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A 62-year-old woman with good past health with no previous neurological symptom was admitted to the Prince of Wales Hospital in Hong Kong after a sudden onset of left upper limb and lower limb weakness along with unsteady gait and numbness. Examination upon admission revealed brisk reflexes over both lower limbs, reduced pinprick sensation with a sensory level at T3 over left side. Her blood pressure was 141/74 mmHg with no history of smoking, hypertension, diabetes or dyslipidemia. An MRI scan of the whole spine showed extensive intramedullary hyperintense signals at C2 to T3 levels; most discrete at the T2 level. On the other hand, no lesions were found on the MRI of the brain. The severity of the spinal lesions and lack of brain lesions prompted an immunological investigation. The patient tested positive for anti-aquaporin-4 antibody and anti-SSA antibody using immunofluorescent test which confirms the diagnosis of NMO. In addition, her rheumatoid factor and antinuclear antibody were both negative; and there were no clinical features of keratoconjunctivitis sicca or dry month suggestive of Sjogren's syndrome.\nA magnetic resonance angiography (MRA) scan of the brain revealed a bilateral distal occlusion of the internal carotid arteries. Occlusions of the MCA were noted with fine collaterals in the region—reminiscent of moyamoya vessels. The ICAs and ACA were preserved; and the CCAs were normal without atherosclerotic changes. She was diagnosed with moyamoya disease as well (Fig. ).\nDespite treatment, leg weakness showed no significant improvement and further deterioration of leg power was witnessed with the left leg deteriorating more than the right. Intravenous methylprednisolone 1 g daily was given for 3 days. Afterwards, she was transferred to rehabilitation after 19 days in hospital with oral prescription of 30 mg of prednisolone daily together with azathioprine 50 mg daily. On follow-up 1 month later, she had suboptimal recovery with lower limb power remained Medical Research Council (MRC) Grade 3+/5 with spasticity and walk with frame for short distance. Blood test also revealed elevated alanine aminotransferase (ALT) 161 U/L while on azathioprine 50 mg daily. Therefore, azathioprine was stopped. Then, she received six courses of monthly cyclophosphamide (750 mg every month). She had gradual improvement in her neurological status with lower limb power MRC Grade 5/5 with reduced spasticity and able to walk with stick. Interval MRI brain and whole spine with contrast showed resolution of spine lesion and one new small non-contrast enhancing lesion over right cerebral peduncle. Clinically she remained well and she was re-challenged with low-dose azathioprine 25 mg daily since September 2013. Prednisolone was currently kept at a low dose 10 mg daily and planned to further taper down if she could tolerate higher dose of azathioprine. |
A 72-year-old male presented with a history of weakness and constipation, for which he underwent colonoscopy, revealing a submucosal mass in the right colon, compressing on the lumen extrinsically. The mucosa overlying the mass was intact and colonoscopy showed an adjacent 12 mm polyp, which turned out to be a tubular adenoma. He had history of removal of previous tubular adenomas on prior colonoscopies. Computed tomography (CT) imaging showed a 7.0 cm lobulated mass arising from mid-ascending colon with exophytic growth and foci of calcifications. CT findings were consistent with a mesenchymal tumor and carcinoma was less likely. No fatty component was reported (). Subsequently, the patient underwent laparoscopic right hemicolectomy and tumor was resected.\nGross examination of the right hemicolectomy specimen demonstrated a firm and calcified intramural mass in the posterior wall of ascending colon, extending into the mesocolic fat, without involvement of the mucosa. Microscopically, distinct histological patterns were identified, with the majority of the tumor being composed of a central ossified core surrounded by a minor component of a high-grade mesenchymal nonlipogenic tumor and adipose tissue. The central bone forming component showed areas of osteoid matrix with highly atypical cells resembling a high-grade osteosarcoma as well as areas of more mature appearing woven bone trabeculae with intervening mildly atypical spindle cells similar to a low-grade osteosarcoma (LGOS). A thin rim of a high-grade sarcoma composed of atypical spindled and pleomorphic cells was identified in the periphery of the bony mass. In addition, small foci of mature-appearing adipose tissue containing rare atypical hyperchromatic stromal cells were detected adjacent to the high-grade sarcoma and at the retroperitoneal resection margin, raising consideration of a WDL (Figures and ). The findings suggested that WDL may have arisen in the retroperitoneum and secondarily involved the colon with a DDL component with heterologous osseous differentiation.\nOn immunohistochemical (IHC) studies, SATB2 was diffusely positive in the atypical cells between bony trabeculae and in high-grade sarcomatous areas without evident bone formation. High and low molecular weight keratin (pancytokeratin), CD117, DOG-1, desmin, SMA, and S-100 stains were negative. Fluorescence in situ hybridization (FISH) revealed high-level amplification of MDM2-gene, supporting the diagnosis of retroperitoneal WDL with DDL showing heterologous osteosarcomatous differentiation and forming a mass involving the ascending colon. |
A 57-year-old right-handed female presented with a 4-year history of right leg weakness with equinovarus, and a reduction in grip strength in the right hand. Weakness was such that she had to lift her right leg in and out of her car with her hands. The following year, she began to experience numbness in the right hand as well as low back pain and urinary urgency. A course of intravenous methylprednisolone provided no benefit. Her condition slowly progressed but remained unilateral after 18 years, with no evidence of bulbar dysfunction. There have been no persistent sensory symptoms, though she has complained of cold extremities and acrocyanosis.\nThe patient was an ex-smoker. Her only past medical history of note was of curative (local) treatment for ductal breast carcinoma (11 years after onset of neurological symptoms). There was no family history of neurological disease.\nThe gait was spastic and hemiparetic, but ambulation was unaided. There was a pyramidal catch in the right upper limb and obvious spasticity in the right lower limb. Mild pyramidal weakness (Medical Research Council (MRC) grade 4) and hyperreflexia were noted in the right upper and lower limb.\nThere was an asymmetrical spastic paraparesis, worse on the right, and requiring a frame to ambulate. There were early flexion contractures of the fingers in the right hand with marked hypertonia in the right upper and lower limbs. Pyramidal weakness was noted in the right upper (MRC grade 4 proximally and grade 3 distally) and lower limb (MRC grade 3). Pathological hyperreflexia was now also evident in the left lower limb, but the left plantar response was flexor whereas the right was extensor.\nThe patient had begun using a wheelchair after fracturing the right radius and ulna in a fall, and had been catheterised due to impaired mobility. She had evolved significant amyotrophy in the right hand and forearm. |
A 36-year-old Brazilian male patient was admitted to the hospital with a palpable lump in his right breast, located at the junction of the upper quadrants of the right breast (Fig. ). On physical examination, the lesion appeared firm with irregular margins. Axillary lymphadenopathy was negative and there were no palpable supraclavicular nodes. On breast imaging, ultrasonography showed a hypoechoic mass with partially defined contours measuring 4.0 × 3.0 cm, located at the upper region of the right pectoralis major muscle at the 12 o’clock position with muscle infiltration (Fig. ). Histological examination of core biopsy samples revealed a malignant tumor. Preoperative exams, such as X-rays and chest CT scan, abdominal US did not show any signs of disease. Radical mastectomy was then performed, due to pectoralis major muscle infiltration, consisting in removal of the breast along with the major and minor pectoralis muscles. Biopsy of the sentinel lymph node was performed. Gross examination revealed a solid tumor measuring 3.7 × 3.5 cm with a yellowish-tan cut surface and local foci of hemorrhage. Histopathology showed intravascular papillary proliferation of endothelial cells, spindle cell areas and necrosis, atypia and prominent mitotic figures, consistent with the diagnosis of high-grade angiosarcoma with areas of infiltration of the pectoralis major muscle (HE staining, magnification of 400×) (Fig. ). Histopathology also demonstrated a surgical specimen with clear margins, absence of angiolymphatic and perineural invasion, in addition to sentinel lymph node free of metastasis. Immunohistochemical study revealed a tumor positive for CD31 marker (Fig. ), confirming the vascular nature of the tumor. At the two-week follow-up of the surgical procedure, adequate wound healing was observed, without any evidence of the disease. The patient was transferred to the clinical oncology department, where he presented with severe headache and seizures after the second cycle of adjuvant chemotherapy with paclitaxel. Magnetic resonance imaging of the brain was ordered, revealing a right frontal parasagittal lesion, measuring 1.3 × 1.1 cm with a hemorrhagic component and perilesional edema, suggestive of brain metastasis. The disease progressed rapidly, culminating in the patient’s death at 20 days after the onset of neurological symptoms. |
A 49-year-old male patient hypertensive, smoker with positive family history of premature coronary artery disease who presented with heavy retro-Sternal chest pain.\nAt admission, the patient had no chest pain. Physical examination revealed a blood pressure of 130/85 mmHg and heart rate of 85/minute. Cardiac and respiratory examination were unremarkable and there was no carotid bruit. Electrocardiogram showed sinus rhythm with Q waves and T-wave inversion in lead II, III, and aVF (). Echocardiogram showed a left ventricular ejection fraction of 60% with no regional wall motion abnormalities, moreover, it showed normal structure and function of the cardiac valves. No intracardiac thrombi were found. Cardiac biomarkers were elevated.\nThe patient underwent cardiac catheterization and was found to have one vessel disease with 90% stenosis of the right coronary artery. A drug-eluting stent was successfully implanted in the right coronary artery ().\nOne hour after intervention, the patient reported acute painless blurred vision in the left eye associated with a lower visual field defect. History from the patient revealed no prior visual complaints or amaurosis fugax. Ophthalmological examination of the left eye showed visual acuity of 6/60 with normal anterior segment and intraocular pressure. Visual field testing showed complete inferior visual field defect in left eye. Fundoscopic exam showed moderate macular edema and a small yellow arterial embolus at the level of the optic nerve head ().\nFundus fluorescein angiography () revealed a 7-s filling delay in the superior-temporal retinal artery; findings consistent with hemi-central retinal artery occlusion.\nA pars plana vitrectomy eye surgery under local anesthesia was performed. Massaging of the superior retinal artery wall over the embolus using a silicone-tipped aspiration cannula was performed in conjunction with induced ocular hypotony, which resulted in dislodgment of the embolus and migration into the peripheral circulation. The patient noted an immediate marked improvement in his vision. Four weeks later, the patient had a visual acuity of 20/25 and normal visual fields. There was complete resolution of the embolus and the artery occlusion and restoration of retinal blood flow ().\nThe study was approved by the Institute Research Board at King Abdullah University Hospital and Jordan University of Science and Technology. The work has been reported in line with the SCARE 2020 criteria []. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. |
A 67-year-old man with a 40 pack-year smoking history initially presented with chills and a productive cough and was given antibiotics for presumed pneumonia. When his condition did not improve, a computed tomography (CT) of the chest was performed and revealed a large right hilar mass with extensive mediastinal adenopathy as well as surrounding infiltrate and atelectasis. Bronchial brushings and a right hilar node FNA were consistent with SCLC. The remainder of the workup, including brain magnetic resonance imaging (MRI), bone scan and positron emission tomography (PET)-CT, was negative for distant metastatic disease, establishing a diagnosis of limited stage (LS) disease. His medical history was significant for numerous coexisting medical conditions including a remote history of suspected but unconfirmed connective tissue disorder (CREST), colitis, esophagitis, duodenitis, livedo reticularis, Hashimoto’s thyroiditis, multinodular goiter, arthritis, glaucoma, hypertension, multifocal motor neuropathy and atrioventricular (AV) nodal reentry tract for which he had undergone AV nodal ablation. Of note, neither the Hashimoto’s nor CREST were active for multiple years leading up to his diagnosis of SCLC. The latter diagnosis had been suspected by the Rheumatology Service but after a negative workup, he was discharged from their clinic.\nAfter completion of staging, he was advised to undergo definitive chemoradiation. He was also advised to re-establish follow up with the Rheumatology Service, but declined. After a detailed discussion of the potential for increased risk of complications from radiation with an underlying connective tissue disorder, he elected to proceed. He was treated with intensity modulated radiation therapy to the right lung and mediastinum in 33 daily fractions of 200 cGy to a total dose of 6600 cGy with two cycles of concurrent cisplatin (cis) and etoposide. After 3000 cGy, another CT was performed to allow for decrease in treatment field after initial response. RT was completed in 8 wk and 1 d. A representative image from his intensity modulated radiation therapy (IMRT) radiation plan is presented in (Figure ). His treatment course was complicated by pancytopenia (for which he received filgrastim and one unit of packed red blood cells), as well as dysphagia and odynophagia. He received two cycles of chemotherapy during the radiation and two cycles in the adjuvant setting after concurrent chemotherapy and radiation therapy, though the last three cycles were dose-reduced because of hematologic toxicities. During chemotherapy he was treated for clostridium difficile colitis and was briefly admitted for generalized weakness. Approximately three months after completion of thoracic RT, he received prophylactic cranial irradiation (PCI) which was given as 10 fractions of 250 cGy.\nDuring PCI, he required admission due to inability to tolerate daily travel. Shortly after completion of PCI he developed recurrent clostridium difficile colitis and within weeks of completion of PCI he was readmitted and remained hospitalized for two months. While admitted, he experienced episodes of hypoxemic respiratory failure requiring repeated use of a nonrebreather and for which he underwent intubation twice. Chest imaging demonstrated development of worsening bilateral interstitial and airspace opacities (Figure ). He was aggressively treated with broad spectrum antibiotics and high dose steroids. Eventually he developed tachycardia, respiratory distress, hypotension and suspected disseminated intravascular coagulation. In accordance with his family’s wishes he underwent palliate extubation and expired shortly thereafter.\nAn autopsy was performed and the report described extensive, diffuse, bilateral alveolar damage consistent with post-radiation changes, as well as small cell carcinoma in multiple foci within septal capillaries and contiguous alveolar spaces. |
A 35-year-old man presented at a local hospital with epigastric pain. Esophagogastroduodenoscopy (EGD) showed that an IVC filter strut had penetrated the third portion of the duodenum (arrow, Fig. ), and this was confirmed by computed tomography (CT) (arrow, Fig. ). In order to retrieve the IVC filter, the patient was referred to our department. He had a history of testicular cancer with para-aorta lymph node metastasis. Left renal vein thrombosis developed because of neoadjuvant chemotherapy before RPLND, and anticoagulants were administered before RPLND. Three years previously, he had undergone left orchiectomy, retro-mediastinal lymph node dissection, and RPLND at the previous hospital. The left common iliac vein was intraoperatively damaged during RPLND. Because the previous surgeon was worried about the high incidence of postoperative DVT and PTE, anticoagulant therapy was continued after RPLND. However, because DVT developed in the left common iliac vein after the initial surgery, a retrievable IVC filter (ALN, France) was placed in the IVC caudal to the renal vein to prevent PTE, and the patient had been receiving anticoagulant therapy. Because follow-up CT after IVC filter placement showed that DVT persisted at the left common iliac vein despite anticoagulant therapy, the IVC filter could not be retrieved at the previous hospital.\nEnhanced CT also revealed that DVT remained in the left common iliac vein. Because ultrasound examination showed organized DVT, an IVC filter was considered unnecessary. An endovascular approach was considered unfeasible for retrieval because two of the filter struts had penetrated the duodenal wall. An extensive discussion with an internal medicine specialist was performed. Because DVT remained with no remarkable changes in CT images for 3 years and the incidence of PTE caused by DVT in the left common iliac vein would be low, we chose surgical treatment for this patient in order to prevent bleeding at the duodenum.\nThe IVC filter was retrieved through cavotomy, and the duodenal penetration site was repaired using intraoperative EGD clipping. The operation lasted 5 h and 54 min, and the intraoperative bleeding volume was 1172 mL. Because it was not possible to mobilize the duodenum due to adhesions resulting from the previous surgery, the IVC at the sites caudal to the renal vein could not be explored. However, a mesenteric incision caudal to the third portion of the duodenum enabled encircling and taping of the IVC (Fig. ). After clamping the IVC cranial and caudal to the duodenum, a 5-cm vertical incision was made on the IVC cranial to the duodenum and the IVC filter was retrieved (Fig. a). Although the head of the IVC filter had penetrated into the IVC intima, we were able to bluntly peel the filter head from the intima. The IVC incision was closed using a continuous 5-0 Prolene suture (arrow, Fig. b). The IVC clamping time was 22 min. Intraoperative EGD revealed no bleeding at the duodenal penetration site (Fig. ). To prevent duodenal bleeding or perforation, the penetration site was repaired by EGD clipping. Retrieved IVC filter was presented as (Fig. ). The patient did not develop any postoperative complications and was discharged on postoperative day 16. |
A 24-year-old female presented to the emergency department shortly after a fall from a fourth floor window in 2012. On full assessment, there were multiple lower rib fractures on the right side (), with a right hemopneumothorax, a flexion-extension injury of 11-12th thoracic vertebrae, and a pelvic bone fracture. An intercostal drain was inserted and emergency vertebroplasty and internal fixation of the pelvis were performed. She was making excellent recovery; however, on the 13th postoperative day, after incentive spirometry exercise and coughing, she became acutely unwell and complained of breathlessness. On examination, she was found to be hypotensive and tachycardic, with a dull right hemithorax and decreased air supply over the right lung field. She was resuscitated and a chest radiograph confirmed a white-out of the right hemithorax (). Initially, 1.5 L of blood was drained from an inserted intercostal drain, and drainage continued at more than 100 mL/h. As the nature of the underlying injury was uncertain, we conducted an urgent computed tomography that revealed absence of intrathoracic vascular injury. Fluid resuscitation for hemodynamic stabilization was administered due to a suspicion of bleeding from intercostal vessels. This was followed by an urgent thoracoscopic exploration with a double-lumen endotracheal tube, performed through the previous intercostal drain site, and 2 additional working port sites in the 4th and 8th intercostal space along the posterior axillary line. The right pleural cavity was full of blood clots, amounting to approximately 2 L. The mediastinum, intercostal vessels, and lungs had no evidence of penetrating injury or inward displacement of rib fragment. Active arterial bleeding was found from a tear of musculophrenic artery in the dome of the right hemidiaphragm, corresponding to a broken right 11th rib end. We used a 10-mm aspiration/irrigation catheter that enabled us to perform an accurate lavage of the pleural cavity, and thus rapidly identify the bleeding sites with sufficient accuracy. Immediate hemostasis was achieved with thoracoscopic bovie cauterization (). The patient made a completely uneventful postoperative recovery. |
A 34-year-old woman of Caucasian origin was referred to the outpatient clinic of our department with a rash on the right side of her face. At the age of five years, multiple asymptomatic skin-colored firm papules developed in a unilateral configuration on the right side of her face. The rash was asymptomatic but of cosmetic concern to the patient. The family history was unremarkable. The patient was otherwise healthy.\nPhysical examination showed skin-colored papules ranging from 2 to 8 mm in size localized on her mid-forehead, the right eyebrow, the eyelids, down the right cheek, and the right nasolabial fold (). The lesions were nontender, smooth, and firm. No other skin lesions were noted. Contact polarized dermatoscopy revealed the presence of bright white linear streaks on an ivory-white background as well as yellow and light brown dots and clods ()\nHistological examination of a skin biopsy showed a well-demarcated dermal tumor composed of anastomosing lobules of basaloid cells of uniform size arrange in an organoid pattern. The cells were small, with regular nuclei, surrounded by prominent cellular stroma. Small keratinous cysts lined by stratified squamous epithelium were also noted. No connection with the overlying epidermis was noted ().\nA lesion in the nasolabial fold was excised in toto and the patient was started on topical treatment with imiquimod 5% cream. The patient applied a thin layer of imiquimod 5% cream prior to normal sleeping hours, left on the lesions for about 8 hours, and then removed by washing the area with mild soap and water, 3 times per week. At the follow-up visit, after three months of treatment with imiquimod 5% cream less than 25% of the lesions at the baseline examination were still present but we also observed a size reduction of the remaining lesions by 1 to 2 mm. The patient was satisfied from aesthetic point of view as she considered that the lesions were less prominent. No side effects were reported during treatment (). |
A 17-year-old unmarried female presented to our resource-constrained clinic in a remote part of northern Pakistan, with a history of painless and progressive abdominal distension. Over the past four months, the patient’s abdomen had progressed to a diameter of 130 cm, restricting her ability to breathe and causing shortness of breath on exertion and on lying down. There was no significant personal or family history.\nSocioeconomic factors such as lack of healthcare access and poor health literacy led to a delay in presentation. Also, it was important to acknowledge the sociocultural factors involved that affected the patient and her family. Community allegations of extramarital pregnancy had resulted in ostracization of the family and significant psychological distress to the patient, who appeared with a flat affect, depressed mood, poor eye contact, and reported frequent suicidal ideation due to the social pressure.\nOn examination, the patient was hemodynamically stable but dehydrated and pale. The abdomen was massively distended (130 cm), tense, non-tender, and prominent veins were seen on the anterior abdominal wall. However, the huge mass was palpable due to its sheer size the upper and lower limits could not be defined. Chest examination revealed symmetrical expansion with adequate air entry bilaterally, however, the patient was unable to take deep breaths because of likely splintage of the diaphragm.\nChest X-ray revealed rib crowding and elevation of the diaphragm to T5 (from its usual position of T8-T12). Abdominal ultrasound revealed a huge cystic and lobulated mass with internal septations occupying the whole abdominal cavity, most likely arising from the right ovary, along with a mild amount of fluid in the peritoneal cavity. An abdominal CT scan and CA-125 were also offered but rejected by the family due to cost restrictions as these investigations could not be performed locally.\nSurgical excision was planned under regional anaesthesia due to the impact of the abdominal mass restricting respiration and lung capacity relatively contraindicating general anaesthesia. Incision extended from xiphisternum to symphysis pubis. A huge mass was seen originating from the right ovary with flimsy adhesions between mass and intestine/omentum which were easily freed. It was removed in total, sparing ipsilateral fallopian tube and ovary. Surrounding viscera were found to be uninvaded, which favoured the benign nature of the lesion. Furthermore, no metastases were observed in any organ. Due to pressure effects, the posterior aspect of the anterior abdominal wall was very hyperaemic. Two drains were placed in the peritoneal cavity that were removed on the third post-operative day. The patient’s recovery was uneventful, and she was discharged on the seventh post-operative day with suture removal and good wound healing observed on the 14th post-operative day. The patient and family were extremely satisfied and relieved. During pre-operative work, the father had mentioned several times that the patient had developed suicidal thoughts due to social stress. There has been no recurrence of tumour growth at four months follow-up.\nMacroscopic examination revealed a 13 kg specimen (30 cm x 30 cm x 26 cm), however, histopathology was refused by the family who instead requested to take the specimen home to demonstrate as evidence to their family and community members to prove that her abdominal distention was secondary to a tumour and not a foetus (Figures -). |
A 40-year-old nulliparous woman of South Indian origin reported to our outpatient clinic with complaints of a mass in the lower abdomen for one year and lower abdominal pain for three months. The patient was apparently asymptomatic one year previously, and then she noticed a mass in the lower abdomen that gradually increased in size. She provided a history of a rapid increase in size for the past three months. She also had associated lower abdominal pain, which was dull and aching in type, dragging in nature and continuous with no aggravating or relieving factors. Her menstrual cycles were regular and normal. She had no history of genital bleeding.\nOn examination, pallor was present, and the patient's vital signs were normal. She was thinly built. On abdominal examination, an irregular midline mass arising from the pelvis was present. The upper and lateral borders of the mass could be made out; the lower margin could not be ascertained. The mass was firm to hard in consistency with restricted mobility and nontender with no free fluid. There was no hepatosplenomegaly.\nOn vaginal examination, the patient's uterus was enlarged to 20 weeks' gestational size and nodular, occupying the whole pelvis. No mass could be appreciated separate from uterus. Computed tomography (CT) scan findings were suggestive of a large, multiloculated, multiseptate growth containing both solid and cystic elements arising from the pelvis and extending into the abdomen, measuring 25 × 17 cm, adherent to both the uterus and bladder. CA125 was 94.80 ng/mL (0-35 ng/mL). Other results were normal, and the patient was posted for exploratory laparotomy.\nIntraoperatively, omental adhesions to the mass were noticed. A mass of 20 weeks' size was arising from the fundus of the uterus (Figure ). It was lobulated (Figure ) with solid, cystic and hemorrhagic components. Both ovaries were normal, and the fallopian tubes were edematous (Figure ).\nTotal abdominal hysterectomy (along with tumor) with bilateral salpingo oophorectomy was done (Figure ). The uterus measured 8 cm × 5 cm × 3 cm with a subserosal bosselated growth from the fundus measuring 18 cm × 15 cm × 11 cm with variable consistency. Omental biopsy and external iliac lymph node biopsy were taken and sent for histopathologic examination. Histopathologic examination showed a cellular tumor arranged in interlacing bundles of spindle cells with elongated hyperchromatic nuclei. The tumor cells were exhibiting moderate pleomorphism and bizarre nuclei with multinucleate tumor giant cells. There were scattered areas and normal and abnormal mitotic figures (> 4/high-power field) with marked nuclear atypia suggestive of uterine leiomyosarcoma (Figure ). The omentum and external iliac lymph node were free from tumor cells. |
