyst too demonstrated the salivary gland tissue []. |
A 60-year-old male with metastatic RCC treated with nivolumab and palliative radiation therapy presented to our institution in 2016 with shortness of breath and was found to be in acute respiratory failure. Computed tomography (CT) of the chest was significant for multiple new ground-glass opacities throughout bilateral lungs concerning for therapy-induced pneumonitis (Figures , ). The etiology of ground glass opacities includes but is not limited to infectious pneumonitis, bronchioloalveolar carcinoma, or interstitial disease. Given the timing of symptom onset as well as lack of response to infectious treatment, therapy-induced pneumonitis remained high on our differential.\nHe initially presented in 2011 with gross hematuria and right-sided flank pain and underwent right radical nephrectomy and lymph node dissection of a 9 cm Fuhrman grade IV RCC with negative margins and lymph nodes. Two years later, surveillance imaging and biopsy were significant for metastatic RCC in the lungs. He was initially treated with one year of sunitinib, a multi-targeted receptor tyrosine kinase inhibitor. However, given the progression of disease, he was transitioned to one year of pazopanib followed by six months of axitinib, one month of everolimus, and five months of sorafenib. Pazopanib, axitinib, and sorafenib are also tyrosine kinase inhibitors. Everolimus is an inhibitor of mammalian target of rapamycin. Given the lack of response to these therapies, our patient was started on nivolumab at 3 mg/kg in May of 2016. Over the course of four years, he received targeted palliative radiotherapy including 1900 centigray (cGy) to a left upper lobe lung mass in May 2016 and 800 cGy to an L5 lesion in September 2016.\nHe complained of chronic shortness of breath for three months felt secondary to anemia and a left pleural effusion before presenting to our hospital in acute respiratory failure with CT evidence of new diffuse ground-glass opacities occupying the majority of both lungs (Figures , ). Given high suspicion for therapy-induced pneumonitis, he was started on a treatment course of high dose steroids. However, the patient’s respiratory status continued to decline and he passed away on comfort measures.\nPathology was significant for organizing diffuse alveolar damage with hyaline membrane formation in all lobes of both lungs away from the metastatic RCC (Figures , ). There was no evidence of an infectious process from cultures and pathologic evaluation. This histologic reaction pattern is a typical finding in patients with a clinical diagnosis of acute respiratory distress syndrome (ARDS) concerning for therapy-induced pneumonitis. |
A 45-year-old Greek man with IACF was prospectively evaluated. The patient presented with right closed IACF in March 2007 at our Orthopaedic and Traumatology Department. The treatment consisted of a closed reduction technique combined with balloon-assisted fracture augmentation with CPC. The mechanism of trauma was axial loading. Standard radiographic evaluation and MDCT scans were performed preoperatively (Figure and Figure ). The calcaneal fracture was classified as a type III according to the Sanders classification [].\nA calcaneus traction wire was placed from the posterolateral area of the calcaneus perpendicular to the longitudinal axis of the calcaneus and the varus, valgus malalignment was corrected (Figure ). Fluoroscopy was used to determine the quality of the calcaneal alignment and the degree of fracture reduction. A stylet and cannula were placed into the calcaneus followed by insertion of a bone tamp attached to a digital manometer (Kyphon®, Medtronic, Minneapolis, MN, USA) (Figure ). The balloon was inflated gradually under fluoroscopy (Figure and Figure ). At this time the bone cement was prepared immediately prior to its injection into the defect and the balloon was removed. The fracture void was cleaned of blood with NS solution and a bone filler device was inserted through the cannula to augment the calcaneal body. No arrest of blood supply was needed and no cast was applied.\nPassive ankle ROM was applied at the end of the procedure. The duration of surgery was 20 min. The patient showed immediate pain relief from the 1st postoperative day. Limb elevation and foot pump were used to prevent ankle swelling.\nClinical follow-up was performed 6 to 24 months postoperatively, using the Maryland foot score for the calcaneal fractures []. Postoperative MDCT scan was obtained for the evaluation of the cement position, the integrity of the joint surface and the calcaneal alignment. Active and passive motion exercises were performed under physiotherapeutic support. The patient was able to walk with partial weight-bearing beginning on second day. Full weight-bearing was performed after the first week postoperatively when the ankle swelling decreased. No superficial or deep infections were recorded. There was no need to undergo a secondary reconstruction procedure. Bone healing was seen on plain radiographs at 12 weeks. The immediate postoperative radiographs and CT scan revealed no loss of fracture reduction and good calcaneal alignment. Complete filling of the bone defects was seen. Radiography and CT scan evaluation at 2 year follow-up showed good fracture alignment (Figure and Figure ). No significant difficulty in toe-walking, heel walking or impingement of the peroneal tendons was observed. |
We describe a case report of a 40-year-old man who presented to the ED of a level-one-trauma center in 2016. He first was assigned to the neurological area on the base of suffering from several neurological symptoms. The patient was a normal weighting young man with a reduced general condition. The symptoms were present since two days. He had no additional chronic diseases except for intravenous drug use of heroin about ten years. The last use of heroin in his left groin was about ten days ago. The first examination showed a patient with normal conscious level. He felt “not well” and in the morning of the day he presented at the hospital he also noticed a dry mouth with a numb tongue.\nHe had a fever about 38,7 °C and suffered from several neurological symptoms, especially from cranial nerve dysfunction like ptosis, dysarthria and dysphagia. He had no paresis or paraesthesia in the upper or lower extremity and the Babinski sign was negative. Furthermore he had a normal blood pressure, no dyspnea or meningism. The clinical examination showed a typical abscess formation from about 10 × 10 cm in the left groin region and a tenderness of palpation of the inguinal lymph node. The first time the patient noticed this formation was ten days ago and he remarked that it has grown and is more painful in the last days.\nThere were a few differential diagnoses like Myasthenia gravis, including Fishers-disease, Lambert-Eaton syndrome, encephalitis and wound botulism. That’s why we made a CT-scan of the cerebrum and a cerebrospinal fluid examination first. Both of them were without any pathological results. Especially the liquor showed normal high of leukocyte, erythrocyte, protein, lactate and glucose. The examination of the blood serum showed a high CRP (C-reactive-protein) of 10,8 mg/dl but normal leucocytes of 10,8 /nl. All other blood tests were inconspicuous; except the toxic screen for heroin, methadone and paracetamol, which were all positive.\nThe following CT-scan of the pelvic region showed a typical abscess formation in the subcutaneous and also in the adductor muscular region along with typical inguinal and paraaortal lymphadenopathy. An immediate operation with incision, abscess debridement and insertion of a drainage in general anesthesia was performed.\nAfter the operation we took surveillance on the intensive care unit for one day. The patient showed no further dyspnea, normal blood gas results and no general complications. Complementary we gave a high dose of intravenous Penicillin G (5 M four times a day) for in total nine days. Additionally we gave 2 times trivalent Botulinum antitoxin, which made no complications like allergic reaction or other. The intraoperative taken sample gave the evidence of Clostridium botulinum Serotype B in the culture and it also showed up in the patient’s serum. It was sensitive for metronidazole (minimal inhibitory concentration 4 μg/ml) and penicillin (minimal inhibitory concentration 0,06 μg/ml).\nIn the following days the cranial nerve dysfunction decreased and the patient left the hospital nine days after admission.\nA second presentation, 14 days after the patient’s discharge of the hospital, showed a complete regression from the neurological symptoms and no wound healing problems. |
A 73-year-old female was referred to our Trust by her general practitioner with a 5-month history of a painless vaginal mass, which extruded from the introitus on straining, but was otherwise asymptomatic. This was originally thought to be a vaginal prolapse; however, examination revealed a soft, well-defined pink mass occupying the upper vagina and an MRI of the pelvis was requested for further characterization.\nMRI was performed using a 3.0 T system utilizing axial T1 weighted fast spin echo; small field of view axial, coronal and sagittal T2 weighted fast spin echo; and T1 weighted fat-saturated sagittal images before and after gadolinium contrast administration, obtained in the arterial and portal venous phases. Diffusion-weighted imaging was also acquired. The images demonstrated a 47 × 40 × 44 mm well-circumscribed, oval mass in the upper vagina. On the T1 weighted images, the signal intensity of the abnormality was intermediate, similar to that of the skeletal muscle (). However, on T2 imaging, there were discrete zones within the lesion; the anteroinferior aspect was of high T2 signal with no enhancement, whereas the posterosuperior aspect was of low T2 signal with avid enhancement (–). There was no restricted diffusion. The posterior wall of the retroverted uterus was demonstrated to abut the superior surface of the lesion and the vaginal lumen was deviated anteriorly. Normal vaginal wall was seen to extend around the lesion’s anterior and posteroinferior surfaces. The lesion appeared to be arising within the left posterolateral vaginal wall and there were areas of loss of definition of the outer margin of the vagina. There was no involvement of the rectum, urethra or bladder; however, there were hazy low T1 and T2 signal changes in the left paravaginal fat.\nThrough a MDT discussion, it was agreed that owing to the suspicious imaging features of enhancement and tissue inhomogeneity, a staging portal venous phase CT scan was required to look for evidence of metastatic spread. Again, the vaginal lesion demonstrated fluid and soft tissue attenuation areas with regions of enhancement (). Significantly, there was no evidence of distant spread or lymph node enlargement. After further MDT discussion, the mass was still thought to be suspicious for malignancy and the patient underwent surgery.\nThe uterus, ovaries, cervix and upper vagina were removed en bloc and macroscopic examination revealed a well-circumscribed 45 mm polypoid mass arising from the paracervical upper vaginal tissue. The cut surface of the lesion was fleshy grey and white in colour, and was mainly solid in nature.\nMicroscopic examination () revealed an unencapsulated lesion with a spindle cell morphology arranged occasionally in fascicles. Beneath the surface epithelium, there was a grenz zone. The spindle cells were set within finely collagenized stroma and were bland in nature, with no conspicuous mitoses identified. Areas of oedema and myxoid change were also present, with no evidence of haemorrhage or necrosis.\nImmunohistochemistry demonstrated that the lesional cells expressed desmin, vimentin, oestrogen and progesterone receptors. The Ki67 proliferation index was low. Immunohistochemistry for MNF116, alpha smooth muscle antigen, smooth muscle myosin, h-caldesmon, S100 and CD34 were negative in the lesional cells.\nThe morphological and immunohistochemical profile was considered consistent with a superficial cervicovaginal myofibroblastoma, which is also known as superficial myofibroblastoma of the lower female genital tract. |
A previously healthy 51-year-old Hispanic female with a significant history of hypothyroidism presented with acute onset chest pain for 1 day. She described the chest pain to be pressure-like in nature, retrosternal, and radiating to her left arm and shoulder without associated shortness of breath. She reported to be sitting at rest when the pain occurred suddenly. She has been an active individual most of her life, but has had intermittent chest pain with exertion for the past 2 months. She has had to stop exercise for relief and has minimized her physical activity. The chest pain on presentation was reported to be similar in nature to the chest pain she had previously. According to the patient, she did not have any syncope, palpitations, racing of the heart, lightheadedness, anxiety, diarrhea, weight loss, or brittle hair. The reason she sought medical care this time was due to increased severity of pain.\nTwo months prior to presentation, she was diagnosed with hypothyroidism with a thyroid-stimulating hormone (TSH) level of ~500 U/mL and given levothyroxine 137 mcg daily. The dose was reduced a few days after labs indicated supranormal triiodothyronine (T3) and thyroxine (T4) levels, but low TSH after starting the medication. She had most recently been on levothyroxine 100 mcg daily, which she was compliant with. She also reported taking omeprazole 20 mg daily for gastroesophageal reflux disease. She did not use oral contraceptive pills. She has never had hypercholesterolemia and had a negative fasting lipid panel from ~1 year prior to presentation. She denied any other past medical or surgical history. She does not have allergies. She was a lifelong non-smoker, did not consume alcohol, and did not use illicit drugs.\nOn presentation, her vitals were normal. Her BMI was 27.3 kg/m2. The physical exam is only significant for a woman who appears in her stated age in mild distress due to chest pain. The electrocardiogram was consistent with normal sinus rhythm without any abnormalities. Troponins were elevated and trended upward from 0.080 to 0.120 to 0.222 ng/mL each 8 hours apart. TSH was nearly undetectable at <0.015 U/mL consistent with hyperthyroidism. T3 and T4 were not measured on presentation. Levothyroxine was stopped. Chest x-ray was unremarkable. Bedside 2D echocardiogram showed a normal left ventricle with EF of >60% without any regional wall motion abnormalities. The left atrium was mildly dilated, and right ventricular systolic pressure was 32 mm Hg. Non-ST elevation MI was diagnosed and patient was given atorvastatin 80 mg, heparin drip, clopidogrel 600 mg, and aspirin 324 mg. Sublingual nitroglycerin 0.4 mg was given, which improved the chest pain from a 10/10 on presentation to a 6/10. Coronary angiogram was done on the day of presentation, which showed an anomalous RCA arising from the left coronary cusp of the sinus of Valsalva, but no evidence of occlusion ( and ).\nA follow-up coronary computed tomography angiogram was done, which confirmed this finding and showed the anomalous RCA taking an inter-arterial course originating from the coronary ostium with a slit-like deformity and at least 50% luminal stenosis ().\nTroponins trended downward and chest pain resolved. Due to known poor outcomes with this type of symptomatic coronary anomaly, the patient was referred to a cardiothoracic surgeon for definitive surgical correction. The patient was discharged home with levothyroxine 50 mcg daily, metoprolol 25 mg BID, and recommendation against aggressive physical activity.\nInformed consent was obtained from the patient for publication of this case report and any accompanying images. |
A 24-year-old, obese, Latin American man with a history of measles, scarlet fever, and appendectomy, and taking no medications, consulted a private odontological care centre because of two days of toothache on the right side of his mouth. Evaluated as a common painful third molar, surgical extraction was attempted, but resulted in partial extraction due to the dentist's inexperience. The patient experienced mild haemorrhage and pain which was controlled by conventional haemostatic measures and analgesia. A prophylactic antibiotic was prescribed to the patient, and a later appointment was scheduled for the extraction of the residual tooth.\nOn the following day, the patient continued to have mild haemorrhaging and local swelling. The same supportive measures were prescribed since these were considered normal outcomes of a traumatic procedure. On the third day after surgery, persistent pain accompanied by malaise, moderate haemorrhaging and progressive local swelling prompted hospital care, where management included conventional haemostatic measures, analgesic, one-day hospital surveillance and early discharge. No laboratory analysis was ordered.\nThe next day, the patient complained of mild local pain, accompanied by trismus, minor local bleeding and clots, ecchymoses and mild jaw enlargement. Surgical extraction of the tooth residues and wound closure were performed, without apparent hemorrhagic complications. The dentist assumed that the patient had a coagulation disorder, based on such slow healing, and recommended a blood test and medical evaluation at the hospital because the private odontological clinic lacked the necessary laboratory resources. That same day, the patient experienced exacerbated pain, poor response to analgesia and continued local bleeding, which required emergency hospital care and a stay in the internal medicine department.\nPhysical examination revealed that the patient had pallor, right-sided jaw enlargement, ecchymoses (Figure , panel A) and an oral cavity with active gingival haemorrhage from surgery (Figure , panel B). There was no gingival enlargement, local abnormal colour, or clinical hepatosplenomegaly and/or lymphadenopathy.\nThe patient's complete blood count (CBC) (Table ) showed pancytopaenia with differential count of neutrophils 0.489 × 109/L (31.5%), lymphocytes 0.924 × 109/L (59.5%), monocytes 0.122 × 109/L (7.84%), eosinophils 0.006 × 109/L (0.38%), and basophils 0.012 × 109/L (0.76%). The patient had normocytic normochromic anaemia (Hb 107 g/L, MCV 84fL, MCH 29 pg and MCHC 351 g/L), thrombocytopenia (platelets 6.3 × 109/L) and abnormal coagulation times (prothrombin time (PT) 16 s with control sample 11 s, INR 1.43, activated partial tissue thromboplastin (aPTT) 39 s with control sample 32 s). Additional laboratory data revealed dyslipidaemia, hypoalbuminaemia and high levels of lactate dehydrogenase. An abdominal ultrasound revealed mild splenomegaly.\nDuring a short stay of three days in the internal medicine department, a peripheral blood smear revealed a 6% blast cell content with Auer rods and promyelocytic cells. The patient was referred to the National Cancer Institute, where M3-APL was diagnosed based on bone marrow samples, with hypercellularity and 80% neoplastic promyelocytes (Figure ). The findings were subsequently confirmed by fluorescence in situ hybridisation (FISH) for chromosomal translocation t(15;17) (Figure ).\nSupportive measures were established based on the patient's stay at the internal medicine department. The patient experienced transfusion-associated fever that occurred after he was transfused with erythrocytes and platelets. Thrombocytopenia was refractory to platelet transfusion and he maintained platelet counts between 6.3 × 109/L and 30 × 109/L. The patient further developed neutropaenia and fever, and the protocol for a high risk of opportunistic infection included isolation and antibiotics (ceftriaxone, metronidazole and fluconazole). At the National Cancer Institute, specific treatment was started immediately after diagnosis. His induction therapy included ATRA (all-trans retinoic acid) and daunorubicin. Laboratory analyses showed platelets at 8 × 109/L, delayed coagulation times and a fibrinogen level of 43 mg/dl (normal range 159 to 317). Further supportive measures included cryoprecipitates in addition to fresh frozen plasma (15 mg/kg q.i.d.) when the patient's fibrinogen level was lower than 100 mg/dl, and platelet transfusion when his platelet count was below 50 × 109/L. His CBC and coagulation parameters (PT, aPTT, thrombin time and fibrinogen levels) were monitored daily.\nDespite early specific treatment, the patient suffered generalised seizures secondary to intracranial bleeding caused by DIC, which was confirmed by delayed coagulation times, low serum fibrinogen, high fibrin degradation products and D-dimer. The patient died after four days of hemorrhagic complications. |
A 48-year-old otherwise apparently healthy Caucasian woman presented to our clinic complaining of headache, nausea and vomiting for 5 days. Physical examination and laboratory tests were within normal limits. She was diagnosed with tension headache and sinusitis and discharged on trimethoprim-sulfamethoxazole. The patient returned to the clinic 3 days later with resolution of her headache but continued vomiting, approximately twice per day. She was treated with an over-the-counter proton pump inhibitor for suspected gastroesophageal reflux.\nOver the following week, she developed ataxia, diplopia and recurrence of her headache. She presented to the emergency department, at which time a T2-weighted magnetic resonance image (MRI) scan of the head showed increased signaling of the left temporal lobe, bilateral pontine areas, left peridentate nucleus and cervical spinal cord. These findings were thought to represent an acute demyelinating process. At this point, clinical suspicion for myasthenia gravis was high, and she was administered a trial of pyridostigmine without improvement.\nDuring her hospitalization, she developed a blood pressure of 190/90 mmHg, a serum sodium level of 123 mEq/l, a glucose level of 176 mg/dl, and a white blood cell count of 12,800 cells/ml. Clinical examination during her stay showed progression of neurological symptoms with decreased sensorium, hallucinations, decreased attention span, and gait disturbance. A lumbar puncture showed increased opening pressure. Based on the radiographic, laboratory and clinical findings, a diagnosis of acute demyelinating encephalopathy was made; suspicions of infectious encephalitis, collagen vascular disease, and toxic encephalitis were high. She received plasmapheresis without improvement.\nA follow-up T2-weighted MRI was performed, which showed increasing enlargement of the bilateral pontine regions with hydrocephalus and mass effect on the fourth ventricle. Because of the absence of pathological enhancement of the pons, this was felt to be an aggressive demyelinating process or a low-grade glioma. The patient continued to deteriorate neurologically, became comatose and died approximately 4 weeks after admission.\nAt autopsy, the brain was edematous with a weight of 1375 g. The pons and medulla showed an infiltrative mass with extension into the cerebellum. Histological examination revealed a poorly differentiated infiltrative astrocytoma with nuclear pleomorphism, increased mitotic activity and focal necrosis (Figures and ). Staining for glial fibrillary acidic protein (GFAP) and MIB-1 were diffusely positive. The tumor microscopically extended into the middle cerebellar peduncles and the upper cervical spinal cord. |
A 39-year-old woman was referred to the Department of Oral and Maxillofacial Surgery with the chief complaint of a mass in the right buccal mucosa, which had been formed more than 2 months ago. The patient was also suffering from a severe trismus due to the lesion. In the intraoral examination, an ulcerative sessile painless exophytic mass in the right buccal mucosa was observed. Lymphadenopathy was not detected. No past medical or allergic history was found. Computed tomography scan (CT scan) of the patient showed large mass in right buccal mucosa which was attached to superficial skin and also due to bone depression in right zygomatic bone (Figure ). Incisional biopsy was carried out. The histopathologic examination revealed a neoplasm composed of small hyperchromatic basaloid cells arranged mostly in cribriform, and occasionally in solid and tubular patterns within a fibromyxoid stroma. The cyst-like spaces among the tumoral cells contained eosinophilic or basophilic material. The tubular pattern consisted of small ducts lined by several cuboidal cells which contained hyalinized material (Figure ). Nuclear pleomorphism, atypia, and mitotic activity were very low. These histomorphologic features were in favor of adenoid cystic carcinoma. Immunohistochemistry (IHC) was performed to confirm the diagnosis and to rule out other adenocarcinomas containing basaloid cells. C-kit antigen was diffusely positive in the tumoral cells. P63 was positive and scattered. Proliferation activity was also evaluated. Ki67 was positive in about 10% of the tumoral cells (Figure ).\nThese results were compatible with adenoid cystic carcinoma with a salivary gland origin. The patient underwent examinations to rule out distant metastasis by computed tomography of the chest and abdominal areas. Fortunately, no evidence of distant metastasis was observed.\nThe lesion was excised completely, and reconstruction of the surgical area was done by pedicle temporalis muscle flap (Figure ). Adjuvant radiotherapy was also carried out. After 24 months of follow-up, the patient is alive without any noticeable problems (Figure ).\nA 37-year-old man was presented to the Department of Oral and Maxillofacial Surgery with a chief complaint of a mass in the buccal mucosa of the left side. Intraoral examination revealed a pink submucosal lesion with duration of 8 months. No noticeable past medical and dental history was detected. There was so sign of lymphadenopathy in extraoral examination. Incisional biopsy was conducted. The histopathologic examination indicated a neoplastic lesion composed of islands of myoepithelial and ductal cells that were arranged in the tubular pattern in some areas. The tumor cells were small and cuboidal exhibiting basophilic nuclei and scant cytoplasm with minimal atypia and mitotic figures. The IHC findings were compatible with adenoid cystic carcinoma (Figure ).\nThe lesion was surgically excised, and postoperative radiotherapy was performed for the patient. The patient situation after the treatment was different from the case number one. Trismus was the most important complication after treatment due to surgery and radiotherapy. No distant metastasis was eventually detected in the patient's workups every three months during 2 years after treatment. |
A 62-year old Caucasian female, a retired nurse with known rheumatoid arthritis, presented to the clinic for acute onset severe jaundice, nausea, vomiting, and pruritus of less than 1-week duration without any other constitutional or systemic symptoms. The patient was not currently on any disease modifying medications for rheumatoid arthritis for the past 8 months. Her regular medications included loratidine for allergic symptoms, multivitamin and vitamin D as supplements, tramadol as necessary for joint pain, and primidone for essential tremors. She also did not have any history of liver disease and was not an alcoholic.\nOn physical examination, her vital signs were normal and stable. Scleral conjunctiva showed visible severe jaundice. Skin showed markings from intense generalized pruritus. She had also noticed a fine rash on her skin, which resolved spontaneously. Abdominal examination was normal without any hepatosplenomegaly, or signs of liver failure or portal hypertension. There were no abdominal masses felt on palpation.\nIn-depth exploration of the history revealed that the patient had undergone dental work 3–4 weeks prior and was given a 10-day course of amoxicillin-clavulanic acid, which she completed 3 weeks prior to presentation. She had no symptoms or signs until 1 week prior to the presentation to the clinic. She had no previous history of amoxicillin-clavulanic acid related reactions, but had allergy in the form of skin eruptions to other antibiotics like sulfa drugs, nitrofurantoin, penicillin, gold salts, and adalimumab.\nLaboratory tests showed markedly elevated liver transaminases, alkaline phosphatase, and elevated total bilirubin with normal glucose, electrolytes, and blood counts. Viral hepatitis panel was negative. This included acute hepatitis panel, which included hepatitis C antibody, hepatitis B surface antigen and core antibodies immunoglobulin M (IgM) and immunoglobulin (IgG), and hepatitis A IgM and IgG antibodies. Ultrasound of the liver and gall bladder did not show any specific pathology to explain the jaundice and abnormal liver functions.\nThe patient was seen by a gastroenterologist. The gastroenterologist ordered a magnetic resonance cholangio-pancreatogram, after initial evaluation. It showed a normal biliary system and no extra or intrahepatic biliary duct dilatation.\nThe gastroenterologist concluded that this was amoxicillin-clavulanic acid induced hepatitis and no further investigation was required. The patient and all physicians involved mutually agreed to wait and watch the liver functions over a course of several weeks. The only medication given to the patient was cholestyramine for the pruritis.\nSerial measurement of her liver functions over a course of 3 months showed complete resolution of the elevated transaminases and bilirubin (, , ). The patient clinically improved and made a complete recovery. |
A 31-year-old man suffered an out-of-hospital cardiac arrest during the night while asleep. His girlfriend started CPR immediately. Upon arrival of the emergency medical team, the patient was found in VF and defibrillation could restore spontaneous circulation. He had no significant past medical history and was taking no medications. Family’s medical history revealed that the patient’s sister had implanted a pacemaker due to sick-sinus syndrome, at the age of 22 years. Plasma electrolyte levels, including potassium and calcium, were within normal ranges. Toxicological screening was negative. The ECG at presentation showed sinus rhythm and an ER pattern with substantial J-point elevation in most of the ECG-leads with notching of the terminal part of the QRS complex in the lateral leads and slurring in the inferior leads (Fig. ). Coronary angiography revealed an anomalous origin of the left anterior descending (LAD) coronary artery arising from a separate ostium of the right sinus of Valsalva without any other concomitant congenital anomaly. In the absence of neurological recovery or purposeful movements, a targeted temperature management protocol with a goal temperature of 36 °C was started in the intensive care unit []. Three hours later, the patient began to experience very frequent episodes of spontaneous VF. The episodes of VF were induced by short-coupled monomorphic premature ventricular complexes (PVC) falling into the vulnerable descending part of the T-wave (Fig. ). The PVCs showed a right bundle block morphology in V1 and a right-inferior axis in the limb leads, suggesting an origin in the left anterior fascicle. The patient was shocked for more than 10 VF episodes, but the PVC kept reoccurring, sometimes just a few beats after cardioversion (Fig. ). The patient’s condition was finally stabilized by isoproterenol infusion, which successfully suppressed the PVC’s at higher heart rates in sinus rhythm. During the course of the isoproterenol infusion, a reduction of the ER pattern on the 12-lead ECG was observed (Fig. ). Compared to the ECG at presentation (Fig. ) the notch in the lateral leads disappeared and the slur in the inferior leads was markedly attenuated. Remarkably, new T wave inversions were present, possibly attributed to cardiac memory. Two days later, the patient was extubated and showed a good neurologic recovery. Cardiac magnetic resonance (CMR) imaging demonstrated normal cardiac structure and function and no late gadolinium enhancement. Regarding the anomalous origin of the LAD from the opposite sinus CMR revealed an interarterial course, in which the LAD passes between the aorta and pulmonary trunk. The left circumflex (LCX) artery was located in its usual expected position at the left sinus of Valsalva (Fig. ). A dual chamber ICD was implanted allowing for continued atrial pacing. A myocardial perfusion imaging with bicycle exercise stress testing was performed four weeks after the hospitalization and showed no signs of ischemia. The patient has not experienced any further arrhythmias during the first two months of follow-up. |
A Caucasian male aged 14 years attending an orthodontic clinic for a routine assessment presented with an incidental radiographic finding. A well-defined calcified mass was visible at the apex of the maxillary right permanent canine on the orthopantomogram (OPG) radiograph ().\nA lateral cephalometric radiograph showed that this mass was significantly superior to the dentition in the antral inferior nasal region (). The mass was a well-defined spherical lesion, measuring 1.5 by 1 cm with a radiopaque, homogeneous internal structure. It lay superior to the canine fossa and anterior to the maxillary sinus anterior wall.\nThis calcified mass had not been present on the OPG radiograph taken previously, at age 10 years ().\nFrom the age of 12, it was reported that the patient suffered with facial AV. He attended a consultant dermatologist who treated the condition successfully. The calcified mass was previously assessed at dermatological review. Despite the fact that it was a solitary lesion, it was diagnosed as dystrophic calcinosis cutis. The dermatologist advised no intervention as there was a significant risk of facial scarring if surgically removed. The mass was asymptomatic and presented little risk of infection. The patient was placed on annual review with his dermatologist.\nAlthough the mass was palpable and mobile, the patient's dermis was intact and acne-free. The mass was not visible clinically on extraoral examination. Facial profile and contour were normal. Orthodontically, he presented with a Class III incisor relationship on a Class III skeletal base with crowding in the maxillary arch. Both the patient and his parents were given the option of orthognathic surgery to treat his malocclusion and underlying significant skeletal relationship discrepancy. After extensive discussions and reviews with a maxillofacial surgeon, they opted for orthodontic treatment only, namely, alignment of the maxillary arch teeth on a non-extraction basis, followed by permanent retention. The orthodontic treatment was uneventful and successful. |
A 75-year-old female presented acutely with a history of sudden onset paraesthesia and ischemic rest pain involving the left foot and ankle. Motor function was retained, capillary and superficial venous return were reduced, the foot was cold, and there were no palpable pedal pulses. The calf remained soft to palpate. There was no history of palpitations or arrythmia, and the patient was in sinus cardiac rhythm both clinically and on electrocardiography. Plain chest radiography and computed tomography angiography (CTA) excluded a proximal arterial source of embolism. Furthermore, there was no previous trauma, (local or systemic) infection or inflammation, collagen, connective tissue or genetic syndromes, or arteritis; nor was there a family history of aneurysmal disease. Positive cardiovascular risk factors included hypercholesterolaemia and hypertension. Baseline haematological and biochemical analyses were normal, as were the renal and liver function. The creatinine kinase was within normal limits. The right foot was well perfused with a palpable PTA pulse.\nCTA demonstrated no significant stenosis in the supragenicular vessels bilaterally. On the symptomatic left side, the tibioperoneal trunk (TPT) and anterior tibial artery (ATA) were patent proximally, with the ATA and peroneal artery occluding in the distal calf. The dorsalis pedis was occluded; however, the plantar arteries reconstituted. Dominant flow to the foot was via the PTA, which occluded in the distal calf (). These findings were considered reflective of embolic disease within the left-sided tibial arteries. The asymptomatic right leg demonstrated dominant flow to the ankle via the PTA. The ATA and peroneal arteries occluded in the midcalf.\nDue to the acute nature of the patient's symptoms, emergent thromboembolectomy of the popliteal and tibial arteries was performed via a medial approach to the distal popliteal artery. Selective thromboembolectomy using Fogarty catheters for each tibial artery was unsuccessful. Urokinase was also injected into each tibial artery without effect. Subsequent exposure of the dominant PTA at the ankle demonstrated a thrombosed 10mm diameter aneurysm. A PTA thrombectomy was performed and subsequent angiography demonstrated flow to the foot via the aneurysmal PTA (). The aneurysm was opened and demonstrated a mixture of old, organised, and fresh thrombus (). The PTA aneurysm was excised and thrombectomy established adequate inflow and backflow to the excised PTA segment. The PTA was repaired using an interposition long saphenous vein graft (). Postoperatively, the PTA pulse was present, the foot had brisk capillary refill, and motor and sensory function normalised. Follow-up at 16 months demonstrated the patient to have a well perfused left foot with a palpable PTA pulse.\nAlthough the patient's contralateral foot was asymptomatic, she opted to have this repaired electively considering the aneurysmal right PTA aneurysm was the single arterial supply to the foot. Repair was performed via excision of the 8mm PTA aneurysm and a spatulated primary end-to-end PTA anastomosis. Completion angiography was satisfactory, and the right foot was well perfused with a palpable PTA pulse postoperatively. Histopathology of the right-sided PTA aneurysm demonstrated findings consistent with atherosclerotic degenerative aneurysmal disease. Specifically, there was intimal thickening, extensive lamina disruption, and broad areas of media replaced by collagenous fibrous tissue, with calcification of the media. There was no inflammation or mural necrosis seen. |
A 68-year-old woman presented to the Department of Oral and Maxillofacial Surgery at Nagoya Ekisai Hospital (Nagoya, Japan) with a chief complaint of malaise and a 7-month history of swelling of the left buccal mucosa. The patient had no congenital swelling of the left buccal mucosa at birth and no history of systemic disease or relevant family history. The patient had undergone maxillary molar restoration treatment 2 years earlier, after which she reported biting regularly on her buccal mucosa. On most occasions, the wound had healed within a week, so she had not sought medical treatment. An extraoral examination revealed no facial swelling or asymmetry. However, an intraoral examination revealed an area of diffuse swelling on the left buccal mucosa measuring about 15 mm × 30 mm and containing a papillary lesion with multiple red, blue, and clear pebble-like vesicles (Figure ). On palpation, the lesion was nontender and soft. The swelling had not expanded to the veins and was pulsatile. An orthopantomogram confirmed that the adjacent bone was intact. Magnetic resonance imaging revealed a soft tissue mass with a clearly distinguishable outline of the buccinator muscle (Figure ). The lesion was surgically excised under local anesthesia with a margin of 3 mm and a depth of 2 mm via the inside surface of the fascia of the buccinator muscle. The outcome was favorable. Pathologic examination of the specimen revealed expanded lymphatic vessels lined by thin endothelial cells and containing lymphatic fluid. A diagnosis of lymphatic malformation was confirmed on histopathology and immunohistochemical studies. Immunohistochemistry was negative for vascular markers such as CD31 and CD34, and the lymphatics stained specifically for D2-40 (podoplanin) (Figures , , ). On follow-up, the wound was found to have healed with no evidence of trismus or recurrence. The patient continued to be recurrence-free at her 2-year follow-up. Informed consent was obtained from the patient, and the procedures were in accordance with the Helsinki Declaration. |
The 70-year-old male patient was admitted to the Department of Neurology with isolated left upper extremity weakness and clumsiness. He had wrist drop and was unable to grip with the fingers. He complained of no sensory loss or numbness. His past medical history included long-term oral anticoagulation due to recurrent lower extremity deep vein thrombosis, glaucoma, and a non-significant aortic valve stenosis, with vascular risk factors including a 5-year history of treated hypertension, hypercholesterolemia, and a history of non-significant stenosis of the left anterior descendent coronary artery. On admission, no abnormality could be detected by physical examination, apart from the neurological signs, which included a wrist drop on the left side with decreased grip strength (Fig. ). Pronation and wrist dorsiflection were lost, as well as the ability to form a ring with the thumb and the index. The reflexes of the left upper extremity were slightly brisk, with not pathological reflexes present and no sensory deficits. Laboratory parameters were without abnormal findings except for a slightly elevated fasting blood glucose level. Cranial CT revealed contrast-enhancing, irregularly shaped lesions with diameters of 7, 10, and 9 mm (in the temporal, parietal, and frontal lobes, respectively), surrounded by perifocal edema. Carotid duplex ultrasonography did not demonstrate signs of focal atherosclerotic plaques, circulatory disturbance or stenosis on either side. In accordance with the findings of the CT scan, the cranial MRI confirmed the tumor in the right precentral gyrus, corresponding to the ‘hand knob’, accompanied by further similar lesions posteriorly in the parietal and the temporal lobes, and in the left frontal lobe (Fig. ). Electromyography and nerve conduction studies did not support a peripheral origin underlying the paresis. The neuropsychological examination revealed a deficit in the Luria three-step test as a single alteration, which performance could, however, be remarkably improved by verbal clues. Chest X-ray performed as part of the search for primary tumor revealed no abnormality. Non-contrast and contrast-enhanced chest CT detected an irregularly shaped mass in segment 10 of the left lung, with inhomogeneous enhancement of the contrast agent and multiple associated lymph node conglomerates, suggesting lung cancer as the primary tumor. Abdominal ultrasonography did not detect malignancy. The patient received palliative steroid therapy, with no improvement in the paresis during the observation period. Based on the recommendation of the tumor board, the patient was transferred to the Department of Pulmonology for bronchoscopic tissue sampling. The verification of the diagnosis by this means, however, was unsuccessful. The diagnostic process had to be suspended due to an acute bleeding duodenal ulcer, and the patient received blood transfusion and local hemostatic treatment in the Department of Surgery. In association with the antibiotic treatment, the patient developed pseudomembranous colitis caused by Clostridium difficile. In addition to the persisting colitis, fever occurred accompanied by hemoptysis and coughing. The subsequent chest X-ray did not confirm pneumonia. Despite the applied antibiotic and oxygen therapy, respiratory failure developed, and the patient passed away 2 months after the onset of the neurological symptom. The autopsy and the histopathological analysis identified an adenocarcinoma (mucinous tubular adenocarcinoma with solid anaplastic parts) both in the brain and the lung; (Fig. ). |