The patient was a 36-year-old male, admitted to a specialist trauma center on 24 December, 2019, at 21:00, having suffered wounds caused by a cold weapon. He had exhibited hemodynamic instability during transport, but after fluid resuscitation he was stable when he arrived at the hospital. The most notable findings of the physical examination conducted in the emergency room were subdued vesicular murmur in the left hemithorax, heart rate of 125 bpm, mean blood pressure (MBP) of 70-75 mmHg, and multiple wounds to the anterior and posterior chest ().\nAfter administration of packed red blood cells and prophylactic antibiotics, angio-CT of the thorax was conducted (\n), showing contrast leakage from the thoracic aorta and left sided hemothorax. Left pleural drainage and arteriography of the thoracic aorta were performed.\nThe decision was taken to proceed directly to definitive endovascular treatment. Intraoperative aortography () was performed, confirming the damage to the thoracic aorta (at the level of T8) with adjacent tamponade, and a 20/20/82 mm straight thoracic endograft was deployed in the descending thoracic aorta. The endovascular intervention was conducted at 01:00 on December 25, 2019. Control aortography performed after implantation () showed no evidence of contrast leakage.\nThe patient remained in the intensive care unit during the immediate postoperative period. On the first postoperative (PO) day, the patient suffered acute myocardial infarction of the lateral wall, but without compromise to cardiac or hemodynamic function. He was discharged to the wards on PO day 3. The patient had a collection in the left hemithorax due to retained hemothorax, and underwent videothoracoscopy and decortication, conducted by the thoracic surgery team. His recovery was otherwise uneventful and he was discharged from hospital on the 10th day after admission. He is currently in outpatients follow-up with the specialties and is asymptomatic. He had a control chest angio-CT 30 days after the trauma, which found no evidence of complications related to the surgical procedure. |
A 67-year-old woman presented with half a year history of dizziness. The dizziness worsened over the last 6 months, and the dizziness could be initiated by moving backward the neck. At the beginning of the disease onset, She had no other presentations, including vomiting, cognitive impairment, unconsciousness, and dysphagia. 6 months before admission, she was hospitalized at a local community hospital, and then, she was scanned with routine brain CT and neck MRI. The neck and brain imagings showed the disk herniation of the C1-C2, C3-C4, and some lacunar infarction in the bilateral basal ganglion, respectively. The local community hospital gave her routine anti-platelet and antihyperlipidemic medications for the lacunar infarction.\nAfter 4 weeks of treatment, the dizziness symptom did not improve, and the patient was then referred to the department of neurology of the Shanghai Sixth people's hospital for further neurological consultation. She complained the dizziness worsened over the last 2 months and weakness of her right lower limb. An additional brain MRI enhancement scan and brain CT angiography scan were performed. The MRI scan indicated that there was an increased signal intensity of the bilateral frontal-temporal-parietal cortex on the diffusion-weighted imaging(DWI) sequence and brain atrophy (Figure ). In contrast, there was a decreased signal intensity of the counterpart cortex on the apparent diffusion coefficient (ADC) sequence. Brain CT angiography showed normal. At that time, the doctor of the radiology department considered it as acute brain infarction. There were no abnormalities on her full blood test, renal, liver, thyroid function, erythrocyte sedimentation rate, and antinuclear antibody panel. The electrolyte examination showed that her blood potassium was 3.1 mmol/L (reference range 3.5-5.5). The previous history revealed that she had half a year of diabetes, and she regularly took metformin to treat diabetes. She did not have a history of smoking and any other drug or alcohol overuse. She also had no significant family history of neurodegenerative diseases. The ultrasound showed there were plaques on her bilateral carotid arteries. The physical and neurological examination showed she had normal blood pressure and weakness of the right distal lower limb (Medical Research Council grade 4/5 ankle dorsiflexion and eversion and grade 4/5 ankle plantar flexion and inversion). The other tests were all within the normal range. Then, the patient was continued to be treated as acute cerebral ischemia with clopidogrel, atorvastatin, and butylphthalide to anti-platelet and lower the level of cholesterol for the next 2 weeks. However, no remarkable improvement was observed during the period of hospitalization. The patient was discharged after 2 weeks of treatment.\nFour months later, the patient experienced a hip fracture and underwent a hip replacement operation. After that, the patient was unable to ambulate and was confined to the bed at home. Meanwhile, her cognitive impairment progressed significantly and became fearful, nervous, and mute. The symptoms worsened progressively in the next 2 weeks. The patient was referred to our department again. The physical evaluation revealed she had startle-related myoclonus, mutism, and hyperreflexia. The patient had little interaction with the family members, and had her eyes open but not tracking a face moving across the visual field. She could not cooperate with the Mini-Mental State Examination. The patient sometimes moved and hold her bilateral upper extremities voluntarily in the air for no purpose. The electroencephalography (EEG) test was performed, and previous MRI images were reviewed. The EEG showed there were classical synchronous triphasic sharp waves over the bilateral frontal, temporal, occipital regions. (Figure ). Creutzfeldt-Jakob disease seems probable based on the new-onset symptoms. However, the family member refuses to do the lumbar puncture for further CSF analysis to confirm the diagnosis. Due to the lack of pathological results, the patient was finally diagnosed as probable sporadic CJD based on the previous medical history, neurological findings, and MRI scan, which fulfilled the diagnostic criteria of sCJD. The patient was empirically treated for the muscle rigidity with eperisone hydrochloride and then transferred to the local center of disease control (CDC) to accept further treatment due to the infectivity of this disease. |
A 47-year-old male, Turkish-Muslim patient living in Germany received a hair transplant for baldness. Three months after the operation, he started having feelings of guilt, reluctance, decreased interest in life, inability to accomplish his religious responsibilities and had decreased levels of energy. He had no previous history of psychiatric disease. He believed that his state of baldness was how God had created him; therefore, he was feeling sinful for having acted against God’s will by receiving a hair transplantation. He talked about his religious struggle with a Turkish imam in Germany but that did not comfort him. The imam advised him to see a psychiatrist. However, the patient did not want to see a non-Muslim physician. Therefore, he was brought to Turkey for psychiatric treatment by his brother in order for a Turkish-Muslim physician to be able to persuade him and change these religious guilt–related thoughts. He was examined by the first author. He was looking older than his age and had decreased eye contact. He had depressed affect congruent with his depressed mood. His speech was monotonous. He had ruminations about having made a sin with his decision to have a hair transplant. He stated that, he was self-conscious when he made his decision to get the operation without anyone forcing him to do so. He believed he was guilty because getting the operation was not an unforeseen event but a planned rebellious act against the way God had wanted him to be. The physician tried to reframe these thoughts as cognitive distortions related to depressive disorder and offered antidepressant drug treatment combined with cognitive psychotherapy. However, the patient refused any treatment because he believed he had deserved this suffering as a way of punishment. He returned to Germany; therefore, he was referred to a German psychiatrist who suggested hospitalization of the patient. Unfortunately, the patient declined all treatment options and killed himself via hanging. |
A 61-year-old man was presented with a large incisional ventral and left-sided groin hernia. He had a history of open cholecystectomy through a vertical midline laparotomy due to acute gangrenous cholecystitis two years ago. The patient was re-operated on due to evisceration on the next day. The postoperative course was complicated by a deep wound infection and secondary wound healing subsequently. Incisional hernia formed at the incision site later. The patient had a history of heavy smoking and controlled chronic obstructive pulmonary disease.\nOpen simultaneous repair of both hernias was done on April 23, 2007. A mesh sized 225 cm2 was applied for the incisional hernia, and 66 cm2 for the groin hernia repair performed by the Lichtenstein method. Starting from the first postoperative day, the patient`s temperature was elevated and hit a high of 38.0 °C in the evenings. Therapy with Gentamicin 240 mg was administered parenterally once daily, starting from postoperative day one. A brownish turbid exude through the drain appeared on the second postoperative day; besides, the drain fell out late in the same evening. Sutures were removed from the inguinal wound on the 4th day postoperatively because of infection signs, but the patient`s temperature did not normalize. A blind puncture of the incisional hernia site was done on the 7th postoperative day in order to examine the fluctuating mass. Brownish pus was aspirated. Bacterial testing confirmed the same microorganism growing as in the groin. Treatment according to the stepwise protocol () was initiated. A gradual improvement was observed. By the 6th day after the first puncture, the aspirate looked like seroma. The patient was discharged from the hospital on the 20th day postoperatively. The incisional hernia wound had healed by primary intention, and the open inguinal hernia wound caused by superficial infection healed spontaneously in six weeks.\nThe follow-up at 12 years after the treatment did not reveal signs of infection or both hernia recurrences. |
A 75-year-old woman complained of pain in her right hip. She had no history of trauma or other diseases. When she stood on her tiptoes and extended her hip joints to pick nuts from a high place, she developed right hip pain and fell. She was transported to the hospital by ambulance.\nOn initial examination, she was unable to move her right hip joint; it was fixed at 0° flexion, 30° external rotation, and 10° abduction. A plain radiograph revealed anterior dislocation of the right hip joint (). We performed a closed reduction using intravenous anesthetic 4 hours after trauma. The center-edge angles were 34° and 35° in the right and left hip joints, respectively, in a plain radiograph after closed reduction. These angles were normal. However, the vertical center anterior margin (VCA) angle was 20° on the right and 33° on the left in the false-profile view (); normal values are greater than 25° []. These findings revealed developmental dysplasia of the right hip. Slot radiography of the spine in the sagittal plane in a standing position revealed vertebral fractures of the thoracolumbar spine (). The T10-L2 thoracolumbar angle was 50.5°, compared to the normal mean angle of 8.6° []. This finding suggested thoracolumbar kyphosis. The sagittal pelvic tilt angle was 67° (the angle between a line connecting the sacral promontory to the pubic symphysis and the vertical line; the normal tilt angle is 19.47 ± 6.26° in men and 24.4 ± 5.93° in women []). This finding indicated severe posterior pelvic tilt. In the fluoroscopic examination, her lower extremity was extended manually and showed no mobility in the femoral head. Because she could resist the manipulation, we judged that there was no severe muscle atrophy around her hip joint. Computed tomography (CT) of the hip joints (axial plane) revealed no fracture or free body of bone. Furthermore, the bilateral femoral neck anteversion angles were −5° in both the right and left sides. These findings revealed a retroversion deformity of the proximal femur. Acetabular anterior coverage in the sagittal plane in a natural standing position and standing on tiptoes with hip extension was measured with a tomosynthesis imaging system (SONIALVISION safire with a flat panel detector of 17 × 17, Shimadzu Corporation, Kyoto, Japan). First, bilateral femoral heads were positioned perpendicular to horizontal line of the image under fluoroscopy. A tomosynthesis image was then taken with the beam centered on the femoral head. Angle measurement was performed with a three-dimensional work station (Virtual Place Lexus 64: AZE, Tokyo, Japan). Similar to analysis of the false-profile view, we measured the VCA angles at the slice where the anterior edge of the acetabulum was seen most clearly; the VCA angle of right hip joint was 17.0° in a natural standing position and 9.2° while standing on tiptoes with hip extension (), and it was 29.3° and 21.7°, respectively, in the left hip joint ().\nGeneral joint laxity was evaluated according to scores described by Carter and Wilkinson [], with negative results. There were no abnormal values on laboratory examination. Bone mineral density (BMD) of the lumber spine (L1-4, 0.61 g/cm2, T-score: −2.68 SD) measured with dual energy X-ray absorptiometry indicated the presence of associated osteoporosis.\nAfter reduction, the patient started partial weight-bearing walking exercises wearing a hip brace. Full weight-bearing walking exercises were started one month after reduction. A parathyroid hormone formulation was administered for treating osteoporosis.\nOn her final follow-up one year after the reduction, the patient was free from pain and was able to walk freely. She had full range of hip motion. Magnetic resonance imaging of the hip joints (T1-weighted image) revealed no osteonecrosis of the femoral head. The Harris hip score improved to 92. She has had no recurrence of dislocation and was extremely satisfied. |
A 40-year-old woman presented with a painless movement restriction of both shoulder joints, complicating activities in daily life such as personal hygiene and lifting her child.\nOne year ago, 5 days postpartum, respiratory insufficiency developed. She was admitted to the ICU and diagnosed with metastasized choriocarcinoma (lungs, liver, and kidney). Curative chemotherapy was started. She developed ARDS, further complicated by multiple organ failure, critical illness polyneuropathy, and bowel perforations, necessitating mechanical ventilation, alternating prone, and supine positions for several months for a better ventilation of the lungs. After 3 months, she was discharged and rehabilitation was initiated.\nOn examination, there was a global atrophy of shoulder musculature, with a fixed position of both shoulders at 20° of abduction and 45° of internal rotation. Shoulder movement was exclusively scapulothoracic. Active abduction was 60° bilaterally, and anteflexion was 45° in the right shoulder and 90° in the left shoulder (). No neurovascular deficits were present.\nX-rays of both shoulder joints showed an identical osseous mass between the humerus and scapula (). Magnetic resonance imaging (MRI) () and three-dimensional reconstruction computed tomography images () showed complete bony bridges, located in the teres minor muscles. Also, isolated ossifications in the subscapularis muscles and avascular necrosis (AVN) of both humeral heads were demonstrated. The AVN of the humeral heads was thought to be a consequence of the corticosteroid therapy at the ICU or, less likely, changes in vascularization due to compression or traction by the bony bridges.\nSurgical resections of the osseous bridges via posterior approaches in 2 separate sessions were planned, starting with the left, nondominant side (). Five months later, her right shoulder was operated in the same manner.\nA posterior vertical incision was made from the posterolateral edge of the acromion in the direction of the axilla. After dissecting the subcutis and fascia, the posterior part of the deltoid muscle was mobilized. A large ossification appeared, which coursed the route of the teres minor muscle. A release of the triceps muscle was performed to gain full insight into the ossification. The native scapula was identified and dissection continued to the posterior side of the humerus, craniolateral to the axillary foramen. The bony bridge between the humerus and scapula was completely freed. A vertical osteotomy was performed along the lateral margin of the scapula, using an osteotome. The radial nerve coursed flush behind the ossification and was carefully protected while removing the bone, sometimes necessitating the use of a punch (). Nearly complete resection of the bony bridge was achieved without nerve damage, which improved the abduction immediately in contrast to rotation, which showed no improvement. The absence of rotation was thought to result from a frozen shoulder, since the ossification of the subscapularis muscle did not connect with surrounding bones, as seen on the MRI. After gentle manipulation, full rotation could be achieved. The shoulder joint was infiltrated with a corticosteroid and a local anesthetic, and the deep and superficial layers were closed with soluble sutures. Postoperative treatment was functional without any movement restrictions, guided by pain and under the supervision of a physiotherapist. We prescribed a 2-week course of a nonsteroidal anti-inflammatory drug (NSAID; diclofenac 2dd 75 mg) to prevent recurrence of heterotopic ossifications.\nAt follow-up visits, the patient demonstrated a significant improvement of shoulder function with 160° of abduction bilaterally, of which 90° glenohumeral motion, 90° of internal rotation and 10° of external rotation. She was able to take care of her hygiene and lift her child again (). Strength of the rotator cuff was graded 5/5 when evaluated with the Medical Research Council Scale, and no postoperative complications, pain, or recurrence of HOs were noted on 1-year follow-up X-rays (). The AVN of the humeral heads was managed conservatively due to lack of symptoms. |
A 68-year-old female patient presented to the emergency department with refractory neuroglycopenic symptoms. In the emergency room, her glucose level was noted to be <50 mg/dL on multiple occasions despite repeated administrations of IV dextrose and PO intake. She described a 3-week history of episodes characterized by confusion, diaphoresis, and severe weakness which were improved with ingestion of food. She also described a weight loss of 30 pounds over the last 4-5 months despite normal appetite. She had no history of type 1 or type 2 diabetes mellitus and did not use insulin or antidiabetic medications. Medical history included hypertension and gastroesophageal reflux, and she did not endorse a history of smoking, alcohol, or recreational drug use. No pertinent family history was identified. On physical examination, she was found to have a palpable, mildly tender right upper quadrant mass and palpable hepatomegaly. A CT scan demonstrated evidence of an 18.6 cm mass projecting inferior to the liver and superior to the right kidney, in the approximate location of the right adrenal gland (). The mass was described as lobular and formed by 3 confluent mass lesions. No other suspicious lesions were described in the report. In addition, venogram showed severe extrinsic compression of the inferior vena cava secondary to the retroperitoneal mass.\nClinical workup of her hypoglycemia proceeded as follows (). Hypoglycemia was initially prevented with a maintenance dextrose drip; however, this was discontinued to allow clinical observation of hypoglycemic episodes ideally proving or disproving Whipple's triad. Whipple's triad includes a venous glucose level less than 55 mg/dL, symptoms of hypoglycemia, and resolution of symptoms after glucose administration. Dextrose drip was discontinued, her glucose level decreased to 47 mg/dL, and she became symptomatic. At that time, C-peptide, insulin, pro-insulin, and beta-hydroxybutyrate levels were measured and all were found to be low, suggesting IGF-2 secretion. IGF-2 was measured during that hypoglycemic episode but resulted within the normal range. This is classified as inappropriately normal because IGF-2 levels are expected to be suppressed in the setting of hypoglycemia (). The patient was given a 1 mg dose of glucagon, and her glucose level increased to 82 mg/dL with resolution of symptoms. Increase in glucose value of greater then 25 mg/dl represent adequate glycogen stores in the liver, yet repeated and persistent hypoglycemia infers that the patient's hepatocytes were not secreting glucose despite severe hypoglycemia. Dextrose drip was resumed to maintain safe glucose levels.\nScreening for use of oral hypoglycemic agents to rule out factitious hypoglycemia was negative. Antibodies to insulin were also undetectable. Our surgical colleagues recommended ruling out adrenal hypersecretion, as elevated precursor hormones are often seen in rapidly dividing adrenocortical carcinoma. Serum renin, aldosterone, 11-deoxycorticosterone, and 17-hydroxyprogesterone were all within normal limits. 5-HIAA and urine metanephrine levels were normal as well, which lessened suspicion for pheochromocytoma or neuroendocrine tumor. Although these conditions are not classically associated with hypoglycemia, we felt it would be useful to exclude additional causes of a large retroperitoneal mass.\nGiven the in-depth negative workup other than the abdominal mass, the decision was made to pursue resection. The gross pathology specimen () showed a mass weighing 2270 gm. Following removal, the patient's hypoglycemia resolved and 2 days later, IGF-2 was re-measured and found to be significantly lower than that measured preoperatively. During recovery, she had episodes of hyperglycemia, which could be explained by the sudden decrease in IGF-2 levels triggering a new equilibrium for glucose homeostasis.\nUnfortunately, IGF-1 level, which was drawn at the time of a hypoglycemic episode, could not be resulted because the sample was hemolyzed en route to the laboratory. By the time we were alerted, the patient was in pre-op for surgical resection. It would have been ideal to have this value, but this case highlights that clinical suspicion in the setting of appropriate imaging, low insulin, C-peptide, and beta-hydroxybutyrate, and an increase in glucose following glucagon injection point to an IGF-2-producing tumor. Finally, in the setting of an acutely ill patient, we anticipate a low/suppressed IGF-1 level.\nA follow-up CT scan one month after surgery demonstrated interval resection of the previously noted large, lobulated right suprarenal mass. There were no findings to suggest residual or recurrent disease. |
A 12-year-old girl presented with a 7-month history of frontal headaches and intermittent left-sided proptosis. On examination, she had dilated and engorged scleral veins on the left eye (evident on inspection), mild dysdiadochokinesia and past pointing on the left side. A brain computed tomography (CT) with contrast showed an enlarged left superior ophthalmic vein and a prominent left cavernous sinus. An interventional radiography (IR) cerebral angiogram under general anaesthesia was performed, which revealed evidence of an indirect CCF fed by ophthalmic branches of the left internal carotid artery and filling from multiple ethmodial branches of the distal internal maxillary artery. From the fistula, the ophthalmic vein was markedly hypertrophied and drained the cavernous sinus anteriorly. The fistula was also feeding from the right side through ethmodial branches into the right cavernous sinus and across to the left cavernous sinus (Fig. ).\nAfter a discussion that involved the neuroradiology team, neurosurgical team, the patient and her family, a decision was made to proceed with endovascular treatment of the fistula. Following informed consent from the patient's parents, the procedure was performed under general anaesthesia. Axis to the right common femoral artery was achieved with a 6-French micropuncture kit. A 6-French sheath was inserted into the right common femoral vein. A 6-French guide catheter was placed in the left internal jugular bulb. Thereafter, 4-French sheath was placed in the left common femoral vein. Then, a 4-French Weinberg catheter was placed in the left common carotid. A Headway microcatheter was advanced into the cavernous sinus trough the petrosal sinus into the cavernous sinus and subsequently into the dilated ophthalmic vein. Multiple coils were then placed into the ophthalmic vein throughout its course in the orbit back as far as the anterior section of the cavernous sinus. This resulted in near complete occlusion of the fistula. No immediate complications occurred, and the patient woke without any new neurological deficit in recovery (Fig. ).\nThe patient was discharged 3 days later and a follow-up brain magnetic resonance imaging (MRI) was conducted 4 months afterward, which showed some residual proptosis. The superior ophthalmic vein was prominent but clearly less prominent than previous imaging. On a routine outpatient follow-up, her proptosis had significantly improved with reduction of the corneal injection, she reported no headaches and normal vision. A follow-up IR cerebral angiogram was conducted 4 months later that revealed no evidence of a residual CCF. |
A 27-year-old male presented to our clinic complaining of pain in his right shoulder. The pain was constant but did not compromise his range of motion. Preoperative radiographs and computed tomography (CT) of the humerus revealed a lytic–sclerotic cortical lesion located at the lesser tubercle. The lesion was ~1 cm in diameter, and nidus formation was also apparent on CT (). Preoperative MRI revealed central sclerosis and edema of the surrounding bone. No cortical expansion or soft-tissue lesion was evident (). Whole-body Tc-99 bone scintigraphy revealed increased uptake by the lesser tubercle of the right proximal humerus ().\nThe lesion was excised with curette and high-speed burr with the radiological diagnosis of osteoid osteoma/osteoblastoma. The pathology was reported as “benign osteoblastic tumor”. However, the symptoms recurred after 2 months. A limited curettage was performed in the form of incisional biopsy. The pathological specimen again exhibited features consistent with osteoblastoma with epithelioid cells lining the trabeculae in focal areas. However, such an early local recurrence was found to be suspicious for a benign osteoblastic tumor, and a new Tc-99 bone scan was performed. This new whole-body scan revealed increased uptake in the right proximal humerus, as expected due to recent recurrence and surgical intervention and also in the lateral border of the body of left scapula, to the authors’ surprise (). Excisional biopsy with wide margins was performed for the scapular lesion, and the result of the pathology was consistent with OBLOS. The surgical margins were negative. Chemotherapy was initiated.\nFollow-up MRI after neoadjuvant chemotherapy revealed possible intraarticular and periarticular involvement of the right shoulder joint. Wide resection of proximal humerus including extraarticular resection of the glenohumeral joint was performed, and the defect was reconstructed with tumor prosthesis. The pathological specimen from the right proximal humerus was consistent with OBLOS (). The tumor necrosis rate was 90%. At 23 months post operatively, the patient was alive with no local recurrence or metastasis. |