A 65-year-old postmenopausal lady (Patient A) and a 32-year-old homemaker (Patient B) presented with history of exertional dyspnea of 1-year duration and 6-month duration, respectively. Both gave history of occasional cough with expectoration. They also gave history of recurrent respiratory infections. However, neither of them had a history of tuberculosis or associated comorbid illness like diabetes mellitus. On presentation, both were clinically stable and maintained normal saturation at room air. On examination, they had mild deviation of trachea to the left with reduced breath sounds in the left hemithorax and laterally placed apex beat. Their chest X-ray (CXR)/scanogram demonstrated shift of the mediastinal structures to the left and hyperlucent right lung []. A possibility of left lung collapse/left lung hypoplasia was considered. Contrast chest computed tomography (CT) showed hypoplastic left lung with bronchiectatic changes in the left lower lobe with herniation of right lung. Mediastinal structures were to the left []. Further on CT, Patient A had a rudimentary left pulmonary which was interrupted/abrupt termination within 1 cm of origin from the main pulmonary artery. The diameter of the left pulmonary artery and main pulmonary artery of Patient A are 7 and 23 mm, respectively. Patient B CT showed a left pulmonary artery of about 9 mm in diameter but was also abruptly terminated within 1 cm of origin from the main pulmonary artery. A possibility of left-sided pulmonary artery agenesis with left lung hypoplasia was suspected and both the patients underwent a perfusion scan which revealed no perfusion in the left lung with normal perfusion in the right []. Fiberoptic bronchoscopy showed narrowed segmental openings of the left lung. The left main bronchus and lobar bronchus were normal. Bronchial washings were collected from all the narrowed segments, but no organism was isolated. Patient B also had a more distally placed right upper lobe bronchus probably due to hyperinflation. Electrocardiogram of both the patients were normal. Echocardiography showed no associated anomalies or pulmonary hypertension (PHT) in both patients. A diagnosis of isolated left-sided pulmonary artery agenesis with left lung hypoplasia was made. Both the patients were counseled regarding their condition and are currently on regular follow-up. |
A 39-year-old woman, who had been taking medication of quetiapine as an antipsychotics for depression, experienced mild dysarthria and visited the department of neurology in our hospital. Her symptom was diagnosed as drug-induced lip dyskinesia, which disappeared in a week. Screening head magnetic resonance imaging (MRI) at this time revealed stenosis of the left MCA with no brain parenchymal lesions (), why she was consulted to our department. We performed angiography, confirming moderate M1 portion stenosis (Figures and ). SPECT showed no apparent laterality in CBF, thereby we decided to observe her with no treatment.\nSeven months later, the patient experienced mild weakness and numbness in her right hand and visited our department. Although MRI showed no apparent ischemic change in her brain, arterial spin labelling (ASL) of MRI detected the decrease of CBF in the left cerebrum (), which was thought to well correspond for her symptoms. She was admitted and treated with an antiplatelet agent. Two weeks later, she still complained of numbness in her right hand; thereby, we decided to perform left STA-MCA anastomosis to prevent deterioration of her symptoms. Preoperative SPECT showed no apparent laterality in CBF (). On operation, left temporal craniotomy was performed, and the parietal branch of the STA was anastomosed with the M4 portion on the temporal lobe (Figures and ). The intraoperative course was uneventful, and the patient recovered from anesthesia without any new neurological symptoms\nPostoperatively, her speech was normal until postoperative day 1 (POD1). On POD2, she exhibited mild speech disturbance, which worsened day by day finally resulting in complete motor aphasia on POD6. Her comprehension was kept normal. On POD3, generalized convulsion occurred, which ceased quickly by diazepam, and levetiracetam was initiated. On the same day, she presented with mild weakness of right upper extremity, which improved gradually and disappeared on POD7. MRI and CT showed no ischemic or hemorrhagic changes, but ASL and SPECT revealed remarkable increase of CBF in the left cerebrum (Figures and ), by which the symptoms were diagnosed as CHPS. Despite the treatment with strict blood pressure and the administration of edaravone and minocycline, complete motor aphasia remained unchanged on POD21. MRI showed no abnormality except slightly hypointense changes on T2 weighted images and FLAIR (). At this point, the patient was discharged partly because of the request from the patient, and we continued to follow her in outpatient visit. One month after the surgery, the patient started to utter words that were not fluent, when SPECT and MRI showed normalization (). Thereafter, the improvement of her speech was slow, and totally more than three months was taken for full recovery after the surgery. |
An 18-year-old female presented to the gynaecology clinic at Bugando Medical Centre, Mwanza, Tanzania, with a history of recurrent painful huge genital swelling in her left labia majora for one year which initially started as a small swelling, then increased in size, and became painful. She gave a history of pus discharge, and due to its recurrent and persistence swelling, it was associated with fever and inability to walk properly and was accompanied with painful micturition. She had a history of being treated unsuccessfully several times through suction with a needle syringe though she noted a temporary relief. However, she had a history of long-term use of antibiotics without getting relief. The swelling recurred at intervals of less than one or two months over the last year. She has no history of receiving counselling for HIV and testing.\nOn examination, there was tender large mass involving the left labia majora and minora, shiny and with a smooth surface, discharging pus at the small sinus, erythematous, fluctuant, and measuring approximately 10 cm in length and 6 cm in width. Her vaginal examination revealed normal vaginal wall, no any offensive discharge, with the healthy cervix; she had negative cervix excitation test and normal sized uterus; neither adnexal mass nor tenderness was elicited (). She had a working diagnosis of huge left Bartholin's gland abscess. On general examination, she had no peripheral lymph node enlargement. Her vital signs were a blood pressure of 120/70 mmHg, pulse rate of 88 beats per minute, and temperature of 37 Celsius. Other systemic examination was normal. Cardiovascular system examination revealed audible first and second heart sounds and no murmurs. Respiratory system examination showed a respiratory rate of 21 breaths/minute, normal chest contour, trachea centrally located, normal chest expansion, and normal breath sound on auscultation. Per abdominal examination, abdomen was flat, moving normally with respiration, and there were no visible superficial veins. The abdomen was soft and nontender. The liver, spleen, and kidneys were not palpable. No shifting dullness and bowel sounds were normal on auscultation.\nThe patient was counselled for emergency marsupialization, and informed consent was obtained. Preoperative antibiotics were given: ciprofloxacin 500 mg orally 12 hourly for 5 days, metronidazole 400 mg orally 8 hourly for 7 days, and doxycycilline 100 mg orally bid for 7 days. In theatre, surgical intervention by marsupialization was done under spinal anaesthesia (5% lidocaine 50 mg preparation). The patient was placed in the lithotomy position, and left huge Bartholin's gland abscess was exposed. The incision was made through the gaped skin covering the abscess whereby pus was drained and cleaned with normal saline until the underlying fresh tissue edge of a gland was identified and was oozing fresh blood. A lot of foul-smelling pus was drained from the abscess, approximately 30 ml. The edges of the gland were grasped gently using a forceps. The sutures material used was a vicryl number 2-0, and repair by the marsupialization method was done. In the postoperative care, she continued with already prescribed antibiotics, and the analgesia given was paracetamol 1 g orally 8 hourly for 3 days. The patient was discharged on the 3rd day and linked to reproductive health clinics for counselling about the knowledge of sexually transmitted infection prevention and treatments. |
The patient was a 65-year-old gentleman with a past medical history of hypertension and diabetes mellitus type 2 who was admitted to the hospital with progressive weakness, headache, dizziness, and confusion. His general and neurologic exam was grossly normal. Blood laboratory data were within normal limits. A nonenhanced computed tomography (CT) of the brain showed a large mass within the right parietal lobe with surrounding vasogenic edema. A brain magnetic resonance imaging (MRI) showed an irregularly enhancing mass measuring 5.8 cm anteroposterior × 3.2 cm transverse × 4.4 cm craniocaudal within the right parieto-occipital lobe, with significant vasogenic edema (Figures and ). He then underwent a right parietal craniotomy with maximal safe resection of the tumor.\nThe surgical pathology report showed a high-grade glioma with foci of necrosis and microvascular proliferation consistent with GBM (WHO Grade IV) (). His postsurgical course unfolded without complications.\nHe then went on to receive adjuvant radiation therapy with a total of 60 Grays divided into 30 fractions during the course of 44 days. He additionally received temozolomide at 75 mg/m2 daily while on radiation therapy, with essentially no toxicity. Temozolomide was subsequently continued at 150 mg/m2 daily five days out of a 28-day cycle.\nA follow-up MRI of the brain approximately 5 weeks post completion of combined therapy showed increased enhancement and diameter of the parietal mass, mass effect on the adjacent right occipital horn of the right ventricle, and diffuse vasogenic edema on the right cerebral hemisphere.\nSubsequently, the patient was transferred to an outlying institution for additional management where he underwent reopening craniotomy with microsurgical resection of the tumor with Gliadel wafer placement approximately 17 weeks from his initial resection. The surgical specimen showed progressive glioblastoma. His postsurgical recovery was complicated by hydrocephalus, hemiparesis, and an acute non-segment elevated myocardial infarction. He returned to the operating room for a right ventriculoperitoneal shunt placement on postoperative day 12.\nAbout 4 weeks after the procedure, the patient was started on bevacizumab every 3 weeks. He received 5 treatments with this agent which had to be discontinued due to proteinuria and hypertension. A follow-up brain MRI five months post second resection showed postoperative changes without evidence of recurrent and/or progressive disease.\nApproximately five weeks after his most recent follow-up MRI, he was hospitalized with excruciating lower back pain radiating to his right hip and anterior thigh. MRI of the pelvis showed extensive lesions with involvement of femurs, iliac bones, and sacrum (). A CT chest, abdomen, and pelvis was negative for visceral metastatic disease. Bone scan (Tc 99m) showed subtle uptake in the bilateral humeri and increased uptake in the region of the greater trochanter on the right. Subtle increased uptake in the trochanteric region on the left was also reported. A biopsy of a right femoral lesion showed numerous tumor cells in a reactive stroma replacing much of the bone marrow (). The tumor cells stained positive for glial fibrillary acidic protein (GFAP) (). The conclusion was that it was representative of a high-grade astrocytoma (i.e., glioblastoma). Given the limited therapeutic options and poor performance status, the patient elected for supportive care only and died 12 days after the biopsy. No brain imaging was performed during the hospitalization. |
The patient’s course is outlined in Fig. .
A 58-year-old, previously healthy, white female presented to her primary care provider (PCP) with the desire to remove a right posterior scalp cyst for cosmesis. This non-inflamed, non-draining, painless, 1–2 cm cyst had been present for close to 10 years without change in size or fluctuance. The cyst was initially drained by the PCP, but, when it recurred 6 months later, the PCP excised the cyst and sent the specimen for routine pathology. The initial read of the tissue sample was high-grade invasive carcinoma with squamous features and arising in association with a PTT. Based on the pathology, the PCP referred the patient to a plastic surgeon for a more definitive excision of the lesion and repair of the defect. The lesion was excised with negative margins and pathology read as invasive high-grade squamous cell carcinoma (SCC). Eight months post resection, the lesion recurred locally along with a palpable right posterior cervical lymph node (LN). A positron emission tomography (PET) scan at that time demonstrated hypermetabolic activity in the posterior occiput and in a posterior neck LN. Fine needle aspiration (FNA) of both the primary scalp lesion and LN were completed, and pathology was reported as SCC, similar to the primary lesion.
With a working diagnosis of locally advanced SCC, the patient’s care was referred to a tertiary care center. There, a dermatopathologist re-evaluated the previous biopsy specimens and altered the diagnosis from SCC to MPTT (Fig. ). Subsequently, the patient was referred to a head and neck surgeon for modified radical posterior neck and lymph node dissection. Intraoperative findings uncovered the presence of nodal metastases to the posterior neck with extranodal extension, extensive perineural invasion of the spinal accessory nerve, and jugular venous invasion of the MPTT. After surgery, the case was discussed at a multi-disciplinary tumor board, and a common |
A 50-year-old Caucasian female, with no previous smoking history or pancreatic cancer family history, presented to our clinic for surgical evaluation of a biopsy-proven PDA noted at the junction of the body and tail of the pancreas. The patient had previously undergone a classic pancreaticoduodenectomy (Whipple) procedure with concurrent hemicolectomy 6 years prior (in 2011) for a T3N0M0 adenocarcinoma of the pancreatic head that had invaded the mesentery of the proximal transverse colon. After the 2011 surgery, pathology revealed a poorly differentiated PDA along with a PanIN grade 2. The final pathology showed negative surgical margins, positive perineural spread, and 0/33 specimen lymph node involvement. From June 2012 to October 2012, the patient underwent and completed adjuvant chemotherapy with gemcitabine, capecitabine, and radiation at an outside hospital. She was carefully followed by her medical oncology team with serial CA 19-9 monitoring and abdominal MRIs on an ongoing basis. Due to her young age, in 2011, she underwent genetic screening and no germline mutations were identified. Since the time of the primary resection, the patient had been high functioning and healthy, with the exception of some problems of early satiety and recurrent cholangitis. These sequelae were attributed to close proximity of the gastrojejunostomy and hepaticojejunostomy, with possible reflux of intestinal contents up the afferent limb, all partially managed by diet changes.\nIn 2017, a biannual screening MRI with intravenous contrast showed a new pancreatic lesion measuring 2.3 × 2.2 cm in the tail of the pancreas (). Esophagogastroduodenoscopy and endoscopic ultrasound-guided biopsy identified it as a poorly differentiated adenocarcinoma. From 2011 to 2017, she had had close followup with serial CA 19-9, and a measurement of this marker after identification of the lesion on MRI showed an elevation, which was confirmed on repeat testing (61 and 55 U/mL; normal <37 U/mL). This was the first instance of two consecutive CA 19-9 measurements outside of the normal range since resection of the primary cancer 6 years prior. When the patient presented to our institution a few months later, the CA 19-9 had returned to normal at 32 U/mL (), and there was a moderate increase in CEA (16.9 ng/mL; normal <3 ng/mL). The patient received a second MRI of the abdomen and pelvis with contrast to identify distant disease, which showed the lesion to be confined to the pancreas. Along with the MRI of the abdomen, a CT of the chest was preformed, which showed no gross metastatic lesions. A completion pancreatectomy was scheduled with revision and lengthening of the jejunal limb proximal to the gastrojejunostomy to resect the tumor and treat her episodes of early satiety and recurrent cholangitis.\nIntraoperatively, the patient's three Whipple anastomoses were noted to be grossly intact and there was no evidence of metastatic disease. First, the stomach was divided approximately two centimeters proximal to the prior gastrojejunostomy. The jejunostomy was closed, and gastrocolic and gastrosplenic ligaments were divided. The splenic artery was ligated, and the splenocolic ligament was divided. The spleen and pancreas were mobilized out of the retroperitoneum. The jejunum was divided between the pancreaticojejunostomy (PJ) and hepaticojejunostomy. The proximal jejunum, prior PJ, remaining pancreas, and spleen were removed. The tumor was noted to be grossly confined to the pancreas. The distal end of the stomach was delivered through the mesocolon and a retrocolic gastrojejunostomy was undertaken 60 cm downstream from the hepaticojejunostomy. The anatomy before and after this operation is shown in .\nThe patient tolerated the procedure well and had an uncomplicated hospital course. The patient was closely followed postoperatively and did well. She completed two cycles of adjuvant chemotherapy with gemcitabine and capecitabine from January 2018 to April 2018, but did have some neutropenia at the end of her adjuvant therapy requiring pegfilgrastim. Her intermittent fevers, early satiety, and abdominal pain resolved after the surgery. She became an obligate insulin-dependent diabetic after the completion pancreatectomy procedure and now requires exogenous pancreatic enzymes to support her nutrient absorption.\nThe specimen was found to be consistent with a poorly differentiated invasive adenocarcinoma. Resection margins were negative, and 2 of 17 lymph nodes were positive for metastatic cancer. We sent representative slides of the patient's 2017 tumor and 2011 tumor to Perthera (McLean, VA) for next-generation sequencing (NGS) and histological analysis, which tested for mutations in a total of 315 genes and stained for various predictive biomarkers (). Both lesions showed the same mutations in KRAS (G12R), CDKN2A (splice site 151-1 G to A), and TP53 (Y220C). In addition, her 2011 tumor had a mutation in ACVR1B (S4) that was not present in the 2017 tumor, and the 2017 tumor had amplifications of MYC and mutant KRAS that were not present in the 2011 tumor (). Upon histological analysis, staining for MLH1, MSH2, MSH6, PMS2, pAKT, and HER2 was similar in both samples, but in the 2017 tumor, there was 60% increased staining for RRM1 and 20% increased staining for ERCC1, which changed the classification from low to high staining for ERCC1. |
A 42-year-old white man with a history of chronic back pain and intravenous drug abuse in the past has presented with intermittent fevers for a week and bilateral flank pain for 2 days. He did not measure his temperature at home and denied having any vomiting, diarrhea, dysuria or hematuria. He did have some nausea. He had a bioprosthetic aortic valve placed 1 year prior to presentation for infective endocarditis associated valvular dysfunction and he stayed away from recreational drugs since then. He was seen by a dentist 2 weeks ago for toothache and underwent extraction of multiple teeth. His vitals were stable except for a temperature of 101°F. Physical examination was significant for a systolic murmur with maximum intensity in the aortic area and tenderness in bilateral flanks. Labs demonstrated white blood cell count of 14 thou/mm3, hemoglobin of 11 g/dL, platelet count of 160 thou/mm3 and serum creatinine of 1.1 mg/dL. Serum lactate dehydrogenase was elevated at 684 IU/L. Urinalysis was significant for microscopic hematuria with 24 red blood cells/hpf. A computed tomography (CT) scan of the abdomen with contrast was obtained to rule out any acute intra-abdominal pathology, which demonstrated areas of non-enhancement involving more than 50% of the right kidney predominantly involving the lower pole and also most of the spleen consistent with renal and splenic infarction (). Interestingly, the patient was noted to have an accessory right renal artery providing flow to the upper part and probably accounts for relative sparing of this portion of the kidney ().\nSubsequently, patient’s blood cultures grew Streptococcus mitis and Streptococcus oralis, which are common bacteria inhabiting human mouth. Also, trans-esophageal echocardiogram showed features suggestive of infective endocarditis of the prosthetic aortic valve with vegetations, which supports the diagnosis of renal and splenic infarction from septic emboli. The patient improved with antibiotic therapy and renal function remained relatively stable. |
A 64-year-old woman initially experienced urinary difficulty and felt tightness at upper back. Weakness of right lower extremity developed 2 months later and rapidly progressed to inability of ambulation in following 2 weeks. She visited authors’ institute with presentation of monoplegia of right lower extremity and asymmetrically decreased response to light touch and pain below nipples, more prominent on the left side. Hyper-reflexia and positive Babinski sign at bilateral lower extremities were also noted. The results of routine blood tests were unremarkable and the patient had no family history of cancer or genetic disease. As the clinical manifestation was compatible with Brown-Séquard syndrome, a compressive lesion to spinal cord at the thoracic spine was suspected. Magnetic resonance imaging (MRI) revealed an intradural extramedullary mass at the level of 3rd thoracic (T3) vertebra with severe compression to the spinal cord (Fig. ). The tumor was about 1.5 cm in size and characterized by intermediate signal intensity at T1-weighted images with mildly increased signal intensity at T2-weighted images and evident enhancement after the gadolinium administration. There was no bony involvement or other lesion found in radiologic assessments.\nThe patient received surgical treatment for removal of the tumor mass on the next day after MRI study. During the surgery, the posterior surface of dural sac was exposed after total laminectomy from T2 to T4 vertebrae and then the intradural space was accessed through longitudinal opening of the dura mater. Grossly, the tumor sized 2 × 1.5 × 1.5 cm and was firm, reddish, lobulated, and hypervascular. The mass was attached to the inner surface of dura mater with no involvement of arachnoid or spinal cord. The tumor was removed en bloc after detached from dura mater. Posterior instrumentation and fusion were performed to prevent post-laminectomy kyphosis.\nHistological examination revealed that the tumor was hypercellular and composed of spindle and round cells with a high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix (Fig. a). The tumor cells had ovoid to round nuclei and inconspicuous cytoplasm, arranged in vague fascicles (Fig. b). As for immunohistochemistry, the tumor cells were positive for CD99, desmin (especially on the chondrocyte-like cells) and CDK4 (focally), while negative for S100 protein, CK (AE1/AE3), CD34, MDM2, and myogenin. In addition, reverse transcription polymerase chain reaction (RT-PCR) was positive for HEY1-NCOA2 fusion transcript (Fig. c). Collectively, a mesenchymal chondrosarcoma of possible meningeal origin was diagnosed.\nThe postoperative course was rather smooth with muscle power of right lower limb improved rapidly and completely after surgery. The patient received adjuvant radiation therapy with 44 Gray in 22 fractions. Follow-up MRI showed no evidence of recurrence 5 years after surgery and the patient remains now in complete remission, fully self-dependent with only mild sensory deficits at lower extremities (Additional file ). |
A 58-year-old male patient presented with a gradually increasing painless mass at scalp over the parietal region for 3 months. On clinical examination, the lesion was 5 cm × 4 cm × 3 cm bosselated reddish-yellow mass, fixed to skin. The patient had no anorexia and weight loss and the examination did not reveal any cervical lymphadenopathy. Computed tomography scan brain did not show any intracranial extension or bone involvement by the tumor. Fine needle aspiration was performed with 24G needle attached with a 10 cc syringe and smears were stained by Leishman-Giemsa and Papanicolaou stain. Cytology showed hypercellular smears comprised cohesive sheets and clusters of oval cells as well as scattered single cells in a background of necrotic debris. The tumor cells had moderate amount pale basophilic cytoplasm, hyperchromatic nuclei with irregular nuclear contour, and prominent nuclei [ and ]. Multiple atypical mitosis and few squamous cells are seen in the smears. Cytology was reported as malignant adnexal tumor, possibly eccrine porocarcinoma. Wide local excision was carried out and specimen was sent for histopathology. On gross examination, it was an irregular friable soft to firm mass 4 cm × 3 cm with whitish cut surface. On H and E-stained sections, the tumor comprised polyhedral cells arranged in solid cord, sheets, and lobules [ and ]. Few lobules showed central necrosis. The tumor cells had abundant pale eosinophilic cytoplasm and enlarged round nuclei. The nuclei show mild pleomorphism, hyperchromatia, prominent nucleoli, and frequent mitosis. Epidermotropism and areas of squamous differentiation were also seen in the sections []. Dermal infiltration was deep to reticular dermis. No intercellular bridges and keratohyaline granules were noted and surgical margins were free form of tumor tissue. Histopathological diagnosis was done as eccrine porocarcinoma of the scalp. He was referred for postoperative chemotherapy. He is followed up for 1 year but did not have any local recurrence and lymph nodal metastasis. |
In a work-up of a 40-year-old female with a history of low back pain, a routine MRI of the lumbar spine identified an incidental lesion. The axial T\n1 weighted image showed a 21-mm round, homogeneous lesion of low signal intensity interforaminally in the left S2 segment of the sacrum. The axial short tau inversion-recovery image showed a well-defined lesion with an inhomogeneous signal intensity throughout the lesion with high peripheral rim intensity. The axial T\n1 image with fat saturation after contrast media injection showed moderate enhancement throughout the lesion and in the peripheral rim (). A radiograph of the pelvis was performed to further characterize the lesion. On clinical examination, the sacral area was not painful to palpation. Owing to the atypical appearance of the lesion and the non-specific nature of the MRI signal pattern, a bone scan was performed that showed a solitary lesion on the left side of the sacrum with increased metabolic activity. For further characterization, and in the search of a potential primary tumour, positron emission tomography (PET)-CT was performed, which showed a well-defined sclerotic lesion with mild fludeoxyglucose (FDG) avidity (average standardized value 2.5) in the S2 segment and no other abnormalities (). Owing to the metabolic activity of the lesion, the patient was referred to the orthopedic oncology department for image-guided biopsy of the lesion. The biopsy specimen consisted of a few small fragments of bone marrow, some skeletal muscle, fibroadipose tissue and blood clots. Infiltration of the otherwise normal bone marrow with scattered small groups of big foamy cells was identified (). The foamy cells had vacuolated cytoplasm and small centrally located nuclei. The cells were negative for cytokeratin AE1/AE3, CD68, barchyury, Melan A, HMB 45, desmin and smooth muscle actin but positive for S100 protein (). The cells contained multiple lipid droplets and numerous large mitochondria; the existence of the latter was exhibited with antimitochondrial marker (). A pathological diagnosis of hibernoma was made in correlation with the imaging findings.\nOwing to the clinical assessment in correlation with the history of bilateral lumbago, worse after physical activity, the patient was diagnosed with chronic lower back pain. At 1-year follow-up, the patient was being managed with analgesics and physical therapy. |
A 56-year-old, nulliparous woman, with previous surgical history of an open paraumbilical hernia repair without the use of prosthetic mesh a year ago was referred due to diffuse abdominal pain. The patient was on antipsychotic medication for schizophrenia, a heavy current smoker (30 pack/years) and morbidly obese (body mass index 35). Patient's mental capacity was assessed using the legally relevant criteria, and she was found to be incompetent. Therefore, her brother acted as decision maker and consented for further investigation and treatment.\nThe patient had normal pulse (85/min) and arterial blood pressure (130/82 mmHg), remaining apyrexial. She was initially assessed by the surgical team, and she was found to have an approximately 4-cm irreducible incisional paraumbilical hernia. The content and texture of the hernia sac were round and firm clinically. Tenderness was elicited with deep palpation, and an incarcerated incisional hernia was the initial working diagnosis. Routine hematological and biochemical laboratory investigations were unremarkable, and abdominal X-ray did not show any signs of small bowel obstruction. Lactate levels were within normal range.\nDigital rectal examination identified a large painful pelvic mass and no signs of bleeding. A gynecological examination was requested, but there were limitations for further assessment as the patient was virgin, and she could not undergo a per-vagina examination. Considering all the above findings and body habitus, an urgent abdominal CT scan was performed. The latter showed a 15 cm solid pelvic mass with scattered calcifications originating from the uterus or adnexa suggesting a large uterine leiomyoma. The mass was extending up to the level of a sizable lower abdominal midline incisional hernia. The hernia sac was filled with small bowel loops on imaging. No free fluid or pneumoperitoneum was noted. The patient was discussed in our MDT with gynecological input, and a decision was taken to proceed with an exploratory laparotomy.\nOf note, she was postmenopausal and nulliparous, and a decision had to be made regarding the potential scenario of hysterectomy. Following an extensive discussion with her brother, consent was obtained to proceed with hysterectomy if needed.\nAs a joint care with the Gynecologists, surgical exploration was decided and performed via a midline laparotomy incision along the previous abdominal wall scar. Cefuroxime 1.5 g was given at anesthetic induction for antimicrobial prophylaxis.\nSurprisingly, and against CT scan findings, a large incarcerated solid mass was seen within the hernia sac (Fig. ). There was no small bowel protruding through the abdominal wall defect. The large mass lesion was almost completely into the hernia sac and incarcerated, while the stalk of the tumor was occupying the neck of the hernia defect (Fig. ). Further exploration revealed the origin of this lesion being the uterus, and therefore, total abdominal hysterectomy and bilateral salpingoophorectomy were decided and performed by a Consultant Gynecologist. The incisional hernia was primarily repaired by the Consultant General Surgeon, using no prosthetic mesh, due to the emergent operative setting and infective contamination concerns. A negative pressure drain was inserted above the repaired hernia defect to prevent a seroma formation.\nFinal histopathology of the tumor showed a 19 × 14 × 9 cm uterine leiomyoma with ischemic and hemorrhagic necrosis, calcifications, and hyalinization with no evidence of dysplasia or malignancy (Fig. ).\nThe patient had an uneventful postoperative course, and standard antipsychotic medication was promptly restarted. On the second postoperative day, she accidentally pulled her negative pressure drain off, and a small seroma was formed, which was gradually absorbed. She was discharged on the 7th postop day. |
An 81-year-old woman was admitted to our hospital with mild abdominal pain, nausea, and vomiting. She had a history of open appendectomy and laparoscopic cholecystectomy. She had received conservative therapy for small bowel obstruction twice in the previous year. Thus, adhesive intestinal obstruction was suspected at admission. A physical examination showed a slightly distended abdomen with diffuse and mild abdominal tenderness. A hematological examination showed elevated inflammatory markers (white blood cell, 17,000/µl; C-reactive protein, 0.63 mg/dl). Abdominal X-ray showed air fluid level of the small intestine. Contrast-enhanced computed tomography (CECT) demonstrated stenosis and the wall thickness of the terminal ileum and intestinal distention of the proximal small intestine, which was the site that had been affected in the first bowel obstruction event one year previously (Fig. ). Colonoscopy performed 18 months previously at another hospital showed no abnormalities in the colon or rectum. Based on the above findings, recurrence of the small bowel obstruction at the terminal ileum due to adhesion was suspected. Transnasal decompression was attempted as an initial treatment. X-ray with contrast medium revealed stenosis of the ileum (Fig. ). We planned to perform laparoscopic surgery to treat small bowel obstruction. During laparoscopic surgery, no adhesion of the small intestine was observed in the abdominal cavity and telangiectasia was pointed out on the serosa of the terminal ileum. This lesion was palpable as an elastic hard mass when gently grasping it with laparoscopic forceps. (Fig. ). Threfore, partial resection of the intestine was performed. Macroscopic observation of the excised specimen revealed a thickened wall and circumferential ulceration with scarring in the stenotic segment (Fig. ). A histopathological examination showed active inflammation and fibrotic change with lymphocytic and plasmacytic infiltration in the intestinal mucosa, lamina propria and subserosa (Fig. a). In addition, a thickened venous wall, stenosis, and obstruction of the venous lumen in the subserosa were observed (Fig. b). Elastica van Gieson staining confirmed the presence of elastic fiber at the site of the thickened venous intima (Fig. c). The wall structure of the veins was similar to that of the arteries, while there were no findings of arteritis. Phlebitis and phlebosclerosis were not observed. These findings were consistent with IMHMV. Retrospectively, we recognized that preoperative CECT showed dilation and winding change of the peripheral veins in the ileocecal region with edematous wall thickening, which supported the diagnosis of IMHMV (Fig. ). The patient had no postoperative complications and left the hospital on the seventh postoperative day. At two years and 8 months after the operation, the patient was well without any additional medication. |
A 43-year-old deceased white male was found lying prone in front of his automobile, facing away from the vehicle and wearing only a black tank top shirt and two white socks. He was last seen alive by his brother two days earlier. A large pool of dried blood and spattered blood lay over the car's hood. Due to recent heavy rains, some of this blood had run down the front of the vehicle, onto the bumper, and settled onto the ground beneath. A distinct circular indention was observed on the hood. Beneath the deceased's head was a copious amount of blood, and clutched within his extended right hand was a tubular metal object thickly covered in dried blood. Upon further inspection, it was discovered that this metal object was in fact the housing tube for a vehicle radio antenna, which would extend from the housing when the automobile is running and retract when the vehicle was powered off. Approximately two inches of antenna extended from the housing tube when it was found in the deceased's hand (Figure ).\nA search inside a metal storage shed in close proximity to the body uncovered assorted drug paraphernalia, including a metal spoon and empty syringe. It was confirmed through interviews with family members that the deceased abused illegal narcotics, including methamphetamine.\nA search of the man's bedroom resulted in the recovery of a notebook containing two different handwritten and signed suicide-type notes, the first of which was an apparently compendious life statement, and the second an apology to his family. These notes had been written over two weeks prior to the discovery of the body.\nThe external examination was notable for moderate decompositional change and insect activity, concentrated over the head and chest. A 1/4 inch laceration was superior to the right eyebrow. No other injuries were noted externally. Inspection of the right posterior aspect of the oropharnyx revealed an irregular defect of the mucosa and soft tissues. Due to decompositional change, it was difficult to assess the presence of hemorrhage in the surrounding tissue. Immediately posterior to this injury were fractures and complete displacement of the right transverse processes and interarticular portions of the C2 and C3 vertebrae. Radiographs of the head and neck showed no evidence of a projectile, and no corresponding exit defect was identified. The right vertebral artery could not be visualized due to the degree of decomposition in this area of the body. However, given the fact that the foramina through which this vessel coursed were destroyed, it was surmised that the artery had been perforated. The cervical spine was removed for a more thorough examination of the bony injuries (Figure ). The brain was negative for subarachnoid hemorrhage. The remainder of the autopsy was unremarkable.\nPostmortem toxicology was positive for methamphetamine as well as for cocaine and cocaine metabolites.\nBased upon the autopsy findings and the death scene investigation, the cause of death was ruled as "perforation of the right vertebral artery due to intraoral penetrating trauma with an automotive antenna." The manner of death was suicide. |
The 37 year-old patient from a small town in northern Italy, was welding when he was hit by a spray of liquid metal on the periorbital region that burned the upper eyelid and part of the lower eyelid. The patient was treated by eye-specialists at the local hospital with multiple debridements of the palpebral lesions, that take several weeks to heal, save for the complete loss of the upper eyelid, albeit partially maintaining the medial and lateral canthus, along with a loss of the ciliary margin of the entire lower eyelid. In the weeks which followed, the inflammation of the eyeball, which was undamaged by the metal but irritated due to a lack of palpebral protection, worsened further, partially compromising the eye's functionality. So, surgery was scheduled to reconstruct the eyelid in a single operation, because of the distance the patient had to travel, which would also provide satisfactory functional and aesthetic results. The patient was operated on to reconstruct the entire upper eyelid using, as a posterior lamella, a mucochondrial autologous graft taken from the ala of the nose as a tarsus and conjunctiva substitutes that were sutured to the levator palpebrae\nsuperioris aponeurosis and muscle. On the other hand, to reconstruct the anterior lamella, which consists of skin and muscle, the surgeons used a myocutaneous temporal flap taken from the region immediately lateral to the external canthus of the palpebral region, and which, after being isolated following a drawing of the upper eyelid to be reconstructed (Fig. ), was rotated and then sutured to the posterior lamella using the orbicularis oculi muscle as a pedicle. The donor area was closed, following the ample undermining of the edges, with a linear scar (hardly noticeable) on the temporal area. The incision performed on the lower palpebral rim, necessary for the preparation of the orbicularis muscle as a myocutaneous temporal flap, was used to place a composite graft taken from the right auricular concha in order to lift and provide greater support for the lower eyelid. The patient's postoperative recovery was normal, and within 7 days he was able to return home, to be cared for once more by local eye-specialists in continuing his medical treatment of the eyeball. When the patient returned two months later for a check-up, the aesthetic and functional result appeared satisfactory. In fact, the new upper eyelid with a stable lid margin could open and close properly in spite of the ongoing presence of residual edema (Fig. ). |
A 60-year-old man with a past history of RCC (clear cell type, G2, T1b N0 M0 Stage I) treated by a right nephrectomy in June 2015 was required to have a follow-up examination at 6-month intervals after surgery, without the use of an anticancer agent. In January 2018, a routine gastrointestinal endoscopy found an ulcerative lesion of approximately 10 mm diameter in the greater curvature of the gastric body (Fig. ). An endoscopic ultrasonography (EUS) of this lesion showed the first three sonographic layers were blurred, which suggested submucosal invasion. An endoscopic biopsy of the lesion exhibited clear cytoplasm with prominent nucleoli, which was histologically compatible with metastasis to the stomach of the patient’s known RCC. On the other hand, computed tomography (CT) incidentally detected a well contrast-enhancing round-shaped mass in the fundus of the gallbladder (Fig. ). Additional ultrasonography revealed a sessile polypoid lesion, and gallbladder stone and wall thickening were not observed. Although these findings were lacking conclusive evidence of diagnosis whether the gallbladder tumor was primary or metastatic, the circumstantial evidence potentially pointed to the tumor as a metastasis from the patient’s known RCC. 18F-Fluoro-deoxyglucose positron emission tomography combined with CT (FDG-PET/CT) was performed as a preoperative workup to detect other possible remote metastasis. However, specific FDG uptake was not shown, even in the gastric and gallbladder tumors. The blood examination was unremarkable.\nIn February 2018, a gastric wedge resection via laparoscopic and endoscopic cooperative surgery (LECS) technique was applied to the gastric tumor, and laparoscopic cholecystectomy to the gallbladder tumor was simultaneously performed (Fig. ). The operation lasted 190 min with little intraoperative blood loss. Intraoperative pathologic diagnosis was not performed in this case. The hospitalization period after surgery was not eventful, and the patient was discharged on postoperative day 7. Histological examination confirmed that the tumors of the stomach and gallbladder were both metastatic RCC. Immunohistochemical staining was strongly positive for CAM 5.2 and vimentin, supporting the diagnosis. Macro- and microscopic findings are shown in Fig. . Thereafter, the patient required examination every 3 months without the use of anticancer agents and has survived without relapse to 12 months after the surgery. |
Our case is that of a 61-year-old female with a prior history of serendipitously discovered malignant carcinoid tumor of the anterior duodenum status post-endoscopic polypectomy and pancreatic cancer status post-Whipple procedure. She continued to have chronic abdominal pain after these procedures and received an exploratory laparotomy and Roux-en-Y bypass with a partial gastrectomy. When she was first seen in the pain clinic, she complained of excruciating abdominal pain for six years. Oftentimes, her pain would be a 10/10 and that level of severity would remain for extended periods of time. She stated that this pain has affected many of her daily activities, including walking and sleeping. The pain had been refractory to ibuprofen, gabapentin, and duloxetine, among other non-opioid medications. She had also been placed on an array of opiates that included hydrocodone, tramadol, codeine, and fentanyl. When she was seen by our pain service, we began our plan of management conservatively. First, we provided trigger point injections in and around the abdominal scars left from her abdominal surgeries. This was followed by a trans-abdominis plane block. Neither of these provided relief. We then moved toward a diagnostic bilateral splanchnic nerve and superior hypogastric plexus block. She reported three days of 80% pain relief in her mid-epigastric region, and her lateral abdominal pain remained significantly improved. The next step in our treatment plan was an RFA of the bilateral splanchnic nerve and superior hypogastric plexus.\nThe splanchnic nerve block has demonstrated effective pain control in cholangiocarcinoma patients []. Given this, we planned for a splanchnic nerve RFA for our patient. The patient was positioned prone. Anterior-posterior (AP) and lateral intermittent fluoroscopic views were used to identify the vertebral body and transverse processes of the right and left T11 vertebra. Prior to the radiofrequency (RF) needles being introduced and after negative aspiration, 2 mL of 2% lidocaine was injected to each of the four sites. Contrast was used under real-time fluoroscopy to rule out intravascular spread. Four 20-gauge 10-centimeter curved venom cannulas with active tip RF needles were advanced dorsal to the anterior vertebral body and caudad to the transverse process of the vertebra. AP and lateral radiographs were then taken to confirm proper needle placement, and no paresthesias were confirmed by the patient (Figure ). The technique of needle placement and its confirmation is described in Kahan []. The stylet of each RF needle was removed and an RF probe was inserted through the cannula. The patient was then inquired about the sensory stimulation to an impedance between 300 and 800 ohms at 50 hertz to elicit abdominal discomfort. After both visual and patient-provided confirmation, RF denervation was performed at 60 degrees Celsius for 120 seconds for four cycles. After the RF denervation, 6 mL of 1% lidocaine was mixed with 2 mL of 40-mg/mL methylprednisolone, and 2 mL of this concoction was injected to each of the four sites of RFA.\nFor the superior hypogastric plexus RFA, the patient was again positioned prone. Fluoroscopic guidance in the oblique view was used to identify the right and left sides of the L5 vertebral body, such that the transverse process was partially superimposed along the lateral edge of the vertebral body. Once the target sites were under view and after negative aspiration, 2 mL of 2% lidocaine was injected to each of the four sites. Contrast was used under real-time fluoroscopy to rule out intravascular spread. Four 20-gauge 10-centimeter curved venom cannulas with active tip RF needles were advanced dorsal to the anterior vertebral body and caudad to the transverse process of the right and left L5 vertebral body. AP and lateral radiographs were then taken to confirm proper needle placement, and no paresthesias were confirmed by the patient (Figures , ) []. The stylet of each RF needle was removed and an RF probe was inserted through the cannula. The patient was then inquired about the sensory stimulation to an impedance between 300 and 800 ohms at 50 hertz to elicit abdominal discomfort. After visual and patient-provided confirmation, RF denervation was performed at 60 degrees Celsius for 120 seconds for four cycles. After the RF denervation, 6 mL of 1% lidocaine was mixed with 2 mL of 40-mg/mL methylprednisolone, and 2 mL of this concoction was injected to each of the four sites of RFA. The procedure was well-tolerated by the patient. Upon follow-up after more than one month post-operatively, the patient reported 80% pain relief in both her mid-epigastric region and lower abdomen. Most importantly, she had stopped all opioid medications. |
An 8-year-old boy was brought to the Emergency Department at tertiary trauma hospital in New Zealand, one evening after a dog bite injury. The avulsed tissue was retrieved by family and kept cool in an artificial ice gel bag prior to being seen by the plastic surgery resident. At the time of review 6 h postinjury, however, it was noted that the artificial ice gel bag had become warm. Intravenous amoxicillin with clavulanic acid was given. Patient was up to date with his immunization schedule.\nGross inspection revealed a defect that included several nasal subunits (partial nasal tip, medial third of his right alar, and the soft triangle, Fig. ). The contaminated amputated composite tissue measured approximately, 1 × 1.5 cm2, was composed of skin, subcutaneous fat, cartilage, and mucosa. As the patient did not fast, the procedure was postponed until 8 a.m. the next day. The avulsed nasal tissue was kept at 4°C overnight. The initial surgical plan was to reapply the amputated part as a composite graft. Risk and benefits of the procedure including future reconstruction were discussed with the mother. The possibility of replantation was brought up but mentioned it would be subjected to the intraoperative findings.\nAt the time of surgery, both the wound and the graft were gently debrided under 2.5× loupe magnification. During debridement, a pulsating vessel was observed at the junction of the tip and right alar at the wound edge (Fig. ). The amputated part was carefully positioned within the defect and the mucosal surface repaired. After this, under surgical microscope magnification, an opposing vessel end was found on the amputated tissue where the pulsating vessel was noted. The wound bed end of the vessel was trimmed and irrigated with papaverine and intraluminally with heparinized saline solution until a pulsatile stream of blood was observed. After ascertaining the artery could be repaired without tension, anastomosis was attempted. Four interrupted 11-0 nylon sutures were used in a quadrangular fashion for an end-to-end anastomosis of the artery (Fig. ). The time taken to perform the microanastomosis was approximately 40 min. At the release of the vessel clamp, the vessel was observed to be patent. The replanted tissue initially became pink, though by the end of the procedure, had a blue hue (Fig. ), with demonstrable capillary refill. The skin sutures were loosely, but accurately, tacked. During the first 24-h postoperative period, the operating surgeon manually induced bleeding from the wound by applying heparinized saline.\nThe patient was discharged the next day on oral cephalexin. There was no postoperative infection. At the one-month follow-up appointment, the replanted tissue remained viable (Fig. ). The outcome was excellent requiring no secondary reconstructive procedure with the last follow-up appointment being 5 years postoperatively (Fig. ). Patient (13 years old) claimed no one ever noticed the injury on his nose nor is he conscious of it. |
A 70-year-old male presented to the hospital with complaints of severe headache, confusion, and gait instability. He became progressively obtunded in the emergency department. A non-contrast computed tomography (CT) of the head demonstrated diffuse SAH and ventriculomegaly (Figure ). The patient was intubated and an external ventricular drain (EVD) was placed. He demonstrated mild improvement in alertness after cerebrospinal fluid (CSF) diversion. Cerebral angiography was performed and demonstrated tumor blush in the posterior fossa with an associated intratumoral aneurysm arising from a right posterior inferior cerebellar artery (PICA) branch (Figure ). Magnetic resonance imaging (MRI) demonstrated a 3 cm enhancing lesion in the area of the cerebellar vermis (Figure ). In an effort to reduce tumor vascularity and aid in surgical resection, arterial feeders to the tumor and to the aneurysm were embolized with polyvinyl alcohol particles. After embolization, the patient was transferred to the operating room and underwent a suboccipital craniotomy for resection of the mass. Pathology revealed the diagnosis of hemangioblastoma. The initial postoperative course was benign. MRI demonstrated a gross total resection and magnetic resonance angiography (MRA) showed no evidence of aneurysmal remnant (Figures , ). Due to his neurological status and persistence of lower cranial nerve deficits, the patient was unable to be extubated and ultimately required tracheostomy placement. On postoperative day nine and post-admission day 14, the patient experienced a sudden deterioration in his neurologic status. The patient’s motor exam declined from following commands to extensor posturing. Transcranial doppler (TCD) was performed and demonstrated markedly elevated left MCA and ICA velocities of 207 cm/s and 196 cm/s, respectively. Emergent CT obtained at that time was grossly stable when compared to the postoperative MRI. CT and MRI were repeated three days following the patient’s initial decline to work up a persistently poor neurologic exam and revealed significant left MCA territory infarction (Figure ). MRA at that time demonstrated severe left ICA and MCA flow-limiting vasospasm (Figure ). The patient was placed on vasopressors in an effort to prevent further ischemic injury. His neurological status remained poor. Ultimately, he was discharged to a long term acute care facility and expired approximately one month after discharge. |
A 47-year-old white woman, from Beirut, Lebanon, presented for general surgery consultation for a suspicious left breast mass discovered on routine mammogram. Our patient was premenopausal and had given birth to two children whom she had breastfed for 6 months each. She had had no previous medical problems and no previous surgeries. She had no family history of malignancy. She said she used to take calcium and vitamin D supplementation and denied taking oral contraceptives.\nOur patient had no medical complaints. On physical examination, there was a 3 cm mass in the medial middle aspect of her left breast, with no nipple retraction or discharge, no changes in the overlying skin, and no palpable axillary lymph nodes. A digital mammogram showed a 30×26×29 mm round mass at the junction of the inner quadrants of the left breast.\nAn ultrasound-guided core biopsy of the lesion was performed, and it showed a small cell neuroendocrine tumor on pathology (Fig. ). PET CT scan imaging and brain magnetic resonance imaging (MRI) were conducted to investigate the possibility of any other primary diseases, and the results were negative (Fig. ).\nOur patient underwent a left total mastectomy and sentinel lymph node biopsy. Four sentinel nodes were retrieved using both the blue dye and the radioisotope methods. Pathology revealed a high-grade small cell neuroendocrine carcinoma, a Ki-67 proliferation index of 50%, estrogen receptor-negative disease (Fig. ), 20% progesterone receptor-positive cells (Fig. ), and negative Her2/neu. Chromogranin, CD56, and synaptophysin were all positive in more than 50% of tumor cells with evidence of ductal carcinoma in situ (Fig. ). The surgical margins were free of tumor, and the four sentinel lymph nodes were negative. Our patient was diagnosed with small cell carcinoma of the breast (stage IIA), and she subsequently received adjuvant chemotherapy with four cycles of cisplatin 80 mg/m2 on day 1 and etoposide 100 mg/m2 for 3 days, followed by four cycles of 5-fluorouracil (5FU) 500 mg/m2, epirubicin 75 mg/m2, and cyclophosphamide 500 mg/m2 (FEC).\nOur patient has received adjuvant hormonal treatment with tamoxifen for 10 months so far. She is on regular follow-up in the cancer center, and she remains free of disease to date. |
A 58-year-old woman (gravida 13, para 10) who became menopausal 10 years ago was referred to our hospital because of increasing heaviness in the vagina. Her past medical history was unremarkable except for hypothyroidism, hypertension and hypercholesterolemia which were all controlled by medication. Gynecological examination revealed a firm mass measuring 3 cm in max diameter and protruding from the left side of the vagina. She also had a small endocervical polyp measuring about 1 cm in max diameter. The ultrasound examination did not reveal any other abnormalities and an excisional biopsy from the cervical polyp showed a benign and inflamed endocervical polyp. A resection of the vaginal mass suggested a low-grade spindle cell neoplasm. However, a subsequent panel of immunohistochemical stains showed that the diagnosis is a LG-ESS most probably originating from vaginal stromal progenitor cells. The patient subsequently underwent a successful total abdominal hysterectomy with bilateral salpingo-oophorectomy and lymph node dissection (TAH-BSO-LND). There was no evidence of any primary endometrial neoplasm, metastatic disease or endometriosis in the resection specimen. The patient was prescribed Letrozole/Aromatase inhibitor 2.5 mg daily and discharged home. She remained well and disease free after 7 months of follow-up.\nThe resected vaginal tumor consisted of an irregular and round firm mass measuring 3 cm in max diameter. The cut surface was white, solid and smooth. Histopathological sections showed that the lesion consisted of densely packed cells showing uniform spindle and oval nuclei. The mitotic rate was low (<5 per 10 high-power field), nuclear pleomorphism mild and there was no evidence of necrosis. The overall lesion appeared lobulated and intercepted by areas of hyalinization and increased vascularity. A diagnosis of low-grade spindle cell neoplasm was rendered pending the immunohistochemical stains (Fig. A and B). A large panel of immunohistochemical stains was done and it showed that the tumor cells are strongly positive for vimentin, CD10, estrogen (Fig. A and B) and progesterone with focal areas of positive staining for CD99. The proliferation marker Ki67 (MIB1) was positive in 10% of the tumor cells. The lesional cells were negative for h-Caldesmon, calponin, desmin, smooth muscle actin, CD68, CD34, Bcl-2, NSE, S100 protein, cyclin-D1, GFAP, HMB45, inhibin, EMA and synaptophysin. The morphological and immunohistochemical features in addition to the absence of endometrial stromal lesion elsewhere were entirely in keeping with primary LG-ESS. |
A 68-year-old Japanese man was admitted to our hospital because of sudden hematuria and abdominal pain. Endoscopic and imaging techniques including CT, MRI, and PET revealed a bladder tumor. Transurethral biopsy and clinical cytology revealed malignant tumor composed of giant cells, and cystectomy was performed. Macroscopically, the bladder contained a large polypoid and ulcerated reddish tan tumor (5 × 6 cm).\nFor histological evaluation, 17 tissue specimens were obtained from the tumor. Histologically, the tumor consisted of the following three elements: giant cell sarcomatoid carcinoma (70% in area), squamous cell carcinoma (20% in area), and papillary urothelial transitional cell carcinoma (10% in area) (Figure ). The giant cell sarcomatous element and squamous cell carcinoma element were invasive into the peribladder fat tissue, while the transitional cell carcinoma element was invasive into the submucosa.\nThe sarcomatoid element was composed of malignant polygonal and spindle giant cells with frequent multinucleated giant cells (Figure ). The giant cells had hyperchromatic nuclei and showed many mitotic figures. The giant cell sarcomatoid carcinoma area was homologous, and did not show heterologous elements such as bones and cartilages. Specific structures including herring bone and storiform appearances were not recognized. The tumor cells did not show plasmacytoid features. Lymphocytic infiltration was absent.\nThe squamous cell carcinoma element was composed of malignant squamous cell with individual keratinization and intercellular bridge formation. Many mitotic figures were recognized in the squamous cell carcinoma element. There were gradual merges between the giant cell sarcomatoid and squamous cell carcinoma elements (Figure ). There were occasional lymphovascular permeations by the giant cell sarcomatoid carcinoma and squamous cell carcinoma elements.\nThe papillary urothelial transitional cell carcinoma was grade II carcinoma with a few lymphovascular permeations. No transitions were observed between the transitional cell carcinoma element and the other two elements.\nAn immunohistochemical study was performed with the use of Dako Envision method, as previously reported [,]. The reagents and results are shown in Table . The giant cell sarcomatoid carcinoma element was positive for various kinds of cytokeratins, epithelial membrane antigen, and vimentin. It was negative for other mesenchymal markers such as desmin and S100 protein. P53 protein was positive and Ki-67 labeling was 72%. It was negative for CD68. The squamous cell carcinoma element was strongly positive for various types of cytokeratins and epithelial membrane, but was negative for vimentin and other mesenchymal markers. Neuroendocrine antigens and melanoma antigens were absent. P53 was positive, and Ki-67 labeling was 72%. The papillary urothelial transitional cell carcinoma element showed the similar results to those of the squamous cell carcinoma element, but the former was positive for cytokeratin 20 (Table ). Both the epithelial elements were positive for p53 and showed high Ki67 labeling (squamous cell carcinoma, 67%; transitional cell carcinoma, 32%). Other antigens examined were negative (Table ).\nA diagnosis of bladder carcinoma composed of three different elements was made. The patient was treated by palliative chemotherapy and radiation, but died of systemic metastases 32 months after the operation. Autopsy was not performed. |
A 27-year-old woman at 6 weeks gestation was admitted with 3 days of severe colicky periumbilical abdominal pain. This was associated with nausea. There was no vomiting, no reflux or dysphagia and the patient had reported normal bowel habit. Her observation parameters were within normal limits. Abdominal examination revealed tender periumbilical area without peritonism. This patient has had laparoscopic gastric band insertion 4 years prior. Her pre-procedure weight was 103 kg with body mass index (BMI) of 35.6 kg/m2. The band has been deflated completely 2 weeks prior to presentation as part of her antenatal care. She has no other comorbidities and no other prior surgical history. Her weight on admission was 75 kg with BMI of 25.9 kg/m2. Laboratories workup includes leukocyte count of 8.3 × 109/l and C-reactive protein of 22 mg/l. Ultrasound of the abdomen was unremarkable with normal intra-abdominal organs and viable single intrauterine pregnancy.\nGiven her ongoing symptoms, she underwent diagnostic laparoscopy. Prior to the procedure, the patient has requested for the gastric band to be removed instead of revised if the issue had been related to the device given that she had achieved satisfactory result (excess weight loss of 91%). Intraoperatively, it was found that the middle part of the tubing was wrapped around a loop of small bowel, creating an adhesion band around the bowel. The tubing and the gastric band were removed in a routine fashion with adhesionlysis of the adhesion band. The loop of bowel appeared compressed but viable and therefore left alone. The patient recovered well from the procedure and she was discharged home on Day 1 post procedure following a normal ultrasound confirming viable pregnancy. The patient was subsequently reviewed in clinic 4 weeks after the operation and she reported no further symptoms. Furthermore, her pregnancy was uneventful and she delivered a healthy baby at term (Figs and ). |
A 59-year-old Caucasian male presented with a 10-year history of a neglected slowly growing red lesion on the right anterior chest wall. Aside from tuberculosis treated in childhood and a remote right clavicle fracture he had no relevant past medical history, took no medications, and denied tobacco or alcohol consumption. He sought attention when the lesion’s growth rate increased and it began weeping serosanguinous fluid. He required transfusions for a presenting hemoglobin of 55 g/L. On examination there was a striking 20 x 15 cm non-odorous geographic lesion centered over the right chest wall, with heaped edges lying 3 cm proud of the normal skin contour and an ulcerated, weeping center (). It extended superiorly to the supraclavicular fossa, inferiorly below the inframammary fold, laterally to the anterior axillary line, and medially 5 cm beyond midline. In addition there was a 3 x 3 cm raised erythematous lesion suspicious for a satellite metastasis on the right forehead which had developed within the last year, and a long-standing unchanged 2 x 2 cm non-specific erythematous plaque on the left temple at the site of a remote chemical burn. Beyond chronic right shoulder achiness and motion restriction he denied pain, other local or constitutional symptoms and had an ECOG performance status of zero. CT from the chest to pelvis confirmed a large disc-shaped lesion invading the chest wall to a depth of 2 cm, destroying the right anterior manubrium and anteromedial right clavicle. There were calcified pulmonary nodules consistent with prior granulomatous disease, but no regional or distant lymphadenopathy and no visceral metastases. An incisional biopsy of the chest wall lesion confirmed moderately to poorly differentiated squamous cell carcinoma, and he was staged as pT4 cN0 cM1. The size of the lesion was prohibitively large for either surgery or radiotherapy so he proceeded to palliative chemotherapy as follows: Cisplatin 55 mg/m2 and Docetaxel 55 mg/m2 every 3 weeks. He tolerated 12 cycles extremely well with only mild myelosuppression, oral mucositis and tinnitus. The chest lesion showed an early and sustained dramatic response to therapy (). The right forehead and left temple lesions remained stable. He went on to receive palliative radiotherapy to the chest lesion as follows: 5000 cGy in 20 fractions via 9 MeV electrons to a 15 x 15 cm field, and to the right forehead lesion as follows: 4500 cGy in 15 fractions via 9 MeV electrons to a 7 x 9 cm field. Both lesions showed further improvement. He sustained a significant progression-free interval: 16 months from the time of initiation of chemotherapy and 6 months from the time of radiotherapy completion, but then developed multiple in-field recurrent chest nodules () which were re-treated with 3000 cGy in 10 fractions via 9 MeV electrons to a 7 x 14 cm field. The long-standing left temple lesion had also begun to progress, and because of this it was deemed to indeed be malignant and was treated with 3500 cGy in 5 fractions via 9 MeV electrons to a 4 cm diameter circular field. At the time of manuscript preparation, 21 months following the time of initiation of chemotherapy, he had developed new satellite metastases on the neck and left shoulder, but was doing well overall with an ECOG performance status of zero. |
A 10-day-old male infant was referred to our hospital because of suspected congenital hypothyroidism. The patient presented with symptoms of airway obstruction, such as an inspiratory stridor and retracted breathing. A hormonal test revealed subclinical hypothyroidism with a free thyroxine level (1.44 ng/dL) within the reference range, although the thyroid stimulating hormone (TSH) level (34.6 μIU/mL) was increased beyond the normal range. Laryngo fiberscopy revealed a lingual mass compressing the epiglottis (Fig. ). Enhanced computed tomography (CT) and thyroid scintigraphy revealed that the mass was an ectopic thyroid with the absence of a normal pretracheal thyroid gland (Figs , ). The patient received oral levothyroxine at a dose of 12 μg/kg/day for 4 weeks to lower the TSH level and reduce the volume of the ectopic thyroid tissue. However, we observed no reduction in the volume of the thyroid tissue and a concomitant progression in his symptoms of airway obstruction. He underwent surgery to relieve the airway obstruction when he was 2 months old. Under general anesthesia, nasotracheal intubation was performed in a sniffing position, and a transverse skin incision measuring 2.5 cm was made at the level of the hyoid bone. We split the hyoid bone at the midline, dissected the base of the tongue towards the foramen cecum, detected the ectopic thyroid mass, and suspended the mass by suturing it to the hyoid bone (Fig. ). We used 5–0 monofilament absorbable sutures and sutured between the lingual thyroid and the hyoid bone. The bite length of both the lingual thyroid and hyoid bone was about 3 mm. The points of suturing were to the lateral side of the lingual thyroid and to the front of it. The total number of suture threads was 3. The degree of suspension of the ectopic thyroid was guided by an intraoperative laryngo fiberscopy to confirm the complete elevation of the epiglottis. The patient was not extubated until postoperative day 4 and needed noninvasive positive pressure ventilation until postoperative day 22. Laryngo fiberscopy performed 6 months postoperatively revealed the complete disappearance of compression of the epiglottis by the lingual mass, and CT performed 8 months postoperatively also revealed the relocation of the lingual thyroid gland towards the hyoid bone (Fig. ). When the patient was 2 years 6 months old, his height was 94.1 cm(1.4 SD), weight was 14.0 kg(1.0 SD), free T3 was 2.97 pg/mL, free T4 was 1.48 ng/dL, and TSH was 4.178 μIU/mL. He was taking daily levothyroxine 4.5 μg/kg/day and had been kept in the euthyroid state. Since we were able to preserve his thyroid gland (which is his only functioning thyroid tissue), the postoperative control of his thyroid hormone status was relatively easy. The surgery was complicated by the development of a salivary fistula that was spontaneously resolved 5 months postoperatively. |
A 45-years-old male engineer with no history of trauma, presented to us with progressive upper back pain for two months that eventually became severe and disabling. The pain was non mechanical and was present even at rest and night. He denied any constitutional symptoms. He had no significant medical history except that he was a smoker for over 20 years.\nOn neurological examination, patient indicated altered sensation from T8 dermatome and below; however, upper and lower limb motor power was normal. He had an unsteady gait and exaggerated deep tendon reflexes in both lower limbs.\nInitial X-rays of the thoracic spine were unremarkable, but MRI demonstrated abnormal marrow replacement and enhancement of the entire T6 vertebra including its posterior elements and right 6th posterior rib. MRI axial cuts at T6 level revealed right postero-lateral epidural extension of the lesion causing severe spinal canal, right lateral recess and right exit foraminal stenosis (, ). In addition, an irregular right lung nodule was noted. CT evaluation confirmed the vertebral involvement () and the presence of a lobulated right lung nodule with emphysematous changes in bilateral upper lobes. Further radiological assessment revealed no other lesion elsewhere.\nDuring the course of the work up, he developed bilateral lower limb weakness and was unable to stand. A diagnosis of thoracic myelopathy due to cord compression was made and immediate surgical management in the form of T6 decompression laminectomy along with T3-T8 posterior instrumentation was performed (). Surgery was uneventful; patient had good recovery and was ambulating independently.\nTissue biopsy from T6 right pedicle revealed presence of metastatic tumour cells with moderate amount of eosinophilic cytoplasm in the marrow spaces suggestive of a metastatic carcinoma with neuro-endocrine features (). Tumour cells were positive for cytokeratin AE1/3, carcinoembrionic antigen (CEA), chromogranin and synaptophysin. Proliferation marker Ki67 was found to be 40%.\nEarly post-operative MRI showed adequate decompression of the spinal canal at T6 level and the patient had interval resolution of symptoms; but there was abnormal fluid collection from the surgical site extending up to the subcutaneous layer, likely to be seroma, which was conservatively managed. Chemotherapy with Carboplatin and Etoposide was initiated after satisfactory wound healing three weeks after surgery.\nOne month after the index surgery, patient developed significant motor deficit in the lower limbs (Right L2-L5 = 2/5; Right S1 = 4/5; Left L2-S1 = 4/5) and had bowel and bladder incontinence. An urgent MRI was done which revealed recurrence of the lesion causing near total obliteration of the spinal canal ().\nA second surgery at this stage was considered high risk and the decision was made to initiate concurrent site specific T4-T7 radiotherapy (30 Gy in 10 fractions) along with chemotherapy. Interestingly at the end of radiotherapy and four courses of chemotherapy (4 months post-op), there was significant clinical improvement of neurology and complete resolution of the soft tissue enhancement surrounding the spinal cord was evident in the MRI (). With appropriate physiotherapy and rehabilitation, patient gradually regained full power in both lower limbs by 6 months. He continues to be under oncology follow up and is ambulant with support. |
The second case shows a 53-year-old female. Clinical and radiographic examination confirmed a unilateral posterior crossbite due a transverse maxillary deficiency with a significant mandibular skeletal deviation towards the side of the crossbite (Fig. ). Treatment objectives.\nSARME was planned to correct the transverse discrepancy followed by arch leveling with lingual appliances and then a second surgery to correct the mandibular asymmetry.\nSimilar to case 1 impressions were obtained and this time the lingual appliances were manufactured by DW Lingual Systems (Bad Essen, Germany).\nDuring the planning for the production of the lingual brackets, it was noted -similar to case 1- that a surgically assisted rapid maxillary expansion takes place. The transverse width of the upper jaw should be adapted to the lower jaw.\nTwo trans sagittal Benefit mini-implants were inserted in the T-Zone. A silicon impression with the transfer caps was taken. The impression was given to the laboratory together with the lingual molar bands. A Hybrid Hyrax [] was then made and laser welded to the molar bands (Fig. ). Similar to case 1, the lingual appliance was indirectly bonded with a dual cured resin and the maxillary expansion appliance was inserted. In this case the molar bands were cemented with a dual cured resin and the hybrid hyrax was fixed to the mini-implants using the Benefit fixation screws. The first lower arch wire 12 NiTi was inserted while in the upper the brackets were secured with a continuous steel ligature in each quadrant (Fig. ). SARME was performed with an activation rate of two quarter turns twice a day until crossbite correction was achieved at two weeks post-surgery (Fig. ). The Hybrid Hyrax was then blocked. The first upper archwire (12 NiTi) was placed four weeks after surgery (Fig. ). After complete leveling and radiographic re-examination the surgery to correct the asymmetry was performed.\nThe patient has a positive overbite and overjet now. The patient shows a good transversal and sagittal occlusion. |
A 48-year-old lady presented with increasing confusion following a three-day history of fever, shortness of breath, and productive cough. Her background included heavy smoking, heavy ethanol use, and two prior hospital admissions for pneumonia and exacerbation of chronic obstructive airways disease.\nAt presentation she was febrile, delirious, and tachypneic with low oxygen saturations, relative hypotension, and new onset atrial fibrillation with a rapid ventricular response. Her chest X-ray revealed dense right upper and middle lobe consolidation. Initial treatment consisted of oxygen therapy, intravenous fluid resuscitation, noninvasive ventilation, and antibiotics (ceftriaxone and azithromycin) for her community acquired pneumonia. She reverted to sinus tachycardia after treatment with digoxin. She was prescribed benzodiazepines for apparent alcohol withdrawal agitation.\nShe was transferred to our institution after two days in established multiorgan failure with shock, respiratory failure, coagulopathy. Invasive organ support was initiated with intubation, mechanical ventilation, vigorous fluid resuscitation, vasopressors, and inotropes. Blood and sputum cultures taken at the time of her initial presentation grew Klebsiella pneumoniae sensitive to all antibiotics except amoxicillin. Antibiotics were changed to ceftriaxone and ciprofloxacin. Human immunodeficiency virus (HIV) screen was negative. Echocardiography revealed normal valve function, with no sign of vegetations and normal chamber size and function.\nBy day 3 in the ICU, her gas exchange had improved. Though tachycardic and febrile, she maintained sinus rhythm and sustained a mean arterial pressure above 70 mm Hg without vasopressor support. Sedation was then discontinued for the first time since intubation. Her pupils were noted to react to light but she was unresponsive to painful stimuli. There was no further change to her neurological status 24 hours after stopping sedation.\nOn day 5, she had a focal seizure limited to her left arm, lasting less than a minute. Her pupils were equal but noted to be unreactive, her gaze was divergent and a flaccid quadriplegia and absence of deep tendon reflexes were noted for the first time. An urgent CT brain demonstrated advanced cerebral injury [], initially reported as extensive cortical necrosis of the frontal, parietal, and temporal lobes. To further define the extent of the inflammatory process, magnetic resonance imaging (MRI) [Figures and ] was performed. There was widespread nonvascular territory cortical infarction, particularly over both convexities. Given the additional patchy involvement of cortex elsewhere, particularly within the posterior fossa, hippocampi, and occipital horns of the lateral ventricles, the overall pathology was deemed to be cerebritis with hyperaemia and associated meningitis. There were no features suggestive of focal cerebral abscess and the dural venous sinuses appeared patent.\nNeurosurgical advice was sought. It was felt that bifrontal craniotomy to reduce intracranial pressure would confer little benefit. In view of ongoing coagulopathy and thrombocytopenia, the risks of placing an intraventricular drain were considered to outweigh any benefits in guiding therapy. Spinal fluid was not sought for the same reasons.\nOn day 6 the patient developed dilated and fixed pupils, absent corneal reflexes, and absent plantar reflexes bilaterally. She still had a cough reflex and took spontaneous breaths on pressure support. A second CT brain [] showed increasing areas of low attenuation within the frontoparietal and superior occipital cerebral cortex with evidence of mass effect.\nA meeting was held with her family to inform them of her extremely poor prognosis. In keeping with her family's understanding of her previously expressed wishes, mechanical ventilation was withdrawn and palliative measures instituted. The patient died two days later. |
A 61-year-old woman presented with two supernumerary nipples located along the milk line on each side of the upper abdomen. During a few months before referral, the patient had noticed a firm palpable mass in close relations to the supernumerary nipple on the right side (). She had no other symptoms. Bilateral mammogram and ultrasound revealed normal breast parenchyma. Ultrasound of the supernumerary nipple on the right side confirmed a small mass in relation to this nipple, presenting as a hypoechoic, well-defined area, measuring approximately 10 mm in diameter ().\nUpon clinical examination, the supernumerary nipple on the right side presented with inversion and a palpable firm mass underneath this nipple. Ultrasound-guided needle core biopsy (16G) was performed (). The pathology report described elongated epithelial islands composed of cubic cells, with a small centrally located lumen with focally identifiable cuticles. The epithelial structures were surrounded by a dense fibrous stroma. The microscopic analysis indicated the possibility of SyT and the lesion was categorized as a B3-lesion (a lesion with uncertain malignant potential) ().\nFollowing a Multidisciplinary Team Conference, it was decided to recommend resection with a 5 mm rim of normal tissue, which was subsequently performed ().\nMacroscopically the tumour measured 7 x 5 x 5 mm and was described as a firm and grey-white tumour, in close relations to the supernumerary nipple. Microscopically the tumour measured 13 mm in diameter and was localized in the deep part of dermis and underlying subcutaneous tissue with no relation to the epidermis. The tumour was composed of solid trabecular and glandular imitating formations with focal cysts. The epithelium consisted of cells with slightly irregular nuclei and indistinct nucleoli, surrounded by an eosinophilic cytoplasm. The glandular structures were lined by cuboidal cells. The tumour was in close association with the lactiferous ducts and smooth muscle of the nipple (). Foreign body giant cell reactions, due to ruptured cysts, were identified.\nImmunohistochemical analysis showed positive reaction for CK5, CK14, and P63 () in association with the epithelium presenting as solid cords, whereas the glandular luminal cells showed positive reaction for CK7 (). Thus, the immunohistochemical analysis demonstrated the complex nature of this lesion. The diagnosis was SyT based on the pathology report.\nA re-excision was performed due to insufficient rim of normal tissue in the cranial direction in order to minimize the risk of recurrence. |