A 40-year-old Asian-Indian male presented to our clinic with the complaints of sudden onset diplopia of 5 days duration associated with headache and vomiting. He was a known diabetic and was on oral hypoglycemic agents. On examination, his best-corrected visual acuity was OU 6/6, N6. On further examination, it was evident that he had a left pupil sparing oculomotor nerve palsy and loss of corneal sensations along with a firm mass palpable in the left temporal fossa. On questioning, patient gave history of presence of “painless progressively increasing bump” on his left temple for 35 years []. Magnetic resonance imaging (MRI) brain revealed a heterogeneously enhancing mixed intensity dumb-bell shaped lesion measuring 5.64 × 3.12 cm, with fat-fluid level noticed in the left orbit, along the superolateral aspect with the medial end extending up to the superior orbital fissure and anterior cavernous sinus. The lateral end of the cyst was extending into the infratemporal fossa []. In the anterior cavernous sinus, the lesion was compressing the oculomotor and the ophthalmic division of the trigeminal nerve. On the basis of the findings, a clinico-radiological diagnosis of frontotemporal intraosseous dermoid cyst was made.\nPatient underwent total excision of the dermoid cyst using a left infratemporal extradural approach. A formal temporal craniotomy was not performed; instead the lesion was approached through the left temporal bony defect due to erosion caused by the tumor itself. After excising the superficial part of the lesion with capsule, the microscope was used to further navigate and drill the bone to excise the lesion in toto. Under the microscope, left sphenoid bone was drilled up to the superior orbital fissure. The dermoid was extending to the left superior orbital fissure and reaching the left anterior cavernous sinus region where it was compressing the nerves in the lateral wall of the sinus. The dermoid cyst was soft yellowish with semi-solid cheese-like consistency with a thin-walled capsule. The capsule was radically excised and also scrapped off along with the periosteal layer of the bone. In the immediate postoperative period, his ophthalmoplegia and ptosis started improving.\nTwo months postoperatively, patient recovered well with marked improvement in diplopia and ptosis [] Follow-up MRI study showed postoperative changes with granulation tissue at the lateral wall of the cavernous sinus wall, without evidence of any residual mass [].\nFrontotemporal dermoids are the commonest group in the orbitofacial region accounting for 64% of cases in one of the largest series.[] They usually present as discrete slow growing masses, located superficially with no deep extension and can be managed by direct excision biopsy via an upper eyelid incision.[] Deep orbital dermoids may occasionally extend through the lateral orbital wall to secondarily involve the temporal fossa, sometimes in a “dumb-bell” or “collar-stud” pattern. In these cases, the intraorbital component of the dermoid usually predominates relative to the extraorbital component, and ophthalmic manifestations such as proptosis or ocular motility disturbances or both typically are present. Intracranial dermoid cysts typically increase in volume slowly over time and tend to grow in intracranial subarachnoid spaces that offer minimal resistance, such as cisterns, sulci or fissures.[] The growth rate of the dermoid cyst significantly affects the clinical presentation. Primary cavernous region dermoids present with oculomotor nerve palsy early in the course of the disease.[]\nHowever, our patient had the temporal swelling for many years preceding the onset of oculomotor paresis and is therefore a case of primary temporal dermoid extending to the orbit, superior orbital fissure and then anterior cavernous sinus; with MRI revealing the characteristic dumb-bell shaped tumor entering the orbit through a small bony window []. In our patient, the oculomotor nerve and the ophthalmic division of the trigeminal nerve were involved and symptoms were present only for 5 days prior to presentation suggestive of progressive growth and enlargement of a primary temporal dermoid. To the best of our knowledge this unique presentation of primary temporal dermoid has not been described so far in the literature and reiterates the continuous slow growing nature of the tumor.\nMagnetic resonance imaging permits the recognition of these lesions with their typical appearance as hyperintense signals on T1-weighted MRI and hypointense to mixed signals on T2-weighted MRI. Absence of peripheral edema due to slow growth with signal characteristics on T1-weighted and T2-weigted sequences helps in the differential diagnosis of dermoids from cystic glioma, arachnoid cysts or other lesions.[]\nExcision of temporal dermoids can be complicated by factors such as thin capsular wall, liquefied cyst contents, large size, rupture or leakage, adhesions to surrounding tissues, and variable involvement of bone with potential extension to the intraorbital or intracranial compartments. Where the cyst is attached to the bone, careful subperiosteal dissection is prudent as intraoperative rupture is common at these sites.[] If a portion of the dermoid is found to extend through a small bony aperture with pronounced expansion within the intraorbital or intracranial cavity, like in our patient, it is necessary to facilitate exposure by enlarging the bony opening to allow continuing dissection from the original approach. This was performed in our patient. Some surgeons prefer to open the second compartment by a formal lateral orbitotomy or craniotomy. |
A 57-year-old Japanese woman (gravida 4 para 4) presented with a 6-month history of light but intermittent postmenopausal vaginal bleeding without abdominal pain. Pelvic examination revealed a 12-mm hypertrophic endometrium via transvaginal ultrasound, and endometrial cytology revealed a few suspicious atypical endometrial cells; however, these findings did not confirm the diagnosis of EC (Fig. ). An outpatient endometrial biopsy performed with a curette followed by endometrial cytology also suggested that there was not sufficient pathological evidence to diagnose the presence of EC. However, contrast-enhanced magnetic resonance imaging (MRI) of the pelvis showed the disruption of the junctional zone in the posterior wall of the uterus and suggested myometrial invasion of the EC. FIGO stage IB EC was suspected (Fig. ). The discordance between the findings of the endometrial biopsy and MRI warranted a second endometrial biopsy to obtain histological evidence of EC. The second endometrial biopsy was performed with the aid of an outpatient diagnostic hysteroscopical vision using a 3-mm flexible hysteroscope (Olympus Corporation), but no obviously elevated mass was detected. We observed only irregular gradual eminence on the posterior wall (Fig. ). The obtained specimen contained only a small number of fused glands lacking stroma, which was not sufficient to conclusively confirm the presence of malignancy (Fig. ).\nWe subsequently performed an operative hysteroscopy with a resectoscope for tissue sampling under general anesthesia, since we hypothesized that the deep location and the hard consistency of the tumor made endometrial biopsy difficult. An irregularly elevated lesion was observed in the posterior wall of the uterine cavity using an 8.3-mm rigid hysteroscope (Olympus Corporation), and part of this lesion was incised with a loop electrode (Fig. ). This 5-mm-thick collected tissue included not only endometrium but also adjacent myometrium. Pathological examination of the hysteroscopically incised tissue showed a mixture of well-differentiated endometrioid adenocarcinoma of the uterine body and adenomyosis (Fig. ). This finding indicated the possibility of well-differentiated endometrioid adenocarcinoma of the uterine corpus with adenomyosis or well-differentiated endometrioid adenocarcinoma of the uterine body arising in adenomyosis.\nA total of 3 months after her first visit to our hospital, total abdominal hysterectomy and bilateral salpingo-oophorectomy with pelvic/para-aortic lymph node dissection were performed in our patient. Macroscopically, the endometrium was atrophic, and there was no obvious mass in the uterine cavity; however, microscopically, cancer cells were present in the deep myometrium and the final diagnosis was FIGO stage IB, endometrioid adenocarcinoma, G1, diffuse and endophytic type with adenomyosis (Fig. , ). As adjuvant chemotherapy, 6 courses of paclitaxel and carboplatin (paclitaxel, 175 mg/m2; carboplatin, area under the curve of 6) were administered due to deep myometrial invasion of the cancer. The patient has been free from recurrence for over 1 year since laparotomy. |
A 66-year-old man presented with complaints of intermittent high-grade fever and generalized dull-aching abdominal pain for 3 months. He had a history of passing turbid urine, decrease in urine output and swelling of both feet for 10 days. For the current complaints, he was treated with intravenous medications for a week before coming to our hospital. He had type II diabetes mellitus for 3 years and was on regular medication for the same.\nDuring his current admission, physical examination showed that he was conscious, afebrile, and tachycardic with heart rate of 136/min. Blood pressure was 110/70 mmHg. Rest of the vital signs were normal. He had renal angle tenderness bilaterally. Rest of the systemic examination was unremarkable.\nInvestigations revealed high total leukocyte counts with left shift, elevated urea and creatinine levels and pyuria with leukocyte esterase positivity [Tables and ]. In the above clinical setting, possibility of pyelonephritis/renal abscess with acute kidney injury was considered. Activated partial thromboplastin time was prolonged, which was thought to be due to sepsis-induced coagulopathy.\nUltrasound of the kidneys revealed enlarged kidneys with bilateral renal abscesses [ and ]. Further imaging was done with a noncontrast computed tomography scan which showed similar findings. He underwent emergency ultrasound-guided drainage of renal abscesses on both sides after transfusion of blood products in view of coagulopathy. Pus drained was sent for bacterial, fungal, and mycobacterial smear and culture. Sample for GenExpert polymerase chain reaction test for Mycobacterium tuberculosis was also sent, which was negative. X-ray chest and electrocardiogram were normal. Relevant blood investigations and results of urine analysis are mentioned in Tables and , respectively.\nAt admission, he was empirically initiated on intravenous meropenem at doses adjusted for renal impairment. Pus smear from renal abscesses from both sides showed septate fungal hyphae. The patient was advised to take intravenous voriconazole. However, after discussion, he opted for intravenous amphotericin B which was started at a dose of 1 mg/kg/day. Cultures from both renal abscesses revealed growth of Aspergillus fumigatus. The patient's hospital stay was complicated by worsening renal function, acute pulmonary edema, hyperkalemia, and metabolic acidosis. He was initiated on hemodialysis and noninvasive ventilation. The patient had a sudden cardiac arrest, probably secondary to aspiration on day 9 of hospital stay and he succumbed to his illness. |
A 60-year-old man was admitted with a hepatic mass that was incidentally detected during a screening examination. There was a history of treated hypertension and gout. His laboratory values of the liver function were normal. The serum α-fetoprotein and liver enzymes levels were normal. He had no history of extrahepatic malignant neoplasm or exposure to any carcinogens.\nInitial contrast-enhanced screening CT demonstrated a 1.1 cm mass in the hepatic dome (). Three phase contrast-enhanced CT demonstrated a low attenuated lesion without definite enhancement on the arterial phase image (), some peripheral nodular enhancement on the portal-venous phase image (), and progressive and delayed filling enhancement on the delayed image (). Although the enhancement pattern of the mass was slightly different from the typical enhancement of hemangiomas, which has a similar density to the contrast-opacified blood in the aorta or hepatic artery during all phases of imaging, the presumptive diagnosis was a hemangioma because of the lesion's small size and the delayed and homogeneous enhancement on three phase contrast-enhanced CT. MR images three months later showed a 2 cm well-circumscribed mass with surface retraction of the hepatic dome (). The T1-weighted MR image () revealed a hypointense mass with a hyperintense focus. The T2-weighted MR image () showed a heterogeneous hyperintense mass with hypointense areas. The hyperintense focus on the T1-weighted image was considered as hemorrhage within the mass. The gadolinium-enhanced MR images showed heterogeneous enhancement with peripherally rim enhancement on the arterial phase image (), centrally septal-like enhancement on the portal venous phase image (), and persistent enhancement on the delayed image (). The size of the lesion increased on the follow-up MR images. The preoperative diagnosis was that of a malignant mass, and surgical resection was performed.\nThe gross specimen showed a 2×1.5×1.5 cm white to red solid mass with an area of hemorrhage. Microscopic examination revealed numerous, irregular and anastomosing vascular spaces separated by fibrous septa, and the spaces were lined by variably pleomorphic and hyperchromatic endothelial cells. Tumor cells grew along the vascular channels, particularly along the sinusoids, with a multilayering and papillary growth pattern (). The final histopathologic diagnosis was hepatic angiosarcoma.\nTwenty-five months after undergoing surgery, there is no evidence of tumor recurrence and metastasis on the follow-up CT. |
A 37-year-old man with a history of HME was admitted to Karatsu Red Cross Hospital with complaints of a 6-week history of gradually worsening numbness in bilateral hands and feet. The motion of the cervical spine irritated pain in the right lower extremity. His father also had had HME.\nPhysical examination revealed multiple palpable exostoses in the lower extremities (). On neurological examination, hypesthesia below the T6 dermatome on the right and the C7 and C8 dermatomes on the left were demonstrated. There were bilateral hypoactive triceps tendon reflexes and hyperactive deep tendon reflexes in the right lower extremity, and a Babinski response was elicited on the right. No weakness of extremities strength was observed. The patient's Japanese Orthopaedic Association (JOA) score was 12 points.\nOn plain radiographs (X-rays), an osteochondroma arising from the lamina of C5 was suspected. Computed tomography (CT) showed a bony outgrowth from the anterior surface of the C5 lamina on the right with growth into the spinal canal anteriorly (). Magnetic resonance image (MRI) demonstrated an extradural mass arising from the lamina of C5 on the right and a herniated disc on the left. The spinal cord was severely compressed anteroposteriorly in the shape of a gourd ().\nThe patient underwent an open-door laminoplasty at the C5 and C6 with excision of the tumor. The operative findings showed a dural tube that was severely compressed by the bony lesion arising from the lamina of C5. The bony lesion, in which a cartilage cap and osseous stalk extended into the spinal canal, was completely en bloc removed by a hemilaminectomy of the C5 on the right (). The opened laminas of C5 and C6 were reconstructed using hydroxyapatite spacers. A small hole was made at the lateral mass and opened lamina to allow passage of a non-absorbable suture from the spacer at each site and the spacer was sutured tightly for bridging between the opened lamina and lateral mass (, ). Removal of the herniated disc was not performed.\nThe histological examination confirmed the lesion to be an osteochondroma. The clinical symptoms were immediately relieved after surgery. The patient was discharged on the 16th day after surgery; he returned to work four weeks after surgery.\nAlthough the patient complained of mild numbness in the right hand and foot, he had no restriction of daily activity and his work one year after surgery. On MRI evaluation, a sufficient decompression of the spinal cord with a spontaneous regression of the herniated disc at the C5-6 was observed one year after surgery (). The patient's JOA score improved four points from the pre-surgery level of 12 to 16 points at the three-year follow up. Plain radiographs showed no appearance of the kyphosis and segmental instability of the cervical spine and there had been no recurrence of the tumor at the latest follow-up (). |
A 52-year-old man with a past medical history of gastroesophageal reflux disease, chronic pancreatitis, chronic pain syndrome, hypertension, anxiety and depression presented to the ED for evaluation of worsening swelling in his upper and lower lips onset three hours prior to arrival. His symptoms began with spontaneous right lower lip swelling that quickly progressed to encompass both lips and caused mild voice changes. Review of systems was negative for difficulty breathing, difficulty swallowing, rashes, nausea, vomiting, diarrhea, and abdominal pain.\nThe patient denied personal and family history of angioedema reactions, prior red tide exposure, known food or drug allergies, seafood or nut intake, and new medication exposure. He reported no changes to his medication regimen and endorsed compliance with his bupropion, pantoprazole, and methadone. The patient had started taking vitamins B12 and D regularly two days prior and had taken these vitamins in the past without issue. He did not recall any insect bites or stings and had been desensitized to bee stings in childhood. The patient’s only notable exposure was to an outbreak of red tide algae at a Palm Beach County beach that morning just prior to symptom onset.\nVital signs showed that the patient was afebrile with a blood pressure of 163/119 millimeters mercury, heart rate of 68 beats per minute, respiratory rate of 15 breaths per minute, and pulse oximetry of 94% on room air. Physical exam was notable only for isolated, severe bilateral lip edema () not involving the soft palate, tongue, and uvula.\nThe patient was placed on two liters per minute of oxygen via nasal cannula, which improved his oxygen saturation to 96%. Initial doses of diphenhydramine, methylprednisolone, and famotidine were administered with no improvement. While still in the ED, his edema progressed, and the patient began to experience difficulty swallowing. Intramuscular epinephrine was then administered with only mild improvement two hours later. He was diagnosed with angioedema of unclear etiology and admitted to the intensive care unit (ICU) for further management. Labs were significant for leukocytosis at a white blood cell count of 10.3 thousand cells per microliter (thousand/mcL) (reference range: 5.0–10.0 thousand/mcL) with increased neutrophils. Immunology/serology showed no abnormalities: C4 complement level of 37 milligrams per deciliter (mg/dL) (16–38 mg/dL), C1q Qn of 12.3 mg/dL (11.8–23.8 mg/dL), C1 esterase inhibitor 39 mg/dL (8–40 mg/dL) and C1 ester inhibitor function of 111 (reference range: greater than 67).\nIn the ICU, the angioedema improved after additional doses of diphenhydramine, methylprednisolone, and famotidine. He did not require higher oxygen supplementation or intubation. Repeat complete blood count in the morning showed resolution of the leukocytosis. With the angioedema subsided, the patient had no difficulty breathing with an oxygen saturation of 98% on room air. He felt well enough the next day for discharge and did not experience symptom recurrence over two years. |
A 48-year-old man was admitted to our hospital due to pain and tenderness in the upper and central part of the abdomen when fasting. There were no pertinent symptoms. His past medical history included benign prostate hyperplasia. He was a current smoker for 42 pack-years. Physical examination revealed nothing significant except for tenderness in the upper and central part of the abdomen.\nAbdominal computed tomography (CT), upper gastrointestinal endoscopy, and complete colonoscopy revealed no abnormality. However, chest X-ray revealed a massive shadow in the right upper lung field (Fig. ), which was confirmed by chest CT showing a 65-mm mass in the right upper lobe with chest wall invasion (Fig. ). 18F-Fluorodeoxyglucose (FDG) positron emission tomography-CT showed abnormal accumulation of FDG in the lung tumor, as well as in a portion of the jejunum. Intestinal fluoroscopy revealed stenosis without obstruction in the distal part of the jejunum (Fig. ). There was no evidence of lymph node involvement and distant metastases other than the jejunum. The clinical diagnosis was right lung cancer with metastasis to the jejunum. Transbronchial lung biopsy revealed no evidence of malignancy. The laboratory data revealed slight elevation in gamma GTP (80 IU/L) and C-reactive protein (0.78 mg/dL). The levels of tumor markers like carcinoembryonic antigen, cytokeratin-19 fragments, and pro-gastrin-releasing peptide were not elevated. Because the metastatic tumor in the jejunum was symptomatic and was localized in a single organ, surgery was planned for both the primary and metastatic sites. Partial resection of the jejunum was performed initially, followed by right upper lobectomy with chest wall resection within a month. The patient had an uncomplicated postoperative course.\nPathologic analysis of the lung tumor revealed atypical spindle cells with irregular and hyperchromatic nuclei invasively proliferating and arranged in interlacing patterns (Fig. ). Epithelioid cells with tubular differentiation (components of adenocarcinoma) were also noted in a small part of the lung specimen (Fig. ). Immunohistochemical studies revealed that the tumor cells were positive for vimentin, AE1/AE3, and TTF-1 but negative for c-Kit, alpha SMA, S-100, and CD34. The pathologic diagnosis was pleomorphic carcinoma of the lung (Fig. ). The tumor in the jejunum was similar in both morphologic and immunohistochemical analyses, confirming a metastasis from the pleomorphic carcinoma of the lung (Fig. ).\nSince the patient did not want to receive adjuvant chemotherapy, we advised careful observation. The patient remained alive without recurrence for 6 years.\nPleomorphic carcinoma is one of the subtypes of sarcomatoid carcinoma among the four other subgroups, including spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. It is defined as a poorly differentiated NSCLC (squamous cell carcinoma, adenocarcinoma, or large cell carcinoma) containing spindle cells and/or giant cells, or a carcinoma that comprises spindle or giant cells alone, in at least 10% of the tumor []. Pleomorphic carcinoma occurs in only 0.1–0.4% of all lung carcinomas and generally has a more aggressive clinical course than the other types of NSCLC. According to the Japanese Joint Committee of Lung Cancer Registry data, its 5-year overall survival was 20–40%, whereas that of the other histologic types was 61.4% [–]. For such aggressive cancers, multimodality therapy needs to be considered, but the optimal treatment strategy remains undefined because of its rarity. The effective regimen for pleomorphic carcinoma, which is often diagnosed in the advanced stage, is not well established at the moment; in addition, adjuvant chemotherapy does not appear helpful.\nMassive coagulation necrosis and lymph node metastases were identified as prognostic factors for a poorer outcome of pleomorphic carcinomas [, ]. However, pleomorphic carcinoma with pN0 has poorer prognosis than other variants of NSCLC with pN0 []. Frequent vascular invasion is the cause of this unfavorable prognosis, even in early-stage disease.\nSome reports showed that a vigorous treatment strategy that included surgery had the potential to offer long-term survival, despite an advanced pleomorphic carcinoma with distant metastasis to other organs. In NSCLC, careful patient selection for complete resection of both primary and metastatic sites can result in a favorable outcome. Hasumi et al. reported on 35 surgically resected NSCLC with oligometastasis and concluded that patients without lymph node metastases had long-term survival []. For pleomorphic carcinoma, Aokage et al. reported two patients who survived for over 4 years after resection of the primary site and the gastric metastasis [] and Yamanashi et al. reported a patient with brain metastasis who was successfully treated by brain metastasectomy followed by chemotherapy and resection of the primary lesion []. Similar to our present case, these reported pleomorphic carcinoma patients did not have regional lymph node metastases by both clinical and pathologic diagnoses. It is important to note that this unexpectedly good outcome should not become a rationale for surgical metastasectomy in all cases. However, complete primary lung tumor resection with metastasectomy might be a treatment option for pleomorphic carcinoma in node-negative patients. |
In 2014, a 14-year-old girl was admitted to our unit with a chief compliant of HMB during her second menstrual period. She had a history of severe gingival bleeding and epistaxis which led to hemorrhagic shock at the age of 8. At that time, she visited a local hospital and was diagnosed with GT. Her first period lasted only 3 days with less bleeding, while the second episode of menstrual bleeding started 8 weeks after menarche and required 10 tampons per day. The bleeding continued for 10 days, and the patient had symptoms of dizziness, weakness and mild abdominal pain. She was admitted to a local hospital where platelet (200 ml) and erythrocyte transfusion (400 ml) was infused to stop the bleeding. After basic treatment she was shifted to our hospital. She had no family history of bleeding disorders, and her parents have a non-consanguineous marriage. A physical examination revealed no abnormalities. Gynecological examination by speculum showed moderate bleeding. Laboratory examination revealed a normal platelet count; PT and PTT; while an abnormal platelet aggregation and altered hemoglobin level of 6.4 g/dL was observed. We recommended the LNG-IUS for this girl, but as her parents refused, oral contraception was prescribed to control her bleeding symptoms. The patient received oral contraceptive therapy (Marvelon) for five courses each for 21 day cycles. After the course of treatment, her Hb levels improved to 10.7 g/dL. However, during treatment, the child often forgot to take the pills. Given the poor patient compliance, the parents decided to allow the placement of a LNG-IUS, and the procedure was performed in 20 min under general anesthesia. However, the LNG-IUS fell out after the next menstrual cycle because of excessive menstruation. Therefore, she once again received oral contraceptive therapy. During the medication period, the girl had a severe headache, and we therefore, decided to place another LNG-IUS. Four months later, the patient was amenorrheic and her Hb levels rose to 12 g/dL. An ultrasound examination showed an endometrial thickness of 5 mm, compared to 8 mm prior to insertion of LNG-IUS. At the 2-year follow-up, she showed normal growth and development and had no complaints. |
The 8-month-old boy was born at term without any unusual birth history (38 weeks, 3,150 g, by Cesarean delivery) to a 45-year-old father and 36-year-old mother. He had one brother (12-year-old) and sister (8-year-old). None of the family members had any medical history during the growth period.\nHe was admitted to the pediatric department due to an initial seizure event following aspiration pneumonia and was referred to our clinic for the evaluation of unexplained neuroregression. Although he was hypotonic from birth, he achieved a social smile at 3 months and started head control during the first 4 months. He rolled over, and nearly grasped his toys with prone position at 6 months. Generalized tonic–clonic type seizures at 6 months were his first clinical symptom, a detailed history revealed delays in developmental milestones after that. Electroencephalogram (EEG) findings showed abnormal awake and sleep recordings due to slow background activity, suggestive of diffuse cerebral dysfunction with symptomatic or cryptogenic seizures. Magnetic resonance imaging showed cerebral hypoplasia especially in the frontal and temporal lobes at approximately 4 years of age. He was observed at the outpatient clinic for developmental delays associated with encephalopathy and seizure events, which occurred hundreds of times for 2 years and were fairly well-controlled with valproic acid, phenobarbital, and clonazepam.\nAt 26 months after surgery for bilateral cryptorchidism, progressive respiratory difficulty persisted and weaning from the ventilator was not possible; repetitive aspiration pneumonia occurred as he was unable to proceed with sputum expectoration. Therefore, tracheostomy was performed and night-time breathing using a ventilator was maintained subsequently. At the time of admission, repetitive hand flipping without purpose and lip smacking was observed during examination, although epileptiform discharges were not observed during EEG, we decided to proceed with additional evaluation other than that previously considered at this point. The various clinical features of the patient are described in Table .\nThere were no abnormal findings based on laboratory investigation, and genetic analysis of mutations including Prader-Willi gene, spinal muscular atrophy gene, and other chromosomal aberrations. Chromosome analysis revealed a 46, XY karyotype. A muscle biopsy also demonstrated no abnormal findings. |
The patient provided informed consent for publication of this case.