A previously healthy 35-year-old African American male presented with a one-month history of worsening lower back and bilateral lower extremity pain, intermittent night sweats, and 32 kg unintentional weight loss over the course of a year. He did not have saddle anesthesia or urinary or fecal incontinence. He was initially seen in a primary care clinic and was diagnosed with sciatica. As symptoms continued to worsen, he underwent a computed tomography (CT) scan of the lumbar scan as an outpatient that was concerning osseous spinal metastasis. He was started on prednisone 10 mg daily and was referred to the oncology clinic at our center. Prednisone gave him minimal symptomatic relief. While waiting to be seen in the oncology clinic, the patient had an episode of leg weakness with near-fall prompting him to present to the emergency department of our hospital and was admitted for further evaluation. His vital signs were stable. He had no palpable cervical, supraclavicular, axillary, or inguinal lymph nodes. Neurological exam was normal with intact strength and sensation in both lower extremities.\nHis complete blood count and serum electrolytes were normal including a normal serum calcium level at 8.1 mg/dL. He tested negative for human immunodeficiency virus 1 and 2 antibodies. Magnetic resonance imaging (MRI) of the cervical, thoracic, and lumbar spine showed several enhancing lesions in T11, T12, L3, L4 vertebral bodies, right sacrum, and ilium that were concerning metastatic disease. There was effacement of the right lateral recess and right neural foramen at the L3-L4 and effacement of the left lateral recess and left neural foramen at the L4-L5 due to tumor retropulsion (Figures –). In addition, a small epidural tumor was noted at the T5 vertebral level without significant spinal canal stenosis or cord compression. Imaging was also concerning osseous metastasis involving the sternum and multiple ribs. Incidentally, narrowing of the neural foramen at left T2-T3 and right C7-T1 and T5-T6 levels was also noted. Since the findings were concerning diffuse metastatic disease, a CT scan of the chest, abdomen, and pelvis were performed and showed bilateral hilar and mediastinal adenopathy, mild cardiomegaly, and dilated main pulmonary artery measuring 3.6 cm (Figures and ). Enlarged liver measuring 18.1 cm, enlarged spleen measuring 12.4 cm, and multiple bilateral enlarged pelvic sidewall, external iliac, and inguinal lymph nodes concerning lymphoma or metastatic disease are shown in . Ultrasound of the scrotum did not reveal any testicular masses.\nHe underwent extensive screening for hematologic and solid tumor malignancies including serum protein electrophoresis, urine immunofixation, beta-human chorionic gonadotrophin hormone levels, and fecal occult blood test that were all negative. He subsequently underwent a CT-guided core needle biopsy of the left iliac crest lesion that was significant for noncaseating and necrotizing granulomas. Histochemical stains for Grocott's methenamine silver (GMS) and Ziehl-Neelsen stains were negative for fungal elements and acid-fast bacilli, respectively. Due to high suspicion of malignancy, he also underwent an endoscopic bronchial ultrasound with transbronchial needle aspiration of the inferior mediastinal lymph node which found non-necrotizing granulomas but did not reveal any malignant cells (Figures –). Fungal culture and acid-fast bacilli culture from the transbronchial aspirate were again negative. Serum ACE level was 62 U/L (normal 14–82 U/L).\nNeurosurgery was consulted, and they did not recommend any acute neurosurgical intervention. The patient was discharged with follow-up in pulmonology clinic. Since there was concern that his steroid therapy prior to admission could have masked lymphoma, he had a left inguinal node excisional biopsy, a month later, that showed necrotizing and non-necrotizing granulomatous lymphadenopathy and was negative for acid-fast or fungal microorganisms. Since there was concern for a process with high metabolic activity, he also had an 18F-labeled fluorodeoxyglucose (18F-FDG) positron electron topography (PET) scan that was significant for extensive hypermetabolic osseous and nodal disease (). |
A 14-year-old male, previously diagnosed case of typhoid two months back, for which he was admitted in a hospital presented to the medical consult service with ongoing fever for one week and vomiting for three to four days despite the completion of treatment. The fever started one week back and was acute in onset, rising in a stepladder fashion. It was high grade, peaked at 104°F, intermittent in nature, and did not present with any rigors or chills after being administered paracetamol. It was also associated with a constant, generalized and bilateral headache, relieved by pain killers for two to three hours only. The patient also complained of abdominal pain, which he described as diffuse, gradual in onset, aching in character, nonradiating, aggravated by food intake, intermittent and associated with vomiting which occurred just after food intake, three to five times a day. It was around one to two cups per episode, watery in consistency with no particular odor and contained food particles in it. In addition, he had mild chest discomfort which developed after admission and myalgia and fatigue.\nOn admission, he was fully responsive, alert, and oriented with normal effect. The patient was febrile with a temperature of 104°F, pulse rate of 110 beats per minute (BPM), blood pressure (BP) of 90/60 mmHg, and a respiratory rate of 16 per minute. His abdomen was nondistended, mildly tender with normoactive bowel sounds, and no organomegaly. The neurological exam for bulk, tone, power, and reflexes was insignificant for any finding. Sensations and joint position sense were intact. Cerebellar signs of co-ordination and cranial nerves two to twelve were intact, with no observed nystagmus or visible tremors. The cardiovascular exam showed tachycardia. The cardiac rhythm was normal with no added sounds or murmurs. The pulmonary exam did not yield any abnormal findings, with bilateral audible breath sounds clear to auscultation and no added wheeze. The patient had conjunctival pallor with no lymphadenopathy, edema, jaundice, rashes, or tightening of the skin.\nThe complete blood count (CBC) showed a hemoglobin (Hb) of 9.8 g/dL and a total leukocyte count (TLC) of 9.7 x 109/L with neutrophils being 82% and lymphocytes being 15%. Platelets were 436 x 109/L. Erythrocyte sedimentation rate was raised up to 84 mm/h (normal: 0-22 mm/h). The coagulation profile was normal as well. The electrolyte panel indicated normal potassium, calcium, and sodium levels. The blood urea nitrogen (BUN) and creatinine were also marginally raised.\nThe liver function tests (LFT) were within normal limits. Viral serology for hepatitis viruses B and C, dengue virus nonstructural protein 1 (NS1), and dengue IgM were all negative. Coagulation profile was also insignificant. The blood culture showed the growth of Salmonella typhi. The disk diffusion method for susceptibility disclosed the Salmonella typhi to be susceptible to ceftriaxone, cefixime, vancomycin, tazobactam, but resistant to ciprofloxacin, nalidixic acid, amoxicillin, and norfloxacin.\nThe patient was admitted to the medical inpatient service where he was resuscitated with intravenous fluids. Empirical antibiotic therapy was started with intravenous ceftriaxone. His condition seemed to be improving when on day three, he abruptly developed shortness of breath which worsened gradually. It was associated with orthopnea and paroxysmal nocturnal dyspnea. This raised the suspicion for pulmonary edema and hence, urgent arterial blood gases (ABGs) and chest X-ray (CXR) were ordered.\nArterial blood gases showed a pH of 7.41, partial pressure of oxygen (pO2) of 31.0 mmHg, partial pressure of carbon dioxide (pCO2) of 31.0 mmHg, bicarbonate (HCO3) of 20.7 mmol/L, oxygen saturation (Sat O2) of 49.6%, and base excess (ABE) of -4.0.\nThen, the patient was shifted to the intensive care unit (ICU) where he was kept on oxygen support. On examination, he was partially responsive, alert, and oriented with normal effect. The patient was febrile with a temperature of 100°F, pulse rate of 122 BPM, BP of 70/50 mmHg, and respiratory rate of 24/min. His cardiovascular and pulmonary systems were re-examined which showed tachycardia with normal rhythm and a slightly raised jugular venous pressure (JVP). The apex beat was also found to be displaced. On auscultation, there was a third heart sound (S3 gallop rhythm) and bilateral end inspiratory crepitations up to the mid-zone of the posterior chest. During the course of his illness, he also developed bilateral pedal edema.\nThe CXR showed cardiomegaly with prominent upper lobe vessels and alveolar edema. The costophrenic angles were blunted with right costophrenic angle showing septal lines (Kerley B lines) due to interstitial edema. These findings were highly suggestive of pulmonary edema secondary to heart failure.\nElectrocardiography, echocardiography, cardiac enzymes and a Urine Detailed report (Urine D/R) were carried out for further diagnosis.\nThe cardiac enzymes showed creatine kinase (CK) of 56 IU/L (normal: 20 to 180 IU/L), creatine kinase muscle/brain (CK-MB) of 32 IU/L (normal: 4 to 24 IU/L) and Troponin I of 0.168 of ng/ml (normal: 0 to 0.39 U/L). The Urine detailed report (Urine D/R) showed a marked presence of ketones and proteins.\nElectrocardiography (ECG) findings revealed sinus tachycardia with mild QT prolongation and T wave inversions which raised the suspicion for myocarditis.\nEchocardiography findings revealed an ejection fraction (EF) of 25% with generalized, moderate to severe left ventricular (LV) systolic dysfunction. The left and right ventricles were dilated with right ventricular (RV) dysfunction. ECHO was also significant for mild tricuspid and mitral regurgitation. There was no pericardial effusion or a thrombus in the dilated ventricles. The pulmonary artery systolic pressure was 25 mmHg. These findings were suggestive of dilated cardiomyopathy secondary to myocarditis (Figure ).\nCardiac magnetic resonance (CMR) imaging and endomyocardial biopsy were not done owing to unavailability of procedures at our set-up and financial constraints.\nThe patient was then admitted to the critical care unit (CCU) where intravenous furosemide and dexamethasone were given. He was kept on oral enalapril, metoprolol succinate, and spironolactone for five weeks to counter the neurohormonal arcade of myocarditis. Empirical antibiotics were continued with intravenous ceftriaxone for 10 days.\nThe patient was then discharged after being admitted for three weeks in the CCU and was followed up in medical consults service twice weekly, then once monthly for six months with echocardiography imaging. After six months, his EF improved from 25% to 45% and the patient made a full recovery with no limitations in his daily life activities. |
A 68-year-old male was admitted to hospital with a history of dyspnea at rest for 2 d, a cough producing mucus, loss of appetite and diarrhoea. Pneumonia was suspected, and the patient received intravenous antibiotic treatment. During the patient's second night in hospital, he was found groggy and walking near his room by the nurses. They took him back to his room and connected a new antibiotic dose to his venous catheter. A few hours later, he was found unconscious, lying on the floor next to his bed. His peripheral venous catheter was connected to a nasal cannula delivering O2 from the wall, with a flow rate of approximately 2 L/min, and the antibiotic dose was found in the garbage can. As post-mortem lividity was present, no resuscitation attempts were performed by the clinicians and the death was pronounced. The body was immediately brought to the University Center of Legal Medicine (Lausanne, Switzerland).\nBefore any manipulation of the corpse, a native CT scan was carried out at around 10 h post-mortem using an eight-row CT unit (CT LightSpeed 8, GE Healthcare, Milwaukee, WI). All scanning parameters are detailed in . A forensic pathologist immediately viewed the native CT images. To evaluate the distribution of gas because of physiological changes in the body after death, the radiological alteration index (RAI) was used as proposed by Egger et al. []. The RAI was based on the analysis of samples from seven sites (heart cavities, liver parenchyma and vessels, left innominate vein, abdominal aorta, kidney parenchyma, L3 vertebra and the subcutaneous pectoral tissues).\nAll images were interpreted in a consensus reading by one board-certified radiologist and one forensic pathologist who were trained in forensic imaging. A post-mortem radiological report was prepared and described all findings from the native CT scan. The native CT scan revealed the presence of subcutaneous, intramuscular and intravascular gas, a pneumoperitoneum, a bloated heart with the right cavities filled with gas and a left pneumothorax ((a–d)). The maximum RAI score is 100, and RAI scores greater than 50 are usually seen in cases of severe changes []. Therefore, given the short post-mortem interval (<12 h), the elevated RAI obtained in this case (75) strongly suggests an exogenous source of gas in the tissues.\nCT-guided gas samples were immediately taken from multiple sites (carotid artery, jugular vein, right auricle, thoracic aorta, pectoral muscle, thoracic cavity, abdominal cavity, scrotum and gluteal soft tissues, (e,f)) according to the protocol for gas analysis described by Varlet et al. [].\nThe external examination revealed massive subcutaneous emphysema with audible crepitations on the entire surface of the body. Additionally, there was a bruise on the left part of the forehead, a small contusion near the left eyebrow surrounded by a purplish-blue bruise and some bruises of different ages on the inferior part of the thorax and on the legs. On the left arm, a venous catheter was still in place.\nSamples for toxicological and biochemical investigations (blood and urine) were collected in S-Monovette® tubes with sodium fluoride or ethylenediaminetetraacetic acid as a preservative (Sarstedt, Nümbrecht, Germany). Biological samples were collected as soon as possible on arrival of the body at the morgue (vitreous humor) and during autopsy (femoral blood, pericardial fluid and urine). During sampling of femoral blood by incision with a scalpel, multiple gas bubbles were visible in the blood ((a)).\nToxicological analysis of the collected samples revealed acetone at a physiological level in the blood, as well as caffeine and paracetamol in both blood and urine. Post-mortem chemistry analysis of the serum samples collected during the autopsy revealed elevated values of C-reactive protein (175 mg/L), consistent with an inflammatory state and a procalcitonine level compatible with a bacterial infection (0.69 μg/L). The results also revealed signs of cardiac dysfunction with a very high level of N-terminal prohormone of brain natriuretic peptide (>35 000 ng/L) and cardiac necrosis with a very high troponin-T level (>56 000 ng/L).\nThe autopsy was performed by one board-certified forensic pathologist and one forensic pathologist in training. Opening of the thoracic cavity revealed numerous gas bubbles in the fatty tissue covering the heart. The pericardium was opened and filled with water, and the heart floated. An incision was made in the right ventricle with a scalpel, and blood with gas bubbles escaped. This technique was performed mainly for training purposes and not for gas sampling, as the gas from the cardiac cavities had already been sampled during CT imaging. The autopsy revealed the presence of numerous gas bubbles throughout the vascular system, even in the small vessels of the brain ((b)). Additionally, changes to the pulmonary parenchyma were consistent with pneumonia. No other major findings were made.\nAn Agilent 6890N GC (Agilent Technologies, Palo Alto, CA) combined with a headspace gas autosampler and equipped with an Agilent Select Permanent Gases column arrangement was used. This column arrangement is specially designed for gas analysis and contains a molecular sieve 5 Å PLOT capillary column (10 m × 0.32 mm i.d.) and a Porabond Q column (50 m × 0.53 mm i.d.) in parallel, which allows for separation of carbon dioxide (CO2). The column temperature was maintained at 45 °C for 13 min. The injector temperature was 100 °C, and the injection was conducted in splitless mode. Helium was used as the carrier gas at a constant flow rate of 8 mL/min. The gas detection and quantification were performed with a thermal conductivity detector set at 150 °C. The system was calibrated for each gas with standard gases of H2S (Multigas, Domdidier, Switzerland), O2 and N2 (from laboratory air), and CH4 and CO2 (Carbagas, Lausanne, Switzerland). With this system, all the gases could be detected in the same run. The gas compositions for the different intracadaver sampling sites are displayed in .\nSamples of the brain, heart, lungs, liver and kidneys were taken during autopsy and stained following a standard haematoxylin and eosin protocol. The heart samples were taken from both ventricles, and the interventricular septum exhibited epicardial vessels deprived of red cells and small intramyocardial haemorrhages. The lung samples taken from each lobe contained numerous clusters of leucocytes, mainly neutrophils, and, to a lesser extent, some macrophages, predominantly in the left lower and right upper lobes. These findings are consistent with the pneumonia suspected on admission to the hospital. There were no significant findings from the other organ samples. Samples of the heart were prepared and analysed for deposition of the plasma antigen fibronectin and the terminal complement complex C5b-9 to look for early cardiac damage, especially the right ventricular ischemia. None of the samples showed reaction for C5b-9, and only small groups of cells stained for fibronectin. Based on these results, cardiac ischemia was not likely in this case.\nIn light of the different results, the cause of death was attributed to a fatal O2 embolism. This was caused by the infusion of pure O2 from the wall into the vascular system through a venous catheter connected to a nasal cannula. Although from a medico–legal point of view the circumstances of the death (accident, suicide and homicide) remained unclear, police investigations led to the suspicion that the patient had connected the nasal cannula to the venous catheter. The wound on the forehead and the bruises observed during the external examination could be explained by a fall from or next to the hospital bed, as suggested by the police. |
A two-day old female born at 39 week’s gestation was referred from a community hospital to a tertiary level neonatal intensive care unit (NICU) with the chief medical concern of bloody stools. Antenatal history was unremarkable. The infant was born after a relatively uncomplicated pregnancy to a 28-year-old G1P0 mother. Maternal serologies for rubella, herpes, HIV and syphilis were protective and group B Streptococcus testing was negative. Maternal cytomegalovirus status was not known. The only maternal medication was prenatal vitamins. There was no history of infections or diabetes. Gestational hypertension developed in the last month of pregnancy did not require intervention. There was no maternal or paternal family history of any gastrointestinal issues, no allergies and no family history of cow’s milk protein allergy (CMPA). Additionally, there was no history of familial bleeding disorders and parents were non-consanguineous. There was no major blood loss at the time of delivery, no bloody discharge from the mother’s nipples and there was no blood loss associated with a neonatal ankyloglossia release performed on the first day of life. The infant was born via spontaneous vaginal delivery with no instrumentation after an induction of labour for pre-eclampsia. Membranes were ruptured for twelve hours and active labour was under two hours. APGARS were 8 and 9 at one and five minutes respectively. The umbilical cord gas had a pH of 7.17 and a base excess of − 7.7 mmol/L. She received standard newborn care including a subcutaneous injection of vitamin K and did not receive any additional medications.\nDuring the first twenty-four hours of life the infant was breastfeeding with latch issues and required an ankyloglossia release. She subsequently continued to breastfeed and was additionally given formula three times. The family recalled that the newborn had approximately ten dark green to black, loose, foul smelling bowel movements and gradually became less interested in feeding. This was brought to the attention of the physician later the following day. On day of life two initial vitals check at mother’s bedside showed a heart rate of 180 beats per minute and a temperature of 38.3 °C. Upon re-assessment in the nursey the heart rate was 130 beats per minute with a temperature of 37.3 °C and the remainder of her vitals within normal limits. On physical exam baby was reported to be less active but responsive and appropriate with handling, otherwise exam was consistent with a healthy newborn exam; bowel sounds were present, abdomen was soft and there were no hemangiomas present on the infant’s skin. There was a questionable anal fissure at the 12 o’clock position. Initial complete blood counts showed a white cell count of 8 × 109/L, hemoglobin of 19.2 g/dL, platelets of 258 × 109/L and there were no eosinophils present. From a capillary sample, gas and electrolytes were within normal limits; the C-reactive protein was 21.5 mg/L and plasma lactate was 4.2 mmol/L. Over the next ten hours the baby had approximately four moderately bloody stools with blood mixed throughout the stool. Additionally, a coagulation profile was performed and was within normal limits. Blood cultures were drawn (which were subsequently negative) and antibiotics were started. A single view abdominal X-ray was performed (Fig. a) that showed a nonspecific bowel gas pattern, without pneumatosis, nor dilated bowel loops. The baby was subsequently transferred uneventfully to our tertiary NICU.\nUpon arrival at the tertiary level NICU, at approximately 55 h of life, while hemodynamically stable on room air, the baby started to produce copious bilious emesis without exhibiting signs of discomfort. Abdominal x-ray (Fig. b) showed pneumoperitoneum. Repeat blood work showed a white cell count of 8.8 × 109/L, hemoglobin of 16.4 g/dL, platelets of 200 × 9/L and a hematocrit of 46.2%, INR of 1.44, lactate 1.9 mmol/L and a CRP of 83 mg/L. The infant was urgently transferred to the operating room where laparotomy revealed a full thickness perforation of the anterior antimesenteric transverse colon that was two centimeters in diameter, in the mid transverse colon (Fig. ). There were patches of green-grey discoloration and suspected full thickness necrosis extending to the cecum and appendix suggesting the pathological epicentre was in the mid transverse colon with additional satellite lesions radiating outward. There was no evidence of intrabdominal calcifications to suggest a remote intrauterine perforation. There were no ischemic or necrotic lesions in the distal transverse colon, descending colon, or the sigmoid colon and rectum. Based on these findings, as the etiology was thought to affect the entire ileocolic vascular distribution, an extended right hemicolectomy was performed, with formation of an end ileostomy and distal transverse colon mucous fistula. During resection, no evidence of vascular malformation or anomalies was noted.\nThe pathological evaluation of the ileocolic resection specimen was consistent with classical features of necrotizing enterocolitis with perforation and acute peritonitis (Fig. ). The perforation site in the colon showed transmural necrosis with heavy acute inflammation as well as numerous empty, dilated spaces in the submucosa, consistent with pneumatosis intestinalis. The rest of the resected bowel showed patchy partial (in ileum and colon) to full thickness necrosis (in appendix), transmural acute inflammation and pneumatosis intestinalis in the terminal ileum and distal colon.\nThe infant had an uneventful postoperative recovery. Initially her nasogastric tube was placed to low intermittent suction then transitioned to straight drainage. When the nasogastric output was minimal, she was worked up of oral feeds of expressed breast milk. She progressively reached full feeds with breastmilk and was discharged home within 2 weeks. A contrast study was performed at 7 weeks, which revealed patency of the distal colon with no evidence of stricture. Five months after her first operation, the patient underwent uncomplicated elective laparotomy for reversal of both stomas and primary anastomosis. Mild adhesions were found at repeat laparotomy with no distal atresia. She was advanced to a full p.o. feeds on postoperative day 5. The resected end-ileostomy specimen submitted for histologic evaluation, showed an unremarkable bowel tissue with no evidence of fibrosis or scarring from the neonatal episode of NEC. |
A 38-year-old man with no significant past medical history presented with left-sided chest pain, dyspnoea on exertion and left flank pain in April 2011. He was found to have left lower lobe segmental pulmonary embolus on a VQ scan, together with an incidental finding of a large soft tissue mass (15.5 × 12.0 × 16.7 cm) with no arterial phase enhancement, immediately superior to the left kidney in the CT scan. No local or distant metastases were noted in the staging CT scan ().\nHe underwent an open laparotomy for removal of the tumor, left kidney and spleen. Pulmonary embolism was treated with therapeutic enoxaparin.\nMacroscopically, a large, well circumscribed tumor (210 × 160 × 100 mm) with soft granular tan cut surfaces was found to be displacing the left kidney posteriorly and inferiorly (). No adrenal gland was identified in the specimen or within the tumor. A fibrous pseudo-capsule separated the tumor from the planes of resection. Tumor was separated from the kidney by renal capsule, and found to be pressing on the renal vein without extension into it. The spleen and the left kidney were of normal appearance.\nMicroscopically, the tumor completely replaced the left adrenal gland, and was composed of sheets of large pleomorphic cells with eosinophilic cytoplasm (). It demonstrated all the risk factors for development of metastatic disease with diffused growth pattern, vascular invasion into small venules but not into the renal vein, extensive tumor necrosis, foci of coarse stromal calcification, > 10 mitoses per 100 high power fields, cellular pleomorphism and capsular invasion. There was no evidence of malignancy in one left renal hilar lymph node and one splenic lymph node.\nTumor cells were strongly positive with Vimentin (), weakly positive with Melan-A (), and were negative with AE1/AE3 and S100.\nThe above findings classified the tumor as stage III (T3N0M0) undifferentiated large cell ACC (as per both AJCC and ENSAT staging system).\nSince the initial presentation there were no signs and symptoms of hypercortisolism, though urine androsterone, etiocholanolone, tetrahydrocortisol and tetrahydrocortisone level were high. The latter went back to normal after the radical surgery.\nAdjuvant mitotane monotherapy (3 - 6 g/day) together with cortisol replacement was initiated after the surgery. The patient enjoyed disease-free period of 7 months until he developed metastatic disease in bones, lungs and peritoneum (, ). At time of presentation of relapse he was complaining of thoracic back pain which precipitated MRI spine which showed T12 cord compression requiring palliative radiotherapy (30 Gy in 10 fractions). Six cycles of etoposide, doxorubicin and cisplatin (EDP) were added to mitotane over a period of 7 months, resulting in excellent partial response to treatment, i.e. complete response of all peritoneal nodules, near complete response of the multiple pulmonary nodules except one sub-centimeter nodule in each lung and stable bony metastases (, ). The patient is now on mitotane monotherapy only (3 - 6 g/day) with stable disease. Therapeutic enoxaparin was ceased after 27 months of treatment for pulmonary embolism with no further thromboembolic complications. |
A 38-year-old male presented to the emergency room with a complaint of abdominal pain around the umbilicus for one day; it was associated with nausea and vomiting. He stated having similar episodes of pain intermittently for the last one year, and the pain was relieved with analgesics after a few hours. He didn’t report any fever, change in bowel habits, or blood in stool or vomitus. He had a past medical history of diabetes mellitus, which was well controlled with medications, and his past surgical history was significant for an exploratory laparotomy with bowel resection for a small bowel obstruction done in 2002 in his home country. His vitals were stable at presentation. On examination, his abdomen was soft with mild tenderness in the left side of the abdomen with no guarding or rigidity. Bowel sounds were heard and were normal. His laboratory workup was unremarkable. A computed tomography (CT) scan of the abdomen and pelvis suggested two blind-ending radio-dense drains/catheters coursing within the peritoneum with proximal tips in the left mid abdomen and distal tips located in the lower pelvis adjacent to the dome of the urinary bladder (Figure ) The rest of the findings were unremarkable.\nThe patient was planned for the laparoscopic removal of the foreign body from the peritoneal cavity and informed consent was obtained. A diagnostic laparoscopy was performed using three 5 mm ports. The first port was placed at Palmer’s point using the Visiport technique, the other two 5 mm ports were placed under vision in the upper midline and the left anterior axillary line at the level of the umbilicus. Adhesions were found between the left abdominal wall and the omentum. Adhesiolysis was performed using LigaSure (Medtronics, Minnesota, United States) and a sharp dissection, and two pieces of drains were found entrapped between the adhesions and wrapped around the omentum. Careful dissection was performed and the drains were removed and retrieved through the 5 mm port (Figure ). The peritoneal cavity was inspected for any injuries or bleeding. No bowel or visceral injuries were seen. The skin was closed after releasing the pneumoperitoneum and removing the ports. The procedure and postoperative course were uneventful. The patient was discharged from the hospital on postoperative day one. |
A 57-year-old man who had primary hyperparathyroidism presented to the Ear, Nose, and Throat clinic because of fatigue and agitation. A 4D CT scan showed a 2.6 × 1.3 cm ovoid mass at the sternomanubrial junction with contrast enhancement on both arterial and portal venous phases in the area where a sestamibi parathyroid study showed focal activity. A right inferior substernal parathyroid adenoma was excised. The excised parathyroid was markedly enlarged and had adenomatous characteristics including hypercellularity and gross enlargement. Pathology examination of the surgical specimens showed a hypercellular area of parathyroid tissue that was surrounded by a fibrous capsule, distinct and separate from adjacent normocellular parathyroid tissue, characteristic of parathyroid adenomas. The surgery was believed to be successful.\nThe patient returned three months later because of extreme fatigue. The parathyroid hormone (PTH) level was found to be elevated to 304. The diagnosis of persistent hyperparathyroid disease was made and reoperation was recommended because of probable parathyroid gland hyperplasia. A 4D CT scan of the neck showed two parathyroid glands (one right and one left) (Figure ). Three-dimensional reconstruction (Figures , , ) sharply demonstrated the two parathyroid glands in relation to the other major structures. This approximated well the anatomic features of the glands, trachea, great vessels etc., which are a precise representation of the human structures. For example, the left parathyroid gland was more posteroinferior than the right parathyroid gland and the left parathyroid gland lay just lateral to tracheoesophageal groove. The 3D post-processing was performed by 3D imaging laboratory staff on the 4D CT scan for parathyroid gland surgical planning (Vital Images VES version 6.5.5, Minnetonka, Minnesota). Anatomic segmentation was obtained by 3D software automation and hand contouring of specific anatomy to localize the parathyroid glands with respect to surrounding anatomic structures including arterial vessels, venous vessels, trachea, esophagus, and thyroid gland. Skin surface rendering was included to provide location references in the x, y, and z planes and 3D box scale provided with the 3D imaging software. Each anatomic structure was given a unique color and transparency to provide rapid identification within the 3D model. Image batches were created with localization references and sent to the radiographic picture archiving and communication system for review and use on screen in the operating room.\nAt revision surgery, the parathyroid glands were localized as expected from the preoperative 3D imaging studies. The two parathyroid glands were tan, oval shaped, and similar to each other in size. Biopsies showed that the right and left parathyroid glands were hypercellular. After histologic confirmation of parathyroid tissue, intraoperative blood PTH level showed 50% reduction- 76. The total operative time was 48 minutes, and estimated blood loss was 10 milliliters. Pathology demonstrated hypercellular parathyroid tissue composed of chief cells that gradually transitioned to areas of normocellular parathyroid tissue and intermingled hypercellular areas composed of oncocytes. The patient had no postoperative complications and his preoperative symptoms resolved. |
A 60-year-old, left-hand dominant female with cirrhosis (Model for End-Stage Liver Disease [MELD] score 16) and type II diabetes mellitus on insulin presented to the emergency room with left forearm burning, pain, and swelling. She denied any history of trauma or new exposure to the hand. After receiving a diagnosis of cellulitis and a course of cephalexin, she returned four days later with fever as well as increased dorsal hand swelling and erythema. Computed tomography (CT) imaging demonstrated no evidence of necrotizing soft tissue infection and abscess. She did not present any motor or sensory deficits of the digits or wrist. She was prescribed a course of ceftriaxone and vancomycin. One month later, she presented again to the emergency room for altered mental status. While receiving treatment for hepatic encephalopathy, she underwent imaging of her left forearm due to continued pain. CT imaging now showed diffuse patchy lucencies and severe cortical thinning of the distal radius with a differential diagnosis of osteomyelitis, bone infarct, or aggressive osteopenia. Contrast-enhanced magnetic resonance imaging further showed significant cortical bone destruction as well as bone marrow edema with enhancing endosteum and periosteum consistent with osteomyelitis of the distal radius (Figure ).\nThere was no overlying soft tissue ulceration or fluid collection, suggesting a potential hematogenous spread of infection. A CT-guided biopsy confirmed bone necrosis inundated with fungal hyphae, which was morphologically consistent with Mucor via Grocott methenamine silver and periodic acid-Schiff staining (Figure ).\nThe infectious disease and hand surgery teams were consulted for management of Mucor osteomyelitis of the left distal radius with no evidence of direct inoculation.\nShe was immediately started on amphotericin. Her first surgical intervention entailed irrigation and debridement of the necrotic radius extending from the radiocarpal joint (including a portion of the lunate) to the proximal radial shaft via a dorsal approach. The bony defect was filled with Stimulan® (Biocomposites, Keele, UK) anti-fungal cement beads. A uni-plane external fixator was placed from the mid-radius to the second metacarpal to span the bony defect while the infection was managed (Figure ).\nShe returned to the operating room one week later for repeat irrigation and debridement followed by application of a Stimulan® amphotericin polymethyl methacrylate cement bone spacer contoured to the lunate distally and the diaphysis of the radius proximally. Postoperatively, the range of motion of her left digits were limited though her sensation to light touch remained intact. While recovering, she experienced significant acute kidney injury from amphotericin therapy and was transitioned to isavuconazole treatment. The plan was for more definitive reconstruction involving internal fixation. However, the patient was lost to follow-up as she sought care for her hepatic encephalopathy and extended-spectrum beta-lactamase bacteremia secondary to a urinary infection.\nSix months after the placement of the antifungal cement bone spacer, she presented again to the emergency room with left elbow pain and forearm tenderness. X-ray imaging showed inadequate alignment of the carpus and distal radius/ulna with extrusion of the antifungal spacer that was no longer communicating with the radius. She underwent repeat irrigation and debridement, removal of the bone cement, and replacement of the uni-plane fixation with a multi-plane external fixator. At this time, her liver condition had decompensated to a MELD score of 22, which correlated with a 30-day mortality greater than 50% with a major operation. Due to her significant comorbidities and chronic immunosuppression, she was not a candidate for an osseous free flap or cadaveric tissue implant to reconstruct the distal radius. A distal ulnocarpal wrist fusion was performed with a spanning bridge plate from the ulna to the third metacarpal and an iliac crest bone graft (Figure ).\nHer long-term postoperative course was complicated by cirrhosis, acute renal failure, and uncontrolled diabetes. Eleven months postoperatively from wrist fusion, her wounds were well healed. Despite occupational therapy and thermoplastic splinting, she developed significant digital stiffness with limited flexion and extension at the metacarpophalangeal and interphalangeal joints. She retained sensation to light touch in the median, ulnar, and radial nerve distributions. She now uses the left forearm as a helper hand to her right counterpart during her daily activities. Per infectious disease recommendations, she was treated with isuvaconazole for 12 months. |
A 21-year-old female patient came to our service with a complaint of unilateral right-onset headache associated with diplopia initiated 6 months earlier. She had no personal or family remarkable antecedents. She never smoked. Six months earlier, the patient started to experience one-sided right throbbing headache. She denied nausea, vomiting, or photo- or phonophobia. Fifteen days after the pain onset, she noticed double vision and medial deviation of the right eye, which forced her to wear an eyepiece to perform her activities and drive. She went to several centers and used various medications such as paracetamol, nonsteroidal anti-inflammatory drugs (NSAIDs), opioids, and triptans without improvement. Three months earlier, she started using dexamethasone 4 mg daily with partial pain control but maintenance of diplopia.\nAt the examination, the patient had cushingoid face, violaceous striae, and right VI cranial nerve palsy with no other neurological changes. Blood tests were normal (). A contrast-enhanced MRI scan of the brain did not show any remarkable features (). A spinal tap released crystalline cerebrospinal fluid (CSF) with an opening pressure of 14 cm of water. Biochemical, microbiological, and cytological analyses of the CSF were normal (). CT scan of thorax did not show any evidence of lymphoma or sarcoidosis.\nPrednisone 1mg / kg was then started. With one week of treatment, complete reversal of ocular paralysis and remission of pain were observed. However, when the corticoid was gradually withdrawn, the patient returned to pain and returned to paralysis of the VI right pair. The prednisone was increased again to 1 mg / kg this time with reversion of ocular paralysis but without pain control. Several prophylaxis attempts were made with beta-blockers, calcium channel blockers, topiramate, and tricyclics without any symptomatic control that would allow corticosteroid withdrawal.\nThe pregabalin 150mg daily was then introduced. With 7 days of medication onset there was already an important remission of pain. With 15 days of pregabalin initiation, the retitration of prednisone was started without any intercurrence and the patient reversed the exogenous Cushing syndrome. Pregabalin was maintained for one year and retracted. Currently, the patient has been free of pain for 2 years. |
A 26-year-old male patient had road traffic accident. He was immediately transferred to the hospital, where he was assessed by an emergency medical officer who found that he was having difficulty in breathing, conscious, and following command, the findings observed were SpO285% with O24 L/mt by face mask, heart rate 124/mt, and RR 26/mt. There was significant subcutaneous emphysema all over the chest and face. He was not moving his right upper limb and there was swelling over the right scapula and shoulder. Movement on the right side of the chest was significantly reduced and breath sound was absent. Abdomen was soft and there was no evidence of intra-abdominal trauma or bleed. He was resuscitated by intravenous fluid, oxygen therapy, and other conservative measures. Routine investigations were sent; chest X-ray revealed hydropneumothorax. Ultrasound abdomen suggested collection in the right pleural space and no evidence of abdominal injury. It was confirmed by computed tomography (CT) thorax which revealed large lung parenchymal tear, hydropneumothorax, multiple rib fracture on the right side, and associated subcutaneous emphysema []. Immediately, chest tube was placed on the right side and about 1200 ml blood came out. Gradually, consciousness level started deteriorating and he had an episode of convulsion, after which he was intubated and put on ventilator. CT scan brain was done to find any cause related to change in consciousness and convulsion. CT scan brain revealed pneumocephalus in the multiple sites of cerebrum [], which was suggestive of brain air embolism due to systemic embolization of air secondary to lung tear as there was no evidence of direct brain injury. Two-dimensional echocardiogram was done and it ruled out intracardiac shunt. He was managed by ventilation with high FiO2, anticonvulsant drugs, antibiotics, and other supportive measures. After 72 h of ventilation, CT scan of brain was repeated and it was found that all hypodense lesions due to air embolism disappeared and were replaced by multiple infarctions. The patient remained on ventilator for 2 weeks with the tracheotomy tube and then gradually weaned off from ventilator. His chest tube was removed and was discharged in the 4th week with complete recovery. |