A 42-year-old woman presented with a history of chronic hepatitis B virus (HBV) and was being evaluated for antiviral treatment. As part of her work-up, ultrasonography of the liver was performed and showed an incidental liver mass. A confirmatory magnetic resonance imaging (MRI) showed a huge mass with several metastases surrounding the right liver with right portal vein invasion that measured 9.6 × 9.1 cm (Fig. ). The tumor characteristics were combined with a markedly elevated alpha-fetoprotein level (>20,000 ng/mL) and no evidence of splenomegaly or portal hypertension, which were consistent with HCC. Because of the presence of tumor thrombus, the patient was not a liver transplant candidate. MRI volumetrics of the liver showed that if the required right hepatectomy was done, the FLR (segments 1, 2, and 3), which constituted 19% of the total liver volume, would likely be insufficient for postoperative recovery (Fig. A and B). As a result of complete right portal vein tumor thrombosis (type II portal vein tumor), right PVE or portal vein ligation would not be necessary.
Plans were made to use RALPP without portal vein ligation (RALP) with the laparoscopic approach to induce rapid hypertrophy of the FLR. After appropriate preoperative preparation, the patient was taken to the operating room for stage I of RALP. After the induction of general anesthesia, the patient was placed in the supine position. Pneumoperitoneum (carbon dioxide at 12 mm Hg) was established, and the abdomen was explored with a 30° laparoscope through a 10-mm umbilical port. Another 10-mm subxiphoid port was created at the midline of the abdomen. One additional 10-mm right or left lateral subcostal port was placed. Laparoscopic cholecystectomy was performed beforehand. Under ultrasonographic guidance, a line for the liver partition on the surface of liver was created by using a laparoscopic electric kn |
A 23-year-old male was feeding his horse, and while stroking the horse's hair, the animal chewed the fourth finger of his left hand causing violent pain and total functional impotence of the finger. Both the patient and the horse were up to date on their required vaccinations at the time of the incident. The patient was transferred to an emergency department and was admitted six hours after the incident. He was conscious, in good general condition, and apyretic. An examination revealed a crush injury of the fourth finger with tendons and bone exposed ().\nCopious irrigation with normal saline (2 liters) at the injury site was performed along with injection of 0.5 ml tetanus toxoid and 500 IU of human tetanus immunoglobulin. Postexposure rabies prophylaxis (rabies immune globulin human 20 IU/kg) with the first-dose rabies vaccine was injected into the depth of the wound as well as around the wound. The remaining rabies immune globulin was injected into the deltoid muscle. The patient was also treated with prophylactic antibiotic therapy with intravenous amoxicillin-clavulanate, gentamicin, and metronidazole.\nAfter this initial treatment, radiography revealed a fracture dislocation of the proximal interphalangeal joint of the fourth finger with a third fragment (), prompting the patient to undergo surgery. Surgical exploration under locoregional anesthesia found that the ulnar digital pedicle was sectioned and thrombosed, the radial digital pedicle was intact, the flexor and extensor tendons were sectioned and shredded, and the skin was irreparably shredded ().\nSurgical procedures included removal of foreign bodies and excisional debridement of devitalized tissue, collection of bacteriological samples, copious irrigation with saline serum (3 liters), tendon striping, and finger amputation with coverage of the bone by the radial digital flap using separate stitches (). The surgery was followed by careful clinical and biological monitoring.\nA clinical assessment of the patient 1 day postoperatively showed that he was apyretic with no necrosis of the flap and no purulent discharge. A neurovascular examination was normal. Biological findings at this time showed a C-reactive protein level of 30 mg/dL and a white blood cell count of 11000/μL.\nThree days postoperatively, bacteriological samples found an evidence of Pasteurella species and Staphylococcus sensitive to amoxicillin + clavulanic acid with C-reactive protein levels of less than 10 mg/dL and a white blood cell count of 7000/μL. At this time, the patient received the second dose of rabies vaccine.\nOne week postoperatively, the patient was discharged with a prescription for a course of 10 days amoxicillin-clavulanic acid treatment to be reviewed weekly. At a three-month follow-up (Figures and ), the patient showed no sign of infection; he returned to his usual activities and was discharged from care. |
A seven and half year-old boy visited the outpatient clinic of Pediatric Dentistry Department, Faculty of Dentistry, Cairo University in June 2015 with a chief complaint of pain on the lower right molar area. The patient’s mother stated that the pain was at times throbbing in nature, and child is not able to chew on this side.\nClinical examination showed a badly decayed, lower second primary molar with related localized intraoral abscess, where the lower first primary molar was intact. The patient had poor oral hygiene; he had not received any professional dental care, and was very apprehensive.\nRadiographic examination revealed root resorption and bone rarefaction related to lower second primary molar. The interesting finding was a considerable amount of root resorption of the distal root of the adjacent lower first primary molar (\n).\nThe case was managed by performing pulpectomy\n to the lower second primary molar, with root canals filled with calcium hydroxide paste with iodoform (Metapex, Meta Biomed, Republic of Korea). The tooth was then restored with high viscosity glass ionomer (GC Fuji IX GP capsule, GC corporation, Tokyo, Japan) (\n). The lower first primary molar was not touched and instead monitored. No antibiotics or analgesics was prescribed.\nUnfortunately, the patient’s mother did not want follow-up appointments in person, however, she was contacted on the phone, after 2 weeks, 3 months and 6 months, and she said everything was fine and there was no swelling or pain.\nAt about 8 months from the treatment appointment, the patient’s mother visited the outpatient clinic with the patient for other reasons, and decided to pass by the Pediatric Dentistry Department for patient follow-up. Clinical examination showed no signs or symptoms, occlusal restoration was intact, and radiographic examination revealed arrested root resorption, on both molars, and an increase in the density of bone although this was not at a normal level yet (\n).\nshows the patient’s timeline of symptoms, treatment and follow-up. |
A 41-year-old man with a history of pontine hemorrhage that occurred on May 2014 was admitted to our rehabilitation unit 1.5 years after the event. The brain computed tomography (CT) revealed hemorrhagic lesions in the bilateral pons, and additional lesions were detected in the right than in the left side. The brain magnetic resonance imaging (MRI) scan, which was conducted 6 months after the onset of hemorrhage revealed bilateral hypertrophic olivary degeneration (HOD). The cranial nerve examination revealed normal pupillary reaction, normal corneal and gag reflexes. In addition, the patient did not present any nystagmus, uvular deviation or impaired sense of taste. However, mild facial weakness and facial sensory changes were notable on his left face.\nIn the initial videofluoroscopic swallowing study (VFSS), bilateral myoclonic movements of the soft palate, pharynx, and larynx were clearly observed at a frequency of 1 to 2 Hz. He revealed premature bolus loss (PBL) in the oral preparatory and oral phase, and decreased laryngeal elevation and protraction during the pharyngeal phase due to PM. He also presented with large amount (7 mL) of dysphagia diet level 3 (mildly thick, honey-like consistency), and a score of 4 on the penetration-aspiration scale (PAS) (). We defined the dysphagia diet level by administering the standardized dysphagia diet developed by Han et al. [].\nFollowing the initial VFSS, the patient was exposed to a combination of behavioral and valproic acid treatments for 2 weeks. In the behavioral treatment component, the patient performed oromotor exercises focusing on the soft palate, deliberate swallowing, and double and multiple swallowing techniques. Further, he was instructed to maintain a chin-tuck posturing while having meals. Valproic acid was administered at 450 mg/day in the first week and doses were increased up to 900 mg/day in the second week.\nTwo weeks of combined treatment resulted in a significant decrease in palatal myoclonic movements occurring at a frequency of a 0.5 Hz during the follow-up VFSS assessment, which occurred 14 days after the initial assessment. He showed a penetration of large amount of dysphagia diet level 5 (thin, water consistency), which corresponds to a PAS score of 4 (). His score on the American Speech-Language-Hearing Association National Outcome Measurement System (ASHA-NOMS) swallowing scale was also improved from 4 to 5. |
Mr. A is a 33-year-old married male, a pharmaceutical sales executive, presented with a history of consuming 200–300 ml/day of cough syrup containing codeine (10 mg/100 ml) for the past 28 months. Our patient reports having been married for the past 2 years and reports having ejaculation occurring with 5 min of penetration and difficulty controlling it during intercourse with his wife. He reports of not having problems with erection or pain during ejaculation and says he did not have this issue before marriage while masturbating. Three months after marriage, Mr. A suffered from acute bronchitis for which he was prescribed a codeine-containing cough syrup. He noticed his ejaculatory response after consumption of the syrup and started consuming 100 ml of the cough syrup (one bottle) each day after purchasing it OTC from different medical shops. Over the past 25 months, Mr. A found he had to increase the amount of cough syrup consumed to improve his time to ejaculation and consumed up to 200–300 ml/day (2-3 bottles). He would also have a craving for the syrup and would have episodes of headaches, nausea, and sweating upon abrupt cessation and subsided upon consumption of the syrup. Mr. A and his spouse did not report on any mood changes, increased sedation, or any other problems at work or family due to his cough syrup consumption. He decided to seek treatment for his problem after attempts by himself to abstain from consumption of the cough syrup were unfruitful during the past 3 months.\nThere was neither any history or family history of mental illness nor any conflicts with wife, other family members, and at workplace. There is no history of any medical illness, use of other medications, or other substance use disorders.\nOn mental status examination, he was adequately groomed with normal psychomotor activity. He was conscious with euthymic mood, and there were no ideas of guilt, depressive ideas/death wishes, formal thought disorders, psychotic symptoms, or cognitive impairment. As per the 10th revision of International Statistical Classification of Diseases and Health Related Problems, a diagnosis of mental and behavioral disorders due to use of opioids, dependence syndrome, continuous use, and premature ejaculation was made.\nHis physical examination and vital signs revealed no abnormalities, including normal pupil size and respiratory rate. He was admitted and laboratory investigations on the day of admission, including, hemoglobin, total and differential count, serum sodium and potassium, erythrocyte sedimentation rate, routine urine examination, renal and liver function tests, and random blood sugar were within the normal limits. No physical abnormalities of the genitourinary system were found upon consultation by a urologist.\nMr. A was started on oral clonidine of 0.1 mg three times a day and oral lorazepam 2 mg at night. The next day the patient reported having sweating, rigor, and mild muscle cramps of his legs following which he was given intravenous lorazepam and clonidine dosage was increased to 0.2 mg, three times a day. His symptoms gradually subsided and he did not report any withdrawal symptoms the next day. Oral naltrexone of 50 mg/day was started on the 4th day. He was discharged on the 14th day of admission after gradual tapering and stopping of oral clonidine and lorazepam tablets and motivational enhancement therapy sessions during his hospital stay. Liver function tests were normal on discharge. He was maintained on oral naltrexone of 50 mg/day over the next 3 months along with monthly monitoring of liver function tests. Sensate focus exercises along with stop and squeeze technique for practice were taught to Mr. A and his spouse following which Mr. A's premature ejaculation improved. After being abstinent from opioids for 6 months (confirmed by urine opiate screening test), oral naltrexone was stopped. |
In the case shown in , the difference between a normal 64-year-old male participant and a patient with disease can be easily identified. The ABI is 1.26 on both sides, and no laterality is observed. Furthermore, clear dicrotic waves are noted on the ankle pulse waveforms with normal upstroke time (UT) and percent mean arterial pressure (%MAP).,, As such, the forms of both ankles are normal, and arteriosclerosis obliterans (ASO) of the lower extremities (LE) are not observed. Therefore, the baPWVs of both sides are accurate, and a higher value of 1224 cm/s is within the normal range (standard deviation value of the population without any atherosclerotic risk factors). Laterality is not observed as well. Therefore, this is an ideal result for measuring indices using the Vascular Profiler.\nThis typical case of a 49-year-old male individual () shows a laterality between the normal lower limb and that with ASO in pulse waveforms, pulse amplitudes, oscillometric envelope, baPWV, and so on. The pulse waveform of the right ankle is normal, and its aspect is similar to that of both ankles as shown in . On the contrary, the left ABI (0.55) is not normal, and this result is consistent with the abnormally higher UT and %MAP.,, The pulse waveform of the left ankle is blunted and triangular, and no clear dicrotic waves are observed. When the Vascular Profiler is used, the height of the pulse waveform is drawn according to pulse pressure, and a pulse pressure of 10 mmHg is expressed as one grid. Thus, a low left ankle pulse waveform is observed because it reflects a pulse pressure of 26 mmHg and is averagely drawn using 2.6 grids in heights. The pulse amplitudes of the right ankle and both arms are perfect, and a strong pulse is observed. On the other hand, the pulse amplitude of the left ankle is significantly low and clearly indicates a weak pulse. The width of the oscillometric envelope is significantly narrow only in the left ankle. This also indicates a decrease in BP and pulse pressure caused by stenosis. The width of the oscillometric envelope reflects a pulse pressure as a whole. Furthermore, the baPWV of the left side is remarkedly lower than that of the normal right side because of the stenosis. In addition, the value of the left baPWV is expressed within a parenthesis, which means it should be cautiously interpreted. Moreover, a baPWV of 1554 cm/s in the right side is accurately measured. Nevertheless, this value should not be used, at least as a prognostic predictive marker. ASO in the left leg indicates a prognostically high-risk condition, and this significantly overweighs the significance of the baPWV as a prognostic predictor. In other words, a baPWV of 1554 cm/s indicates a prognostically middle-risk condition, but an ABI of 0.55 indicates a high-risk condition. Thus, they are inconsistent.,, It would be marginally possible to use a baPWV of 1554 cm/s as a marker of arterial stiffness and/ or function. Nevertheless, the exclusion criteria for patients with ASO have been generally accepted recently when using baPWV for any purpose. Moreover, in this case, a remeasurement is not necessary because the BPs and ABIs are accurate based on the information in the oscillometric envelopes.\nIn this case, a pseudo-underestimation phenomenon of baPWV caused by ASO was explained. The fundamental requirement in PWV measurement is the absence of a significant physical obstacle in the measuring pathway. Therefore, a similar phenomenon of inaccurate baPWV estimation, which is similar with this case, can be considered in patients with aortic diseases, such as aortic aneurism and dissection.\nIn , the case of a 71-year-old male individual with diabetes mellitus is presented. The ABIs are 0.98 on the left side and 1.00 on the right side, but this patient had LE-ASO in both sides. This is a typical case of pseudo-normalized ABI due to a strong arterial calcification of the lower limbs. The ABIs range from borderline to normal, but the pulse waveforms of both ankles are triangular. The UTs are more than 180 ms, and the %MAPs are more than 40% on both sides. Thus, ASO in both sides can be strongly suspected. Furthermore, the baPWVs are significantly high, reaching more than 2000 cm/s, even though they are likely underestimated due to ASOs. As a result, arterial calcification and the pseudo-overestimation of ABI will likely occur. In fact, the toe-brachial index (TBI) is 0.36 on the right side and 0.43 on the left side. Thus, LE-ASO on both sides is confirmed in this patient.,\nIn , the case of a 64-year-old male individual is presented. The ABI in the right side is 0.75, but this is a result of an inaccurate measurement of the right ankle systolic BP (SBP). Thus, this does not indicate LE-ASO. On the other hand, an arterial stenosis of the left UE is most likely to occur. To focus on the oscillometric envelope of the right ankle, the determined timing of the SBP is remarkedly close to that of the maximum pulse amplitude. In other words, the timing of the SBP is too late, which means that the SBP is close to the mean BP. The right ankle SBP has to be determined much earlier (an equivalent timing of the oscillometric envelope in the left ankle), but this is mistaken for some reasons. The aspects of the oscillometric envelopes of both ankles are similar. The UTs and %MAPs of both ankles are normal, and no laterality is observed. Furthermore, dicrotic waves are clearly observed in the pulse waveforms of the right ankle, and no laterality is observed in the baPWVs. In general, the baPWV in the right side should be significantly lower than that of the left side in an individual who presents with ASO in the right LE. A pulse pressure as low as 35 mmHg causes a diminished waveform in the right ankle (due to a falsely measured SBP, that is, the height is averagely expressed in 3.5 grids). shows the comparison of the ankle pulse waveforms with the adjusted heights, and no obvious laterality is observed. The pulse amplitude is lower in the right ankle than in the left ankle. However, the following results are consistent and considered more important: 1) There is no significant laterality in the shape of the oscillometric envelopes; 2) no significant sign of stenosis is observed in the pulse waveform of the right ankle; and 3) the baPWV in the right side is not lower than that of the left side. Improper cuff positioning is considered to have caused the inaccurate measurement. Thus, in this case, a remeasurement with a firmly placed and appropriately positioned cuff should be conducted.\nThe pulse amplitudes in both arms are perfect. However, there are lateralities of 21 mmHg in the SBP and 5 mmHg in the diastolic BP (DBP), and the width of the left arm oscillometric envelope is somewhat narrower. Thus, an ASO in the left UE is suspected. There are no clear literalities in the pulse waveforms and %MAPs in both arms. Nevertheless, there are frequently cases in which a dynamic change is not observed in the brachial pulse waveform unless the UE-ASO causes a quite large decrease in the brachial BPs. As a reference, details on the oscillometric envelope of this device are depicted in .\nIn , we present the case of an 80-year-old female individual. The baPWVs on both sides exceed 3400 cm/s. However, the values are significantly inaccurate. The SBP is lower by 19 mmHg, and the DBP is lower by 3 mmHg in the right arm than those of the left arm, and the pulse waveform of the right arm is blunted. The UT of the right arm pulse waveform is evidently longer than that of the left arm (no value is recorded in the result sheet). Moreover, a lower pulse amplitude and narrower oscillometric envelope are observed in the right arm than in the left arm. Therefore, a right UE-ASO likely exists. However, the brachial base of the baPWV measurement remains in the right arm. In this condition, the pulse transit time between the heart and the right arm becomes longer because of the stenosis, and the right arm pulse waveform slightly shifts to the right side. Therefore, the pulse transit time between the right brachium and the ankles, which is the denominator used to calculate the baPWV, becomes shorter. As a result, the baPWVs are overestimated. The Vascular Profiler has an automatic program that validates the existence of ASO in the right upper limb and that switches the base of the baPWV measurement. Unfortunately, the condition does not match the algorithm in this case. The automatic program requires both of the following: 1) the pulse amplitude of the right arm should be lower than that of the left arm (a slight difference is acceptable); and 2) the inter-arm BP differences must not be less than 16 mmHg in SBP and 5 mmHg in DBP, or 20 mmHg in SBP only. shows the remeasurement results with the base of the arm automatically switched by the program. The baPWV is approximately 2080 cm/s, which is accurate. Moreover, the values of the ET and the ET/PEP (pre-ejection period) have changed. The total systolic interval corresponds to the time between the onset of the Q wave of the electrocardiogram and that of the II sound of the phonocardiogram. Moreover, the total systolic interval is equal to the sum of the ET and PEP (ET+PEP). The ET corresponds to the time interval between the onset and dicrotic notch of the brachial pulse waveform. The PEP corresponds to the time of QII – ET. Thus, the total systolic intervals are both 384 ms in the first and second measurements (290 ms + 94 ms = 384 ms, ; 298 ms + 86 ms = 384 ms, ). The ET and ET/PEP increased (290ms→298ms and 3.09→3.47, respectively). Therefore, when there is ASO in the UE artery, the indices related to the systolic time intervals will be affected. In addition, there are several cases in which the conditions do not match the requirement in switching the brachial base. For the maneuver in such a situation, the cuffs of the arms can be set oppositely. Nevertheless, BPs and the pulse waveforms of the arms show those of the opposite side if this maneuver will be carried out. Thus, this must be reflected in the result sheet and/or medical record.\nThis case is considerably rare in Japan but would be more common in Western countries because of the higher prevalence of cardiovascular diseases, including peripheral arterial disease.– Furthermore, a similar phenomenon will likely occur in the carotid-femoral PWV (cfPWV) when there is a carotid artery stenosis in the cfPWV pathway. Therefore, caution is needed in interpreting cfPWV especially when a carotid stenosis is suspected. And the prevalence of carotid arterial diseases will be higher in western countries than in Japan, with a similar prevalence of cardiovascular diseases.–\nIn , the case of a 73-year-old male individual with a hemodialysis access in the right arm is presented. The ASOs in the left arm and both legs are clearly observed regardless of ABIs, and the progression of calcification in the LE and aortic stiffness also exists because of hemodialysis. However, both ABIs and baPWVs are not reliable at all. For this reason, all the pulse waveforms are triangular. All the pulse amplitudes are extremely low, and the %MAPs on both ankles are more than 45%. In addition, the oscillometric envelope of the left arm is significantly narrow, and a left brachial SBP of 63 mmHg, which is the denominator used to calculate the ABI, is remarkedly low. As a result, the ABIs are overestimated. Moreover, the baPWVs are also overestimated, which is similar to the phenomenon shown in (this effect seems to far more exceed the possible underestimation of baPWVs caused by LE-ASOs in this case). Therefore, the ABIs and baPWVs are never accurate regardless of the remeasurements that are carried out. Nevertheless, for the significance of the measurement, stenotic diseases can be easily considered using the pulse waveforms. Moreover, the absolute values of the BPs could be used as a reference value, considering the influence of the LE arterial calcification because the timing of BP measurement in each oscillometric envelope is acceptable.