The patient is a 78-year-old African American male with a history of B2 predominant lymphocytic thymoma, stage IIIa, diagnosed in 2007. He originally presented with chest pain and was found to have a mediastinal mass. This mass was biopsied and diagnosed as a stage III widely invasive malignant thymoma with bronchial and vascular invasion. He underwent surgical resection of the mass, including part of the lung, and radiation therapy. Post-surgery, the patient experienced vocal cord paralysis and was treated with thyroplasty.\nHe was doing well and had no recurrent disease until April 2012, when he started having chest pain. Repeat computed tomography (CT) scan of the chest showed multiple new pleural nodules in the patient’s left hemithorax. Biopsy results were interpreted as recurrent thymic carcinoma. In May 2012, he was started on the ADOC chemotherapy regimen- intravenous (IV) cisplatin 50 mg/m2 and doxorubicin 40 mg on Day 1, 0.6 mg/m2 vincristine on day 2, and 700 mg/m2 cyclophosphamide on day 3, every 4 weeks. He also received continuous IV fluids, and on Day 5 received pegfilgastrim. He was started on sandostatin due to high chromogranin levels, and given dexamethasone and antiemetics prior to starting chemotherapy. The patient tolerated his first cycle of chemotherapy well with minimal side effects.\nRepeat CT of the chest in June 2012 showed the patient was having a response to the chemotherapy with marked decrease in size of the metastatic disease, with one pleural nodularity decreasing from 7.3 × 1.8 cm to 5.0 × 1.3 cm. The patient completed three additional rounds of ADOC therapy for a total of four cycles, the last one being in August 2012. More therapy was not pursued due to adverse effects of fatigue and dizziness, and it was decided to observe the patient for 2 months.\nIn October 2012, PET/CT scan showed progression of the metastatic disease with pleural thickening and new bilateral lung nodules. Different treatment options were discussed, and in late October 2012 the patient was started on erlotinib 150 mg daily. Improvement was evident after a few months of treatment. PET/CT scan in December 2012 revealed that the small right sided lung nodules had resolved. Repeat PET/CT scan in April 2013 showed a positive response with significant improvement in the patient’s pleural metastases with a decrease in size and FDG uptake. Side effects of erlotinib experienced by the patient included diarrhea and a macular papular rash on the face and nose. He was started on loperamide for the diarrhea, and for the rash he was given cetaphil lotion, doxycycline 100 mg twice daily, and hydrocortisone 0.2% cream. As of April 2013, the patient continues to take erlotinib 150 mg daily and has no evidence of recurrent disease. |
A 32-year-old male with no significant past medical history presented to our facility with a history of worsening pain and swelling in the medial aspect of his proximal left lower extremity over the past several years. Anteroposterior (AP) and “frog leg” lateral radiographs of the left lower extremity revealed a dense lesion consistent with dense matrix, adjacent to the medial aspect of the femur (). An MRI of the left lower extremity revealed that the lesion was of extraskeletal origin, arising from the adductor magnus, without any involvement of the femur (, ). The solid heterogeneous mass was located in the posterior compartment of the proximal-to-mid thigh and measured 9.6 × 7.3 × 13 cm in the transverse, AP and vertical dimensions, respectively. Multiple tiny hypointense foci, which corresponded to the calcific density noted on the prior plain radiographical studies, were noted centrally. The mass was otherwise of intermediate soft-tissue intensity on T\n1 weighted images and moderately hyperintense to muscle on T\n2 weighted images. Following intravenous administration of gadolinium, moderate enhancement of the mass, excluding the central portion, was observed. Superiorly and inferiorly, the margins of the mass were indefinite, and the mass demonstrated peripheral vasculature, which was most prominent at the cephalad and caudad margins. The mass was centred within the adductor magnus muscle fibres that were displaced around the mass. Anterolaterally, the mass was very closely approximated to the posterior cortex of the femur and the fascial margins that separated the quadriceps and the posterior compartments (Supplementary videos). No obvious signal abnormality was noted within the femur to indicate invasion or primary osseous origin. Post-excision pathology confirmed the presence of mesenchymal chondrosarcoma.\nIn the interval, surveillance imaging for metastatic disease with CT scan of the chest, abdomen and pelvis was performed. At 18 months, multiple bilateral non-calcified pulmonary nodules concerning for metastasis were identified (). The most prominent pulmonary nodule was present within the posterior right base, measuring 2.3 cm in the greatest dimension and abutting the pleura (). With regard to the abdominal and pelvic series, a new right adrenal lesion appearing as a hypodense pedunculated mass measuring 1.6 cm in the greatest dimension was noted. There was no evidence of osseous metastases. |
The patient is a 7-month-old twin boy who presented to our institution's emergency department with increased work of breathing and desaturations (70 s). He was born at 33 weeks gestational age with Down syndrome, developed chronic lung disease (CLD) of prematurity, and was also found to have a moderate size secundum atrial septal defect (ASD) as a newborn. Prior to the current illness, he had been in the hospital multiple times for failure to thrive and respiratory distress, requiring mechanical ventilation with high amount of supplemental O2 and inhaled nitric oxide (iNO) as he developed pulmonary hypertension (PH). Echocardiography showed progressive enlargement and hypertrophy of his right ventricle and at times bidirectional shunting across his ASD. A diagnostic cardiac catheterization as a preoperative evaluation was performed, which showed elevated pulmonary vascular resistance indexed (PVRi) at baseline (8.8 WU·m2), which decreased with inhaled oxygen alone and iNO (3.8 WU·m2). Additional catheterization data at baseline condition showed a right atrial mean pressure of 6 mmHg, right ventricular end diastolic pressure of 6 mmHg, and pulmonary artery pressure 51/19 mmHg with mean 32 mmHg. The patient was started on home O2 therapy with nasal cannula. The current hospitalization occurred prior to a planned fenestrated patch repair of his ASD.\nHe was initially admitted to the general ward and soon transferred to the pediatric ICU for severe hypoxemic respiratory failure requiring mechanical ventilation. Respiratory syncytial virus (RSV) infection was diagnosed with the positive antigen test. He continued to have paroxysmal severe hypoxic events compatible with PH crisis. He was treated with sedation and neuromuscular paralysis, increased FiO2, optimization of O2 carrying capacity with packed red blood cells transfusions, and iNO. Milrinone infusion was added as the right ventricular function was depressed on echocardiogram (TAPSE 6 mm, Z-score −4), which demonstrated evidence of systemic to suprasystemic right ventricular pressure and bidirectional shunting across the ASD (Figures and ). No other cardiovascular intravenous drips were given during the ICU stay. Sildenafil was initiated enterally and escalated to maximal dose (2 mg/kg/day) without hemodynamic compromise. He was on diuretic therapy (bumetanide infusion up to 10 mcg/kg/hr) as chest X-ray demonstrated evidence of bilateral interstitial edema with bilateral pleural effusions on admission () and confirmed by chest ultrasound. Bilateral chest tubes were placed after failure of diuretic therapy to reduce effusions on hospital day #6. The drained fluid was milky in appearance bilaterally, with a white blood cell of 1,004/mm3 with lymphocyte predominance (88%) and elevated triglycerides (1008 mg/dl), and hence a diagnosis of chylothorax was made. Low IgG level (249 mg/dl) and hypoalbuminemia (2.5 g/dl) were noted at the time of pleural effusion drainage. Intravenous immunoglobulin and 25% albumin solution were administered. His feeding formula was changed to medium-chain triglyceride formula. The milky drainage became serous; however, the volume of chest tube drainage remained unchanged. Enteral feeding was discontinued and total parenteral nutrition was initiated, which decreased the volume of pleural effluent but small to moderate amount of pleural effusion was intermittently observed by chest X-ray for over sixty three days until the patient's death. Venous Doppler ultrasound of the upper extremities and the neck was performed on hospital day #7 and 4 weeks later, and compression, thrombosis or obstruction of the superior vena cava, and upper extremity were ruled out. A central venous catheter was placed in the right jugular vein soon after admission and was removed on hospital day #7 and replaced by a peripherally inserted central line. The patient required chest tubes for drainage until hospital day #22. Since then, intermittently small to moderate pleural effusion was observed by chest X-ray, but chest tubes were not placed.\nHe continued to be critically ill with persistent hypoxemic respiratory failure without improvement in PH with several PH crisis episodes. Therapy with an endothelin (ET) receptor antagonist (Bosentan) was added. The hospital course was complicated by bacterial tracheitis from Pseudomonas and E. coli. The patient remained on mechanical ventilator support for 6 weeks due to failed weaning of ventilator support from hypoxemia despite high levels of supplementary FiO2 and iNO. Cardiac catheterization performed 6 weeks after admission showed PVRi of 7 WU m2 on 100% FiO2 and 20 ppm of iNO under general anesthesia, pulmonary venous desaturation, and bidirectional shunting through ASD. Additionally, interval increases in right atrial pressure (mean 13 mmHg), right ventricular end diastolic pressure (12 mmHg), and pulmonary artery pressure (52/24 mean 36 mmHg) were noted. Given his severe and irreversible lung injury from mechanical ventilation in addition to baseline chronic lung disease, he was deemed not a candidate for lung transplant. Considering that the patient had Eisenmenger physiology due to severe PH and poor prognosis, the palliative care team was also consulted. Weaning from the mechanical ventilator was tried multiple times, but failed. At 9 weeks of his ICU hospitalization, he developed severe hypoxemia unresponsive to medical therapy that ultimately caused his death.\nAn autopsy showed bilateral small straw-colored pleural effusions (right 17 ml and left 10 ml), and the lung parenchyma was red-brown, poorly aerated, and diffusely congested with focal consolidation. The heart had an ASD (0.8 × 1.2 cm) with right ventricular hypertrophy secondary to PH. Microscopically, both lungs showed subpleural cysts lined by pneumocytes and containing macrophages, sloughed pneumocytes, and neutrophils. Acute multifocal bronchopneumonia was present with neutrophils in the bronchioles and alveoli. Chronic interstitial lung disease is diffusely present with alveolar septal thickening, capillary disorganization, and hemosiderosis. Small pulmonary arterial branches demonstrate moderate to marked medial smooth muscle hypertrophy with lumen narrowing, while large pulmonary arteries were normal with minimal changes. No lymphatic dilatation was observed on H&E or D2-40 immunostained slides; therefore, lymphangiectasia was ruled out (). From the autopsy results, hypoxia due to progressive PH was considered as a cause of death. |
A 61-year-old Caucasian British gentleman with a five-year history of stable left calf claudication at 100 yards was referred to our unit with recent onset of incapacitating left calf and thigh claudication as well as sudden onset of rest pain in his left foot. His only risk factor was smoking. He had no other comorbidities and was taking aspirin and simvastatin. Clinically he had a weak left femoral pulse with absent distal pulses on his left leg. His left foot was cool to touch but there was no evidence of ulceration. He had all his right lower limb pulses palpable. Ankle Brachial Pressure Indices (ABPI) were 0.37 on the left and 1 on the right ankle.\nMagnetic resonance angiography (MRA) revealed occlusive disease of the left common femoral artery (CFA) and of the proximal superficial and profunda femoral arteries (Figures , ).\nThe patient underwent an endarterectomy and a prosthetic patch angioplasty of his left common, superficial and profunda femoral arteries. The main operative findings were occluded common, proximal superficial and profunda femoral arteries with sub-plaque hematoma. The occluding plaque was found at the bifurcation while the proximal SFA and PFA with a patent SFA and PFA were found beyond the plaque.\nThe endarterectomy was performed openly through an inverted Y arteriotomy extending from the left CFA onto the proximal SFA and then onto the proximal PFA (Figure ). Tacking sutures of 7/0 prolene were applied to the distal end of the PFA and SFA. The arteriotomy was closed with a Dacron patch with its distal end bisected into two to patch the PFA and SFA simultaneously (schematic diagram of the surgical repair is shown in Figure and the final intraoperative repair in Figure ).\nAfter the operation, the patient made an uneventful recovery with an improvement in his ABPI to 1.03 and a full resolution of the symptoms on his left leg. |
36-year-old female with no past medical history developed lower limbs and para-spinal muscle spasms that progressed over 3 months. Severe muscle spasms leading to arching of her back were triggered by sudden noise or cutaneous touch. Her symptoms continued to progress and she became wheelchair-bound 6 months later.\nMRI of the neuroaxis and CSF examination were normal. Anti-GAD antibodies were positive (> 2000 U/ml). She was diagnosed with the classical form of SPS. She responded partially to plasmapheresis at the referring centre but continued to require very frequent treatments and was therefore started on IVIG.\nWhen she was reviewed at our institution, she was severely disabled by her symptoms requiring regular IVIG treatments at a dose of 90 g every 12 days. She was taking regular diazepam at a dose of 30 mg per day and morphine up to 60 mg a day to control pain. On examination, she had brisk reflexes and severe clonus. She was exquisitely touch-sensitive which induced severe prolonged painful muscle spasms. The muscle spasms were severe enough to compromise her breathing and she required intermittent oxygen. EMG showed continuous muscle fibre activity. Blink reflex study was abnormal with marked amplification of the R2 component recorded following test stimulus in keeping with brainstem hyperexcitability (Fig. A). Given her extreme stimulus sensitivity, she underwent EEG/EMG polygraphy recording which captured exaggerated startle response to auditory stimuli in keeping with brainstem hyperexcitability (Fig. ). The rest of her work-up and immunology screen were negative.\nShe received auto-HSCT 8 years from the onset of her symptoms. Transplantation proceeded with no unexpected complications apart from routine toxicities (Table ).\nWhen reviewed 5 months after auto-HSCT, the majority of her neurological symptoms had improved significantly. She was ambulating independently and required no further IVIG but continued to take diazepam. She had mild startle responses. Anti-GAD antibodies remained positive at > 2000 U/ml.\nRepeat EMG undertaken a year post-auto-HSCT remained abnormal with continuous motor unit activity. Blink reflex study with short interstimulus intervals was not possible as immediately following the first electrical stimulus protracted contraction of facial muscle tended to emerge. Therefore, no meaningful comparison could be made with the previous study. Overall, the neurophysiological assessment continued to show features in keeping with SPS despite the clinical improvement.\nShe was reviewed again 2 years following auto-HSCT and was noted to have remained off all immunotherapy and was able to walk independently. She reported occasional muscle spasms affecting her arms and legs and poor exercise tolerance. Neurological examination was normal. She declined repeat neurophysiological assessment. Her anti-GAD antibodies remained positive. |
An 18-year-old unmarried young Asian female came through the emergency department (ER) with a history of hematemesis for last twenty-four hours. The patient reported that this was the third episode of hematemesis in one day. She was managed in ER for non-variceal upper gastrointestinal bleeding (UGIB) with fluid support and urgent transamine injections. Her blood pressure was 100/60mm Hg and pulse were 100 per minute. After stabilization, she was transferred to the inpatient ward for further investigation and management.\nOn examination, the patient was in acute distress due to her recurrent bleeding episode. She had elicited tenderness on palpation in right upper quadrant (RUQ) and left lower quadrant (LLQ) of her abdomen. Rest of the physical examination is unremarkable. Her weight was 45kg and height was 162.5cm. She had never been to school and her education took place at home by her family.\nPreviously, she had severe headaches with 8/10 in intensity for three months. She had a computerized tomography (CT) scan done two months ago for her headache in a different hospital where she was admitted due to her complaints of a headache and blurry vision. She further claimed that CT scan showed the blood clot in her head and she was taking a blood thinner (unknown anti-platelet agent) for it. The patient was unable to present the reports when a physician asked for it. She took a blood thinner which was available over the counter (OTC).\nAccording to her, she had first episode of bleeding from the ear when she was two years old. She never had any bleeding episode afterward. She had menarche at an age of thirteen years and she had regular menstruation and reported the use of one pad per day for four days every month. For last three months, she had menometrorrhagia and she also had watery discharge along with it. Complete work up for bleeding disorders (bleeding time, prothrombin time, quantiative analysis of platelets) and serology (autoimmune disorders) ordered came back normal.\nLater, the patient also mentioned about seizure episodes associated with her menses. She told that she had three to four episodes of generalized tonic-clonic (GTC) seizures, each lasting for 10-15 minutes. Her last event of seizures was in the emergency department. The event was not witnessed by anyone (physicians, nursing staff or family member). She experienced a severe postictal headache in the frontal region.\nShe got symptomatic relief in a headache with acetaminophen. Upon admission, baseline labs were ordered including complete blood count (CBC), basic metabolic panel (BMP), liver function test (LFT), complete bleeding profile, antinuclear antibody (ANA), electroencephalogram (EEG), electrocardiogram (ECG), chest X-ray (CXR), stool detailed report and urine detailed report was ordered. All her tests came back normal.\nShe again had an episode of upper gastrointestinal bleeding on the third day of admission. This time patient had fresh blood on her shirt. This event was concerning for the possibility of a qualitative platelet defect or a rare collagen disorder. She underwent qualitative analysis which did not show abnormalities on Adenosine 5’- diphosphate (ADP) and collagen test. The patient was evaluated by a gastroenterologist and had an esophagogastroduodenoscopy (EGD) which showed mild gastritis. In addition, a viral panel (Hepatitis B and Hepatitis C) and a CT scan of the abdomen and the chest were non-suggestive of any pathology.\nThe patient was given omeprazole oral tablets twice a day and was managed symptomatically. Next day she had blood in tears as shown in figure #1 which was an alarming symptom and raised the concern of haemolacria secondary to ophthalmological disorder. Ophthalmology consult was made and after ruling out every serious possibility like trauma, tumors, etc., she was put under close surveillance for haemolacria episode. On the same night, she bled again from eyes with each layer of bloody tears coming from eyes. She also mentioned blood oozing from nipples and vagina. Repeat physical examination was done and a bottle was found beneath her bed which she refused to give initially and kept insisting that the bottle has medications. Upon opening the bottle, a nurse found blood in it which she said she collected from her nipples. There was no mark of any fresh or dried blood on a body orifice and her breast examination was also satisfactory.\nOn her sixth day of admission, the patient complained of continuous chest pain and she said she had it for three to four times per week since last three years. She added that she forgot to address it on the day of admission. Chest pain was sharp in quality and diffuse in nature. ECG had no acute changes. When asked about any previous episodes of chest pain, she told that she had an episode before and that always relieved by getting some fresh air.\nOn the eighth day of admission, the patient complained about dribbling of urine since eight months. According to her, she used to take bath five times in a day. But on examination, she had not changed her clothes since admission. On observation, she was in acute distress and in severe need of any kind of management. Patient demanded medical or surgical treatment to relieve her different symptoms. She was from the low socioeconomic group and family was concerned about her illness and prompt treatment. They had incurred a huge financial loss for last eight months in seeking medical opinions about her various conditions. She was given different treatments but none of them ever helped.\nSuch plausible and fabricated medical history raised our concern for a psychiatric consult. After having a long session individually with the patient alone and then with her relatives revealed the fact that she was the only female in her family and therefore; the center of attention and care. Our psychiatrist advised close camera surveillance and vigilant observation of the patient after getting consent from the family. It was found patient used that bottle of blood as a source for her tears. They counseled family members about her symptoms in detail and advised to keep her occupied in different activities with paying less attention to her complaints. A conclusive diagnosis of Munchausen syndrome was made after a detailed assessment. We discharged her after one week and followed-up with serial cognitive behavioral therapy (CBT) sessions. |
A sixty years old male was admitted to our multidisciplinary ICU with complaints of aggression, altered sensorium, tremors, sweating, clonus in legs (myoclonus) and irrelevant talks. Initially he was managed in medicine emergency where a working diagnosis of gastroenteritis induced hyponatremia with sepsis was made. The patient received infusion of 3% NaCl, antibiotics and oxygen supplementation @ 6 L/min through venturi mask. In view of aggression and altered sensorium, CT brain was done, which was normal. An assessment by neurologist was done, who advised for MRI Brain for further evaluation. Patient was kept nil per oral in view of possibility of further deterioration and need for mechanical ventilation. Since there was no improvement, patient was shifted to ICU. In ICU, the intensivist took a detailed history from the attendants of the patient. His medical history included long standing hypertension, diabetes mellitus type 2, coronary artery disease with PTCA for triple vessel disease and a major depressive disorder. Relatives denied any allergies or illicit drug abuse. Patient was taking metoprolol 25 mg once daily, olmesartan 10 mg twice daily, ecospirin 75 mg once daily, atorvastatin 10 mg at night, metformin 1 g twice daily twice daily, pioglitazone 50 mg once daily, regular insulin 16 U and 20 U subcutaneous, pantoprazole 40 mg once daily along with some herbal powder for his comorbidities for the past 10 years. For last two years, patient's depression was controlled with escitalopram (SSRI) 10 mg and clonazepam (benzodiazepine) 0.5 mg. The patient had an acute episode of anxiety five days before admission to ICU, for which psychiatrist had prescribed another SSRI paroxetine 20 mg and alprazolam 0.5 mg. Symptoms did not subside, rather patient developed restlessness in his legs with current like sensation along with loose motions. His symptoms worsened over next 2 days, and he presented to the medical emergency with severe agitation and presenting complaints as described above.\nIn the ICU, patient was hemodynamically stable but severely agitated and restless and not responding to commands. Laboratory parameters measured included Hb 12.8 g%, TLC 10800/mm3, platelet count 2.7 lakh/mm3, INR 1.0, Na+ 117meq/L, K+ 4.5meq/L, serum creatinine 0.9 mg/dL and ABG pH 7.424/PaO2 109/PaCO2 28.9/HCO3 18.6/BD −4.3/SpO2 99%/lactate 0.7/Na+ 117/K+ 3.9/iCa+2 3.55/glucose 90 mg/dL/Cl− 83 mEq/L. In view of the recent escalation in the doses and addition of other antidepressants (SSRI) and benzodiazepines with concomitant intake of drugs like metoprolol, pantoprazole and some herbal medications associated with typical features, presumptive diagnosis of SS was made as per Hunters criteria. All antidepressants were stopped. Sedation using titrated doses of propofol (10 mg/hr) to maintain Ramsay score of 2–3 was started to alleviate the symptoms and allow the complete excretion of drugs. Patient received oxygen by face mask and was monitored continuously. Over the next 24 hours his agitation subsided and patient was awake and started responding to commands. Later propofol sedation was gradually tapered off. Thereafter, patient was discharged from ICU and referred back to psychiatrist for further management. |
Patient 1 is a 68-year-old married white British male with a diagnosis of TRS. He first presented with psychosis to psychiatric services in 1965 at age 17. Over the next four decades he was treated with a number of antipsychotic agents including sulpride, aripiprazole, haloperidol and olanzapine; all were at up to maximal British National Formulary recommended doses and a minimum of 12 weeks’ duration with compliance assured during inpatient episodes; he experienced a number of relapses over time. He did not achieve remission and was started on clozapine in 2002 (dose 400 mg daily). Following that, he was functional and relatively stable for 11 years, aside from some minor residual psychotic and anxiety symptoms.\nHis medical history at this time included ischaemic heart disease (IHD), cardiomyopathy with an ejection fraction of 35%, congestive heart failure (NYHA II) and bilateral pedal oedema. In 2014, following a decline in his cardiac function, his maintenance clozapine dose was reduced from 350 mg to 300 mg daily. Unfortunately, this was followed by a severe psychotic relapse characterized by prominent thought disorder, paranoid delusions, self-neglect and increasingly aggressive and challenging behaviour. His clozapine dose was increased back to 350 mg but his mental state continued to deteriorate and due to violence and aggression he was transferred to a psychiatric intensive care unit in March 2015.\nIn August 2016 he was admitted to the National Psychosis Unit, having been an inpatient for over 2 years. His symptoms at this time included persistent persecutory delusional beliefs, obsessive-compulsive traits, anxiety, mood lability, prominent difficulties in concentration and memory and irritability with outbursts of aggression. Psychotropic medication on admission included lurasidone 111 mg (which he had been treated with for over 1 year), sertraline 100 mg and diazepam 6 mg daily. On admission, he scored 115 on the PANSS total score and 29/30 on the Mini Mental State Exam (MMSE). An MRI brain scan was within normal limits; blood tests on admission were mostly within normal limits, except for a raised urea of 13.9 mmol/L (3.3–6.7) and an increased prolactin level of 894 mIU/L (100–410), attributed to lurasidone. The pharmacotherapeutic approaches considered included a trial of high-dose olanzapine, switching from sertraline to vortioxetine because of the added benefit in attention and cognitive flexibility, or adding either memantine or donepezil as adjuncts to aid cognition.\nIn August 2016 it was decided to increase his lurasidone dose to 148 mg daily. Sertraline was switched to adjunct vortioxetine 10 mg, which was gradually titrated to 20 mg over the next couple of weeks. By September 2016 there was noticeable improvement in symptoms, and by December 2016 he was in remission, scoring 44 on the PANSS total score. Pregabalin 25 mg BD was prescribed as an anxiolytic after stabilization on lurasidone and vortioxetine, and benzodiazepines were down-titrated and stopped. He was successfully discharged home in January 2017. Psychotropic medication on discharge included lurasidone 148 mg, vortioxetine 20 mg and pregabalin 25 mg BD. He described his wellbeing as 9 out of 10, compared to having been 1 out of 10 at admission. The family described his change as ‘miraculous, and that he had not been so well, even while being treated with clozapine’. Subsequent follow up confirmed a sustained functional and symptomatic recovery. |
A 49-year-old gentleman came to our institute with dysphagia for the last two years. The dysphagia was gradually progressive in nature and was more for solid than liquid. There was no associated pain during deglutition. The patient had been treated by several physicians for one and a half years for the same complaint but without any relief. The patient lost 5-6kg of weight during this period. He was earlier diagnosed with cervical spondylosis and treated with a cervical collar, analgesics, and neck exercises. However, nothing could relieve his main complaint of dysphagia. When the patient arrived in our clinic, his body posture was stooping forward and he was having difficulty in straightening his torso over his pelvis ().\nThe patient was a known hypertensive and was prescribed tablet telmisartan 40mg since 5 years. The patient was also on metformin 500 mg twice daily for type II diabetes mellitus.During a detailed clinical examination, there was no obvious pathology found in the oraphaynx and laryngopharynx. No neck nodes were clinically palpable. All other systemic examinations were not significant. All the routine blood investigations were normal. Barium swallow oesophagus was suggested and it showed a narrowing of the lumen in the hypopharynx and cervical part of the oesophagus. Ossification was seen in the anterolateral aspect of the C3-T1 vertebrae ().\nThe rest of the oesophagus was normal. This was confirmed by computed tomography of the vertebral column showing syndesmophytes and anterior longitudinal ligament ossification at the cervico-dorsal spine. This was impinging upon the posterior pharyngeal wall and oesophagus confirming the diagnosis of Diffuse Idiopathic Skeletal Hyperostosis (DISH) ().\nUpper Gastro-intestinal tract endoscopy, another important investigation for dysphagia, was not performed in this case as there was no intraluminal mass lesion detected in the CT scan. There was another segment showing ossification of the lumbar spine from L1-L5 vertebrae (). We advised the patient to have a surgical removal of the osteophytes; but he refused to do any sort of surgical intervention. So we advised him to avoid solid food and ingest soft semisolid food in a small bolus instead. After a 3-month of follow up, the patient wasdoing well and was satisfied with his diet therapy. |
A 32-month-old Middle Eastern boy was born full term at a community hospital in Michigan with birth weight of 3135 g (15.0 percentile). He had normal prenatal ultrasounds. He passed meconium at birth and had no other complications including prolong neonatal jaundice or dehydration. His CF NBS showed serum IRT 139 ng/ml and was negative for the 40 gene mutations panel. At 1 month of age, he developed a wet cough without any other symptoms. He was followed by his primary care provider (PCP), and no treatment was given at the time. His symptoms continued on and off until 1 year of age. At 1 year, the mother noticed increased frequency of productive cough, lack of appetite, and poor weight gain. His weight-for-age percentile ranged from 0.3 to 5.0. His stools were reportedly normal. He had no excessive sweating. He was referred to an outside asthma/allergy specialist for evaluation of asthma. He was prescribed budesonide without any improvement. He had frequent pharyngitis and otitis media that were treated with oral antibiotics that reportedly helped treat acute infection, but the cough persisted. He was also prescribed a H2 blocker for possible gastroesophageal reflux disease, but no improvement in symptoms was noted. Family history was negative for CF.\nAt 30 months of age, he was seen by his PCP for one week of cough and fever. He was treated with amoxicillin. His symptoms continued to worsen despite oral antibiotics, and he had two episodes of small-volume hemoptysis. He was subsequently admitted for community-acquired pneumonia and influenza B. Chest X-ray showed diffuse ill-defined opacities in the perihilar area and diffuse bronchiectasis. During the hospitalization, pediatric pulmonary consult was obtained. Given the negative NBS, it was stated that CF was unlikely and no sweat chloride test was recommended. He had a normal videofluoroscopic swallow study. Immunodeficiency workup revealed elevated immunoglobulin levels, protective vaccine titers, and normal lymphocyte counts and response to phytohaemagglutinin, concanavalin A, and pokeweed mitogen. HIV test was negative. Pediatric gastroenterology was consulted for failure to thrive and recommended to continue high-calorie diet. He was discharged home on augmentin.\nTen days following discharge, he was seen at the immunology clinic. He was noted to have digital clubbing, worsening tachypnea, and crackles. With the concerning physical exam findings, a sweat chloride test was done with a result of 90 mmol/L (normal 0–29 mmol/L; intermediate 30–59 mmol/L; abnormal ≥60 mmol/L) []. He was referred to pediatric pulmonary clinic the same day. He was then admitted and treated for a CF exacerbation. Throat culture grew Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus (MSSA). Fecal elastase-1 was <50 mcg E/g stool (normal >200 mcg E/g stool). Lab results including comprehensive metabolic panel and vitamin A and E levels were normal. He completed two weeks of cefepime and tobramycin.\nAfter notifying MDHHS with the false-negative NBS results, the blood spot that was available at the NBS lab was retested using the new and expanded mutation panel (60 mutations). He was found to be homozygous for R1066C (c.3196C > T; p.Arg1066Cys) mutation. His care was transferred to our CF center, as per parents' request. Two weeks later, he was admitted for worsening respiratory symptoms and treated for a CF exacerbation. Vitamin D level was low at 25 ng/ml (normal ≥30 ng/ml). High-resolution computed tomography of the chest showed diffuse bilateral bronchiectasis (). Flexible bronchoscopy showed airway erythema and significant thick green secretions () that was positive for MSSA. |
A 79-year-old Asian man was admitted to our emergency department (ED) after accidental falling with syncope from 2nd floor to the ground at his house. On examination, he had an E3V3M5 Glasgow Coma Scale score with restlessness, a presenting blood pressure of 104/55 mmHg, heart rate of 35 beats/minute, and a respiratory rate of 24/minute on admission. He immediately received initial trauma resuscitation on the basis of the Advanced Trauma Life Support guidelines []. Initial electrocardiogram showed complete AV block in the ED (). Though he underwent administration of 0.5 mg of atropine and transcutaneous pacing immediately in primary care, his heart rate showed no improvement. Focused assessment with sonography for trauma and chest X-ray in the ED were interpreted as normal. Pelvic X-ray showed pelvic fractures in the sacrum, right pubic rami, and the ileum. Contrast-enhanced computed tomography (CT) scan of the chest, abdomen and pelvis showed hematoma on the right side of the pelvic cavity that compressed the bladder in the obturator internus, the iliacus and in the gluteus medius muscle of the fracture area and extrapelvic hematoma with contrast medium extravasation (). The dislocation of the right elbow was also determined. The injury severity score, which is based on the abbreviated injury scale (AIS) severity values and derives from the sum of the squares of the highest AIS values from each of the 3 most severely injured body regions, was 20.\nDespite administration of initial fluid resuscitation using Ringer’s lactate, his systolic blood pressure (SBP) deteriorated to 70 mmHg and heart rate of 30 beats/minutes. We were concerned about cardiogenic shock or hemorrhagic shock, and therefore decided to place a transvenous pacer for complete AV block and to perform transcatheter arterial embolization (TAE) for the pelvic injury with massive fluid resuscitation in primary trauma resuscitation. We initially transferred him to the angiography room and initiated IVR to place the transvenous pacer because we found no improvement of his heart rate in spite of administration of atropine and placement of a transcutaneous pacemaker in the ED. The transvenous pacer was successfully placed for 1 hour, and his SBP had recovered to 100 mmHg. After completion of placement of the transvenous pacer, his heart rate was controlled at 60 beats/minute. However, his hemodynamic status soon deteriorated to a SBP of 79 mmHg. We continuously initiated TAE for secondary deterioration of his hemodynamic status concerning of pelvic injury. Initial pelvic angiography showed multiple contrast medium extravasation from the branches of the right internal iliac artery (). On completion of TAE, using stainless coils and gelatin sponge particles for the pelvic fractures, angiography showed no findings of bleeding (). The patient required hemodynamic stability with a SBP of 150 mmHg and heart rate was controlled at 60 beat/minutes. After completion of TAE, he was admitted to our intensive care unit (ICU). At 12 hours after admission, he received a transfusion of 5 units of red blood cells and 10 units of fresh frozen plasma and his hemodynamic status remained stable. He was discharged ICU on hospital day 2, and his blood culture from admission to 24 hours after admission was detailed in . |
A 14-week-old female presented to the otolaryngologist's office for evaluation of hearing loss. She was born at 36 weeks gestation following a pregnancy complicated by type II diabetes requiring insulin. She failed the newborn hearing screen (ABR) prior to discharge from the hospital and failed the two-week follow-up screen in her pediatrician’s office. A natural sleep auditory brainstem response (ABR) evaluation was performed at 10 weeks of age, which showed bilateral severe to profound sensorineural hearing loss, absent distortion product otoacoustic emissions, and no evidence of auditory neuropathy spectrum disorder (ANSD). There was no family history of early onset or congenital hearing loss and no other risk factors. Her physical examination was unremarkable. Non-contrast MRI of the brain and internal auditory canals was significant for hypoplastic bilateral internal acoustic canals (IACs), a hypoplastic right cochlear nerve, and a left cochlear nerve that could not be visualized (Figure , ). A follow-up CT of the temporal bones confirmed narrow internal auditory canals bilaterally and normal course of bilateral facial nerves. She was cleared by pediatric ophthalmology, had normal EKG and renal ultrasound, and was found to have a normal microarray and GJB2 testing - a test for connexin 26 protein, a common genetic cause of nonsyndromic hereditary hearing loss - by pediatric genetics.\nAt four months of age, she was fit with binaural behind the ear hearing aids using average real-ear to coupler difference (RECD) values and fit to DSL targets. Post hearing aid fitting she was referred for auditory verbal therapy (AVT). Between four and six months of age, she developed intermittent right>left facial twitching independent of hearing aid use.\nAt seven months of age, she developed salmonella gastroenteritis, which was treated with intramuscular ceftriaxone. Her gastrointestinal illness improved, but within one week of developing fever and diarrhea, she was noted to have left facial droop and 4/6 left facial paralysis with healthy middle ears. A two-week prednisone taper was prescribed starting at 2 mg/kg, and she had near complete resolution of facial palsy within three weeks.\nShe had close audiologic follow-up and regular AVT. The hearing aid trial period indicated that the patient received little to no benefit from consistent use of bilateral hearing aids and that the use of traditional amplification was not providing the patient with adequate access to sounds across the frequency range. She met multidisciplinary cochlear implant team criteria for cochlear implantation (CI). A decision was made to implant the right ear first, due to the more prominent cochlear nerve on that side, and previous left facial palsy.\nAt 13 months of age (prior to right-sided cochlear implantation) she was found to have asymptomatic bilateral otitis media with effusion. She promptly underwent bilateral pressure equalizer (PE) tube placement with Paparella 1.02 mm tubes. Five days later, the patient underwent right-sided cochlear implantation (Cochlear Americas slim straight electrode CI522) with intraoperative facial nerve monitoring for three hours. The operation was performed uneventfully via a round window approach. Intraoperative impedances were acceptable on all 22 electrodes, and neural response telemetry (NRT) was completed on all 22 electrodes, with responses obtained on eight of 22 electrodes. The implant was activated three weeks postoperatively and CI mapping was performed over the following three months. CI mapping was completed using a combination of NRT responses as well as behavioral observation audiometry in the programming room (typically with two testers). Booth testing results were also incorporated into programming changes as the patient became more reliable and consistent. Data logging was consistent with > 6h of CI usage daily. Behavioral audiologic testing revealed that she reliably responded to speech at 25 dB and Ling 6 recorded stimuli were obtained between 20-25 dB. She continued with AVT, and demonstrated increasing accuracy of speech sound imitation.\nAt 18 months of age, the patient underwent left-sided CI (Cochlear Americas slim modiolar electrode CI532) with intraoperative facial nerve monitoring for three hours. The operation was performed uneventfully via a round window approach. Intraoperative impedances were acceptable, but NRI elicited facial nerve stimulation across the length of the electrode array from apical to basal. Her postoperative course was unremarkable. CI was activated three weeks later, and she was noted to have left facial stimulation at a level of 160CUs. A map was created based on NRT, and the patient was comfortable in live mode without facial stimulation. Follow-up booth testing revealed reliable responses to narrowband noise and Ling 6 sounds at 30 dB. Left-sided cochlear implant mapping challenges with this patient have revolved around providing as much access to sound as possible, while of course ensuring that facial stimulation does not occur. The most recent behavioral testing with the left CI in isolation yielded responses to Ling 6 sounds in the range of 20-30 dB.\nThe patient continues to tolerate bilateral CIs and demonstrates increased accuracy with identifying and imitating Ling 6 sounds and following basic one-step directions. She also has developed some simple words such as “hug” and “mama”. |