\nHerein, one of the pitfalls in using ABI is presented, that is, the BP and pulse waveforms in the left arm are not within the normal range. On the other hand, there are several cases in which the change in the brachial pulse waveform is not remarkable, although a stenosis exists. Thus, stenosis cannot be easily identified in such a situation. For example, the pulse waveform of the left brachium is similar to that of the right brachium (). Let us suppose that the right arm is not measured as shown in . Furthermore, the laterality cannot be validated using the information provided, including that of the pulse waveforms of both arms in hemodialysis patients, because BP is generally measured only in the contralateral side of the hemodialysis access. However, even if the decrease in SBP caused by UE-ASO is not significant, for example, 10 mmHg, the interpretation of the ABI could significantly change (i.e. 135÷140=0.96 and 135÷130=1.04). Thus, the only way which could avoid these pitfalls (and not perfectly) is to carry out measurement in both arms. BP measurement in the arm with hemodialysis access is generally contraindicated, and it is also explained in the device manual. Nevertheless, there is important information on this issue according to an oral presentation of the Japanese Association of Cardiovascular Intervention and Therapeutics in 2013. A total of 169 hemodialysis patients who agreed to have their BP measured in both arms with the Vascular Profiler were recruited. BP measurements were obtained in both arms in 147 patients (19 patients declined, and three patients had unsuccessful measurement due to involuntary movements). Forty patients (27.2%) had an inter-arm difference of ≧10 mmHg in their SBP. Among them, 15 patients underwent further examination as requested (ultrasound imaging). Stenosis and occlusion in the UE were confirmed in 12 out of 15 patients, and these were observed in the side where the BP reading was usually obtained (non-hemodialysis access side) in five patients. In addition, failed hemodialysis access was not observed in 150 patients. Thus, they conclude that measurements must be obtained in both arms in hemodialysis patients, particularly when LE-ASO is suspected, because the likelihood of measuring appropriate ABIs could be improved. Therefore, regarding the increased ABI in the hemodialysis patients with ASO, the phenomenon explained above is causing this to a certain extent as well as the LE arterial calcification,\nHemodialysis patients are highly recommended to undergo periodic ABI measurement. However, the pitfalls may likely be overlooked even if information from the pulse waveforms is provided at the same time as using the device because only one arm is generally used in measuring the BP of hemodialysis patients. Therefore, full attention in confirming all the information is always required when interpreting the measurements in hemodialysis patients.\nMoreover, this case also implies that the measurement of the ABI would be more difficult in hemodialysis individuals when the conventional Doppler method is used. There is no information from the pulse waveform regarding the Doppler method (except the Doppler waveform). And it is impossible for the Doppler method to synchronize the SBP measurement of all the limbs in the same way that the Vascular Profiler performs. The statement document of the American Heart Association (2012) points outs that there were two studies that obtained a left ABI of 0.03 significantly lower than the right ABI (in the general population, not hemodialysis cohort). Moreover, according to a meta-analysis on the prevalence of laterality in brachial BPs, a significant increase in the odds ratio of the inter-arm difference (i.e. SBP ≧ 10 mmHg) was observed when the measuring process was compared (sequential versus simultaneous). This information implies that ABI measurement using the Doppler method is more prone to accidental errors than the synchronized measurement with the information from the pulse waveforms.\nIn , the case of a 76-year-old female individual is presented. The features of her disease are as follows: 1) high ABI (i.e. >1.3) and pulse pressure in the UE and LE; 2) low baPWV for the patient’s age and/or BP (in the absence of LE-ASO), and in the result sheet of the Vascular Profiler; 3) a high pulse waveform of the ankle and an oscillometric envelope with a significantly long width (reflecting a wider pulse pressure); 4) a tendency of lower UT and %MAP; and 5) continuous noise of the phonocardiogram depending on the case and the setting. These are considered as compensatory mechanisms. First, the high ABI is a result of the altered condition in the heart systole and the systemic arteries to rapidly increase the peripheral BP during early systole to compensate for the regurgitation during the late systole. Second, the low baPWV is primarily a result of the dilated aorta due to an increased blood mass in the aorta and arteries compared to the normal condition for the compensation of the regurgitation and of the concurrent tendency of decreased DBP. The wider the internal diameter of the artery, the lower the PWV. After the patient underwent an operation to replace the aortic valve, the ABIs and baPWV (both ABI=1.19; the average baPWV is 1457 cm/s) and the other parameters normalized, except for the electrocardiogram results and systolic function ().\nshows the case of a 63-year-old male individual. The features of his disease are as follows: 1) a major noise in the systole on phonocardiogram; 2) low baPWV for the patient’s age and/or BP (in the absence of LE-ASO); and 3) prolongation tendency in ET and UT. The exact cause of the decreased baPWV cannot be fully elucidated. Nevertheless, the considerable mechanisms are as follows: 1) the energy of the systole does not adequately propagate because of the aortic valve abnormality and the turbulence in the ascending aorta; 2) the characteristics of the systemic arteries, including the aorta, could be temporally altered, such as the aortic regurgitation described previously. shows the result of the post-aortic valve replacement. baPWV increased to approximately 300 cm/s, which is within the reference range, although there is a slight increase in the brachial DBP. In addition, the heart noise disappeared. presents a typical case in which both features of the regurgitation and stenosis of the aortic valve are clearly observed (in a 58-year-old male individual). The following similarities are observed: 1) baPWV tends to be low; 2) ABI and pulse pressure tend to be high; 3) the width of the oscillometric envelope is wide; and 4) there is a strong noise during systole on phonocardiogram.\nThe oscillometric envelope varies with different conditions and diseases. In summary, when stenotic diseases exist, an oscillometric envelope with a narrow width is observed. Moreover, when severe LE arterial calcification or aortic regurgitation exists, an oscillometric envelope with a wider width is noted. However, the shape is different. The information in the oscillometric envelope will support the validity of the BP readings and ABI (see ).\nThe phenomena discussed in this article and other phenomena are summarized in , except a low ABI caused by ASO. Furthermore, a low ABI and a borderline ABI measured by the Vascular Profiler are frequently observed in healthy young individuals in Japan.– For example, the left ABI of female students (age; 9–17 years old, mean 14.7) is 1.03±0.09 (range; 0.73–1.33). The prevalence of ABI of <1.0 among medical university students was 18.1% in males and 25.6% in females. The prevalence of ABI of ≤0.9 and age showed a J-curve relationship among women in the general population. In this study, the prevalence of ABI of ≤0.9 was 0.9% in the group under 40 years old, which decreased to 0.2% in the group of 60–69 years old, then increased to 1.6% in the group over 70 years old. These phenomena are likely caused by a weak reflected wave of the flexible artery at the ankle. This information is also included in . This categorization would also help us to understand the results of the Vascular Profiler, and also to judge the validity of the indices. |
A 42-year-old male with hemodialysis-dependent (HD), end stage renal disease (ESRD) underwent a second deceased donor kidney transplant (DDKT). His past medical history included glomerulonephritis, the etiology of his ESRD, for which he underwent an initial DDKT at the age of 30. Following this first transplant, the patient was diagnosed with donor-derived metastatic melanoma. Immediate discontinuation of immunosuppression was followed by a donor nephrectomy that same year. The patient was disease-free without evidence of metastasis for the following 11 years. In lieu of a prior malignancy, he did not undergo center-standard induction therapy with alemtuzumab and instead received 500 mg of intravenous methylprednisolone and 40 mg of intravenous basiliximab. The standard criteria donor was a 20-year-old male that suffered from head trauma following a motor vehicle accident. Cold ischemia time was 4 hours. Incision and placement of the allograft was in the left retroperitoneal iliac fossa due to the patient's prior right sided surgeries. The donor had an accessory renal artery and a single arterial anastomosis was completed using a single aortic cuff receiving inflow from both tributary vessels. The venous anastomosis was completed in standard fashion. Operative time was 1.5 hours. Intraoperative urine output was 225 mL and postoperatively continued at a rate of 40–500 mL/hr for the following 10 hours. Urine output then began to decrease acutely from 25 mL/hr to 10 mL/hr. Renal ultrasound obtained at postoperative hour 11 demonstrated elevated velocity at the main renal artery anastomosis. A loss of tardus parvus waveform with a decreased resistive index was concerning for external compression, kink, stenosis, or impending thrombosis. No perinephric fluid collection was visible. The concern for a technical error prompted immediate return to the operating room on postoperative day (POD) 1. Intraoperatively, the allograft was torsed medially across the vascular pedicle with mild discoloration suggestive of ischemia. No evidence of frank necrosis was observed. After detorsion, intraoperative ultrasound assessment confirmed reestablishment of arterial and venous flow. A biopsy was not performed at the time of reexploration. Nephropexy of the superior pole to adjacent soft tissue and the lateral abdominal wall was then completed. The iliac fossa space was more intimately closed to decrease the potential space.\nThe patient remained oliguric requiring hemodialysis with ongoing elevations in serum creatinine. With a concern for rejection, on hospital day 6 the patient underwent an ultrasound-guided biopsy with final pathology revealing Banff 2B acute cellular rejection. A 5-day cycle of anti-thymocyte globulin treatment was initiated with the patient producing urine after 18 hours of treatment. The creatinine level markedly decreased from 6 at initiation of treatment to 1.35 upon discharge. Throughout his perioperative and postoperative course, the immunosuppression regimen consisted of prednisone, mycophenolate mofetil beginning on POD 2, and tacrolimus was initiated on POD 8. |
A 32 year old female Cameroonian gravida 4 para 3 at 34 weeks of gestation presented to the labour and delivery unit of Mbalmayo district hospital with 8 h history of severe generalized headache, expressive aphasia and right sided paralysis in an afebrile context. This was associated with blurred vision but no convulsions. There was no epigastric pain and no difficulty breathing and no history of trauma or fall. For this current pregnancy, antenatal care (ANC) was started at 18 weeks with a booking blood pressure of 100/70 mmHg. She did four ANCs and all were uneventful. During her routine four ANCs here blood pressure was always less than 140/90 mmHg and her urine dipsticks done during the four ANCs were all negative for proteinuria. She refused neurological symptoms such as headache during pregnancy. She has a history of gestational hypertension in her third pregnancy. There was no family history of chronic hypertension, diabetes and chronic kidney diseases. On examination she was afebrile with a blood pressure of 182/126 mmHg and pulse of 112beats/minute. Neurological examination revealed Glasgow coma score of 13/15, right sided hemiparesis and expressive Broca’s aphasia, no signs of meningeal irritation. The abdomen was distended by a gravid uterus with a fundal height of 35 cm, foetus in a longitudinal lie and cephalic presentation. The cervix was long, posterior, soft and closed with a station of − 1. We had a working diagnosis of severe pre-eclampsia complicated by stroke. Shown on Table are laboratory investigations done and their results.\nAn emergency obstetric ultrasound showed a life foetus with an estimated foetal weight of 2300 g at 33 weeks of gestation. Emergency cerebral non contrast-CT scan showed a 3.2 cm hyperdense region in the left parietal lobe with surrounding hypodensity due to clot retraction as shown on Fig. . Emergency management by the obstetrician consisted of MgSO4 using the Pritchard protocol [], which consisted of 14 g loading dose then 5 g maintenance every 6 h until 24 h after caeserean section; bethamethasone 12 mg intramuscular and reduction of blood pressure with nicardipine 5 mg/h. Four hours later an emergency caesarean section was done by the obstetrician under spinal anaesthesia and it let to the extraction of a life female with APGAR 8 and 10 at the 1st and 5th minute respectively and weight 2200 g. The management after caesarean section consisted of hospitalization in the intensive care unit with nicardipine titrated in an electric syringe at 2.5 mg/hour, ceftriaxone 2 g intravenous, Paractamol 1 g 8 hourly, and ringers lactate 6 hourly for 24 h. Post-operative management was done by a multidisciplinary team including a neurologist, cardiologist, intensive care physician, obstetrician, neonatologist and physiotherapist. On postoperative day 2 she was transferred from the intensive care unit to the maternity where she spends five additional days on nicardipine slow release 50 mg 12 hourly and paracetamol 1 g 8hourly and was later release after the ten days on nicardipine 50 mg daily and daily physiotherapy. Six weeks during routine postpartum visit the blood pressure was normal and patient was no longer aphasic and shet has regained the muscle strength partially. The baby was hospitalised in the neonatal unit for 10 days and discharged alongside the mother. |
The 8-year-old boy patient presented with painful red lumps, pus discharge, and alopecia on the back of the head since 2 weeks ago. In the past two weeks the patient complained of pruritus and rashes on his head which were especially severe in the afternoon and evening. The patient was not given any treatment. However, 1 week ago, the patient’s mother noticed that the patient’s alopecia in the pruritic area and the rash had increased. The patient also began to complain of pain in the head area. Therefore, the patient decided to come for treatment at a dermatology and venereology specialist clinic. History of fever is denied. Similar complaints were also found in the patient’s twin, that consist of alopecia, red lumps, and pus discharge. But we did not found other family member with the same complaints. The patient has a history of playing with stray cats in front of his house and is said by the family to have poor hygiene. Informed consent was obtained from the patient and his family for his case and images to be published in this paper. This case details of this manuscript also has been approved to be published by the Department of Dermatology and Venereology, Faculty of Medicine, Universitas Sumatera Utara.\nDermatological examination revealed multiple erythematous nodules with sinuses in the scalp of the parieto-occipital area and alopecia. There is tenderness in the area of the lesion. () On palpation, there is no palpable enlargement of the lymph nodes. There is no lesion in the other parts of the patient’s body. Based on the history and physical examination, the differential diagnosis in the patient was kerion type tinea capitis and impetigenic scabies.\nWe examined skin scrapings in the lesion area using KOH 10% and examined it under light microscope, which did not show any fungal elements. The culture also showed a negative result. Through microscopic examination we found the Sarcoptes scabiei mite. The examination was done in Department of Microbiology, Faculty of Medicine, Universitas Sumatera Utara (). Based on the laboratory examination results, we conclude that the diagnosis in this patient was impetigenic scabies. The patient received treatment in the form of applying 5% permethrin cream once for 8 hours, cetirizine syrup 1 x 1 teaspoon and amoxicillin oral. In this case, the patient’s twin also given the same treatment as the patient. We also followed up both of them each week.\nDuring the first until third control, the patient felt improvement in his pruritus and pain. By the third week, the patient’s hair was starting to grow. Dermatological examination revealed multiple erythematous macules. There is new hair growth in some areas that were previously alopecia. We followed up the patient for In the last visit, the patient’s hair has grown completely. () Similar result was also seen in patient’s twin. () |
81-year-old woman with a history of hypertension treated via Enalapril and dyslipidemia treated via diet. She had been evaluated some years ago by an otorhinolaryngologist and was diagnosed with bilateral hypoacusis, which was severe in the right ear and moderate in the left ear, where she had a hearing aid.\nFour months prior to the consult, the patient had her first episode of MH. It consisted of childhood songs, which she had learned and liked (Manolo Escobar, folk songs). She heard them in her left ear, which was the only one that still retained partial audition with a hearing aid. She had not heard anything with her right ear for several years. The songs could last for hours and did not bother her; she assumed they came from the outside environment.\nIt was noted that the patient did not have any previous incidents of psychiatric illness, she had not shown symptoms of cognitive deterioration, and she had no changes in her regular treatment; she lived alone and was independent in her daily life. After the patient’s symptoms began, her family was worried about with the possibility that she had a psychiatric or neurological illness; following a consult with her general practitioner the patient was given treatment with sertraline. The family also started considering the possibility of sending her to a nursing home believing that she was showing signs of the onset of dementia.\nIn the neurological examination, aside from the hypoacusis, the patient did not present with alterations of interest and, in her cognitive evaluation, the tests were within normal ranges (Minimental-test, Fototest).\nBecause of the presence of MH, the patient was evaluated by psychiatry, which did not encounter any pathology.\nIn the tests performed: analytical, Holter EEG, and MRI of her brain, only diffuse cerebral atrophy was identified in the image study, and there were no other points of interest. The audiometry test noted a moderate loss of neurosensory hearing in the left ear and severe loss in the right.\nOnce diagnosed with auditory hallucinations secondary to sensory deprivation, she was placed on carbamazepine (200 mg every 8 hours). About 15 days later there was a partial improvement of the clinic: the music came less frequently, although of equal intensity. After explaining the symptoms to the patient and her family, she continued living alone and having personal autonomy |
A 35-year-old male, manual laborer by occupation, with left hand dominance, presented to the casuality department, sustaining an accidental injury due to entrapment of his left upper limb in concrete mixer while working. He presented after a delay of 24 h as the patient was referred from primary health center. The patient was transiently hypotensive with blood pressure below 90 but responded well to intravenous fluid.\nHe sustained severe crush injury of the left forearm, wrist and hand. There were multiple lacerated wounds, around 15-20 in number, criss-cross in shape, each approximately 3 cm × 2 cm in size serially longitudinally arranged on both volar and dorsal aspect of forearm, wrist and hand, on the radial side of limb, sustained due to entrapment of limb in rotating concrete mixer, caused by the rotating motor blades. The skin and muscles were severely crushed over the radial side of forearm, wrist and palmer aspect of the thenar eminence. The thumb was attached with skin tags only. The tendons and muscles were normal over hypothenar area. Multiple small bone pieces of the radius were seen and can be palpated from the wound, whereas surprisingly the ulna was felt intact. The wound was grossly contaminated. Surprisingly all active finger movements were present though reduced, in all four fingers except the thumb. Ulnar pulse was palpable whereas radial pulsations were absent clinically as well as on color doppler. Capillary reflow was sluggish on the radial side. Hypoesthesia was present only on dorsum in first web space, whereas ulnar and median nerves were intact. The elbow, arm and the shoulder were normal. The mangled extremity severity score (MESS) of the limb was 8 (age group – 30-50 [1 point], shock group - transiently hypotensive [1 point], skeletal/soft tissue group - medium energy injury [2 point], ischemia group – moderate [2 point], ischaemia time >6 h [2 point]).\nPrimarily, gross contamination was removed by thorough irrigation of the wound and primary splintage done, which was followed by administration of third-generation cephalosporin antibiotics and tenatus toxoid. Thus after stabilizing the patient haemo-dynamically, anteroposterior (AP) and lateral radiographs of the entire affected forearm, wrist and hand were taken, which showed severely comminuted fracture with multiple bony pieces arranged in criss-cross pattern in middle and distal third radius (bag of bones), along with intraarticular extension. Ulna and all the carpals were intact with maintenance of the inter-carpal relation. The radiological examination of the hand showed comminuted fracture of 1st and 2nd metacarpal, with crushed thumb and severely comminuted fracture of the proximal and distal phalanx of the thumb [].\nAlthough MESS score of the patient was >7 a score validate for amputation, we, on the contrary, planned the patient for limb salvage reconstructive surgery. Under brachial block, in the supine position, initial debridement along with thorough irrigation of the wound was done, with removal of all dead necrotic tissues and refreshing the muscles and skin, till fresh bleeding points. The free loose bone pieces seen, were removed. As the thumb was severely crushed and was attached with only skin tag, which was impossible to reconstruction and hence the thumb was amputated from the metacarpophalangeal joint. After the debridement and thumb amputation, multiple lacerated wounds on the volar and dorsal aspect were closed in layers, whereas the wound on the dorso-radial surface of the wrist and hand was left open for regular dressing as it was not possible to close it primarily. This was followed by stabilization of the fractures, by application of a radio-metacarpal distracter maintaining the length of radius by passing two 3 mm schanz pins proximal to the fracture, in middle third radius at mid forearm between the brachioradialis and extensor carpi radialis brevis muscles and the two schanz pin distally, in the neck of the 2nd metacarpal, as the base of the 2nd metacarpal was also fractured [].\nRegular dressing and antibiotics continued till 5 days and as the dorso-radial wound improved, split skin grafting was done. Sutures were removed after 2 weeks. Patient continued on radio-metacarpal distracter till 6 weeks followed by below elbow cast for further 4 weeks. Range of motion (ROM) exercises for elbow and fingers started with below elbow cast in place. ROM exercises for wrist also started after cast removal. Sound bony union was seen at 7 months with wound healing with no signs of infection, although with some amount of malunion and ulnar variance []. Elbow had full ROM whereas wrist had 30° of each palmar-flexion and dorsi-flexion. Fingers had near normal ROM. Pronation of the limb was about 70° and supination was restricted to 50°. Grip strength was about 75% compared to contra lateral side even without the thumb as tested objectively with dynamometer. Patient is being considered for policisation of the index finger for better functioning of the hand. At final followup of 7 months, both the dorsal and palmar sensation was intact except at dorsal first web space. Further, as thumb was amputated the strength, prehension ability and performance was reduced. |