A 61-year-old male was presented to our clinic reporting episodes of headache and hypophyseal mass for nine months and right eyelid ptosis for four months. In January 2019, he started with an intermittent headache, blurred vision, weakness, and anorexia. He was then admitted to a local hospital for further examination. His serum sodium level was 127 mmol/L with normal blood pressure. The biochemical indexes indicated that he suffered from hypopituitarism. The symptoms of diabetes insipidus were not observed. Pituitary MRI showed a suspicious low signal in the right-wing of the pituitary. Further, he had a history of partial thyroidectomy ten years ago due to a thyroid nodule, with unknown postoperative pathological results. Based on the above evidence, an initial diagnosis of autoimmune hypophysitis was made. The patient then started the replacement therapy using 30 mg hydrocortisone per day and Euthyrox at 50 µg qd, which alleviated headache and improved the right eye acuity.\nFive months later, the patient developed sudden right eyelid ptosis. A new MRI disclosed that the pituitary mass had invaded the right cavernous sinus. Glucocorticoid impact therapy with an initial dose of 500 mg qd was initiated, which improved his right eyelid ptosis on the second day of therapy. Nevertheless, a relapse of ptosis occurred three months later and another MRI revealed a 1.3 × 0.9 cm sellar mass with extension to the right cavernous sinus and internal carotid artery. Immunology tests, including ANA, ANCA, serum IgG4, and ACL were reported negative. Lumbar puncture and thoracoabdominal CT scan did not show any remarkable findings. The diagnosis of autoimmune hypophysitis could not be excluded and the cortisol treatment was continued (prednisone 50 mg qd) in addition to 75 mg azathioprine per day.\nIn October 2019, he was referred to our clinic with several tests, including an electrolyte, serum and urine osmolality, sex hormone (FSH, LH, E, P, T, PRL), IGF-1, thyroid (TSH, FT3, FT4), immunology (IgG4, ANCA, sACE, ESR) and tumor markers tests (CEA, AFP, βhCG), which were all within the normal range. The MRI indicated a possible sellar macroadenoma (12.7 × 6.7 × 11.1 mm) with Knosp IV; the progression of the sellar lesion is shown in . Physical examination revealed truncal obesity and diabetes mellitus due to glucocorticoid treatment. His right eye showed ptosis with loss of pupil’s direct/indirect light reflex and ocular motility. FDG-PET/CT revealed a hypermetabolic lesion in the right sellar with infiltration to the right sphenoid sinus, suggesting invasive pituitary adenoma. The systemic disease was, therefore, excluded.\nThe patient underwent a sellar biopsy using an endonasal transsphenoidal approach and the mass on the right side of the sellar displayed a grayish-white hard tissue that looked similar to fish-meat with insufficient blood supply on tissue modality. Pathological analysis established the diagnosis of diffuse large B-cell lymphoma. Cell immunohistochemistry was positive for p53, Bcl-2, Bcl-6, C-MYC, CD20, and CD5, whereas the index for pituitary hormones and epithelial cells tested negative. Cell proliferation index Ki-67 was found to be 70%. Fluorescent in situ hybridization (FISH) of EBER ISH was negative, and no abnormality was found in CSF and bone marrow. The diagnosis of primary pituitary lymphoma was eventually established, and the patient received chemotherapy (rituximab 800 mg iv d1, MTX 7 g iv 4 h d2, lenalidomide 25 mg po d1–14) with 5 mg intrathecal dexamethasone and 50 mg cytarabine. After two courses of chemotherapy (R2-MTX), the patient achieved remission from prolonged headache and ptosis. MRI confirmed a significant reduction in the size of the sellar mass (). |
An 83-year-old Caucasian man (a national of the United States of America) with a nine year history of myasthenia gravis presented with complaints of difficulty swallowing. The patient complained of having trouble swallowing liquids and his pills. He felt as if there were painless secretions in the back of his throat inhibiting swallowing. He also had intermittent coughing and choking. He had had similar symptoms in the past with past MG relapses. There was no associated double vision, droopy eye lids, or extremity weakness, and he had no complaints of shortness of breath. He had been stable on a combination of Mestinon® (60 mg po q 4 hours) and Immuran® (50 mg po bid which he had been on for the last 8-9 years) and was intolerant to past prednisone. His last major myasthenia gravis relapse which required intubation was approximately 8 years ago and he responded well to plasmapharesis at that time. On this current admission there had been no preceding febrile illnesses and no recent vaccinations. His past medical history was significant for diabetes mellitus type 2, degenerative arthritis and cervical spinal stenosis, a past left nephrectomy secondary to prior staghorn calculi, previous lumbar spine surgery, previous cholecystectomy, previous bariatric surgery, and past peptic ulcers with a previous gastrointestinal bleed secondary to past steroid use. In addition to the above, his medication included insulin and lansoprazole.\nOn examination he was awake and alert with mild dysarthria but no nasal speech patterns. There were no dysconjugate gaze or nystagmoid patterns, nor was there any evidence of ptosis, facial asymmetry, or fatigability. No tongue atrophy, fasciculations, or furrowing was noted, and the patient was able to puff both cheeks adequately. There was no observed use of any accessory respiratory musculature. His motor strength revealed grade 5/5 on the MRC (Medical Research Council) scale in his bilateral upper extremities proximally and distally with a negative "pump handle" test. Lower extremity testing revealed 5/5 strength. Neck flexors and extensors were 5-/5. Reflexes were 1/4 throughout. No coordination abnormalities were identified.\nLaboratory investigations revealed a white blood cell count of 7.77 10 × 3/cumm (normal range 4.0-10.8 10 × 3/cumm) without a left shift. C - reactive protein was 0.33 mg/dL (normal range 0.00-0.80 mg/dL). Sodium level was 138 mEq/L (normal range 132-152 mEq/L), potassium was 4.6 mEq/L (normal range 3.4-5.4 mEq/L), blood urea nitrogen was 21 mg/dL (normal range 5-25 mg/dL), creatinine was 1.0 mg/dL (normal range 0.6-1.8 mg/dL), and blood glucose was 309 mg/dL (normal range 70-120 mg/dL). A creatinine kinase level was normal (83 mg/dL). Acetylcholine receptor antibody assay was elevated at 7.94 nM/L (normal less than .03 nM per liter) and smooth muscle antibodies were negative. Vital capacities were all normal (2.65/2.61/2.61 L) along with maximal inspiratory pressure trials (-40/-50/-50 cm H20). A computerized tomography scan of the neck was performed which did not reveal any abnormal mass in the hypopharynx or larynx.\nIt was thought that the patient was suffering from an MG exacerbation presenting as a predominant dysphagia, and his dose of Mestinon® was subsequently increased along with initiation of IvIg. It was not felt there was any cholinergic crises component secondary to lack of other symptoms commonly seen in this condition. He did not tolerate the IvIg secondary to hypertension so this was stopped. He was subsequently given plasmapharesis but his dysphagia continued.\nUnfortunately esophageal manometry was not performed. It was decided however to obtain a barium swallow X-ray which showed a prominent cricopharyngeus sphincter with incomplete relaxation and no evidence any cricopharyngeal bar. The patient was then scheduled for direct laryngoscopy followed by esophagoscopy with mechanical dilation of the cricopharyngeus sphincter under general anesthesia. He tolerated the procedure well and there were no complications.\nThe patients' swallowing markedly improved and he was discharged home on his same pre-admission medications without any change in dose. He remains stable on his current medication regimen without any required dose adjustments, and is followed as an outpatient. |
A 19-year-old male presented in the emergency department with one episode of melena per day, for one week. It was associated with vomiting, shortness of breath and palpitations. His hemoglobin level on initial complete blood count was 5.80 g/dL, signifying severe anemia according to WHO guidelines []. His lab parameters on admission are presented in Table .\nImmediately packed red blood cells (RBCs) were requested from the blood bank. On forward typing his blood group was labeled as O positive and his serum showed strongly positive indirect Coomb’s test with a negative direct Coomb’s. On extended 11 cell panel antibody testing, his serum demonstrated pan-agglutination which matched with monoclonal panel cells having anti-Kell, anti-Lub, and anti-Kpb antibodies. On cross match with four O negative and four O positive packed RBCs, +4 incompatibility was seen with all. Meanwhile a detailed history of the patient revealed two distinct episodes of epistaxis in childhood and a family history of his paternal grandmother having an increased bleeding tendency. In view of his past history of fresh frozen plasma infusions, it was interpreted that the patient may have multiple alloantibodies in blood leading to gross incompatibility. Considering the urgency of the situation, one unit of the least incompatible (O negative) packed RBCs was issued after washing with normal saline thrice, to the emergency department. Transfusion was started under strict monitoring by the emergency department physicians. After slow transfusion of around 10 ml blood, the patient started shivering and his temperature spiked to 101°F with tachycardia and hypotension. The transfusion was stopped immediately and the patient was given intravenous antihistamine and hydrocortisone. Meanwhile, he was transferred to the intensive care unit (ICU) where he received intranasal desmopressin and intravenous factor VIII.\nTransfusion reaction workup revealed a grade 4+ pan agglutination in his serum. During repeat blood grouping, forward typing did not demonstrate any reaction to anti-A and anti-B antisera, like a normal O blood group. However, on reverse typing, his serum showed strong agglutination with group O pooled control cells. His post saline wash incompatibility with O negative red cell concentrate showed minor difference from grade +4 agglutination (pre-wash) to grade +3 clumping (post-wash). A fresh RBCs sample from the patient showed negative direct Coomb’s test, while fresh serum sample remained positive for indirect Coomb’s test. This workup strongly raised the suspicion of Bombay phenotype and his red cells were tested with anti-H lectin, which showed no agglutination. This confirmed his blood group as Bombay phenotype. The reactions observed with Bombay phenotype compared to other blood groups, on forward and reverse typing, are illustrated in Table .\nImmediately, voluntary donor pools were contacted in blood banks throughout the country. Overnight, a donor with Bombay negative blood group was arranged from Karachi. The packed RBCs were airlifted to Islamabad maintaining the cold chain. After crossmatching with recipient’s blood showed no reaction, the donor blood was transfused to the patient. Meanwhile, a distant relative of the patient from a nearby city, with Bombay positive blood group, consented to donate blood at our blood bank. Two days later, another unit of packed RBCs was transfused to the patient. His hemoglobin after two transfusions rose up to 7.40 g/dL. As his melena settled down on supportive therapy, an endoscopy was performed that suggested an underlying hiatal hernia. After surgical consultation, the patient was advised to reduce weight and discharged from the hospital, with a scheduled follow-up visit.\nIn view of the patient’s past medical history and family history, during the follow-up visit, a von Willebrand factor antigen, von Willebrand factor functional activity and factor VIII levels were ordered. His von Willebrand factor antigen level was <2.0%, von Willebrand factor functional activity was <4.0% and factor VIII level was 18.5%, consistent with type 3 von Willebrand disease. The patient and his family were counselled accordingly and referred to the hematology clinic. |
An 18-year-old female patient presented with pain and discolored maxillary central incisors. The patient gave the history of trauma about 10 years back. Medical history was noncontributory. Thermal and electric pulp tests were performed to determine the vitality of all the anterior teeth. Both the maxillary central incisors were found to be nonvital showing no response to thermal and electric pulp tests. Maxillary lateral incisors showed a positive response to thermal and electric pulp tests. Intraoral periapical radiograph of the involved teeth was taken which demonstrated a periapical lesion with bone loss [] and hence conventional root canal therapy was planned. Following isolation with rubber dam, access cavities were prepared on the maxillary central incisors and the working length determined. Canals were cleaned and shaped using K-files by a conventional method. Warm 3% sodium hypochlorite solution was used as the intracanal irrigant which was ultrasonically activated. The files were liberally coated with EDTA gel throughout instrumentation. The canals were enlarged by circumferential filling. Triple antibiotic paste was placed in the canal as intracanal medicament in each tooth and access cavities were sealed with cavit. The patient was recalled after 3 weeks. In the next visit, the canals were cleaned with warm 3% sodium hypochlorite solution and final rinses of 17% EDTA were given followed by normal saline. The canals were dried using paper points, and the apical third of the canals were obturated with MTA. A moist cotton pellet was placed over it, and the access cavities sealed with cavit []. Next day, cavit was removed and the remaining canals were obturated with thermoplastic Gutta-percha []. Access cavities were sealed with composite resin.\nThe patient was recalled after 6 months [] and 1 year intervals []. The clinical and radiographic examination demonstrated that the patient was asymptomatic, and the teeth exhibited proper integrity of the periodontal tissues after 1 year. |
A 33-year-old lady presented to the clinic for bilateral retro-orbital headache with tinnitus most severe upon waking up in the morning for 3 months, without evidence of nausea, vomiting, or insomnia. Vital signs were within the normal range. The neurologic exam was non-focal. Enhanced brain magnetic resonance imaging (MRI) revealed a T1 hypointense, T2 hyperintense, homogenously enhancing Pineal tumor of 2 × 1.89 × 1.61 cm (anteroposterior × transverse × height) compressing the posterior wall of the third ventricle with evidence of moderate hydrocephalus (). The patient was scheduled for craniotomy for resection of the mass. In the operating room, an external ventricular drain was inserted in the right occipital horn followed by craniotomy and resection of the mass through a right occipital transtentorial approach. Gross total surgical resection was achieved under microscopic navigation (). The patient was extubated. Upon extubation the patient started to have chewing-like movement of her lips on the ET tube, afterward, she was transferred to the intensive care unit for monitoring. On postoperative day 1, we noticed that the patient was still having chewing-like movements typical of orofacial dyskinesia with evidence of buccal mucosal injury and swelling of the lips (). She was also complaining of inability to sleep. The patient was on regular postoperative medications (esomeprazole, cefazolin, paracetamol, morphine, dexamethasone, levetiracetam). An enhanced postoperative brain MRI was done for evaluation and confirmed complete resection of the lesion without any bleeding or ischemic changes (). The chewing like movements lasted for 72 hours and spontaneously resolved afterward. Pathology confirmed pineal parenchymal tumor of intermediate differentiation (WHO Grade III). The patient was treated with adjuvant radiotherapy. This is an unreported complication post pineal gland tumor resection in humans. Below we review the literature reporting abnormal facial movements associated with various pineal gland conditions as the data remains scarce concerning dyskinesia post pineal tumors resection in humans. |
A 34-year-old female of Asian origin presented with dull aching pain over the right hypochondrium, radiating to the back for 6 months with no history of fever, jaundice, hematemesis or malena. She had undergone an open cholecystectomy about 5 years back with an uneventful post operative period and was discharged on the tenth day following surgery. Rest of the history was unremarkable. On examination, a tender, firm intra abdominal lump of size 7 × 5 cm was felt in the right hypochondrium. The lump was extending to the lumbar region and was moving well with respiration. It was dull on percussion and continuous with liver dullness. Laboratory parameters were within normal limits except for an elevated serum alkaline phosphatase of 420 U/L (Normal 80- 240). Ultrasonography (US) of the abdomen showed a cystic lesion involving the segment V of the liver with a huge extra hepatic component along with small septations and internal debris. Contrast enhanced computed tomography (CECT) of the abdomen (Figure ) showed a large well defined enhancing smooth walled cystic lesion in the right lobe of the liver adjacent to the porta hepatis. As the lesion was in close proximity to the first part of the duodenum a possibility of a duodenal duplication cyst was considered and an upper gastrointestinal endoscopy (UGIE) was performed. UGIE showed a deformed stomach with an extrinsic compression at the junction of first and second part of duodenum. Hydatid serology and 99 mTc Pertecnate Meckel's Scan for ectopic gastric mucosa were negative.\nDuring the period of investigation, pain and the lump increased in size. The patient was explored through a right subcostal incision under general anesthesia. A large cystic mass of size 10 cm × 10 cm was found in relation to the undersurface of liver. The mass was adherent to the colon and duodenum and was overlying the region of porta hepatis. Aspiration of the cyst revealed bilious fluid. The cyst was excised leaving a cuff of cyst wall, which was densely adherent to the duodenum. Frozen section of the cyst wall showed only fibrocollagenous tissue. After excision of the cyst porta hepatis as exposed and an induration was noted in the region of the confluence of hepatic ducts with a small opening through which bile was trickling. The opening was cannulated and the cholangiogram was done to delineate the anatomy. Since biliary anatomy was not clear on intra operative cholangiography, opening was closed Choledochotomy done and a T tube placed in the bile duct. Abdomen was closed after placing a sub hepatic drain. In the post operative period, the drain output was bilious in nature. The drain output gradually decreased and finally stopped. A T-tube cholangiogram done on day 14 after surgery showed a normal bilio-enteric pathway with no leakage (Figure ). The drain, the T-tube was then removed and the patient was discharged. At 1 year of follow-up is asymptomatic with normal liver function tests. |
A 68-year-old man was referred to our institution with a diagnosis of rectosigmoid colon cancer. Colonoscopy revealed a type 2 tumor at the rectosigmoid colon (Fig. a). Right hemiparesis was present as a result of two cerebral infarctions that had occurred during the 2 months prior to his first admission. After consultation with a neurologist, we considered that it was necessary to wait several months before performing a major operation due to the risk of inducing another cerebral infarction. However, advanced cancer would be expected to grow during the waiting period. We therefore considered that it was necessary to administer systemic chemotherapy to suppress tumor growth. We initially administered five courses of preoperative chemotherapy (mFOLFOX6). No cerebral events or severe adverse events occurred during the chemotherapy. Stable disease, according to the RESIST criteria, was achieved with the preoperative chemotherapy. A laparoscopic Hartmann’s operation with D3 lymph node dissection was performed at 4 weeks after the last course of chemotherapy. The patient’s postoperative course was good without any complications. The final diagnosis of the rectal cancer was Dukes B with well-differentiated tubular adenocarcinoma and rectal tumor invasion to the subserosa (T3) without lymphatic or venous invasion (Fig. b). The resected specimen was free from cancer cells. The patient had complained of a perianal abscess for 5 years before his first admission. However, we gave priority to treating the rectal cancer. Although his anal symptoms did not worsen during chemotherapy, at 1 month after the Hartmann’s operation for rectal cancer, his perianal abscess worsened and induration with two secondary open lesions was detected on physical examination (Fig. a). The pathological examination of the biopsied perianal tissue revealed adenocarcinoma. Because the perianal tumor was localized without invasion of the neighboring tissue on magnetic resonance imaging findings (Fig. b), and considering his physical states, we performed local excision under spinal anesthesia for curative resection. The final pathological diagnosis of the resected specimen was metastatic adenocarcinoma to an anal fistula originating from rectal cancer, and the resected margin was free from cancer cells (Fig. a–c). Immunohistochemistry revealed that both the rectal tumor and anal tumor were cytokeratin 7 (CK7) − and cytokeratin 20 (CK20) + (Fig. ). Based on the pathology of the primary tumor and the absence of lymphatic or venous invasion, metastasis was considered to have occurred due to the implantation of exfoliated cancer cells. Seven courses of postoperative adjuvant chemotherapy with the same regimen have been administered. No recurrence of the tumor has been seen for 31 months after the operation. If a locally recurrent tumor had been found near the rectum and anus without distant metastasis during follow-up, laparoscopic resection of the tumor with the residual rectum and anus would have been attempted after considering the patient’s performance status. |
A 30-year-old obese woman with no medical history presented to our emergency department (ED) complaining of 3 h of right flank pain radiating to her groin. On arrival, the patient was hemodynamically stable but in acute distress, writhing in pain from presumed renal colic. Three hours after arrival to the ED, the patient was noted to be lethargic with systolic blood pressure (SBP) in the 60 s. She was transferred to the trauma bay and administered 2 L of normal saline with SBP up to the 100 s. Emergent computed tomography of her abdomen/pelvis revealed a ruptured 9.2 cm suprarenal AAA with active extravasation. The alpha TTA, the highest level of activation at our institution, was initiated by the emergency medicine attending which mobilized the operating room (OR) supervisor to halt scheduled cases to hold one OR open, the blood bank to activate the massive transfusion protocol (MTP), and the in-house acute care surgeon and anesthesiologist to the trauma bay. The attending surgeon coordinated resuscitation efforts with the MTP as the OR staff set up the room. Within 38 min of the alpha activation, the patient was in the OR with the on-call vascular surgeon for open repair of her ruptured AAA.\nEmergent open AAA repair was performed with the left renal and inferior mesenteric artery sacrificed. Her abdomen was packed and an ABThera™ (KCI, San Antonio, TX, USA) open abdomen negative pressure device was placed with plans for a second look operation as the patient was severely acidotic, coagulopathic, and hypothermic. An hour after transfer to the surgical Intensive Care Unit, the patient was noted with bright red blood per rectum with associated hypotension. Bedside colonoscopy revealed ischemic pancolitis. The patient returned to the OR with the on-call acute care and vascular surgeons for exploration, subtotal colectomy, and ABThera™ replacement. Her remaining hospital course was complicated by acute kidney injury which resolved without the need for hemodialysis. The patient underwent ileostomy creation with abdominal closure on postoperative day 5 and was discharged home on postoperative day 22. Subsequent history revealed that although the patient did not manifest typical Marfan phenotype, she carried an unknown diagnosis of Marfan syndrome with only aortic manifestations of the disease. |
A 32-year-old woman, gravida 1, para 1, who had a caesarean delivery 6 years ago, was admitted to our hospital with an increasing serum ß-hCG level after conservative laparoscopic surgery for ectopic pregnancy. At first, she was referred to the provincial hospital for evaluation of spotting and mild abdominal pain at 6 weeks of gestation. Her serum ß-hCG level was 7000 IU/l, and transvaginal sonography showed an empty uterine cavity. She was diagnosed with ectopic pregnancy and underwent a diagnostic laparoscopy. At laparoscopy, 2 ovaries appeared normal and the infundibulum of left tube had light bleeding. A small amount of free fluid was seen in the pelvis. No trophoblastic tissue was found in the abdominal cavity. The patient was discharged from the provincial hospital on postoperative day 3. At a scheduled visit 1 week later, her serum ß-hCG level increased to 12000 IU/l. Soon after, she was transferred to our hospital.\nOn the day of admission, a physical examination revealed some vaginal discharge, but no vaginal bleeding. The abdominal examination was unremarkable. Her serum ß-hCG level rose to 16088 IU/l on postopereative day 12. Transvaginal Doppler ultrasound showed a 19 × 17 mm mass that contained a yolk sac and located in the right of rectum (Fig. ). A heterogeneous hypoechoic mass measuring 26 × 17 mm was close to this mass (Fig. ). No gestational sac was visualized in the uterine cavity. Abdominal Computed tomography (CT) angiography was then performed to determine the location of her adnexal mass. It demonstrated a right adnexal mass measuring 20 × 20 mm adherent to the rectal wall (Fig. ). No thickening of the rectal wall was seen.\nOn postoperative day 14, another laparoscopic surgery was performed. No blood was detected in the peritoneal cavity. Both ovaries and Fallopian tubes looked normal. A 3 × 3 cm gestational sac coated with blood clots was found behind the uterus in the right pouch of Douglas. The placenta tissue was identified to implant on the anterior wall of the rectum. Removal of the placenta revealed a 1 cm size defect in the rectal wall. A gastrointestinal surgeon was involved to investigate and suture the rectal mucosa. Hemostasis was achieved with electrocautery and surgicel. Two days after the surgery, her serum ß-hCG level decreased from 12000 IU/l to 3700 IU/l.\nHistologic examination of the surgical specimens confirmed the diagnosis of a rectal ectopic pregnancy with direct invasion of trophoblastic tissue into the rectal wall. The patient made an uneventful recovery and was discharged from hospital on postoperative day 20. At a scheduled visit 2 months later, her serum ß-hCG level decreased to less than 5 IU/l. |
A 67-year-old male chronic smoker with medical history significant for chronic obstructive pulmonary disease was admitted for imaging guided biopsy of a 1.2 cm left lower lobe lung nodule found recently on a computed tomography (CT) scan of the chest. The nodule was highly suspicious for primary lung malignancy. The patient was placed in a prone position and lung parenchyma in the posterior lateral left chest was visualized. Under CT guidance, a 19-gauge guide was advanced into the left lower lobe and two separate 20-gauge core biopsy specimens were obtained from the mass. There was no hemorrhage or immediate post procedure complication. However, towards the end of the procedure, the patient started complaining of sudden onset of chest pain and became unconscious. No palpable pulses were identified. A code blue was called and cardiopulmonary resuscitation was begun according to Advanced Cardiovascular Life Support guidelines. The patient subsequently demonstrated ventricular fibrillation which responded to defibrillation shock and epinephrine. The patient had three cycles of chest compressions, one dose of epinephrine, and a shock of 200 J. He was successfully resuscitated and intubated for mechanical ventilation. A CT scan of the chest was obtained immediately after the resuscitation and it demonstrated development of a small anechoic area in the left cardiac ventricle consistent with air embolus (Figure ).\nThe vital signs recorded at the time were as follows: blood pressure of 130/80 mmHg, pulse rate of 90 beats per minute, respiratory rate of 18 breaths per minute, and normal oxygen saturation of 92% on room air. The patient was admitted to the intensive care unit. Bedside transthoracic echocardiogram (TTE) did not reveal evidence of an air embolus. Additionally, no cardiac wall motion abnormalities were noted. The patient remained hemodynamically stable for 24 hours, and he was successfully weaned off from the mechanical ventilator next day and discharged home in a stable condition. |
A 32-year-old man was admitted to our hospital with appetite loss. He had a history of traumatic transverse cervical spinal cord injury at the C5 level due to suicide attempt at the age of 18. As a result of cervical spinal cord injury, he was paralyzed in the lower body. Contrast-enhanced computed tomography (CT) revealed a late-onset traumatic diaphragmatic hernia with strangulated ileum (Fig. ). The small intestine, transverse colon, and omentum were displaced into the left thoracic cavity, and some portions of these organs showed a decrease in blood flow. Left lung collapse and a compressed right lung with mediastinal shift were evident. The patient underwent emergency surgery. After replacing the incarcerated organs to their original positions, scattered areas of necrosis were identified in the small intestine, transverse colon, and omentum (Fig. ). By using interrupted sutures with non-absorbable 1–0 monofilament, the diaphragmatic orifice was closed. Wedge resection with primary closure was performed for the colonic necrosis in two places. Partial resection, 45 cm long, with end-to-end anastomosis was performed for the small intestine. The necrotic omentum was removed (Fig. ). In addition, a gastrostomy tube was placed since delayed initiation of oral intake was expected. The patient developed severe septic shock postoperatively. Treatment-resistant critical hypotension with non-compensatory tachycardia developed, likely due to parasympathetic nervous system damage related to the cervical spinal cord injury. On postoperative days (POD) 3 and 6, cardiac arrest occurred. Fortunately, he was rescued by cardiopulmonary resuscitation with administration of large doses of vasopressin and catecholamine. However, peripheral vasoconstriction, increased intra-abdominal pressure, and ischemia of the gastrointestinal tract developed, which resulted in colonic anastomotic leakage with diffuse peritonitis, abdominal wound dehiscence, and collapse of gastrostomy on POD 6 (Fig. ). The patient was unable to undergo surgical repair because of his poor general condition with continuing severe septic and neurogenic shock. Therefore, he underwent AVS through the open abdominal wound and it was the first procedure at the intensive care unit. The procedure of AVS was as follows: 1. the open wound and peritoneal cavity were rinsed with normal saline and necrotic and/or contaminated tissues were debrided (Fig. ); 2. wound dressing materials (DUOACTIVE® ConvaTec, New Jersey, USA) for protecting healthy skin around the open wound were patched along the abdominal wound in piecemeal fashion so as to adjust dressing materials to the complicated shape of the wound (Fig. ); 3. two drainage tubes with multiple side holes, up to 30 cm from the tip, were placed in the abdominal cavity through the open abdomen and the enteric contents were suctioned through the drainage tubes using a Continuous Suction Unit MERA Sacuum (Senko Medical Instrument Manufacturing CO, Tokyo, Japan) set to 50–75 mmHg continuous negative pressure; and 4) the entire wound was filled with saline-moistened gauzes and covered with polyurethane drape (Fig. ). The colonic anastomotic leakage showed gradual healing over the course of 2 months, followed by contraction and closure of wound dehiscence (Fig. ). Because the gastric fistula remained, a gastrostomy balloon catheter was placed through the gastric fistula. The patient resumed oral intake on POD 112 and left the hospital on POD 190 with the gastrostomy balloon catheter and without incisional hernia. |
A 57-year-old male was admitted to the Pulmonary Oncology Department of University Hospital Centre Zagreb in November 2017 with cough and hemoptysis. He smoked 27 packs/year, had an alcohol abuse history, and displayed no evidence of present or past soft tissue neoplasms or history of radiation exposure. His Karnofsky performance score was 80. Subsequent physical examination revealed reduced air entry in the left lung. His complete blood count and biochemical parameters were within the normal range. Initial chest x-ray showed an extensive consolidation in the left lung suggesting possible malignancy; CT of chest and abdomen was recommended. The CT scan showed an extensive partially necrotic tumor in the left upper lobe (LUL) extending from the left hilum with infiltration of the LUL bronchus to the visceral pleura, measuring 74 mm in the largest diameter (Fig. ).\nThere was no evidence of nodal or visceral dissemination at presentation. Bronchoscopy examination with biopsy was performed, and specimen from the LUL bronchus was taken. Histopathological analysis showed an almost completely necrotic tumor with a share of less than 10% viable pleomorphic tumor cells expressing vimentin and CD99 and focal positive desmin. There was no expression of AE1/AE3, p40, TTF-1, napsin A, epithelial membrane antigen (EMA), CK7, and S100. The most likely diagnosis was poorly differentiated sarcoma.\nFor final staging, PET/CT scan was performed, showing no extra thoracic spread of the disease. The multidisciplinary tumor board deemed that lung surgery was the best treatment alternative, and several weeks later, left pneumonectomy with mediastinal lymph node dissection was done. Final pathohistological analysis noted a tumor size of 100 × 85 × 71 mm, more than 80% of which was necrotic. The pleomorphic tumor cells showed the same immunohistochemical profile as in biopsy specimen. Synovial sarcoma-associated translocation was negative. All surgical margins and dissected mediastinal lymph nodes were tumor free. Based on this, the final diagnosis of undifferentiated pleomorphic sarcoma (grade 3) was made (Fig. ).\nThe patient recovered well from the surgery, and follow-up was planned. At the first follow-up visit 7 weeks after lung surgery, the patient was free of malignant disease as per physical examination and chest x-ray. Three months after the lung surgery, a follow-up CT scan was done which showed a 60-mm metastatic intraabdominal lesion with an evident small bowel involvement, as well as further separate malignant peritoneal deposits. The scan also showed mild upstream dilatation of the small bowel suggestive of partial obstruction (Fig. ).\nTherefore, an abdominal surgery was planned. However, just a couple of days later, the patient presented to the Emergency Department with clinical signs of acute abdomen due to bowel perforation. Urgent abdominal surgery was performed with resection of perforated segment of the ileum, which was infiltrated with tumor. A unipolar ileostomy was then fashioned. The histopathological analysis confirmed metastasis of undifferentiated sarcoma (Fig. ).\nThe patient was discharged from the hospital 9 days later with the Karnofsky performance score of 40; thus, the best supportive care was indicated. The patient died at home 2 months later. |
A 78-year-old woman presented to her general dentist with a chief complaint of an enlarging soft tissue mass of several months duration. The patient stated to be otherwise healthy, with no history of malignancy. She was not in acute distress on presentation.\nIntraoral examination revealed a fluctuant, exophytic lesion of the maxillary anterior gingiva extending from the right lateral incisor to the left central incisor (teeth #12, 11, 21, F. D. I. System). The lesion measured 3.0 cm × 1.5 cm in greatest dimension and appeared dark-red color with secondary tan-gray ulceration []. The dentist described the involved tissue as edematous and hyperemic and stated that on incisional biopsy the tissue partially collapsed under pressure from the forceps. A smaller, similar appearing lesion was identified in the right maxillary vestibule adjacent to the labial frenum []. A periapical radiograph of the area showed no changes in the quality or quantity of bone and no evidence of tooth-related infections []. Based on the appearance of the lesion, a clinical diagnosis of pyogenic granuloma was made before the biopsy.\nHistologic examination revealed soft tissue covered by stratified squamous epithelium. The epithelium appeared focally ulcerated but was otherwise unremarkable. Beneath the epithelium, tumor cells were found to completely efface the lamina propria []. These cells were predominately arranged in lobular aggregates separated by thin fibrous septae []. Some of the aggregates had a perivascular pattern, and the lesion itself had a rich vascular network. On high-power magnification, the cells displayed pink to vacuolated cytoplasm with vesicular nuclei and prominent nucleoli. Significant nuclear pleomorphism was present and the lesion demonstrated brisk mitotic activity []. Based on these findings, the lesion was initially diagnosed as a carcinoma of unknown primary origin.\nA wide panel of immunohistochemical markers was subsequently used to further classify the cells of origin. The tumor cells were strongly positive for pancytokeratin, CK8/18, Pax-8, CD10, CA9, CK19 and vimentin [Figure –] and were focally positive for EMA. The cells were negative for CK20, CK7, p63, p40, CK5, synaptophysin, c-kit, GATA3, TTF-1, S100, CDX-2, calponin, calcitonin, EBER, HMB45, PR, ER and CD31. These findings were consistent with a primary malignancy of renal origin.[]\nThe patient was subsequently referred to her primary care physician and an oncologist for full body imaging. A whole body bone scan was performed which showed uptake in the right kidney consistent with neoplasm. In addition, focal increased uptake consistent with possible metastatic deposits was seen in the right femoral head and greater trochanter. |
A 66-year-old African American female with a past medical history of refractory immunoglobulin G (IgG) lambda MM, essential hypertension, and chronic kidney disease presented to the emergency department with five days of right upper quadrant pain.\nHer MM was diagnosed one year prior when she presented with altered mental status, uremia, hypercalcemia, hypoalbuminemia, and paraproteinemia. A skeletal survey at that time revealed multiple thoracic spinal lytic lesions and an eroding soft tissue mass at the level of T10. Further evaluation revealed a very high IgG level, elevated M protein band, and a kappa/lambda ratio <0.01 (normal 0.26-1.65). A biopsy from the soft tissue mass revealed a plasmacytoma. Radiation therapy was initiated for 10 days. She received three cycles of bortezomib and dexamethasone followed by two cycles of bortezomib, dexamethasone, and lenalidomide. Her disease progressed, and a subsequent bone marrow biopsy revealed hypercellular bone marrow with 70% atypical plasma cells. The patient subsequently received seven cycles of carfilzomib, lenalidomide, and dexamethasone. She was not a candidate for bone marrow transplantation given the high plasma cell burden.\nOn her current presentation, the pain was sudden in onset, intermittent, worse with eating, and without radiation. The pain was associated with nausea and anorexia, but she was without any change in bowel habits. She denied any previous similar episodes. Upon physical exam, the patient was in distress but remained alert and oriented. Her vital signs were all stable. She exhibited right upper quadrant abdominal tenderness without rebound or guarding. Her initial labs are presented in Table . The patient was admitted to the hospital for further evaluation of her abnormal labs and supportive treatment.\nThe patient was started on intravenous hydration and was made nil per os. An abdominal ultrasound revealed a distended GB with sludge (Figure ). The GB wall was thickened up to 9.5 mm, and the sonographic Murphy sign was positive. The common bile duct and common hepatic duct measured 6.3 mm and 3 mm, respectively. The liver measured 18.3 cm and exhibited normal echogenicity. There were no intraparenchymal masses or fluid collections. The portal and hepatic veins were patent.\nThe patient was diagnosed with acute cholecystitis. Intravenous piperacillin-tazobactam 3.37 g every eight hours was initiated, and the patient was referred for open cholecystectomy given her overall condition and lactic acidosis. Intra-operatively, the GB was thickened and firm but not overly distended or perforated. The GB was dissected from the liver edge, and a liver biopsy was performed successfully.\nThe pathology report from the cholecystectomy revealed chronic cholecystitis with involvement of the GB submucosa and serosa by abnormal plasma cells with lambda light chain restriction (Figure ). Subsequently, Congo red stain of the GB sections revealed apple-green birefringence throughout the submucosal areas consistent with amyloid deposits. The liver biopsy exhibited abnormal plasma cells in periportal locations with lambda light chain restriction as well; Congo red stain was not done on the liver sample.\nThe patient declined any further treatment for MM and decided to proceed with hospice care. She was discharged home with comfort measures. |