A 35-year-old woman presented with two month history of a swelling in her left eye which increased rapidly in size to involve the left temporal region. Within this period she developed rapidly progressive proptosis and blurring of vision leading to complete blindness. Also the patient had no pain at the time of presentation and no history of swelling at any other site of body was present.\nUpon physical examination, there was a lobular firm, nontender mass of size 15 × 7 cm, extending from left orbit to the left temporal region. In addition to axial proptosis, the left eye showed restricted movement in all directions. Her palpebras exhibited prominent chemosis []. She was unable to perceive light in her left eye. Her cornea was intact. The round pupil was 6mm across and sluggishly reactive to light. The disc of the left optic nerve was edematous. On extensive examination there were no subcutaneous swellings, café au lait spots or Lisch nodules. Computed tomography (CT) scan showed large lobulated extra-axial mass arising from left sphenoid wing and temporal lobe apex; extending into lateral extra-coanal space of left orbit and temporal fossa destroying temporal lobe []. Magnetic resonance imaging (MRI) of the orbits and brain with and without contrast enhancement showed left sphenoidal based extra-axial marginated in-homoginously enhancing mass at the lateral side of the left optic nerve buckling the ipsilateralantero temporal lobe []. Other systemic observations of the patient were normal. No evidence of any distant metastasis was found on clinical or radiologic evaluation.\nOn this occasion, the tumor was exposed through a left lateral orbitozygomatic approach. The tumor was highly vascularised, densely adhered to the zygomatic bone and had eroded the lateral orbital wall and temporal bone of left side. Optic sheath and the duramater of temporal lobe were intact. Near total dissection of the tumor was done with microsurgical technique. The histopathological diagnosis was MPNST [] which was subsequently confirmed on the basis of immunopositivity for S-100. Immunohistochemically, tumoral cells were positive for S-100 []. Smooth muscle actin, desmin, Leu 7 and myelin basic protein (MBP) were all negative. Postoperatively, the patient recovered rapidly. Post-operative CT scan showed minimal residual mass in the orbit []. At discharge, she had improved cosmoses with stable neurological status []. Vision in her left eye improved to finger counting at one meter and extra-ocular movements were normal. She was further referred to the cancer institute where she received external beam radiotherapy. On one year follow-up, the patient is doing well with no local recurrence or any distant metastasis. |
An 18-year-old girl hailing from Kozhikode, South India, with no past history of any illness, visited our hospital with complaints of fever of 1 week duration. She also had headache that was increasing in intensity and aggravated in the early morning. She also had two episodes of vomiting. She complained of double vision on the third day of admission, which was followed by asymmetric weakness of both proximal distal muscles of all four limbs. There was no history of sensory disturbances and bladder or bowel dysfunction. On examination, her vitals were stable. There was no pallor, icterus, clubbing, lymphadenopathy, rashes or eschar on general examination. Neurological examination on admission revealed normal speech, intelligence and memory. There was no papilledema. She developed bilateral lateral rectus palsy (), with normal upgaze and papillary reflexes. Other cranial nerves were within normal limits. Motor system examination showed normal muscle bulk. Muscle groups in all limbs showed hypotonia with grade 3/5 power in the right lower limb, grade 2/5 power in the left lower limb and grade 4/5 power in both upper limbs. All deep tendon reflexes were sluggish. Sensory system examination was normal. There was neck stiffness with other signs of meningeal irritation. Rest of the systems were within normal limits. Her clinical course is summarized in .\nInitial investigations revealed normal hemogram, with an elevated erythrocyte sedimentation rate (ESR) of 95 mm in the first hour. Blood sugar, serum electrolytes and renal and liver function tests were within normal limits. Computerized tomography of brain was within normal limits.\nWith a history of fever and headache and findings of meningitis, lumbar puncture was done, which showed an opening pressure of 190 mm of cerebrospinal fluid (CSF). CSF analysis revealed a CSF total count of 30 cells with 80% lymphocytes. CSF sugar was normal, and protein was mildly elevated (60 mg/dL [normal 20–40 mg/dL]). Bacterial cultures were sterile. CSF adenosine deaminase and culture for Mycobacterium tuberculosis were negative. India ink stain for cryptococci was negative. Cytology did not show any malignant cells. CSF viral serology turned out to be positive for JE virus with positive IgM antibodies specific for the same insignificant titer. Autoimmune markers in CSF (NMDAR, LGI1, Caspr2, AMPAR [GluR1 and GluR2 subunits] and GABA-B-R) were negative.\nMagnetic resonance imaging (MRI) of the brain showed hyperintensities in bilateral thalami and in the limbic region, suggestive of limbic encephalitis (). MRI of the spine showed T2-weighted hyperintense signal intensities in the spinal cord caudally till the L1 level, predominantly involving the anterior horn cells, and rostrally, they extended upto pons, suggestive of longitudinal myelitis (). Electroencephalogram displayed slowing of delta waves in the frontotemporal region.\nThe patient was treated with supportive measures. However, she deteriorated with the development of altered sensorium and intermittent hypothermia. Oral, axillary, rectal temperature revealed a temperature <94°F on multiple occasions. Electrocardiogram at the time of hypothermia showed bradycardia, prolonged QT interval and Osborn wave (). Other causes of hypothermia such as hypothyroidism and hypoadrenalism were ruled out with normal thyroid function tests and fasting serum cortisol levels. Warm saline infusions and intermittent rewarming were given at the time of hypothermia. She developed intractable seizures and autonomic instability and succumbed to her illness after 2 weeks of hospital care.\nWritten informed consent was obtained from the parents of the patient for the publication of this report and accompanying images. |
A 19-year-old male patient complained of shoulder pain with no trauma history. He reported that the pain started about 6 months before, with progressive worsening. Pain was present in elevation and abduction, especially above 90°. The pain caused him to interrupt his physical activities, although he did not notice worsening during bodybuilding.\nA winged scapula was identified in physical exam, with scapulothoracic grade III dyskinesia, according to Kibler et al. []. He presented infraspinatus atrophy, complete range of motion, preserved upper limb strength, and discrete paraesthesia at the region of the medial border of the right scapula, with no other signs. There were no clinical signs suggestive of rotator cuff injury or glenohumeral instability ().\nThe imaging exams did not show significant changes. Shoulder MRI showed no rotator cuff lesion, labral lesions, cysts, or other soft tissue involvement. The cervical spine MRI did not show cervical discopathy or syringomyelia. Electroneuromyography with evoked potential of the scapular girdle evidenced diffuse axonal involvement of the long thoracic nerve, without other alterations.\nThe patient was then referred to the physiotherapy service, where he initiated a program of shoulder girdle rehabilitation focused on analgesia and passive mobilization. During ten weeks, he remained under the care of physiotherapists twice a week, but he did not notice an improvement in the pain. When he returned to the orthopedic clinic, he was informed about the possibility of surgical treatment.\nThe patient insisted on conservative treatment. We recommended strengthening of the shoulder girdle and swimming. During three months, he practiced swimming three times a week under the guidance of a physical education professional with experience in athlete training. In order to strengthen the periscapular muscles, he tried to practice the four classic styles of swimming, using floats in the lower limbs and increasing the demand on the upper limbs. Progressively, he noticed an improvement in his pain.\nThe swimming program consists of a 60-minute pool training three times a week, with increasing distances. The front crawl, breaststroke, and backstroke were alternated during training. The main set was a target mile, split in 200 m lengths with 1-minute rest (in a 25 m pool) and alternating strokes.\nHe returned after 90 days, free of pain. He presented dynamic stabilization of the scapula during elevation, and dyskinesia was no longer perceived. The force remained unchanged, but atrophy was no longer identified. The patient was satisfied with the progress made and was encouraged to stay in the muscle strengthening program in aquatic activities (). |
A 28-year-old man presented with a painless periodontal swelling in the right side of the mandible. The patient reported five years of evolution, with moderate discomfort during mastication as the only relevant symptom. Oral examination revealed a 2.5 cm sessile tumour on the right side of the alveolar ridge of the mandible, between canine and first premolar (Figure ). These teeth were displaced by the lesion without relevant mobility and positive responses were obtained to thermal test of pulp vitality. The lesion had a firm consistency and was covered by a normal overlying mucosa. There were no clinical signs of inflammation in spite of that surface indentations caused by their upper right canine. On radiographs, it was evidenced a rounded unilocular radiolucent alteration surrounded by a thin radiopaque membrane, with some discrete radiopaque spots. There was not radicular resorption albeit the lamina dura of the affected teeth was not apparent in the proximal aspect to the lesion (Figure ). Puncture of the tumor did not revealed liquid content, and a punch biopsy was performed to obtain a fragment with a myxoid appearance.\nMicroscopically, the sample consisted in a fibrous connective tissue alternated with more vacuolated myxomatous areas. Individual nuclear morphology of the fibroblasts varied from spindle shaped to stellate. Abundant nests and strands of odontogenic epithelium were found, often with a clear or vacuolated cytoplasm, sometimes surrounded by juxtaepithelial hyalinization (Figure ). Calcification, inflammatory cells and mitotic activity were not observed. Correlation of clinical, radiographic and histopathological features leaded to the diagnosis of central odontogenic fibroma.\nThe lesion was then entirely removed by curettage under local anesthesia. The microscopic evaluation of this material reveals the same features of the previous biopsy. All options for rehabilitation were given to the patient, who choice to use a removable partial denture. There was not any relevant event after thirteen years of follow-up. |
An 85 year old African American female presented to her primary care physician with complaint of spitting up blood. Imaging studies of the chest did not identify a cause. Five months later she presented with complaints of sinus symptoms and CT scan identified a "large right nasal polyp". Her primary care physician treated her for sinusitis, but three weeks later she presented again complaining of shortness of breath and difficulty moving air through her right nostril. She also reported a bloody nose of three weeks duration and the self discovery of a new nasal polyp one week prior to presentation. On physical exam a large polypoid lesion filled the right naris and MRI identified an extensive right nasal passage mass eroding through the cribriform plate of the right ethmoid sinus into the cranial cavity (Figure ). A tissue biopsy was obtained and two follow-up biopsies were collected at the request of the pathologist. Arrangements were made for radiation therapy, but one month later, prior to initiation of the therapy, the patient developed progressing neurologic symptoms and subsequently died from a massive intracranial hemorrhage. No autopsy was performed.\nReview of the biopsy materials showed an extensively necrotic tissue with scattered viable epithelial and mesenchymal elements. The different elements blended morphologically into one another. Atypical immature epithelial, mesenchymal and primitive neuroectodermal components were recognized at high magnification (Figure ). Immunohistochemically, the epithelial components were positive for keratins and EMA, and showed focal positivity for AFP. The mesenchymal and neuroectodermal components were positive for vimentin. HMB45, myoglobin, myogenin and bcl-2 were uniformly negative. Neural markers (S100, GFAP, synaptophysin, chromogranin, NSE) were focally positive in all elements, as were CD99 and desmin. p53 stained approximately 10% of the neuroectodermal components.\nBased on the clinical presentation, histologic findings and immunohistochemical characteristics, a diagnosis of teratocarcinosarcoma was made.\nConventional cytogenetic analysis of 20 metaphase cells identified a hyperdiploid clone characterized by trisomy 12, with an additional subclone characterized by a del(1 p). Karyotype of the tumor was described as: 47, XX, +12[]/47, idem, del(1)(p31.1p36.3)[] (see Figure , Table ). |
A 25-year-old female was admitted to the emergency department complaining of left hip pain and difficulty walking since 3 days. She was diagnosed with acquired immunodeficiency syndrome about 2 months ago and was taking prescribed highly active antiretroviral therapy. She gave no history of trauma or surgery. On examination, she was afebrile with normal vitals. Her neurological examination was normal and her lower limb examination revealed mild tenderness over the left gluteal region; however, deep tendon reflexes and power were within the normal limits. Laboratory examination showed normal blood cell counts. Her CD4+ T-cell count was 300 (within normal limits). She was referred to Department of Radio-diagnosis for plain radiograph of pelvis, which did not reveal any significant abnormality. She was subsequently discharged with a prescription of antiinflammatory drugs.\nShe returned a week later with the same complaints. On examination, she was tachycardic and febrile with a body temperature of 38.2°C. Her lower examination revealed pain on flexion and extension of left hip joint. The lower limb pulses were within the normal limits.\nShe was then referred to Radio-diagnosis Department. Since her plain radiographs were within the normal limits in the initial visit, she was investigated with a CT scan of the lower abdomen. Multiple air locules were noted in the left iliac blade and acetabulum with multiple ill-defined hypodense areas in periarticular muscles of left hip joint with surrounding fat stranding [Figure –]. A possibility of emphysematous osteomyelitis was considered and she was subjected for a fine needle aspiration cytology, a specimen of which was also sent for culture and sensitivity tests. She was empirically started on metronidazole and piperacillin. Her gram stain revealed gram-negative rod-shaped bacteria. Culture and sensitivity revealed growth of Klebsiella pneumoniae sensitive to piperacillin, amikacin, and ciprofloxacin. She was put on piperacillin and tazobactam, and metronidazole was discontinued. No complications were noted on follow-up. |
A 70-year-old woman presented in November 2017 to the Emergency Department at Skåne University Hospital, Sweden, due to the rapid onset of fever, shivers, and a suspected skin infection. She had a previous medical history of left-sided ductal breast cancer with lymph node involvement in 1999, which was treated chronologically with neoadjuvant chemotherapy, partial mastectomy, axillary lymph node dissection, and radiation therapy. In addition, in 2001, a right-sided localised ductal breast cancer in situ was identified and was treated surgically with a partial mastectomy. Secondary to her lymph node dissection, she developed lymphoedema of her left arm, which had been continuously treated with compression stockings. The patient was on treatment with an ACE inhibitor and a beta-blocker due to hypertension, and in addition, she had a known systolic murmur, characterized as physiological, as transthoracic echocardiographs in 2011 and 2017 were normal. Since her surgery in 1999, on a total of six occasions prior to her last and seventh visit, of which the first episode occurred in 2008, she had been treated for erysipelas in her left upper arm. The presentation had always been sudden with spiking fever and erythema spreading in approximately the same localisation. Interestingly, on all three out of the three occasions where a blood culture has been drawn on presentation with erysipelas, the cultures have shown growth of a bacterium belonging to the S. mitis group. These first two isolates also had similar MIC values for penicillin of 0.064 and 0.125 mg/L, for vancomycin of 0.25 and 0.5 mg/L, and for gentamicin of 2 and 2 mg/L (). In addition, they were both sensitive to clindamycin.\nOn the present visit, she once again had a sharply demarcated, warm, swollen, and painful erythema measuring approximately 7 × 15 cm in the lymphoedematous area on her left upper arm. No local portal of bacterial entry was found. Vital parameters showed a temperature of 38.0°C, respiratory rate of 16 breaths/min, O2 saturation of 96% on room air, heart rate of 80 beats/min, and blood pressure of 120/70 mmHg. On physical examination, a grade II systolic murmur was heard with punctum maximum I2 dexter. She had no signs of septic emboli, oral examination showed no signs of infection, and examination of lymph nodes was normal. Possibly due to her quick presentation, that is, less than 6 hours from the onset of symptoms, her laboratory results were normal with a white blood cell count of 8.4 ∗ 109/L, platelets of 263 ∗ 109/L, and hemoglobin of 147 g/L. Her CRP was 12 mg/L. She was clinically diagnosed with erysipelas, and due to previous bacteraemia with the S. mitis group in relation to erysipelas and the presence of a systolic murmur, blood cultures were drawn and she was treated with one dose of intravenous penicillin (3g≈5 million IU) followed by an oral penicillin (1g≈1.6 million IU) three times daily, for seven days. Once again, now for the third time, the two blood cultures showed growth of a bacterium belonging to the S. mitis group. The MIC value for penicillin was 0.125 mg/L, for vancomycin 1 mg/L, and for gentamicin 16 mg/L (). Similar to the two previous isolates, it was also sensitive to clindamycin. Her treatment was prolonged for 10 days, and a follow-up visit was arranged. Repeat blood cultures were drawn 14 days after discontinuation of antibiotics and they were negative. To prevent further infections, she has once again been referred to the lymphoedema outpatient clinic as well as to the dentist office. On follow-up, thereafter, the patient had no sequelae to her infection, and she gave informed consent for this case report to be published.\nThe three blood isolates, one analysed in 2015 and two in 2017 (15 and 8 months apart), were initially subgrouped to S. mitis/S. oralis/S. pseudopneumoniae of the S. mitis group by combining the MALDI-TOF MS results (MALDI Biotyper, Bruker) with the information that the three stains were resistant to optochin. To allow a more detailed comparison, the three stored isolates were reanalysed and now ethanol/formic acid extractions were performed on the strains, and the updated and improved Bruker MALDI Biotyper database (DB-7311 MSP Library) was used for the MALDI Biotyper analysis. In addition to the standard log (score), weighted list (scores) was also calculated []. S. mitis was the best match for both the first and second isolates when both log (score) and list (score) were calculated. For the third isolate, the best match was S. oralis for both types of scores (). Next, the mass spectra of the three isolates were inspected manually. All three strains showed the specific peak 6839.1 m/z which is associated with S mitis and S. oralis strains, but only the third isolate showed the specific peak 5822.5 m/z which is associated with S. oralis () []. In addition, no peak profiles typical for S. pneumoniae and S. pseudopneumoniae could be detected in the three isolates [, ]. These results further support that the first two isolates are S. mitis and the third isolate is S. oralis. Many differences were seen in the mass spectra of the third isolate (S. oralis) compared to the first two (S. mitis). On the other hand, no clear differences in the spectra between the first and second isolate could be seen, and one can therefore not exclude that they belong to the same clone. |
A 14-year-old Asian girl came to our hospital with blisters and erosions all over her body and in the oral mucosa. She had been diagnosed with PV by a skin biopsy about 3 months before hospitalization and had been treated with 1 mg/kg/day of oral prednisone. She had no history of diabetes mellitus, cardiovascular diseases, or psoriasis; no psychosocial history; and no familial history of pemphigus diseases. Her parents are not biologically related. The severity of the disease led to the following life-threatening issues: dehydration and electrolyte imbalance secondary to excess fluid loss from the skin wounds, as well as sepsis secondary to infection of the exposed wounds. Due to progressively worsening symptoms, extensive lesions, and high susceptibility to further infections, the patient was admitted to the intensive care unit. The following immediate actions were taken: urinary catheterization and resuscitation with intravenous fluids to correct the circulatory and electrolyte imbalance, early management of sepsis with intravenous antibiotics, and proper pain relief with opioids. The differential diagnosis included allergy eruption [], staphylococcal scalded skin syndrome, and Stevens-Johnson syndrome.\nThe patient was depressed and slightly dull. Her vital signs were as follows; temperature 38.5 °C, blood pressure 90/60 mmHg, heart rate 115 beats/minute, and respiratory rate 24 breaths/minute. Her mucosae were pale. Her capillary refill time was < 2 seconds. Her musculoskeletal system was normal. Her lymph node examination revealed no findings. Her cardiac examination revealed tachycardia with no murmur. Her lung examination revealed no rales or wheeze. Her mental status (consciousness) was slightly obtunded. The result of her cranial nerve examination was normal. Her pupils were equal with normal direct and indirect pupillary light reflexes. Her motor examination revealed muscle bulk and tone were normal. Her strength was full bilaterally. Her reflexes were normal and symmetric at the biceps, triceps, knees, and ankles. Her plantar responses were flexor. Her sensory examination revealed that her light touch, pinprick, position sense, and vibration sense were normal in her fingers and toes.\nThe patient was febrile with numerous flaccid blisters and erosions involving the oral mucosa and more than 75% of the body surface area (face, trunk, and limbs), some covered with slough (Figs. and ). She had oozing from ulceration and hemorrhagic excoriation with the peeling of the skin. Her perilesional Nikolsky sign was positive. The rest of her physical examination was normal.\nSkin biopsy confirmed the diagnosis of PV. Histology showed separated keratinocytes (acantholytic cells) just above the basal layer of the epidermis (Fig. ).\nThe patient’s complete blood count revealed the following values: white blood cells, 18,000 C/UL 18 x 109/L; neutrophils, 86%; lymphocyte count, 10%; hemoglobin, 10 mg/dl 100g/L; hematocrit, 30.3% 0.303 proportion of 1; mean corpuscular volume, 79.5 fl; mean corpuscular hemoglobin, 22.8 pg; platelets, 150 C/UL 150 x 109/L; alanine aminotransferase, 16.3 U/L; aspartate aminotransferase, 53.9 U/L; total bilirubin, 4.3 μmol/L; lactate dehydrogenase, 408 U/L; erythrocyte sedimentation rate, 27 mm/hour; C-reactive protein, 48 mg/L; blood urea nitrogen, 2.8 mmol/L; creatinine, 50.8 μmol/L; potassium, 3.5 mmol/L; and sodium, 136.4 mmol/L. Urinalysis showed no infection. Serum antibodies could not be measured, because the patient could not afford the test.\nAt the time of admission, two blood cultures and many swabs from the erosions on the patient’s trunk and limbs were collected for culture. The cultures were processed in the laboratory as per standard microbiological techniques and were cultured for aerobic and anaerobic bacteria. The organism was an encapsulated, gram-negative, rod-shaped bacterium. The isolates were identified as Pseudomonas aeruginosa. Blood culture and the pus culture from the erosions revealed the presence of P. aeruginosa. The organism was found to be sensitive to polymyxin B or colistin (colistimethate sodium).\nThe patient was treated with oral prednisolone at a dose of 1 mg/kg/day with oral azathioprine at a dose of 150 mg/day (3 mg/kg/day) on admission for 7 days and intravenous ceftazidime 1 g three times per day + oral levofloxacin 500 mg once per day. Treatment with these antibiotics continued until the results of blood and pus cultures were obtained. The bullous skin lesions had breakdown, leaving painful, eroded areas of skin with a similar appearance to the type of wounds found on burn victims. Early assessment by the dermatologists and plastic surgery team resulted in the placement of appropriate dressings to protect the skin wounds and relieve the patient’s distress. One week after the treatment, she did not show any improvement, and she developed fever spikes every day. Blood culture and pus culture from the erosions revealed the presence of P. aeruginosa. On the basis of sensitivity reports, she was started on intravenous colistin (colistimethate sodium) 9 mIU/day in three divided doses for 10 days. Because the disease remained active, the prednisolone dose was increased to 1.5 mg/kg/day 3 days after the start of antibiotics. Ten days after that, the erosions persisted but showed slight evidence of re-epithelialization. The patient’s Nikolsky sign was still positive, and she continued to develop a few new blisters. She also developed edema of the genitalia. Because the disease remained active and she was in sepsis with persistent fever, we could not consider giving her intravenous corticosteroids. Considering all the above factors, she was given four sessions of plasmapheresis over 10 days with a gap of 2 days between the sessions. Two liters of plasma were removed using a plasma filter attached to the dialysis machine and replaced with 1 L of normal saline and 1 L of fresh frozen plasma. The procedure was done by a nephrologist in the nephrology department of Tishreen Hospital. After the fourth session of plasmapheresis, the patient’s Nikolsky sign became negative, and no new blisters appeared. The erosions showed re-epithelialization. Serum antibody levels before and after plasmapheresis sessions could not be measured, because the patient could not afford the test.\nAround 90% of the erosions healed after 1 month of plasmapheresis (Fig. ), and oral prednisolone was gradually tapered over 3 months. The patient was maintained on daily oral prednisolone at a dose 0.5 mg/kg/day, and no new blisters formed (Fig. ). |
A 48-year-old married male was brought by his family in the emergency department with an acute onset of elation, grandiosity, distractibility, pressured speech and hallucinatory behavior for the past 2 months. Patient had a history suggestive of two manic episodes of moderate severity, with complete inter-episodic recovery in last 4 years. There was no past history of any depressive episode or medical comorbidity. There was no family history of medical or psychiatric morbidity or substance use. A diagnosis of bipolar affective disorder was made. The current episode was diagnosed as manic with psychotic features.\nA detailed treatment history and perusal of medical records revealed that 3 years ago, he had experienced persistent painful penile erection when he had been on sodium valproate (1500 mg/day) and risperidone (6 mg/day). This was unrelated to sexual arousal or stimulation and lasted for around 20-24 hours. He had five to six such episodes over a span of 7-10 days. Despite significant distress, he did not report the problem to the clinician. This was the only adverse effect experienced by the patient. He discontinued taking the medication and got relief from the priapism after 3-4 days of the last dose, but had another manic episode 2 years ago.\nIn light of existing medical literature, priapism was attributed largely to risperidone in this instance. However, it was difficult to attribute an adverse effect due to drug combination solely to a single agent. In view of history of priapism possibly due to risperidone and valproate, lithium carbonate was started as the mood stabilizer and olanzapine as anti-psychotic during the second episode. Over the next 6 months, olanzapine was gradually tapered-off. Around 8 months ago (nearly 1½ year after initiation) lithium carbonate was stopped after lithium induced hypothyroidism was detected. Thereafter, he was prescribed sodium valproate again; suspecting that earlier episode of priapism was attributable to risperidone, as sodium valproate has not been reported to cause priapism. Sodium valproate was started and doses were increased upto 1500 mg/day, with corresponding serum levels of 100 mg/l. This time patient adhered to treatment with sodium valproate at doses of 1500 mg/day continuously for a period of 5 months, until he developed priapism. This happened around 3 months ago and attributing this condition to medication, patient discontinued treatment and got relieved of this adverse effect. |
The patient was a 96-year-old Caucasian woman who lived alone. She was visited by carers four times a day. She presented to our hospital two years after a recurrence of a right neck mass.\nIn 1999, she underwent a right partial mastectomy for ductal carcinoma (T2NXM0) and received adjuvant chemoradiotherapy. She remained disease-free and was discharged from follow-up ten years later.\nIn 2011, she was referred by her GP for a suspicious lesion on her right neck. This was diagnosed as a poorly differentiated squamous cell carcinoma and completely excised. She was discharged back to the care of her GP.\nBetween 2013 and 2015, she had been admitted on three occasions to a short-stay ward at a District General Hospital (). Of these, one was for presyncope and the others for urinary tract infections, anaemia, and general deterioration. She had been seen by geriatricians, general physicians, and rehabilitation staff. The patient declined investigation or intervention and was discharged with no follow-up.\nIn 2015, after being alerted to the patient's poor condition by her carers, the patient was visited at home by her GP. From there, the patient was referred to the Head and Neck MDT and to our hospital for admission. She presented to a long-stay care of the elderly ward with a bilobulated, fungating, 8 × 10 cm mass in the right neck (). This mass had grown over a two-year period. Her principle complaints were of pain and the inability to lie comfortably.\nShe appeared cachectic, and apart from the neck mass physical examination was unremarkable. The patient was aware of the possible malignant nature of the neck mass. During the two years from its recurrence to her final admission, she had not sought medical attention for it.\nShe said she wanted to be “left alone,” fearing that surgery and anaesthetic would lead her to “die on the table.” She also expressed a general reluctance to be “interfered with.” When asked, she expressed a wish to be pain-free and to die at home. She was unaware of palliative approaches to treatment.\nInitial blood tests showed Hb of 80 g/L; MCV of 87.9 fL; CRP of 36 mg/L; and WCC of 8.4 × 109/L. Kidney function was deranged with a urea of 10.7 mmol/L and creatinine of 117 mmol/L. Following transfusion of two units of blood, her haemoglobin recovered to 126 g/L.\nA plain chest radiograph showed a left upper lobe mass that was not present on radiographs two months previously. A CT chest/abdomen/pelvis demonstrated a 6 cm left upper lobe lung mass, a lobulated, likely nodal, 5 cm mass in the caecum, and the right neck mass which measured 8 cm × 10 cm.\nA punch biopsy of her neck mass was arranged, which was reported as an invasive, poorly differentiated, squamous cell carcinoma.\nOn admission, the patient was referred to the palliative care team. Pain control was rapidly attained with regular oramorph and subcutaneous morphine sulphate titrated against response.\nControl of agitation was achieved with subcutaneous midazolam, and subcutaneous hyoscine butylbromide effectively reduced respiratory secretions.\nThe patient was reviewed by the Head and Neck MDT who recommended palliative radiotherapy, followed by fast-track referral to a nursing home. She developed a hospital acquired pneumonia and received two days of intravenous piperacillin-tazobactam as per hospital guideline. Spiritual support was provided by the hospital chaplaincy service which was funded internally.\nAfter input from the hospital chaplain, the patient appeared more settled and accepting of the inevitability of the outcome.\nHowever, the decision to commence radiotherapy was subjected to two multidisciplinary team reviews. This had the effect of delaying the onset of this definitive palliative treatment.\nAs a consequence, the patient died before radiotherapy could be initiated. The final agonal event was a hospital acquired pneumonia ten days after admission. |
A 41-year-old woman who complained of chest tightness after performing an activity, presented with aggravation of symptoms in our hospital. She was diagnosed with pulmonary embolism in a different hospital, prior to her transfer in our institution. The patient occasionally had cough, expectoration, blood in the sputum, and nocturnal sweating, but she had no edema in the lower extremities, fever, smoking history, and related genetic history in the family. There were two transient syncope episodes in the course of the disease. Blood oxygen saturation was 72% and carbohydrate antigen 125(CA125) was 230.2 U/ml at admission. Other physical examinations and laboratory data were unremarkable. Computed tomographic pulmonary angiography (CTPA) showed that there are low-density filling defects in both PAs, and then the patient was diagnosed with PTE (Fig. ). However, venous thrombosis could not be found in her lower extremities. Echocardiography showed at the proximal end of the main PA and its left and right branches, a hyperechoic mass of approximately 13 × 13 mm in size, which appeared to have invaded the pulmonary valve, extending down to the right ventricular outflow tract (Fig. A). It also showed an enlargement of the right heart, severe tricuspid regurgitation and pulmonary hypertension. Moreover, the lateral pulmonary branch of the body could be seen to form (Fig. B). Thus, ultrasonographers considered that the mass is a space-occupying lesion involving the right ventricular outflow tract and pulmonary valve instead of PTE.\nBased on these diagnoses and the patient's clinical manifestations, emergency surgery is required. Intraoperatively, the ratio of the aorta to the main PA was found to be 1: 1.3. In the exploration of the main PA under the support of extracorporeal circulation, a large number of polyp like substances, which are irregular in shape, invaded the pulmonary valve, extending down to the septal side of the right ventricular outflow tract, and involving the far end of the left and right PAs. We performed a partial pulmonary endarterectomy and the sarcomatoid material was taken out from the PA (Fig. ). The specimens (Fig. A)were submitted for intraoperative pathological examination, and the mass was diagnosed as an endometrial sarcoma of the PA, the histologic type is high-grade pleomorphic undifferentiated sarcoma (Fig. B). We then performed partial pulmonary valve resection and repair of the pulmonary trunk with a pericardium patch. Afterwards, tricuspidplasty was also conducted.\nPostoperative echocardiography showed that the PA pressure was significantly reduced and the patient's condition was improved. The immunological histological chemistry (IHC) results were as follows: ER(−), PR(+), CD31(−), CD34(−), DOG(−), SMA(+), Desmin(+), H- Caldesmon(+), EMA(-), CK(AE1/AE3)(+), Vimentin(+), S-100(−), K167(30%), LCA(−), ALK(-), CD30(−), CD17(−), DOG-1(−), WT19(−)and calretinin (−). The patient stayed in the intensive care unit (ICU) for 3 days and transferred back to the general ward. However, she refused to undergo radiation and chemotherapy. During the follow-up via telephone 6 month after the operation, the patient reported that she has been feeling well. |
A 67-year-old woman reported an accidental injury to the right eye from a falling chestnut. She visited an eye clinic nearby, and was treated with ofloxacin ointment. On the next day, cell infiltration was observed around the corneal wound, suggestive of deterioration of the corneal infection. Therefore, she was referred to our hospital.\nThe patient complained of mild pain. At first visit, her best-corrected visual acuity (BCVA) was 0.8 in the right eye and 1.5 in the left eye. Slit-lamp examination showed a perforation at the center of the right cornea; leakage of aqueous humor through the perforation was observed (Fig. ). The Seidel test, which evaluates aqueous leakage on the cornea using fluorescein, was positive, although the anterior chamber was deep (Fig. ). Cell infiltration was observed around the wound with conjunctival injection, and moderate cells were observed in the anterior chamber. No foreign body was found in the conjunctival sac, cornea, or anterior chamber. The iris was damaged, and the anterior lens capsule was opacified with pigments (Fig. ). AS-OCT showed a corneal wound involving the endothelial layer (Fig. ). The central corneal thickness was 603 µm. Additionally, slight cataract and early pterygium were observed. A gonioscopic examination showed no hemorrhage or foreign body at the angle of the eye. No fundus abnormality was observed. The patient was in good general health with no significant past medical history. Bacterial examination of corneal scrapings showed gram-positive cocci and a few neutrophils, but no fungi.\nThe patient was diagnosed with a corneal perforation and bacterial keratitis. Levofloxacin 1.5% and cefmenoxime administration every 2 hours, atropine administration once a day, and oral acetazolamide were initiated. A soft contact lens that was made of hydroxyethyl methacrylate was placed on her right eye in order to seal the wound.\nOn day 2 after the injury, the Seidel test was negative and the use of the soft contact lens was discontinued. There was no progression of cataract. However, there was an increase in cell infiltration with conjunctival injection.\nOn day 3, folds of Descemet membrane appeared (Fig. ). BCVA of the right eye decreased to 0.7. There was no improvement in the corneal cell infiltration. Slit-lamp examination and a repeat AS-OCT suggested inner wound closure (Fig. ). The corneal endothelial cell density was 2,778/mm2 in the right eye and 2,793/mm2 in the left eye. Steroid therapy was withheld because fungal infection had not been ruled out.\nOn day 9, the patient reported no ocular pain. Slight cells were observed in the anterior chamber with slight conjunctival injection. There was improvement in cell infiltration. A culture test revealed the presence of Propionibacterium acnes (P. acnes), which is sensitive to both levofloxacin and cefmenoxime. The test was negative for fungi. Because both antibiotics were thought to be effective, we continued the same treatment and decreased the frequency to 6 times a day. Atropine and acetazolamide were discontinued.\nOn day 14, BCVA of the right eye had improved to 0.9. The opacification of the anterior lens capsule decreased with slight cells in the anterior chamber. However, cell infiltration at the deep corneal stroma persisted.\nOn day 26, the patient was completely asymptomatic. The opacification and cell infiltration at the center of the cornea had improved (Fig. ). AS-OCT showed healing of the corneal wound with reduction in the central corneal thickness to 523 μm (Fig. ). The corneal shape analysis suggested that the wound did not affect corneal irregular astigmatism (Fig. ). No progress of cataract was observed and BCVA improved to 1.0. |
We report here the anesthetic management of hemicolectomy for cecal malignancy in a 46-year-old, 71 kg male patient, who had a history of 15 days hospitalization for acute intermittent porphyria (AIP), 11 years ago. The diagnosis of AIP had been earlier confirmed by elevated porphyrin levels in the urine and the presence of an R167Q mutation in the porphobilinogen (PBG) deaminase gene. Neurological examination revealed foot drop in the right lower limb, which was a sequel of the acute attack. Biochemical and hematological reports were now within normal limits.\nThe patient was reassured and premedicated with intravenous (IV) midazolam 1 mg. An IV infusion of 10% dextrose 1000 ml was given overnight. An exhaustive list of drugs documented or suspected of precipitating AIP and a list of safe drugs was prepared from currently available literature and placed in the operation theatre for ready reference. We utilized IV fentanyl 100 μg, IV morphine 4.5 mg and IV propofol 120 mg for induction of anesthesia. IV vecuronium 6 mg was the neuromuscular blocking agent injected and 60 s later when the peripheral nerve stimulator was switched on, the single second twitches had nearly disappeared. The trachea was intubated with a 7.5 mm ID, polyvinyl chloride cuffed orotracheal tube fixed at 22 cm after confirmation of placement by auscultation and capnography. A BIS-guided propofol infusion, desflurane in 66% air-oxygen mixture, peripheral nerve stimulator guided vecuronium infusion at 1 mg/h with two top-up boluses of 1 mg each were utilized for maintenance of anesthesia. The surgery lasted 2 h 30 min. The neuromuscular blockade was reversed with IV neostigmine 2.5 mg and IV glycopyrrolate 0.4 mg. The patient was extubated after he was seen to consistently generate a tidal volume of 450-500 ml for 15 min. He was given 3 mg morphine for pain at surgical site. After 20 min of extubation he became hysterical, complained of difficulty in breathing and developed stridor and had to be re- intubated with a 7.5 mm ID nasal endotracheal tube using awake fibreoptic intubation after 10% lignocaine spray on glottis and transtracheal block with 2 ml 4% lignocaine. We started a 10% dextrose infusion. The patient was extubated over bougie after 3h following full motor recovery. IV morphine 3 mg 6 hourly and IV paracetamol 1 g 12 hourly were utilized for postoperative pain relief. Normal colored urine was observed during hourly urine output monitoring throughout intraoperative and postoperative periods. The patient was discharged after 6 days. |
A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event. |
A 57-year-old lady, a non-smoker and non-drinker with good past health, presented with a week’s history of sore throat, runny nose and productive cough. Upon admission, she was having high fever and sinus tachycardia. Her neck had skin erythema and crepitus on palpation. Oxygen saturation was maintained. Her lateral neck radiograph showed the presence of retropharyngeal gas and chest radiograph noted consolidative changes at the right lower zone. A septic work-up was performed and her laboratory evaluation revealed significant metabolic acidosis.\nThe urgent contrast computed tomography neck and thorax revealed multiple gas locules in bilateral deep cervical spaces, loculated right pleural effusion and right chest wall subcutaneous emphysema. The overall radiological features were suggestive of underlying deep neck space infection by gas-forming organisms complicated with descending mediastinitis and empyema thoracis (see Figs –).\nEmergency surgical debridement and drainage were performed by combined bilateral transcervicotomy and right video-assisted thoracoscopic approaches. All deep neck spaces down to mediastinum were opened. A moderate amount of turbid fluid was drained and necrotic fascia and muscle were debrided. The remaining collection inside the thoracic cavity was drained through thoracoscopic approach. The neck wound was laid open and packed with gauze. Multiple right lower posterior teeth with severe periodontitis were extracted. Chest drains were inserted at the conclusion of operation.\nThe patient required high dose inotropes throughout the operation. She was kept intubated in ICU afterwards. Daily wound examination and dressing was done. Second look wound exploration and debridement were performed on Day 3. Residual collection over the superior mediastinum and right parapharyngeal space was drained. The patient was tracheostomised. A feeding tube was inserted to help build up her nutrition. She was dependent on inotropic and ventilatory supports during the first 4 weeks. Her neck wound was amenable to primary closure 6 weeks after the first debridement. The tracheostomy was successfully decanalised. She was provided with a course of swallowing rehabilitation and was discharged 2 months later.\nHistopathology sectioning of the right carotid sheath, fascia and mediastinal tissue confirmed necrotizing inflammation. Both blood and deep neck tissue cultures recovered C. parapsilosis. The carotid sheath tissue and pleural fluid recovered Peptostreptococcus micros, Bacteroides pyogenes and Prevotella buccae. Intravenous anidulafungin was added to her existing broad-spectrum antibiotics. |
A 4-year-old male child was referred to us for complaint of pain in abdomen. On enquiry, there was intermittent history of bilious vomiting since infancy. There was no previous history as regard to abdominal distension. However, there was occasional history of abdominal pain, which was relieved by medication. On examination, his abdomen was distended, but it was non-tender. The weight of the patient was 9 kg. Thus he had failed to thrive. His ultrasound (USG) of abdomen suggested some fluid filled cystic spaces. A computerized tomography (CT) scan of abdomen was advised for confirmation of the diagnosis.\nOn next day, just after CT abdomen, the patient suddenly developed severe abdominal pain. X-ray abdomen showed pneumoperitoneum. Clinically the patient looked septic. His pulse rate was 130/min with a feeble pulse. An urgent laparotomy was planned. After initial stabilization, the patient was explored under general anesthesia. On exploration, we noticed malrotation of the gut, which was associated with two turns of midgut volvulus. The midgut did not have any evidence of ischemia or gangrene. It was complete malrotation having cecum in left hypochondrium and narrow mesentery. Ladd's bands were also present. The peritoneal cavity was grossly contaminated with fecal fluid. The perforation site was about one foot proximal to ileocecal region. The perforation was at the mesenteric side, and it was associated with a soft mesenteric mass communicating with the normal bowel. The mass was about 10 cm in length. The perforation was just at the junction of the mesenteric mass and the normal bowel [].\nAfter performing the Ladd's procedure, attention was placed towards the perforation. On attempting to resect the mesenteric mass, we noticed two lumens in the bowel distal to the perforation site and single lumen proximal to that [Figures and ]. The duplication appeared to be having all the layers of the bowel wall. When an attempt to ascertain the length of the duplication was made by inserting two Ryle's tube through the lumens, it was reaching up to ileocecal region. As the general condition of the patient was poor, complete resection of the bowel was not done, and a double barrel (triple barrel, to be more precise) was fashioned.\nIn the postoperative period, there was wound infection with superficial wound dehiscence. It was managed conservatively. The patient was discharged after 3 weeks with proper follow-up advise. During the follow-up, we had closed the ileostomy. As the duplication was reaching up to cecum, we had performed ileoascending anastomosis. The patient is fine. |
A 44-year-old woman with a history of metastatic triple negative breast cancer and lung metastases presented with a six-month history of recurrent haemoptysis. She had no other significant medical history. She was initially managed for her right breast cancer with a wide local incision and adjuvant chemoradiotherapy in 2014; however, her malignancy recurred two years later. She had positive margins on subsequent right mastectomy and proceeded to excision of the right pectoralis major and overlying dermis. Six months later she was found to have bilateral pulmonary metastases and underwent initial diagnostic bronchoscopy identifying a bleeding mass in the medial segment of the right middle lobe (RB5), which was subsequently treated with topical adrenaline and biopsied – confirming metastatic disease. Her malignancy progressed despite palliative chemotherapy with epirubicin and cyclophosphamide, during which time she developed worsening haemoptysis of ~1/2 cup (~120 mL) daily. A multidisciplinary decision was then made to perform therapeutic bronchoscopy due to excessive distress caused to the patient because of haemoptysis. She underwent bronchoscopy using a therapeutic video bronchoscope (Olympus BF-TH190, Olympus Corporation, Tokyo, Japan) introduced via a rigid bronchoscope, which provided secure airway access. Endobronchial survey revealed the source of bleeding in the distal right middle lobe, although the actual bleeding source was not directly visible. A volume of 2 mL of TISSEEL was injected into the right middle lobe bronchus via a catheter followed by deployment of a size 6 Spiration (Redmond, WA, USA) IBV to add stability and prevent expectoration (Fig. ). A further 1 mL of TISSEEL was then applied over the valve (Fig. ). The procedure abolished the patient's haemoptysis instantly.\nTwo weeks later the patient developed recurrent haemoptysis; however, repeat bronchoscopy showed a different source of bleeding in the right lower lobe, with the existing combination TISSEEL and IBV still in place in right middle lobe and maintaining haemostasis. To control the new bleeding TISSEEL was injected in the right lower lobe bronchus distal to the opening of RB6, followed by deployment of a size 9 IBV. Further injection of TISSEEL was then applied and haemostasis was achieved. Unfortunately the patient was found to have brain metastases and died of her malignancy 10 weeks later, without recurrence of haemoptysis. |