A 33-year-old male with human immunodeficiency virus (HIV), not on highly active antiretroviral therapy (HAART) for the last two years, presented to an outside emergency department with a three-week history of subjective fever, arthralgias, myalgias, and a diffuse painful papular upper body rash. The rash had progressed from his trunk to involve his face, arms, and upper legs. He reported painless genital lesions preceding the diffuse rash, as well as two unprotected sexual encounters approximately one month prior. The patient took no home medications and had no known medication allergies. In the emergency department, he was found to have a temperature of 100.9°F, a heart rate of 130 beats per minute, and a respiratory rate of 22 breaths per minute. Urinalysis was not suggestive of infection, and chest radiograph demonstrated no infiltrate. Blood and urine cultures were obtained. He was given 1 L bolus of intravenous fluids and 1 g of ceftriaxone intravenously for presumed sepsis. He was subsequently transferred to our institution for further workup and management.\nUpon arrival to our emergency department six hours later, the patient was febrile to 102.8°F with a heart rate of 161 beats per minute and systolic blood pressure initially 89 mmHg. Physical exam was remarkable for normal mentation, tachycardia but no murmurs, and clear lung fields bilaterally. There were erythematous scaly papular and plaque-like lesions over the face, back, chest, arms, abdomen, and upper legs (Figures , ). The rash spared the palms and soles. He also had an erythematous papule on the mucosa of the lower lip. The patient remained hypotensive, with a blood pressure of 82/53 mmHg, despite 3 L of normal saline. A central venous catheter was placed, and a norepinephrine drip was started. The patient also received empiric antibiotic treatment with vancomycin and cefepime for presumed septic shock. The patient was admitted to the medical intensive care unit, where he required less than 24 hours of vasopressor administration with norepinephrine to maintain adequate blood pressure.\nBlood and urine cultures obtained at the outside hospital prior to the first antibiotic dose were negative for bacterial growth. Broad-spectrum antibiotics were discontinued once cultures showed no growth for over 48 hours and the patient remained clinically stable. Blood cultures, fungal cultures, acid-fast bacilli smear and cultures, cerebrospinal fluid (CSF) cultures, cryptococcal serum and CSF antigens, Histoplasma galactomannan urine antigen, Neisseria gonorrhea polymerase chain reaction (PCR), and Chlamydia trachomatis PCR obtained at our facility were all negative. CSF studies demonstrated no red blood cells, no white blood cells, glucose 53 mg/dL (reference range 40-70 mg/dL), and protein 49.5 mg/dL (reference range 10-45 mg/dL), which was not thought to be consistent with aseptic meningitis. Rapid plasma reagin (RPR) was positive, and syphilis serum titer was 1:256 dL. CSF RPR was negative, and CSF Treponema pallidum IgG by immunofluorescence assay was nonreactive. The patient was also noted to have a CD4 T-cell count of 162/mm3 (reference range 338 to 1448 per mm3) and HIV-1 ribonucleic acid (RNA) of 456,000 copies/mL. Dermatology was consulted and deferred skin biopsy as exam findings were clinically consistent with secondary syphilis. The patient was treated with 2.4 million units of intramuscular benzathine penicillin. The remainder of his hospital course was uncomplicated, and he was discharged home after three days with close follow-up with Infectious Diseases with plans to resume HAART as an outpatient. |
A 2-year-old healthy boy underwent an office-based surgical circumcision by his physician. During the procedure, profound bleeding was observed that was not controlled by applying direct pressure. The physician attempted to control the bleeding by multiple deep suturing; the bleeding was stopped successfully and the patient was discharged home. During the postoperative period, the child had progressive painful and difficult voiding with only few drops of urine after straining. During this period the child was prescribed painkillers for his discomfort and no additional evaluation was done. After about a week he was referred to a district hospital with fever (temperature=38.1C), agitation, vomiting, urinary retention, and significant abdominal distension. Abdominal examination showed decreased bowel sounds, dull percussion, and severe guarding. Urgent abdominopelvic ultrasound revealed large volume of free fluid in the abdomen and pelvis, with small amount of urine in the bladder. The initial lab tests also showed leukocytosis with left shift and increased blood creatinine and blood urea nitrogen. Catheterization with a 6-Fr feeding tube failed because it did not pass beyond the subcoronal urethra. The patient underwent emergency midline laparotomy with the diagnosis of acute abdomen and the fluid was drained. A small intraperitoneal bladder rupture was noticed at the dome of bladder. The gastrointestinal tract was inspected precisely and was intact. The diagnosis of intraperitoneal bladder rupture with urinary ascites was made probably due to near-total urethral obstruction. The rupture site was repaired in two layers and a suprapubic cystostomy catheter was fixed.\nTwo months later, the patient was referred to our center for further evaluation and treatment. Antegrade voiding cystourethrography (VCUG) was performed via the suprapubic catheter that showed terminal urethral obstruction (). Urethroscopy was attempted under general anesthesia that failed due to complete obstruction at 1 cm from the meatus.\nDecision was made to explore the area and to repair the urethra. Through a circumferential incision, distal urethra was elevated from the corpus spongiosum. A 3-Fr ureteric catheter also did not pass the obstructed part (). A 5-mm fibrotic tissue was encountered at the site of obstruction (). The corpus spongiosum was dissected free from the corpora cavernosa to prevent iatrogenic chordee after end-to-end urethral anastomosis. The obstructed fibrotic part of urethra was completely resected and an end-to-end urethral anastomosis was performed along with spongioplasty over an 8-Fr silicon catheter in two layers; dartos pedicled flap was used to cover the site of anastomosis. The postoperative period was uneventful and the patient was discharged home with suprapubic and urethral catheter. The urethral catheter was removed seven days following the surgery. The suprapubic catheter was removed four weeks after the surgery following normal urethral voiding and normal ultrasound. A VCUG was performed six weeks after the surgery that showed normal bladder and urethra with acceptable voiding per urethra (). During a 2.5-year follow-up period, the patient was asymptomatic with normal renal function, ultrasound, and voiding pattern. He had a maximum flow rate of 15.3 ml/sec in uroflowmetry study. |
A 28-year-old female patient presented to our clinic for progressive decrease in vision, first the right eye and later the left eye, for the past year. Her medical history was unremarkable in terms of systemic disease. She reported going to another center one year earlier for reduced vision where she was diagnosed with VKH and treated 3 times with high-dose corticosteroid and finally with an injection in her right eye. At time of presentation, the patient was using oral methylprednisolone 16 mg/day, oral cyclosporine 150 mg/day, and oral azathioprine 75 mg/day. Physically, she exhibited cushingoid appearance and complained of excessive weight gain and hair growth on the body. On examination, her visual acuity was light perception with projection in the right eye and 0.6 in the left eye. Anterior segment examination of both eyes was normal except for white accumulations consistent with corticosteroid in the inferior subconjunctival region of the right eye. Intraocular pressure measurement was 13 mmHg in the right and 15 mmHg in the left eye with dorzolamide hydrochloride-timolol maleate twice daily. Fundoscopic examination revealed no cells in the vitreous and exudative retinal detachment from the inferior quadrants to the superotemporal arcade in the right eye. In the left eye, there were no cells in the vitreous, while exudative retinal detachment was observed in the inferior periphery and subretinal fibrin accumulation was noted at the superotemporal and inferotemporal arcades and nasal to the optic disc (). Ultrasonographic imaging (USG) was compatible with exudative retinal detachment in the right and left eyes (). Fluorescein angiography (FA) and indocyanine green angiography (ICGA) in the right eye revealed hypofluorescence in the region corresponding to the exudative retina detachment, as well as early hyperfluorescence increasing in later phases at the peripheral superotemporal arcade surrounded by multiple localized hypofluorescent foci. In the left eye, FA and ICGA revealed early hyperfluorescence increasing in later phases in the macula, superior and inferior temporal arcades, and nasal of the optic nerve (). Enhanced depth imaging-optical coherence tomography (EDI-OCT) revealed subretinal fluid and a hyperreflective band located subretinally in the section taken at the peripheral superotemporal arcade in the right eye; in the left eye, EDI-OCT showed subretinal fluid on the section passing through the macula, and subretinal hyperreflective material with a hyporeflective field and irregularity in retinal pigment epithelium (RPE) in the section taken at the level of the superotemporal arcade (). Subfoveal choroidal thickness in the left eye was determined as 591 µm on EDI-OCT (). Neurologic and otorhinolaryngologic examinations were normal; however, dermatologic examination revealed findings consistent with hirsutism. Based on the results of ophthalmologic examination and auxiliary imaging techniques, the patient was diagnosed with bullous type CSCR exacerbated by corticosteroid therapy. After consultation with endocrinologists, the patient was diagnosed with Cushing’s syndrome associated with systemic corticosteroid use, and gradual methylprednisolone tapering was recommended. Therapy with cyclosporine and azathioprine was discontinued. The subconjunctival corticosteroid particles were removed from the patient’s right eye. After consultation with endocrinology and obtaining the patient’s consent, treatment was initiated with oral mineralocorticoid receptor antagonist eplerenone 25 mg twice daily. Low fluence photodynamic therapy (PDT) (25 J/cm2, 300 mW/cm2) was applied to areas of leakage seen on FA and ICGA in the macula and superotemporal arcade in the left eye due to the potential threat to the macula. Focal laser photocoagulation was applied to areas of leakage in the left nasal and inferotemporal arcades. Exudative detachment was reduced in the right eye and had completely regressed in the left eye at 1-month follow-up. At month 4, visual acuity was counting fingers from 1 meter in the right eye and had improved to 0.7 in the left eye. Anterior segment examination was normal in both eyes. Fundus examination in the right eye revealed regression of the exudative detachment to the inferotemporal arcade and the presence of subretinal fibrosis at the peripheral superotemporal arcade; in the left eye, subretinal fibrosis was observed at the inferior and superior temporal arcades (). EDI-OCT examination revealed subretinal fluid in the right and left maculas, and subfoveal choroidal thickness was 537 µm in the right eye and 335 µm in the left eye (). The patient was lost to follow-up after the fourth month because she moved to another country. |
We report the case of a 14 years old male teenager who presented with gradually worsening intermittent localized dull aching low back pain which was aggravated by prolonged standing, sitting or walking, for the past one year. It was associated with significant weight loss of more than 10kg during that period and loss of appetite. There was history of night sweats, evening rise of temperature as well as multiple episodes of sinuses over the lower back and gluteal region with pus discharge which had resolved for a few months prior to current presentation. There was no history of persistent cough, tuberculosis contact, high risk behaviour or trauma to the lower back. There were no altered bowel or bladder habits. There was no history of dental abscess or procedures done previously. He had no known past medical illness and no previous surgery, blood transfusion or trauma. He lived with his parents along with four siblings and there was no family history of tuberculosis or malignancy. Tuberculosis workout had been done in a district hospital and reported negative. He was referred to a tertially hospital with spine service when there was worsening of low back pain despite management with analgesia and physiotherapy.\nOn examination, there was minimal tenderness over the lower lumbar region on palpation. There were no spinal deformity or gibbus. Range of motion of lumbar spine was restricted due to pain. Neurological examination revealed no motor or sensory deficit. Reflexes were normal over both upper and lower limbs. Examination of cardiovascular, respiratory and gastrointestinal systems revealed no abnormalities. Laboratory investigation revealed raised Erythrocyte Sedimentation Rate (ESR) of 69 mm/hr (the normal range is 0-22 mm/hr for men and 0-29 mm/hr for women), C-Reactive Protein (CRP) of 0.7 mg/dL (Normal values are below 3.0 mg/dL) and White Cell Count of 9.43× 109/L (Normal values range from 4.5 to 11.0 x 10^9/L). Mantoux test and sputum for Acid Fast Bacilli (AFB) were negative on three occasions. Chest radiograph was normal. Radiographs of the lumbosacral spine revealed collapse and reduction in joint space with erosion of end plates over L4-L5 region and loss of lumbar lordosis (). An MRI of the lumbosacral spine showed loss of L4-L5 disc space with minimal collection of extraneous material with adjacent paravertebral extension (). A CT guided biopsy of L4/L5 disc level yielded only obtained a small soft tissue sample which grew a pure culture of Streptococcus constellatus which was sensitive to penicillin and erythromycin biopsy was not repeated.\nHe was treated with intravenous C-penicillin 4 mega unit 4-hourly for total duration of six weeks after consultation with infectious disease team. He recovered well with gradual resolution of back pain and his inflammatory markers returned to normal just two weeks after initiation of treatment. The patient achieved full recovery after completion of six weeks of intravenous antibiotics. |
A 74-year old female farmer had injured her shoulder against the side view mirror of a moving car. After the accident, she was unable to lift her left arm due to shoulder pain and attended our hospital. On physical examination, a deformity was observed on the left clavicle. Standard radiograph revealed a fracture at the distal end of the left clavicle (). Three-dimensional computer tomography (CT) scan revealed in addition a displaced fracture of the proximal end of the clavicle, and a diagnosis of bipolar segmental clavicle fracture was made (). The proximal and distal displaced fractures were extra-articular (Robinson Type 1B1) and intra-articular (Robinson Type 3B2), respectively. The proximal end of the clavicle was displaced anteriorly and the distal end posteriorly (), with floating intermediate fracture fragments. This indicated fracture instability and a high risk of non-union. Therefore, we decided to treat with open reduction, and it was performed seven days after injury with the patient’s consent.\nThe patient underwent surgery under general anaesthesia in the beach chair position. A skin incision was made over the distal fracture site. The distal end of the clavicle was displaced posteriorly. The floating bone fragment was found rotated opposite to the direction of the displacement. As the distal end of the clavicle was reduced, the proximal end was simultaneously reduced to a good anatomical position. A 3-hole locking plate [LCP™ Superior Clavicle Plate, DePuy Synthes Trauma, West Chester, USA] was used to provide fixation between the distal end of the clavicle, as the intermediate floating fracture fragment was secured by more than two screws to prevent rotational displacement. The proximal clavicle fracture was left alone to avoid any critical complications.\nAfter the operation, the patient’s shoulder was placed in a sling and allowed passive range of motion of the shoulder below 90 degrees for one month as the proximal clavicle fracture was not surgically fixed. Gradually active movement of the shoulder was allowed as tolerated. Four months after the operation, the patient was able to elevate her shoulder forward up to 160 degrees. Radiographs and CT scans revealed bony union of both proximal and distal ends of the fracture ( and ). Because of discomfort due to implant irritation, the implant was removed one year after surgery and the symptoms diminished (, and ). Three years post operatively, the patient had neither pain nor crepitation in both her proximal and distal fracture sites. The ROM of shoulder was 165 degrees in anterior elevation and 80 degrees in abduction. The Oxford shoulder score was 47 points and ASES shoulder score was 91.6 points. The Quick DASH score was 5.0 points, demonstrating a better score than previous literatures (8 points without complication case, 66 points with complication case). The patient has continued her activities as a farmer. |
An 18-year-old male high school wrestler with no significant past medical history came to the emergency department (ER) because of cramping right leg pain. The pain started one day after wrestling which he denied any direct below (no history of trauma) to the area or any penetrating injury during the activity.\nIn ER, the patient was found to have a fever of 101.9 along with weakness in the right leg. During the examination, swelling and tenderness over the lateral right leg were evident. The patient was admitted, and workup was initiated including X-ray and CT scan of the right leg and blood cultures. The erythrocyte sedimentation rate was 25 (1–20 normal range), and CRP was 13 (<0.5 normal range).\nX-ray was negative, and the CT scan showed slight edema involving the right gluteus minimus muscle. Two blood cultures were sent which came back positive for methicillin-susceptible Staphylococcus aureus or MSSA. We started vancomycin IV, and infectious disease (ID) was consulted as no known source of infection was found. ID requested MRI.\nMRI results exhibited mild soft tissue edema in between the right iliac bone and the right gluteus minimus muscle, which extended laterally to the superficial aspect of the right proximal rectus femoris muscle and inferiorly to the superficial and deep portion of the gluteus medius muscle at its greater trochanteric insertion. The MRI report suggested myositis () of the right gluteus minimus muscle. Additionally, MRI showed reactive bone marrow edema of the right iliac bone, abutting the right gluteus minimus muscle.\nAs per ID request, interventional radiology (IR) was consulted and the case was discussed with an orthopedic surgeon and general surgeon on service. ID and IR agreed to proceed with ultrasonography joint arthrogram. IR-guided fluoroscopy was performed for possible aspiration of the joint and rule out septic arthritis. Successful access of the right hip synovial space was done, but no significant effusion was found for drainage. No further intervention was recommended by IR, ID, orthopedic surgeon, and general surgeon.\nNext two sets of blood culture came back positive for MSSA. The patient was switched to nafcillin based on sensitivities. Repeat cultures became negative. Further workup included a transesophageal echo that showed no endocarditis. The patient condition improved and was discharged with PICC line and ceftriaxone to be received at medical day stay for four weeks. |
This previously asymptomatic, healthy 7-year-old girl experienced an abrupt onset of several physical, neurological, and psychiatric symptoms increasing in intensity over a 3-week period. The patient's mother reports that strep pharyngitis was diagnosed by a previous physician on three separate occasions with the first episode occurring 180 days prior to the onset of neuropsychiatric symptoms. The first course of treatment was a 10-day course of amoxicillin which resulted in no change in her behavior or functioning. The strep infection recurred, and amoxicillin was again prescribed with no behavioral improvement. The third episode of strep throat (Quest Labs DNase B results of 407), (reference range <376) was treated with a course of clindamycin with a notable improvement in the patient's symptoms (). Neurologists at a university medical center referred the patient to the Psychiatry department. Instead, the patient's mother chose to have her evaluated by a developmental pediatrician who arrived at a diagnosis of Pediatric Autoimmune Neuropsychiatric Disorder Associated with Strep (PANDAS) (, ). Due to the patient's worsening symptoms, a second opinion was sought, and additional blood work performed including a CDC Lyme Western Blot which was positive, leading to the additional diagnosis of Lyme disease. Quest Laboratories' Lyme disease enzyme immunoassay (EIA) with reflex to Western Blot was positive at 1.25, (0.00–0.90 negative index value). The patient was referred to the university's infectious disease clinic where treatment to address the Lyme disease diagnosis began. A PICC line was inserted to facilitate the administration of IV Rocephin. A lumbar puncture (LP) was performed as part of the infectious disease evaluation with normal results. At this point, because the patient had both a diagnosis of PANDAS and Lyme disease, her mother sought the opinion of a pediatric Lyme disease specialist who was also familiar with diagnosing and treating PANDAS. A graphic representation of the timeline from the initial suspected Lyme disease exposure through her complete course of treatment is shown in .\nOn her first visit with the pediatric Lyme disease specialist, the patient presented with crying, anxiety, headache, joint pain, decreased cognitive functioning, fatigue, nighttime awakening and an extreme fear of sleeping alone. Her ASO titer was negative but a DNase B titer was elevated at 407 (<376). Her ESR was normal. The IV Rocephin 1.5 gm QD prescribed by the previous doctor was continued, but her PICC line became occluded requiring removal. Oral Omnicef 300 mg BID was initiated to replace the Rocephin. Zithromax 250 mg BID and Tindamax 250 mg QD (Saturdays and Sundays only) were initiated.\nDue to complaints of right upper quadrant pain, an abdominal ultrasound was ordered which was unremarkable ruling out concerns of possible Rocephin cholelithiasis. Ursodiol 300 mg BID was ordered to help break down cholesterol that had possibly formed in the gall bladder. A CDC Lyme WB was negative although repeated Lyme testing through IGeneX Laboratory revealed a positive Lyme WB IgM with positive double starred bands 31 and 41 and indeterminate bands 39 and 83–93. Lyme WB IgG and Bartonella henselae testing showed negative IgG and IgM results. Babesia FISH (RNA) was negative. Her previously elevated DNase B was now normal as was her ASO titer. An elevated Mycoplasma pneumoniae IgG at 2.01 (reference range ≤ 0.90 ISR) indicated a previous infection and M. pneumoniae IgM was negative. A basic metabolic panel, CBC, urinalysis, ESR, and ANA were completely within normal limits. Genetic testing indicated that the patient was a carrier of the HLA-DRB1, 2, and 4 genes which are reported to occur more frequently in patients with Lyme disease and rheumatoid arthritis (–). It is also been observed that B. burgdorferi can alter the repertoire of self-peptides bound to MHC class II molecules and influence the likelihood of autoreactive T-cells which could lead to infection-induced autoimmune illnesses ().\nThe patient's mother was given a Lyme disease checklist to complete prior to each appointment indicating symptoms and improvement or lack of improvement since the previous visit. The Lyme disease checklist asks the parent to rate the patient on 35 symptoms including (1) unexplained fevers, sweats, chills or flushing, (2) weight change (loss or gain), (3) fatigue, (4) hair loss, (5) swollen glands, (6) sore throat, (7) testicular/pelvic pain, (8) menstrual irregularity, (9) unexplained milk production/breast pain, (10) irritable bladder or dysfunction, (11) upset stomach/abdominal pain, (12) constipation/diarrhea, (13) chest pain/rib soreness, (14) shortness of breath or cough, (15) heart palpitations, (16) joint pain/swelling, (17) joint stiffness, (18) muscle pain/cramps, (19) muscle twitching, (20) headaches, (21) neck creaks/cracks/stiffness, (22) numbness/tingling/stabbing sensations, (23) facial paralysis, (24) double vision/floaters/loss of vision, (25) buzzing/ringing/ear pain/sensitivity, (26) vertigo/dizziness, (27) lightheadedness/poor balance, (28) tremors, (29) difficulty thinking, (30) difficulty concentrating, (31) forgetfulness/poor short term memory, (32) disorientation/getting lost, (33) difficulty with speech or writing, (34) mood swings/irritability/depression, and (35) too much/too little sleep. In addition, the parents were asked to rate symptom severity on a mild, moderate, or severe scale as well as symptom frequency on an occasional, often, or constant scale.\nAt a follow-up office visit 8 weeks later, her Lyme disease checklist indicated overall improvement with no headaches, but increased insomnia and scattered joint sensitivities. Range of motion, biometrics and vital signs were normal except for an oral temperature of 99.2° F. She had no “dark circles” under her eyes, no tremors and her balance was normal. 2 weeks later the patient experienced a sudden symptom recurrence. Omnicef was discontinued, Zithromax continued at the same dosage and Augmentin added at 500 mg BID. EMLA cream (Lidocaine 2.5% and Prilocaine 2.5%) was prescribed as a topical anesthetic to treat her joint sensitivities. Lab values at this visit were within normal limits.\n10 weeks following the regression, at her next office visit, she had no major complaints and her Lyme disease checklist indicated overall symptom improvement. She denied having headaches and reported increased energy, improved cognitive function and enjoyment of her music lessons. Her physical examination was within normal limits. ANA, CRP, ASO, Streptozyme, and M. pneumoniae lab results were all within normal limits. The patient was attending day camp, Monday through Friday, all day, without problems. 2 weeks following this appointment, she suddenly became argumentative, hyperactive, and combative and experienced chills and headache. Bactrim SS BID was added due to a suspected urinary tract infection and to address new IGeneX laboratory results indicating the presence of Lyme borreliosis with positive double starred bands 31 and 41 and double starred bands 39, 83–93 indeterminate. Quest laboratory testing showed an elevated B. henselae IgG at 1:64. B. henselae IgM was within normal limits at <1:20. Both Ehrlichia and Anaplasma testing had negative results.\n2 months later at her next office visit, the patient presented as unhappy and experiencing some facial “twitching.” However, her appetite was good (no previous restrictive eating or food refusal was noted) and she was engaged in activities including dance and tennis.\nTwo additional months later, labs were repeated. Quest laboratory testing indicated negative results for E. chaffeensis, A. phagocytophilum, and B. henselae IgG and IgM. IGeneX testing showed an indeterminate Lyme IgM with double starred bands 31 and 41. Lyme IgG testing was interpreted as positive with double starred bands 31, 41, and 58 reactive. IGeneX interpretation guidance states the IgG WB is considered positive if two or more of the double starred bands are present from either Group 1 or Group 2. Group 1 includes bands 23–25, 31, 34, 39, 83–93. Group 2 includes bands 23–25, 34, 39, 41, 83–93. B. henselae IFA IgG and IgM were both reported as negative. B. duncani IgG was negative but IgM was reported as indicative of active infection at a level of 80 with values > 40 considered elevated. This was presumed to be a co-infection due to the original tick exposure as no additional tick exposure was identified.\nCD45RA testing, which is a marker of naïve T cells, showed an elevated result of 38% (reference range 5–37%) which is an indication of the amount of time elapsed (~10 days) since the most recent antigenic stimulation (, ). Although the patient's CD45RA was not significantly abnormal, as it was only slightly outside of the upper end of the normal range, it may be supportive of the confirmed presence of an additional co-infection with B. duncani. The presence of B. duncani prompted the addition of Mepron 375 mg BID to her medication regimen. Less than 5 days following this visit, she experienced an onset of severe stomach aches and “migraines” which caused her to leave school early 4 out of 5 days each week for several weeks. These symptoms slowly resolved, and her medication regimen was continued without additional changes.\n4 months later at her sixth office visit, her mother reported that she was having panic attacks and was “terrified” to sleep alone. She had some residual combativeness, lack of focus, and cognitive interference. The patient's energy level was improved, and she was engaging in her normal activities. Physical examination and vital signs were normal. Sensory processing problems were evident with heightened sensitivity to lights and sounds. She appeared to manage well at home but struggled with these symptoms in her classroom resulting in the development and implementation of an Individual Educational Plan (IEP). Neuropsychiatric testing was ordered to assess this issue. Quantitative immunoglobulin values were all within normal limits. Quest laboratory testing at this visit revealed negative results for B. henselae IgG and IgM, E. chaffeensis IgG and IgM, and Anaplasma Phagocytophilum IgG and IgM. IGeneX testing showed negative results for B. henselae IgG and IgM, and Lyme WB IgG. Lyme WB IgM was indeterminate.\nThe Cunningham Panel™ of Tests (Moleculera Labs, Oklahoma City, OK) was ordered to assess the presence of antineuronal antibodies against specific neuronal receptors. The patient's Anti-Dopamine D1 Receptor (DRD1) and Anti-Dopamine D2L Receptor (DRD2L) were elevated, her Anti-Lysoganglioside-GM1 (LYSO-GM1) was normal, her Anti-Tubulin (TUB) was elevated, and her Calcium/calmodulin-dependent protein kinase II (CaMKII) was normal (–). Published studies demonstrated that the elevated presence of one or more of these antineuronal antibodies and antibody mediated stimulation of CamKII was strongly associated with autoimmune neuropsychiatric symptoms such as those present in PANDAS and PANS (, ). Based upon the patient's Cunningham Panel™ test results (See ) the decision was made to prescribe intravenous immunoglobulin (IVIg) in accordance with established treatment guidelines for the patient's level of symptom severity (, ). She received IVIg at 2 gm/kg given over a 2-days period for a total of three treatments at 4-weeks intervals.\nA significant evidence base exists for the use of IVIg in PANDAS patients, particularly those exhibiting a relapsing and remitting course of illness with moderate to severe symptoms, although additional randomized, double blind, placebo control studies need to be performed (). One well-known study, based on the hypothesis that PANDAS and Sydenham's chorea have similar group A strep etiology, proposed that immunomodulatory treatments could effectively treat neuropsychiatric symptoms. Comparing IVIg to therapeutic plasma exchange (TPE) showed that reassessment at 1-month following treatment, the TPE and IVIg groups both showed “striking improvements in obsessive-compulsive symptoms, anxiety, depression, emotional lability, and global functioning" ().\nAlthough controlled trials have only evaluated IVIg given as a single course, unpublished data based on the experiences of clinicians and researchers engaged in the PANS Consortium suggest that one to three repeated doses of IVIg may be helpful in children who exhibit a positive response to the initial dose but then experience a relapse as the exogenous antibodies are cleared ().\nIn another randomized, controlled trial of IVIg for PANDAS, it was concluded that IVIg was safe and well-tolerated but differences between groups were smaller than anticipated and the double-blind comparison failed to demonstrate superiority of IVIg over placebo (). It was proposed that the study design may have negated the potential observation of an improvement with IVIg by the administration of antibiotics to both groups prior to either IVIg treatment or placebo. An additional study concluded that children with PANDAS derive a favorable response to IVIg at 12-months follow up “consistent with its role in Ig replacement and immune modulation" ().\nAt the conclusion of her IVIg treatments, the patient, now age 9 and in third grade, was normal in height and weight. She had no complaints and her activity level continued to improve with decreased fatigue. Her appetite was normal, and her Lyme disease checklist indicated overall improvement. She did have some residual obsessions and compulsions, intermittent hand tingling, slight facial tics, and a humming tic. Medications at this time included Mepron 375 mg BID, Omnicef 300 mg BID, Tindamax 250 mg QD (Saturdays and Sundays only), and Zithromax 250 mg QD, all of which were continued due to the presence of her residual symptoms. The protocol utilized regarding the length of time for which antibiotic treatments were administered was based upon on the patient's symptoms and laboratory results. Before discontinuance of antibiotic treatment, the patient must exhibit symptom resolution along with negative laboratory findings for a at least 2 consecutive months.\nNow 21 months into her treatment, at her seventh office visit, her mother reported that she was doing “pretty well” despite a symptom flare the previous month which lasted ~3 weeks but had completely resolved. Her Lyme disease checklist indicated that her status was stable. Vital signs were normal except for a slight elevation in her oral temperature at 99.3 F. The patient was doing well in her new school, had a normal activity level and appetite, and was enjoying her usual activities including music and dance lessons. Physical exam revealed no involuntary movements, no vocal or motor tics, clear lungs, and normal reflexes. IGeneX testing at this time showed indeterminate results for Lyme WB IgM with double starred bands 31 and 41 ++ and double starred bands 39 and 83–93 indeterminate. Lyme WB IgG was negative. B. henselae IFA IgG and IgM were negative. B. duncani IFA IgG was negative, however, B. duncani IgM was positive at 80 with normal values falling < 40. All findings were negative per CDC standards. A Cunningham Panel™ was repeated (Test 2) to assess the post-treatment status of antineuronal antibodies. The results of the five assays were all within normal ranges () (see ).\nAt this point, it was determined that no further IVIg was needed. Because the family was unable to obtain insurance coverage for the IVIg treatments, they incurred the expense out-of-pocket which averaged $12,000 per treatment (, ). At her follow up visit 6 months later, 31 months after her initial visit, the patient's Lyme disease checklist indicated overall improvement. Her final laboratory testing for tick-borne diseases, strep antibodies, and M. pneumoniae were all within normal limits.\nCurrently this patient appears to be fully recovered and has been discharged from the care of the pediatric Lyme disease specialist. She is asymptomatic and performing academically at the “top” of her class according to her mother. A summary of the serological evidence of exposure to tick borne illness and streptococcal infection is included in . |
The second patient (PT2), a black female, was a 43-year-old non-smoker with weight of 300 lbs and height of 6–2" at the time she presented for care at the clinic. She was diagnosed in New York in 2000 with right upper extremity lymphedema (Figure ) following treatment for breast cancer in 1998 including mastectomy, axillary dissection of 24 lymph nodes, radiation, and chemotherapy. A side effect of the radiation was fibrosis of the lateral chest wall and breast tissue. The patient's first CDT began in 2002 after the lymphedema progressed with increased discomfort and recurrent infections. Between 2002 and 2006, she had four episodes of cellulitis treated with multiple antibiotics. She received three courses of intensive CDT, including two to three visits weekly for three months. In 2004, she began using the programmable ProACT 1 pump (Fore Tech Medical, Williamstown, NJ; HCPC code E0652) intermittently for temporary relief of pain when her symptoms were most bothersome. This device did not provide treatment of her truncal lymphedema.\nIn 2006, she stopped using the ProACT 1 pump due to development of a fibrotic cuff (at the limb root nearest the axilla), skin irritation, and lack of sustained reduction of lymphedema. She then moved to Georgia and in March 2007 began a fourth course of CDT, which required treatment five days a week for four months. At the time she began this treatment, arm volume measurements showed that the affected arm had 31% greater volume than the unaffected arm.\nThe patient's self-MLD was not successful, because she was unable to reach all affected areas and did not have anyone at home to assist with her care. The FT system was recommended because, in addition to treating her affected arm, it treated the chest wall, trunk, and shoulder areas that the patient could not reach. In December 2007, she began using FT for in-home treatment. The treatment protocol with the FT device consisted of one full upper-extremity and chest treatment session per day. A single session with the ProACT 1 pump produced a 2.9% increase in the patient's arm volume (Figure ). A session with FT was associated with a 0.68% decrease in arm volume.\nDuring her long-term experience with FT, PT2 reported the device to be comfortable and easy to use without assistance. She experienced softening of the fibrosis in her arm, breast, and chest wall after incorporating the FT system at home. The FT system also effectively treated multiple affected areas, reducing edema in her fingers, palm, forearm, upper arm, shoulder, and back. In addition, with FT usage she did not develop a fibrotic cuff or experience the skin irritation that had previously occurred with usage of the older generation IPC.\nPrior to treatment, PT2 received disability benefits due to pain from the lymphedema and swelling. After treatment with FT, she was able to return to work. She has not required additional in-clinic CDT since initiating FT therapy one year ago. |
A 67-year-old man complained of left shoulder pain for 2 weeks without any history of trauma. Bloody effusions were aspirated 5 times at a local orthopedic clinic during a 2-week period before the patient was referred to us. He had no previous history related to hemorrhagic factors. On examination, he had slightly limited range of motion, with active forward flexion to 160°, abduction to 120°, external rotation to 30°, and internal rotation to the fifth lumbar spine with the arm at the side. Passive range of motion was the same as active range of motion. Neer's impingement test and Hawkins test were positive. The greater tuberosity was seen irregular on radiography. MRI demonstrated a hematoma in the anterior portion of the subdeltoid bursa and bony erosion of the lateral acromion, similar to case 1 ().\nArthroscopy was performed to determine the cause of the hemorrhage. A hematoma was not observed in the glenohumeral joint. The intra-articular portion of the long head of the biceps tendon had completely disappeared. A bursal-side partial tear of the rotator cuff was noted. The size of the tear was 25 mm in the anteroposterior direction. Crater formation on the undersurface of the acromion and irregularity of the greater tuberosity were observed, which seemed to be kissing lesions (Figures and ). Active bleeding was seen at the exposed bone marrow of the acromion when the pressure of irrigation was lowered. The undersurface of the acromion was coagulated and the greater tuberosity was abraded to avoid impingement (). Acromioplasty was not performed in this case. The bursal-side partial tear was not repaired at that time ().\nOne month later, the patient underwent arthroscopic rotator cuff repair. The patient regained full range of motion 3 months after rotator cuff repair and returned to work as a taxi driver. At the final follow-up, 14 months after the first arthroscopy, he had no functional deficit. The UCLA score was improved from 14 points preoperatively to 35 points at the final follow-up. Hemarthrosis did not recur in the 14 months postoperatively. |
A 43-year-old male with a known history of retroperitoneal DDLPS presented to the emergency department complaining of upper abdominal pain with band-like radiation to the back. Associated, he had multiple episodes of vomiting for one day. On physical exam, he was found to have diffuse abdominal tenderness and distention.\nThe patient had undergone exploratory laparoscopy for tumor resection, and right radical nephrectomy for stage IV retroperitoneal dedifferentiated liposarcoma one year before the presentation. The resected mass was 20 cm in size. The surgical and pathologic margins of the resected specimen were negative at that time. The microscopic findings revealed a spindle mass with fibrous stroma consistent with a Grade 3 dedifferentiated liposarcoma (Figure ). After six months of resection, lymph node recurrence was noted on surveillance imaging. A lymph node biopsy at that time showed spindle cell sarcoma with similar morphology to the original tumor (Figure ).\nOn admission, a CT scan of the abdomen revealed enlargement of the previously noted masses. The tumor was reported to be 17 cm in size (identified by yellow arrows), extending into the duodenal lumen, causing a duodenal obstruction (Figure ). The CT scan also revealed acute pancreatitis with significant stranding and free fluid surrounding the pancreas (Figure ). Serum lipase was 3817 U/L at the time of presentation.\nSubsequent esophagogastroduodenoscopy demonstrated a large polypoid mass in the second portion of the duodenum, completely obstructing the duodenal lumen (Figure ). A 22 mm x 12 cm metallic duodenal stent was placed under fluoroscopic guidance (Figures , ). After the duodenal stent placement, his symptoms were significantly improved. After discharge, radiation therapy was attempted which he did not tolerate well. He was also started on doxorubicin and ifosfamide which he did not tolerate due to neurologic side effects. Later, he was treated with pazopanib (a multikinase angiogenesis inhibitor) with evidence of radiographic and symptomatic improvement. |
A 51-year-old woman presented with fatigue, nausea, and vomiting for three days. She was diagnosed with metastatic melanoma to pelvic nodes in July 2014 and was treated with ipilimumab (anti-CTLA-4) in combination with nivolumab (PD-1 inhibitor) between September 2014 and April 2015, receiving a total of 4 cycles of combination therapy and 9 cycles of nivolumab monotherapy with an initial mixed response followed by slow progression of disease. In April 2015 she was enrolled on a study of radiation (to pelvic mass) in combination with pembrolizumab (PD-1 inhibitor), receiving a total of 9 cycles of pembrolizumab with no toxicities. In December 2016 she was found to have new brain metastases and in January 2017 pembrolizumab was added. The day following her second dose of pembrolizumab, she developed fatigue and nausea and began having intermittent vomiting and diarrhea. Abdominal CT scan demonstrated diffuse colitis. Infectious studies including C. difficile antigen, stool culture, viral PCR, and ova and parasites exam were all negative. She was started on methylprednisolone 2m/kg/day. Over four days of hospitalization, her abdominal pain worsened and she developed melena, which progressed to bright red blood per rectum. She was given infliximab at 10mg/kg. Repeat imaging performed 48 hours later due to severe abdominal distension showed large amounts of free air with gaseous distention of large and small bowel loops, consistent with perforation in the context of colitis with ileus. She was taken to the operating room for emergent bowel resection and a perforation site was identified at the transverse colon. The resected transverse colon serosa was congested and dusky with site of perforation identified (). The colonic mucosa revealed diffusely edematous folds as well as confluent areas of yellowish exudate and multifocal ulcers (). Histologic sections confirmed the presence of transmural necroinflammation and multifocal ulceration (Figures and ). The findings were of a fulminant colitis with multifocal ulceration and perforation. No evidence of metastatic melanoma to the bowel was identified. |
A 14-year-old Thai girl was born via cesarean section due to premature rupture of the membrane with a birth weight of 2500 g. She is the first child of a consanguineous (second-degree relatives) couple. Both parents are healthy and have never had fractures. During her first year of life, she had delayed motor development and growth failure. At one year of age, she could not sit by herself and weighed 7.5 kg (< 3rd centile). She presented to our hospital at 14 months of age with fractures of both femora without a history of significant trauma. She was found to have ptosis of both eyes with normal teeth but no blue sclerae. She was small for her age. Her weight was 7.8 kg (3rd centile) and her length was 68 cm (< 3rd centile). Skeletal survey showed diffuse osteopenia, multiple healed fractures of the right humoral shaft, both tibiae and fibulae. Spine radiograph showed flattening and indentation of vertebral bodies (Fig. ). A diagnosis of OI was made and intravenous bisphosphonate therapy (pamidronate 1 mg/kg/dose for 3 days) was initiated and given every 3 months. However, she sustained 1–2 long bone fractures per year from minor trauma. She required multiple corrective osteotomies to correct her deformities. At the last follow-up, she was 14 years old, weighing 20 kg. She could not walk due to her long bone deformity (Fig. ). Remarkably, although she was in a special education class due to physical disabilities, her cognition was appropriate for age. She could talk fluently and do mathematics properly.\nPrenatally, her younger sister was found to have a dilated fourth ventricle by an ultrasonography. She was born at term via cesarean section because of previous cesarean section and was diagnosed with hydrocephalus at birth. At 4 months of age, she had her first fracture without a history of a significant trauma, leading to a diagnosis of OI. Physical examination revealed a head circumference of 38 cm (> 95th centile) with a wide anterior fontanelle (3 × 3 cm.) and blue sclerae. She had global developmental delay (could not hold her head) and hypotonia. MRI of the brain demonstrated a large posterior fossa cyst connecting with the fourth ventricular system, moderate hydrocephalus, hypoplasia of cerebellar hemisphere with absence of cerebellar vermis, and hypoplasia of corpus collosum. She was also diagnosed with vesicoureteral reflux grade V and gastroesophageal reflux requiring tube feeding. The patient had multiple hospitalizations because of recurrent urinary tract infections and pneumonia. She expired at the age of one year.\nSixteen known OI genes, BMP1, COL1A1, COL1A2, CREB3L1, CRTAP, FKBP10, IFITM5, LEPRE1, PLOD2, PPIB, SERPINF1, SERPINH1, SP7, TMEM38B, WNT1, and MBTPS2, were amplified from 200 ng of genomic DNA using the Truseq Custom Amplicon Sequencing kit (Illumina, San Diego, CA). 286 amplicons which covered all the 226 exons (28 kb) of the target genes were sequenced by Miseq (Illumina, San Diego, CA) using 2 × 250 paired-end reads. SNVs and Indels were detected by Miseq reporter software. The proband was found to harbor a homozygous mutation, c.6delG, p.Leu3Serfs*36 in WNT1. The mutation has never been reported in Human Gene Mutation Database (HGMD; ) (Fig. ). The mutation was subsequently confirmed by PCR-Sanger sequencing. Segregation analysis was performed by using primers, WNT1-E1F: GGT TGTTAAAGCCAGACTGC and WNT1-E1R: ACCAGCTCACTTACCACCAT. The results revealed that the patient was homozygous, while her mother was heterozygous for the mutation (Fig. ). |
An 85-year-old woman originally presented to her primary care physician with vague pain in her left upper quadrant. She described it as a sharp, intermittent pain, not associated with meals. The pain was exacerbated by different body positions and by activity. She experienced loss of appetite and five pounds of weight loss. She had no significant past medical history, but had recently had both upper and lower endoscopies, which were normal.\nPhysical exam revealed tenderness in her left upper quadrant. No organomegaly was identified, and no other tenderness or palpable masses were present on examination. A computed tomography (CT) scan of her abdomen was ordered by her primary care physician, and this showed multiple masses within the spleen which were suspicious for malignancy (Figure ). Because of the CT findings and the patient's complaint of pain, she was referred for splenectomy.\nPre-operatively, a complete blood count and electrolytes were within normal limits. A laparoscopic splenectomy was initiated but due to extensive adhesions and multiple tumor implants, the procedure was converted to an open approach. On exploration, numerous omental, mesenteric, peritoneal, and diaphragmatic implants were identified. The splenic capsule, however, appeared to be uninvolved. A splenectomy, omentectomy, removal of peritoneal nodules, and right oophorectomy were performed. The left ovary was not identifiable due to the dense adhesions and implants matted in that area.\nPathologic examination of the gross specimen (Figure ) revealed intraparenchymal splenic lesions. Representative sections from the ovary (Figure ) and spleen (Figure ) are shown here. The tumor at both sites showed a similar morphology of a poorly differentiated adenocarcinoma consisting of irregular nests of large anaplastic polygonal cells with an ill-defined cribriform pattern (Figure , inset). The ovary was almost completely replaced by tumor. There were multiple splenic nodules showing invasive growth. Special stains (not shown) were identical between the sites, with small amounts of intracytoplasmic mucin on mucicarmine stain. Neoplastic cells showed positive immunohistochemical staining for pankeratin, estrogen receptor, and CK7 and were negative for progesterone receptor, carcinoembryonic antigen (CEA), and CK20 in sections from both sites (not shown). This immunophenotype is indicative of a primary ovarian adenocarcinoma.\nA CA-125 level checked postoperatively was markedly elevated at 685.9 units/mL (normal: <35 units/mL). The patient made an uneventful postoperative recovery, and was discharged home. After a consultation with oncology, she is currently undergoing chemotherapy. |
A 62 year-old male had displayed bradykinesia and tremor of his right limbs for one year, during which he was able to perform limited fine movements such as dressing himself, lacing up his shoes and brushing his teeth. His tremors were aggravated by nervousness and relieved when asleep. He had had a history of hypertension and took a daily dose of 5 mg amlodipine. The patient had no history of any other chronic illnesses and was not on any other type of medication. Neither the electrocardiogram nor the Holter monitor showed any abnormalities. His baseline recumbent-upright blood pressure (BP) and heart rate (HR) were normal prior to treatment with piribedil, as shown in Table . He was diagnosed with PD based on the Movement Disorder Society clinical diagnostic criteria []. Initially, he received dopamine replacement therapy of 50 mg piribedil per day. Although there was no significant improvement in symptoms neither did he feel any discomfort. Therefore, starting the first dose change of piribedil, he added extra 50 mg to his dose. About two hours later after the first change in dose, the patient experienced symptoms of dizziness and sweating; he collapsed half an hour later. Whilst in a sitting position, the patient’s BP and HR were measured immediately. The BP reading was 85/48 mmHg and HR was 45 beats/min. His symptoms continued for the duration of the day with sitting BP fluctuating between 80–95 mmHg to 45–68 mmHg. Because his head computerized tomography examination found no abnormalities, the patient received 500 mL of 0.9% sodium chloride solution, after which his symptoms improved. Due to adverse drug reactions (ADRs), the patient was started on a second dose change of piribedil, i.e. an extra dose of 25 mg piribedil was to be taken in the afternoon in addition to the existing 50 mg taken in the morning. After two and a half hours, the patient experienced dizziness, sweating, nausea and vomiting, with a BP reading of 70/45 mmHg and a HR of 47 beats/min when sitting. His BP recovered to 105/65 mmHg and HR to 60 beats/min after elevating his lower limbs and resting for 20 mins. As a result of these incidences, the patient was switched to pramipexole to replace piribedil. After taking pramipexole 0.125 mg or 0.25 mg three times a day (tid), the symptoms of hypotension and bradycardia disappeared but a reduced amplitude of his right arm swing was still observed. Finally, after the pramipexole dose was increased to 0.375 mg tid, the patient showed marked improvements in motor symptoms. The changes in BP and HR are shown in Fig. . |
This case was reported according to the Case REport (CARE) statement and checklist [], and written informed consent was obtained from the patient to publication of this case report.\nA 29-year-old female patient, nonsmoker, who reported not consuming alcoholic beverages, not taking any long-term medication, and having no clinically relevant family history information, complained of pain on touching the maxillary left central incisor (tooth 11). Her dental history included injury to the tooth in question due to a fall from standing height during childhood, and the tooth began to be dislodged over the years. In a specialized dental care clinic, she received endodontic treatment that did not achieve the expected success.\nClinical examination showed grade II mobility of tooth 11. Radiographic examination revealed the presence of external root resorption that resulted in extensive resorption of the buccal wall in the area (Figures and ).\nAfter evaluating the case, extraction of tooth 11 was proposed to the patient, followed by immediate placement of a dental implant associated with guided bone regeneration, because her gingival biotype was classified as thick [], and the remaining bone tissue allowed immediate implant placement []. The management option of choice was a fully digital treatment, from surgical planning to fabrication of the final crown, for the current analysis of digital workflow in dentistry.\nA computed tomography (CT) scan of the complete maxilla was obtained in Digital Imaging and Communications in Medicine (DICOM) format, and intraoral scanning was performed using the Trios 3® pod scanner (3shape) to obtain stereolithography (STL) files.\nOnce these 2 files had been obtained, the coDiagnostiX® software (Dental Wings) was used for virtual planning. After DICOM and STL images were superimposed, surgical planning was performed by placing the implant in the optimal position for future prosthetic rehabilitation (). Once the implant had been virtually planned, a software for manipulation of 3D objects (Meshmixer®) was used to remove tooth 11 from the original STL file in order to fabricate a tooth-supported surgical guide. This new STL file was imported, and the process of guide fabrication was started by using the “Add surgical guide” tool in coDiagnostiX®. The final virtually designed guide () was then printed with a Rapid Shape®-Straumann 3D printer.\nAt the time of surgery, before the sterile drapes were applied, intraoral antisepsis was performed by mouth rinses with 5 mL of 0.12% aqueous chlorhexidine, and extraoral antisepsis was performed with 2% aqueous chlorhexidine. Anesthesia was obtained with 4% articaine with 1 : 100,000 epinephrine (4% Articaine DFL®). Initially, a buccal incision was made 2 mm above the mesial papillae (from tooth 21 to 12), in order to have them preserved, followed by a divergent relaxing incision in the mesial side of tooth 12 extending to the mucogingival line [] (). The flap was then elevated, and tooth 11 was extracted using a minimally invasive approach with periotomes (Quinelato). After extraction, the surgical guide was placed in position to assess proper fitting through the inspection windows. After this stage, the implant bed was prepared with drills from the Straumann ® Guided Surgery–BLT system, as described in the protocol printed by the planning software (). Subsequently, a 3.3 × 14 mm Straumann SLActive® Bone Level Tapered Roxolid implant (NC) was placed, reaching a maximum torque of 45 Ncm (Figures and ). Its respective tap was then inserted. Particulate bone substitute (Geistlich Bio-oss®) was placed on the exposed threads, as planned virtually, for the purpose of guided bone regeneration [, ] (). Once bone grafting had been completed, the flap was repositioned and secured with simple interrupted sutures (4-0 silk suture, Ethicon) for healing by first intention. Postoperative care included administration of antibiotics (amoxicillin 500 mg, every 8 hours for 7 days), anti-inflammatory drugs (dexamethasone 4 mg, every 12 hours for 2 days), and analgesics (acetaminophen 750 mg, every 6 hours for 3 days in case of pain).\nThe crown of tooth 11 was prepared and temporarily bonded with light-cured resin (Tetric N-Ceram®–Ivoclar Vivadent) to the adjacent teeth (teeth 21 and 12) until the time of implant uncovering.\nAfter 45 days, the implant was uncovered. The surgical guide was again positioned in the mouth, and using a 3.4 mm diameter mucosa punch (Straumann Guided Surgery®–BLT), the gingival tissue was removed, and the tap was exposed. A temporary abutment for NC–BLT implant (Straumann) was then placed, while the provisional crown was captured in the mouth (artificial teeth–Trilux–EuroVIPI). After polishing and finishing, the temporary abutment was tightened with manual torque ().\nThe patient was scheduled to return in 4 weeks for evaluation of the peri-implant tissue. At this visit, minor adjustments were also made to the crown to improve the gingival contour. The patient was then scheduled to return in 6 months for fabrication of the final prosthesis. At this return visit, the provisional crown was removed, and a scanbody (NC Straumann) was placed. After checking the scanbody for proper position, another intraoral scanning was performed (Trios 3® pod–3shape) for fabrication of the final prosthesis. During scanning, the patient's tooth shade was determined by using the scanner's shade measurement tool () and the VITA Classical shade guide. Subsequently, both the STL files and the visually selected shades were sent to the laboratory for fabrication of the final prosthesis.\nGiven the precision of the digital workflow, the final abutment (3.8 mm diameter, 5.5 mm height, 1 mm gingival height; NC–Variobase®), preselected during virtual planning, was sent to the laboratory, which was requested to send us the final crown, thus obviating the need for an appointment for metal framework try-in. A specific CAD dental software (Dental System–3shape®) was used to design the crown, which was subsequently milled with the Ceramill® Motion 2 milling machine (Amann Girrbach) (CAM system). On delivery, minor adjustments were made to the prosthesis to eliminate occlusal interference. After polishing and patient's approval of aesthetics and function, the crown was placed on the implant with a torque of 35 Ncm.\nThe coDiagnostiX® “treatment evaluation” tool was used to assess how much the implant deviated from the preoperatively planned position. The STL image produced by intraoral scanning through the scanbody was superimposed onto the software planned implant position, thus generating the deviations ().\nThe patient returned for treatment evaluation 3 months after crown delivery, when she reported satisfaction with the aesthetic and functional results of the final crown (), without any complications since its placement. |
The patient was a 44 years old male, right-handed native English-speaker. He had no past medical history of note, in particular no developmental delay, no delayed speech or language development, no cognitive or behavioral disturbance. He also had no cardio-vascular, neurological or psychiatric history of note, although he had a longstanding gambling addiction. The patient lived by himself and his family described him as a hardworking gentleman, chatty and cheerful, but at times with a short fuse and irritable. He had good friends and many interests.\nIn 2017 he presented to hospital after three weeks of dizziness and daily headache, worse in the morning and associated with nausea. Investigations included a Computed Tomography (CT) brain, which revealed an avidly enhancing posterior fossa mass in the cerebellar midline and a small additional lesion in the right superior frontal gyrus. The subsequent Magnetic Resonance Imaging (MRI) brain showed a 22 mm by 31 mm by 25 mm intra-axial mass lesion centrally within the vermis with surrounding edema, extending more prominently into the right cerebellar hemisphere; with no features of hydrocephalus (Fig. ).\nHe was commenced on Dexamethasone (16mg once daily), which led to improvement in his symptoms. Elective surgery for removal of the cerebellar lesion was planned two weeks later. At admission, the patient was hyperactive and slightly agitated with disrupted sleep, likely as a side effect of the steroids. The surgical technique involved a horizonal fissure approach with paravermian surgical incision of right and left cerebellar hemisphere. No ultrasonic aspiration was used during the surgery. A branch of the Posterior Inferior Cerebellar Artery (PICA) was entering the lesion and was electro-coagulated. There was no obvious swelling at the end of the surgical procedure and no clear hemorrhage. Pathology results of the brain tissue showed malignant melanoma, BRAF V600e positive (sequence variant valine to glutamic acid translocation). The patient underwent further investigations including CT thorax, abdomen and pelvis, which was negative. He was then diagnosed with resected Stage IV melanoma T0N0M1c [].\nThe immediate postoperative course was uneventful. The post-operative MRI (Fig. ) showed no enhancing residual tumor in the posterior fossa. There was high Fluid-attenuated inversion recovery (FLAIR) and Diffusion-weighted Imaging (DWI) signal surrounding the resection cavity, more prominent in the left cerebellar hemisphere and left tonsil, suggesting some post-operative contusion and ischemia. A pseudomeningocele associated with the posterior fossa surgery was noted.\nClinically, on day one after surgery the patient was lethargic but rousable. On day two he was drowsy and obeying simple commands. On gross neurological examination, there was no cranial nerve or eye movement deficit, and no hypotonia or weakness. In the following days the patient tended not to interact, had moderately preserved vigilance with fluctuating drowsiness, and when prompted complained of headache, nausea and dizziness. Improvement in the patient’s level of alertness brought to light the consistent changes in his mood and behavior with predominant negative features (i.e. flat mood, reduced interactions, withdrawn affect). He showed substantially decreased voluntary motor activity and general apathy with disengagement. The patient usually lay with his eyes closed curled up in bed in a fetal position. There was no crying or whining. Auditory-verbal comprehension was intact for daily language use. His interactions were minimal. He was dismissive and abrupt with medical staff, but he was not aggressive. He replied to questions only when prompted to do so, and only spoke spontaneously to family members. Communication was characterized by monosyllables or two-word sentences without articles and determinants. His ate very little in spite of being able to swallow safely. At that time the patient’s weight was 54 kg with a height of 1.7 m (BMI 19). Due to the persistence of the behavioral change, a neuropsychology assessment was requested one month post-surgery. The patient was unable to comply with a structured assessment due to his poor interaction, poor engagement and lack of motivation. However, the psychologist noted the following features: 1) stark contrast with the patient’s behavior prior to surgery; 2) the patient required significant prompting to complete any task and this prompting led to the patient being agitated; 3) the patient had insight into his mood; 4) the patient had insight on discussions with medical staff that caused him annoyance; 5) rigidity and perseverance of thinking on selected topics that caused the patient anxiety; 6) sleep disturbances due to “mind racing”.\nA repeat MRI brain showed an increase in size of the frontal melanoma metastasis and he commenced targeted anti-cancer therapy with Debrafanib and Tramatenib. Forty days post-surgery he deteriorated with increasing drowsiness, headache and nausea. He required an urgent ventriculoperitoneal shunt (attached to Medtronic Strata valve sets) for a sub-occipital pseudo-meningocele, likely aggravated by recumbency, with developing hydrocephalus.\nPost shunt insertion, the patient’s headache and drowsiness had improved, whilst his neuro-behavioral presentation with negative symptoms continued. His speech and language did not show progressive improvement. He was defiant to complete daily tasks that required his collaborations, such as taking medicines, sitting up, standing, engaging in any physiotherapy, but he was otherwise submissive regarding major interventions, such as surgical decisions. Despite many attempts, he only managed to sit at the edge of the bed for 15 s before complaining of headache, dizziness and nausea. He did not show significant ataxia limiting his mobility whilst fatigue continued to increase. His day-to-day physical performances were not consistent and at best, he managed to walk from the bed to the toilet with assistance for balance. Despite stabilization and response to treatment of the right frontal lesion, his general health deteriorated with a progressive loss of weight due to decreased food intake. He lost 8 kg in four months (BMI 16). Attempts to support his nutrition via a nasogastric tube were overall unsuccessful, with the patient repeatedly pulling out the tube. At that point, there were concerns about his capacity to consent, he did not seem to retain information and showed little insight into his physical health. A decision to place a gastrostomy was made under the Adult With Incapacity (AWI) act, almost five months post initial surgery. The family was supportive of the decision and the patient did agree to have the procedure. The improved nutritional intake did not change the patient’s behavior or fatigue. Most days he refused to work with the physiotherapists and even when he agreed to it, the sessions lasted a few minutes at most.\nFour months after the initial surgery, the patient was referred to a Neurologist with an interest in brain tumors and posterior fossa syndrome (author EM). The family members were present at the visit. His collaboration and the neurological examination were limited. He showed fatigue within a few minutes, verbal production was minimal with short sentences, slow, mildly dysarthric, slightly slurred but comprehensible. His mood was fluctuating and he showed some emotions when crying about his inability to be discharged home. Finger-nose test was slow but with no dysmetria. Muscle tone was normal. Gait was not tested at that time. In conjunction with the Psychiatrist (author MO), the patient was started on Sertraline, an antidepressant drug of the family of selective serotonin reuptake inhibitors (SSRIs), at a dose of 50 mg daily. There was no improvement after two months. Given the decline of the patient’s general health and the risk of missing the window when rehabilitation may be beneficial, the Neurologist (EM) and the Psychiatrist (MO) discussed a further medication trial. The patient was started on Modafinil (seven months after the initial surgery) with an increasing dosing scheme up to 100 mg twice daily.\nIn the first week of starting Modafinil, the patient showed impressive improvements in all areas of evaluation, and progressively continued to recover. Specifically, he engaged in conversations not only with the family, but also with members of the hospital staff. He expressed himself with short conversations, which were not characterized by the previous child-like grammar. He engaged with the physiotherapists, he sat on a chair and walked down the corridor of the hospital with assistance. He started having short sessions of physiotherapy of approximately 10 min each, and continued to increase their duration with concomitant progressive reduction of the recovery time in between. He ate by himself and stopped using the gastrostomy tube completely after two weeks, with subsequent tube removal. He did not develop side effects related to treatment with Modafinil, and in particular he did not develop mania or psychosis. Although not specifically measured, the patient did not manifest autonomic-related symptoms such as heart rate or blood pressure issues. A perfusion HMPAO SPECT (single-photon emission computed tomography) CT perfusion scan of the brain during this recovery phase (two and a half months after starting Modafinil) showed expected reduced perfusion within the cerebellar vermis consistent with post-surgical cerebro-malacia, and normal frontal lobe cortical perfusion. In addition, there was focal hyperperfusion in the anterior cingulate (Fig. ). Follow up CT brain confirmed the frontal lobe metastasis to be stable on the maintenance regime of BRAF inhibitors.\nThe patient continued to improve and gain from his physiotherapy and rehabilitation. Ten months post-surgery, and three and a half months following the start of Modafinil treatment, he had a staged return home (i.e. returning home at night). He was deemed fit for discharge home with rehabilitation support 12 months after the initial surgery, and five months after the start of Modafinil. Unfortunately, just a few days before his final discharge, neuroimaging showed progression of the metastatic lesions (progression of the frontal lesion with hemorrhage and new occipital lesion) and he became symptomatic with vomiting and focal motor seizures. Despite the progression of the cancer with unfavorable outcome, the patient maintained the benefits of Modafinil treatment on mood, behavior and engagement.\nOther investigations at the time of the planned discharge included a neuropsychology assessment. In particular, the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) form a (Fig. ), Hayling test and Trial Making A and B were performed. The patient’s performances revealed pretty low scores across the board of tests, but with relative strengths in attentional skills, visual screening ability and processing speed.\nThe MRI brain at the same time showed bilaterally high T2-weighted and FLAIR signal in medulla oblongata at the level of the inferior olivary nucleus (Fig. ), suggesting hypertrophic olivary degeneration as a result of bilateral damage of proximal efferent cerebellar pathways secondary to presumed peri-operative damage to the dentato-rubro-olivary pathways or Guillain-Mollaret triangle.\nFunctional MRI was also performed with three language paradigms for assessment of language centers and activation; this showed normal left hemispheric language activation in Broca’s and Wernicke’s area (Fig. ).\nDiffusion Tensor Imaging (DTI) (Fig. ) was also performed, with fractional anisotropy map obtained and tracts seeded for tractography (Fig. ). Although we could not see any gross abnormality of the tracts, the images were degraded by artifact arising from the right parietal shunt, limiting the value of the examination. |
An 8-month-old female infant had coronary reimplantation at age 3 months for ALCAPA. Postsurgical ejection fraction showed early improvement with subsequent deterioration. A diagnostic left heart catheterization performed as part of a heart transplant evaluation revealed severe ostial stenosis of the LMCA. She was referred for PCI of the left main coronary artery to relieve her heart failure and preempt transplant. Her physical exam was significant for congestive heart failure and failure to thrive. The patient's echocardiogram showed markedly depressed left ventricular function. A cardiac computed tomography angiography (CTA) and initial diagnostic nonselective root aortogram demonstrated stenosis of the LMCA at the site of ALCAPA reimplantation. Selective left coronary angiogram revealed severe kinking of the reimplanted LMCA at the ostium ().\nThe procedure was performed in the Pediatric Cardiology interventional suite after extensive discussion and planning with Pediatric and Adult Interventional Cardiology, Pediatric Cardiac Anesthesia, Pediatric Cardiothoracic Surgery, and Pediatric Cardiac Radiology. General anesthesia and a femoral arterial approach were utilized. An ascending aortic root angiogram was obtained in 2 planes to assess the ostial left main stenosis. Due to the small size of the infant aorta, a 6 Fr JR-4 guide was reshaped to engage the left main coronary artery. A BMW wire was used to cross the lesion. Since the infant left main coronary artery was small but was expected to grow with age, a somewhat larger (3.0 × 8 mm Vision bare metal) stent was carefully implanted in the proximal LMCA at less than nominal pressure to avoid distal dissection. A poststent angiogram showed that the stent had moved during implantation and missed the ostium with residual stenosis of the LMCA origin. Hence, a 3.5 × 8 mm Vision bare metal stent was placed into the LMCA ostium overlapping with the previous stent distally and protruding 1-2 millimeters in the aorta proximally. The ostium and aortic overhanging portion of the stent were postdilated producing proximal flaring. The final angiogram confirmed excellent stent position and normal flow ().\nThe patient tolerated the procedure well and was discharged home on dual antiplatelet therapy (DAPT) with aspirin and clopidogrel one day after PCI. She was followed in Cardiology clinic post-PCI and noted to have no change in her ventricular function. Nevertheless, she continued to do well clinically until six months post-PCI, at which time she demonstrated failure to thrive and required admission for initiation of continuous milrinone infusion. The left main stent was widely patent with normal Doppler flow signals as visualized by echocardiography (). She was listed as status 1A for orthotopic heart transplantation (OHT) and underwent transplantation one year post-PCI, at age 20 months. |
A 46-year-old female was referred from an outside hospital for squamous cell carcinoma of the right ala. The mass measured 2 × 1.5 cm and had an ulcerated portion from a previous punch biopsy site (). A single-stage operation was offered, to which the patient consented. The tumor was excised through all three layers with a 5 mm margin, and the resultant defect measured 2.5 × 2.0 cm. Intraoperative frozen biopsy report was consistent with squamous cell carcinoma. The lining mucosal defect measured 2.0 × 1.5 cm, and the graft for this inner layer was prepared using a septal mucosal hinge flap based on the septal branch of the superior labial artery. The typical incisional boundaries of a mucosal flap are located superiorly, about 1 cm from the nasal dorsum, inferiorly along the maxillary crest, and posteriorly as far as the extent of the defect. The mucosal flap was elevated from the septum in a posterior-to-anterior direction and turned laterally toward the defect, such that the mucoperichondrium was external and the mucosa faced internally () []. A right conchal cartilage graft was harvested, fashioned into a suitable shape, and placed over the mucosal hinge flap to provide structural support. The outermost surface was restored using a nasolabial island flap with a subcutaneous pedicle that was based on a perforator vessel from the angular artery. This flap was designed using a 3 × 2 cm mirror-image template from the contralateral ala, and was elevated in an inferior-to-superior direction. Once rotated over the septal mucosa-conchal graft construct, the flap was sutured into place (). The nasolabial fold was re-established with primary closure of the cheek skin, under which a capillary drain was left for three days and then removed.\nThe postoperative course was uneventful, without any vascular complications. By 6 months, however, scar contraction along the reconstructed rim had resulted in a partially obstructed inlet with mild asymmetry. A nostril implant was used to improve the patency. At 8 months, the patient was satisfied with the operative outcomes. No subsequent interventions were necessary (). |
A 63-year-old female with primary open angle glaucoma underwent combined phacoemulsification and trabeculectomy augmented with MMC in the left eye. Intraoperatively, fornix-based flap was created, and 4 mm × 4 mm partial thickness sclera flap was dissected. MMC (0.2 mg/ml) was applied using four pieces (1 mm × 1 mm) of methylcellulose sponge on the sclera beneath the conjunctival flap away from cornea for 1.5 min. Care was taken to ensure that the edges of the conjunctiva were kept clear of the sponge. All the four pieces were counted and removed using nontoothed forcep. After the sponge was removed, the space between conjunctival flap and episclera was thoroughly irrigated with normal saline. The surgery was uneventful. Conjunctiva was closed using 10-0 nylon suture.\nThe patient was doing well with visual acuity 6/12, and intraocular pressure (IOP) ranging from 10 to 16 mmHg with a diffuse posterior bleb with minimal vascularity extending over 2 clock hours. At 3 weeks postoperatively, the patient presented with severe pain and redness in the operated eye. A granuloma adjacent to the bleb was noted []. Probable diagnosis of suture granuloma was made.\nSuture removal was done. The frequency of topical steroids was increased, and oral steroids were started. After 2 weeks, two whitish areas at the center of the granuloma were seen which was assumed to be either necrosis of the overlying conjunctiva or white foreign body material partially extruding on to the surface from the subconjunctival space []. There was no anterior chamber reaction, and the bleb remained uninvolved so surgical exploration was done.\nTwo fragments of methyl cellulose sponge were retrieved surgically from the lesion []. The underlying sclera was healthy. The microfragment was sent for culture which was found to be sterile. Topical steroids and antibiotics were continued. On the subsequent visits, patient maintained a best-corrected visual acuity of 6/12 with IOP in the range of 10–12 mmHg with a functioning bleb. |
The mother was a 47-year-old woman, para 7, originally from Somalia. The father was 71 years old. The mother presented at 35 + 6 weeks of gestational age after 3 days of decreased fetal movements. Cardiotocography (CTG) was severely pathologic, showing a preterminal pattern. Emergency caesarean section was performed.\nThe antenatal course had been uneventful up until the last few weeks before delivery when the mother had started to complain about fatigue and palpitations. Four days before delivery the mother had fainted and was examined at the maternity ward. Her level of hemoglobin and the electrocardiography (ECG) were normal, as was the CTG.\nThe newborn weighed 2800 gram and had Apgar scores of 0 at 1 minute, 4 at 2 minutes and 7 at 5 minutes. At birth a sample of umbilical arterial blood showed metabolic acidosis. Blood glucose was normal.\nAt first there were vital signs but soon the child turned pale, weak and was without cardiac activity. Cardiopulmonary resuscitation was initiated. The infant was found to be severely anemic, the hemoglobin concentration in the umbilical artery was 2.1 g/dL. She was immediately treated with blood transfusion.\nHereafter she was admitted to the neonatal intensive care unit, where she was intubated, ventilated and treated with nitrous oxide (NO) because of pulmonary hypertension.\nEchocardiography showed severe distention of the right side of the heart as well as a small apical ventricular septum defect (VSD). Both ultrasound of the cerebrum and electro-encephalogram (EEG) were normal. Because of neonatal sepsis the infant was treated with a broad spectrum of intravenous antibiotics.\nThe infant remained hospitalized for 1 month. The serum levels of both alfa-foetoprotein and hCG were at all times normal why no ultrasound, X-ray or CT scans were performed in search for metastases. The infant showed no signs of neurological damage. After discharge she was seen regularly in the out-patient clinic with measurements of serum levels of alfa-foetoprotein and hCG as well of monitoring of her neurological development. The mother was simultaneously examined because of the risk of widespread disease. Chest X-ray revealed no metastatic lesion, neither did ultrasound or CT scan of the abdomen.\nThe mother was referred to a regional oncology center where serial serum concentrations of hCG and alfa-foetoprotein were measured, for the first month twice a week and hereafter twice a month until 2 months after normalization of the serum hCG level. From there on the mother's serum hCG will be measured every second month until the level of serum hCG has been normal for 14 months. On the 17th day postpartum the hCG was 510 iU/L dropping to 16 iU/L 36 days postpartum. Since there were no signs of dissemination, the mother was not treated with chemotherapy. Six months postpartum, both mother and child are doing well, showing no signs of malignant disease. |
Here, we present a case of a 49-year-old man with a history of right lung mass proven by biopsy to be a nonsmall cell lung cancer (squamous cell carcinoma). The first and second dose of paclitaxel (200 mg m2) combined with cisplatin was infused within 1 h with standard premedications. An 18F-FDG PET/CT scan performed 3 weeks after the second cycle of chemotherapy revealed multiple hypermetabolic skin lesions at several parts of the body including scalp, neck, chest, abdomen, and back, right inguinal region with maximum standardized uptake value of 4.31 [, arrows] mimicking probable skin metastases in addition to hypermetabolic mass in the right lower lobe and hypermetabolic mediastinal lymph nodes. On physical examination, severe painful and pruritic rashes on his scalp, neck, chest, abdomen, and back were inspected. It was retrospectively learned that he developed a widespread paclitaxel-induced drug eruption during the therapy within 2 days after the administration of the second cycle. Radiologic and clinical examination by a dermatologist indicated first the drug HSR as the underlying cause of these hypermetabolic skin lesions. Biologic tests revealed no autoimmunity. He reported not to have any skin lesions after the prior cycle of chemotherapy, and there were not any skin lesions at the first 18F-FDG PET/CT performed for staging, as well. Based on the clinical course and physical examination, skin biopsy did not need to be performed. Successful symptomatic management with oral (dexamethasone 4 mg/day) and topical treatment produced prominent regression of the lesions. The patient was re-exposed to taxanes through desensitization protocol, and these allowed him to complete the six courses of therapy that includes paclitaxel. Subsequently, 18F-FDG PET/CT performed after 3 weeks after completion of six cycles of chemotherapy showed resolution of the skin lesions in addition to the regression in primary malignancy [, arrows], which correlated with clinical resolution of the skin lesions without any scarring. |
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