A 57-year-old Chinese woman presented to Tianjin Union Medicine Center, Tianjin, China, with rectal adenocarcinoma at Dukes’ stage C. Before presenting to the hospital, the patient was not taking any drugs and had a completely negative medical history. On 7 October 2008, the patient was treated by low anterior resection, at a distance of 13 cm from the anus and radical resection was performed. Before surgery, it was not known whether the patient had lymph node metastasis or whether the tumor, which was not large in size, could be completely resected by surgery, and so neoadjuvant chemoradiotherapy was not performed.\nFollowing the operation, from 31 October 2008 to 10 April 2009, chemotherapy treatment (oxaliplatin) was carried out five times every month with a total of six cycles. On 4 June 2009, six lesions with a maximum diameter of 0.5 cm were removed by inner lens polyp resection. Six months later, a lesion measuring 2.3 cm × 1.4 cm, at a distance of 0.5 cm from the left side of perianal region, was removed and pathology analysis indicated adenocarcinoma. On 13 March 2010, another lesion measuring 1.5 cm × 1.0 cm × 1.0 cm was removed from the left groin. Pathology analysis indicated lymph node metastasis. Figure a shows the pathological findings of this lesion. On 31 March 2010, the patient could not tolerate chemotherapy with irinotecan and so chemotherapy was terminated. From 12 April 2010 to 18 June 2010, the patient received radiation therapy on the bilateral inguinal region and anus 32 times, including 10 times with enhanced radiation therapy. On 6 August 2010, a perianal lesion measuring approximately 1.3 cm × 0.8 cm × 0.9 cm, 1 cm from the left anterior anal wall (close to the perineum), was removed. Pathology analysis indicated adenocarcinoma with cytokeratin CK20+ (Figure b). There was a small postoperative anal defect in the patient. On 30 September 2010, multiple right inguinal nodules, measuring a maximum of approximately 1.6 cm × 0.7 cm, were removed. Pathology analysis indicated metastatic lymph node adenocarcinoma (5/7 nodules). On 11 November 2010, the perianal nodules (measuring 2.2 cm × 1.3 cm × 0.3 cm), the adenocarcinoma nodules in the skin and subcutaneous tissue, and three right inguinal nodules (CK−) (measuring a maximum 1.4 cm × 1.4 cm), were resected (Figure c).\nOn 23 February 2011, three perianal tumors (measuring 2.0 cm × 2.0 cm × 0.4 cm; CK+, CEA+−), 0.3 cm to 1.3 cm from the anal margin and left posterior anal wall of the external sphincter (left, middle and rear), were resected (Figure a). On 31 May 2011, the left inguinal lymph node was resected and analysis indicated lymphadenia (measuring 1.0 cm × 1.5 cm × 0.2 cm) (Figure b). The anal margin tumor near the location of the nodule, found on 6 August 2010, was resected and pathology analysis indicated adenocarcinoma (measuring approximately 0.6 cm × 0.7 cm × 0.2 cm). On 23 September 2011, tissue measuring 0.9 cm × 0.5 cm × 0.7 cm was removed, but analysis indicated that it was adipose tissue. On 25 October 2011, the perianal lesions (measuring 1.4 cm × 0.6 cm × 0.8 cm; near the last end tailbone) left behind the anal margin were removed. Pathology analysis indicated CK staining was negative (Figure c).\nFollowing the initial resection surgery, the patient attended hospital for surveillance examination every 3 months. B-mode ultrasonography, chest X-ray and blood biomarker tests were performed. Every 6 months, CT scanning was used to find any LR and distant metastasis. Until 9 April 2013, the patient is alive and has good condition. |
A 74-year-old woman presented to our emergency department with abdominal and back pain that had started two days earlier. The patient further complained of nausea and vomiting and described a worsening of her abdominal pain with postural changes. The physical examination of the patient showed diffuse abdominal tenderness, but no rebound tenderness. Her blood pressure was 190/90 mm Hg and the heart rate 78 beats per minute without the use of inotropic drugs. The laboratory findings were as follows: hemoglobin 12.9 g/dL, white blood cell count 8.07 × 109/L, and C-reactive protein, 0.4 mg/L. The patient's medical history included deep vein thrombosis and pulmonary embolism with subsequent placement of Celect IVC filter (Cook Medical, Bloomington, Indiana, USA) approximately eight years ago. The patient had undergone IVC filter implantation in another hospital, and the exact reason for the insertion could not be ascertained.\nComputed tomography (CT) revealed a perforation of the IVC wall by one of the filter's struts and penetration into the duodenum. We did not identify another cause of abdominal pain in our examination (Fig. ).\nOur first attempt to remove the IVC filter through cavography failed. The patient consecutively underwent median laparotomy. On retraction of the duodenum to the right, one strut of the filter could be seen protruding the vessel wall and penetrating into the duodenum (Fig. ). The strut was removed from the duodenum entirely, and the perforation site was sutured primarily with Vicryl (Ethicon Inc., Somerville, NJ). The IVC was dissected at the level of the left renal vein and the confluence of common iliac veins to permit safe clamping. An IVC clamp was then used to clamp the IVC proximally just below the renal vein. A second IVC clamp was placed above the iliac vein confluence. We then opened the IVC and found that the IVC filter was obscured by intimal fibrosis. The IVC filter struts were separated with wire cutters and removed without significant IVC injury (Fig. ). The IVC was repaired with a running 5-0 Prolene (Ethicon Inc., Somerville, NJ) suture, and the abdomen was closed in standard fashion. The patient's postoperative hospital stay was uneventful without any complications. On hospital day 12, she was discharged. We followed her up for six months. We followed the patient up and her CT did not show any abnormal findings six months after discharge. |
An 81-year-old man who had started suffering from dementia after a stroke 3 years prior was being fed through a nasogastric tube long-term and experiencing intermittent shortness of breath. He had been admitted and treated for aspiration pneumonia over the course of 11 episodes within 3 years.\nOn this visit, his shortness of breath had persisted for 3 days. On admission, he was found to have severe dyspnea and a violent cough with mucoid sputum. The patient had no apparent fever or leukocytosis. His vital signs were as follows: heart rate – 112 beats/min, respiratory rate – 32 breaths/min, and blood pressure – 128 / 68 mmHg. A physical examination revealed bilateral coarse crackles without wheezing. We also observed hypersecreted saliva and nasal discharge that caused the patient to frequently choke. His sputum culture was negative for bacteria, tuberculosis, and fungus. A chest radiograph (CXR) revealed bilateral lower lung infiltration []. Amoxicillin with clavulanic acid was administered at 1200 mg every 8 h. One week later, the CXR revealed left lower lung consolidation and bilateral multiple pulmonary nodules []. A chest computed tomography (CT) scan revealed that the nodule reflected water attenuation with a density of −15.28 Hounsfield units without enhancement after contrast administration []. The nodules were located in both central and distal airways and even within the acini of an axial image [] and a coronal reconstructive image []. Metastatic lung cancer could not be ruled out, and thus, a chest CT-guided biopsy was performed. The histopathological findings revealed interstitial fibrosis and mild chronic inflammation with a few multinuclear giant cells and suppurative exudate mixed with necrotic debris, suggesting aspiration foreign body granulomas complicated with an infection []. In addition, we performed a periodic acid–Schiff stain, a Gomori's methenamine silver nitrate stain, and an acid-fast stain, which were all negative. There was no evidence of malignancy. Therefore, to treat the mucoid impaction, a tracheostomy was performed with intensive sputum clearance. Three months later, the CXR [] and chest CT [Figures -] showed nearly total obliteration of the nodules and lung consolidation and some residual peripheral bronchiectasis. The patient recovered well except for frequent constipation with paralytic ileus requiring future admission. Unfortunately, 2 years later, the patient expired due to severe colon volvulus that resulted in septic shock. |
A 65-year-old man was referred by the otolaryngology department to our outpatient clinic due to sudden swelling and mild pain around the right eye. On examination, the patient exhibited what appeared to be severe edema encompassing the upper and lower lids of the right eye (). Crepitus was clearly audible on palpation of the eyelids. An attempt to open the lids was unsuccessful. Visual acuity and intraocular pressure could not be measured due to extreme lid swelling. The patient reported that he had undergone transnasal endoscopic nasal polypectomy through the right nostril 2 days earlier. He said he had been instructed not to cough or strain after the endoscopic nasal surgery and the sudden swelling occurred immediately after severe coughing and straining. We suspected that the sinus wall was weakened due to his endoscopic surgery and the increased pressure caused by straining had forced air in the nose into the periorbital area. B-mode ultrasonography showed trapped air in the periorbital area ().\nConsidering the patient’s anxiety, the severity of periorbital emphysema, inability to conduct a full ophthalmologic examination, and the risk of complications such as compressive optic neuropathy, the patient was re-evaluated for a surgical intervention. After consultation, it was decided to evacuate the air using a 21 gauge needle inserted in the subcutaneous tissue of the upper and lower lids. In sterile conditions, the eye area was cleaned with 10% povidone-iodine. A 21-gauge needle was passed through the skin and subcutaneous tissue of the upper and lower lids parallel to the tarsus about 1.5 cm from the lid margin. Evacuation of subcutaneous air was evident from a significant reduction in lid swelling during the procedure (). The patient’s vital signs were stable and the procedure was concluded. He was discharged with systemic antibiotics (cefuroxime axetil 500 mg twice daily) and moxifloxacin drops four times daily.\nOn follow-up examination the next day, the periorbital emphysema was substantially reduced and the globe could be examined (, ). He had full visual acuity in both eyes; intraocular pressure was 17 mmHg in the right eye and 16 mmHg in the left eye. Dilated fundus examination was normal. No restriction in eye movements was observed. Follow-up examinations at 1 week and 1 month revealed no pathological findings. |
This case involves a 44-year-old woman with uncontrolled type 2 diabetes and severe peripheral neuropathy in both feet. In late 2012, during routine follow-up for her right foot chronic ulcers, a growth was found underlying the plantar aspect of her right first metatarsal phalangeal (MTP) joint. She described this lesion as “sharply painful”; her other ulcers caused her a “more dull pain.” Eventually, the growth became cauliflower-like and drained a pungent, toothpaste-like substance. shows this exophytic lesion within a diabetic ulcer prior to excision. A wide excision of this lesion was performed by a dermatologist in October 2013. Pathology confirmed a diagnosis of VC. The patient was followed up closely by dermatology and podiatry. In December 2014, a biopsy was performed in the area of her previous VC showing recurrence of the disease. She was referred to another dermatologist for confirmation of this diagnosis versus one of Charcot arthropathy. Mohs resection of the lesion was performed in March 2015, with pathology confirming VC. Podiatry concluded that difficulties with wound healing in the area of the first MTP were likely due to VC recurrence rather than Charcot arthropathy.\nThroughout early 2015, the patient and her home care team noted an acutely painful lesion on her left foot on the plantar surface of the 5th MTP joint (). The patient suspected VC; however, due to the rare nature of VC tumor, clinical suspicion was low for bilateral disease. Unfortunately, the patient was not seen for this lesion again until May 2015, when an incisional biopsy was taken of this growth. Pathology confirmed VC; however, as the biopsy was small and the base of the lesion was not visualized, two more biopsies of the lesion were collected between May and July of 2015, all showing VC. Mohs resection of the tumor was performed, showing complete resection of the lesion. shows the left foot after Mohs resection of this lesion. In November 2015, the patient's right second toe was amputated due to complications from her ulcers. Pathology described a prominent verrucous epithelial hyperplasia, with focal atypia that approaches but is not diagnostic of VC. |
The proband was a 34 year-old right-handed man. From the age of 26 years, involuntary movements of the bilateral lower limbs, associated with dysarthria, grinding teeth and drooling, appeared and gradually worsened. At 31, he suffered from epileptic seizures, which were considered to be generalized tonic-clonic seizure, but antiepileptic drugs had never been administered. One year later, involuntary movements spread to his upper limbs and orofacial automatisms including abnormal tic-like facial movements, tongue protrusion and biting his lips appeared. Then he was treated with haloperidol (2 mg three times a day) and baclofen (10 mg three times a day) for 2 years for his choreic and dystonic problems, but he responded poorly to drug treatments. At age of 34, his involuntary movements gradually spread to his whole body and epileptic seizures increased in frequency. Since the disease onset, the patient had never suffer from psychiatric problems. Neurological examination revealed poor muscle tone and absent deep tendon reflexes in all limbs. Additionally, right positive babinski sign was elicited. Laboratory data revealed elevated creatine kinase level in the peripheral blood. Acanthocytes were found in 4% of cells on the peripheral blood smear test. Doppler ultrasound examination revealed splenomegaly. Brain magnetic resonance imaging (MRI) showed progressive, symmetrical, mild atrophy of the caudate heads (Figure ). His 24-h continuous electroencephalography (EEG) showed generalized asynchronous theta and epileptiform activity, which mostly originated from the right temporal lobe. A nerve conduction study showed a polyneuropathy, which revealed the right peroneal nerve, right median nerve and bilateral ulnar nerves were partly damaged. His score of Mini Mental Status Examination (MMSE) was 27. The father of the proband did not show any neurological abnormalities and died from pneumonia at 65 years old (Figure ). The mother of the proband (II-3), a 65-year-old woman, showed mild involuntary movements in her limbs since the age of 45 years (Figure ). The proband's uncle (II-5), a 52-year-old man, showed mild cognitive impairment (MMSE 24), characterized by memory impairment and had seizures history of 31 years, which were simple partial seizures and treated with antiepileptic drugs (Figure ). His another uncle (II-1) and two sisters (III-1, III-2) had no neurological clinical symptoms. Brain MRI and peripheral blood smears of the proband's mother, his uncles and two sisters are normal. The clinical picture of the proband was suggestive for ChAc, but the inheritance mode of this family seems to be autosomal dominant.\nAll patients were of Han nationality from Hunan province, China. Blood specimens and genomic DNA were obtained from family members and 100 control subjects after informed consent. The 73 exons and flanking intronic splice consensus sequences of VPS13A were amplified by polymerase chain reaction (PCR) (, ). By sequencing, we identified a novel homozygous nonsense mutation c.8823C > G (p. Tyr2941*) in exon 65 of VPS13A in the proband (Figure ). Five members of the family including the proband's mother (II-3) and his uncle (II-5) were detected to be heterozygous for mutation c.8823C > G (Figures ). The homozygous nonsense mutation c.8823C > G (p. Tyr2941*) causes the loss of TPR10 domain of the vacuolar protein sorting 13A protein. This homozygous nonsense mutation c.8823C > G was not detected in 100 healthy controls, thus representing a novel etiology in an ChAc Chinese family. Besides, the mutations in genes responsible for Huntington's disease and McLeod disease were screened and the results were negative. |
A 48-year-old woman with morbid obesity and persistent dysphagia after resection of a left jugular foramen schwannoma was referred for a PEG tube placement. The PEG tube was placed uneventfully with the external bolster placed at a 5-cm external mark. The patient presented to the emergency department (ED) four days later complaining of epigastric pain with minimal leakage around the PEG tube site. An x-ray abdomen with contrast showed the tube to be in a good position and flushing well. Three days later, the patient’s primary care physician prescribed clindamycin for the concern of PEG tube site infection.\nAfter a week, the patient presented to the gastroenterology clinic complaining of continued leakage from the site around the tube. The external bolster was at a 6-cm external mark. The tube was re-inserted further with the external bolster tightened to a 4.5-cm external mark, and the patient was instructed to complete the course of clindamycin. The patient presented to the ED three days later with an inability to infuse tube feeding or even water. Physical examination showed normal vital signs. The abdomen was soft with no appreciable tenderness or clinical signs of peritonitis. There was no evidence of distention or palpable masses. There was no evidence of induration at the patient’s PEG site and the external bolster was noted at a 5.5-cm external mark with mild surrounding erythema. The patient underwent an abdominal x-ray with contrast which showed the tube to be outside the stomach with concern for retraction into the gastrocutaneous tract. Subsequently, a CT scan confirmed the retraction of the internal bolster into the subcutaneous fat just adjacent to the gastric wall (Figure ).\nThe patient was admitted to the hospital for intravenous (IV) antibiotics and fluid hydration. The patient underwent an esophagogastroduodenoscopy (EGD), which revealed a hole in the anterior wall of the gastric body (Figure ).\nThe PEG tube was removed externally with simple traction. A plastic trocar (without needle) was used to check the integrity of the tract (Figure ).\nA guidewire was introduced into the stomach and snared, and a PEG tube was placed through the same tract with the external bolster left at a 4-cm external mark. Tube feeding was initiated 24 hours later, with recommendations not to remove the PEG tube for 12 weeks, and to complete the course of the antibiotics. The patient was discharged home in two days. At a follow-up visit several weeks later, she was tolerating tube feedings well without any complication. |
A 49-year-old and otherwise healthy man underwent a traffic accident and was transferred to emergency room in an unconscious state. Brain computed tomography (CT) revealed traumatic subdural and epidural hemorrhage at the bilateral frontal lobes and right temporal lobe. No abnormality in the extrapyramidal motor system including the basal ganglia was present (). The initial Glasgow Coma Scale (GCS) score was 5 (eye opening response [E]=1, verbal response [V]=0, motor response [M]=4). After receiving burr-hole trephination and hematoma drainage, the patient was reevaluated to have a GCS score of 11 (E1, V5, M5). Although verbal and motor responses improved after surgery, he was unable to open his eyes even with a strong painful stimulus. In spite of gradual recovery of mental status and motor function, voluntary eye opening was still not observed. After being transferred to the rehabilitation unit, the patient was examined for the neurological function with mental status being alert and the Mini-Mental State Examination score at 7. He exhibited left side weakness that was graded fair to good in a manual muscle test. The pupils were 3 mm in diameter, symmetrical, and equally reactive with extraocular movements fully intact. With the patient unable to open his eyes on command, an examiner elevated the patient's eyelid and found no abnormality in the ocular motor function. No evidence of other apraxia, such as buccofacial and limb apraxia, was present. He received comprehensive rehabilitation treatment, but AEO did not improve. We planned on treating the patient with levodopa/benserazide 200/50 mg twice a day with the informed consent of the patient. On the fifth day of the treatment, he started to open his eyes on command intermittently (). We confirmed no visual loss since he was able to recognize color and count fingers during spontaneous eye opening. With continued treatment, the interval between command and eye opening gradually shortened while the duration of spontaneous eye opening increased. This result was even more apparent when eating and undergoing physical training. We presumed that the dopaminergic treatment had a beneficial effect on AEO and tried to increase dose frequency from twice to three times daily to investigate a dose-dependent response. However, in the third day of the dosage modulation, aggressive behaviors emerged, and the treatment was withheld for three days. Even though the aggressiveness subsided shortly thereafter, the patient again exhibited difficulty in voluntary eye opening. After the resolution of the behavioral problem, the treatment was resumed twice a day. With AEO ameliorated, as evident in spontaneous eye opening during eating and physical training, the patient was discharged and has been closely followed up on an outpatient basis with the treatment of levodopa/benserazide 200/50 mg twice a day. He is currently capable of voluntary eye opening that is sustained much longer than before being discharged. |
The patient was a 57-year-old man who had been diagnosed with right-sided aortic arch and Kommerell's diverticulum. Since he was asymptomatic at diagnosis, he was being monitored. The patient developed type B acute aortic dissection (DeBakey IIIa), and the dissecting aortic aneurysm diameter rapidly increased. He was referred to our hospital 4 months after onset of type B acute aortic dissection. Multidetector-row computed tomography (CT) showed right-sided aortic arch, an anomaly in which the left common carotid and left subclavian artery originated from Kommerell's diverticulum, and dissecting aortic aneurysm with a maximum diameter of 60 mm. Surgery was indicated (Figs. , ). Although thoracic endovascular aneurysm repair was considered, single-stage surgical repair of extensive thoracic aorta was selected because the proximal landing zone was short and aortic curvature was severe.\nIn surgery, the aneurysm was approached through median sternotomy and right posterolateral fifth intercostal thoracotomy, and extracorporeal circulation was established by infusing blood through the ascending aorta and right femoral artery and draining blood from the right atrium. Kommerell's diverticulum was on the posterior side of the esophagus, with the left common carotid and subclavian artery branching out on the left side of the trachea. Circulation was arrested at a core temperature of 26°C, and the aorta was dissected. The brain was protected by selective cerebral perfusion where a blood delivery cannula was inserted into the right common carotid artery, right subclavian artery, left common carotid artery and left subclavian artery. The aorta was clamped at the periphery of the right subclavian artery, and systemic circulation was resumed by infusing through the femoral artery. The proximal side was anastomosed using a 24-mm Intergard with four branches (St. Jude Medical, St. Paul, MN). The branches for reconstructing the left common carotid and subclavian artery were prepared using the lateral branch, and the left common carotid artery, left subclavian artery, right common carotid artery and right subclavian artery were reconstructed in that order. At this stage, blood was perfused from the first branch to maintain coronary and cerebral circulation. The aorta was clamped immediately above the diaphragm, a distal anastomosis was performed, and the Kommerell's diverticulum inlet was closed from inside the aorta. The duration of myocardial ischemia and extracorporeal circulation was 9 min and 152 min, respectively (Fig. ). The postoperative course was favorable, and the patient was discharged without any complications. He is doing fine as of 4 years after surgery.\nSurgery is not necessarily indicated for Kommerell's diverticulum accompanying aortic anomaly. However, if clinical symptoms are present related to vascular ring, such as respiratory and swallowing impairments, surgery is indicated for aneurysm formation because of the risk of peripheral embolism, aortic dissection and rupture associated with Kommerell's diverticulum [,]. Although the patient was asymptomatic, surgery was indicated because of Kommerell's diverticulum, aortic dissection and aneurysm diameter enlargement. Surgery for descending aortic aneurysm accompanying right-sided aortic arch has only been described sporadically [,]. Hybrid procedures combining with thoracic endovascular aneurysm repair and bypass has been reported in recent years [,]. Although we considered hybrid procedure, we decided on single-stage surgical repair of extensive thoracic aorta because cervical branch bypass was required, the proximal landing zone peripheral to the right subclavian artery was short, and aortic curvature was severe. |
A 66 year old Caucasian, post-menopausal woman was admitted to hospital under the care of the general physicians. She had a three months history of cough and productive green sputum, weight loss, poor appetite and frequent night sweats. Interestingly there was no history of vaginal bleeding.\nClinical examination revealed she was febrile. On auscultation of the chest, crepitations were audible. The abdomen was soft but tender on palpation of the lower abdomen.\nInitial investigations included an elevated CRP (279.8) and white cell count (25.4). She was anaemic with a haemoglobin of 8.2 g/dL. Chest X-Ray showed basal atelectasis but nil else.\nThe patient was transfused with two units of blood and broad spectrum intravenous antibiotics were commenced. A few days later the patient developed vomiting, abdominal pain and was noted to have a brown vaginal discharge. In view of her continuing poor general condition, the lower abdominal tenderness and discharge, imaging of the abdomen and pelvis was arranged.\nA CT scan revealed a huge pyometra extending up to the umbilicus and an intrauterine contraceptive device was noted to be in situ (Figure ). No lymphadenopathy was evident and the remainder of the abdomen was normal. The patient's care was then transferred to the gynaecology team.\nAfter taking a thorough gynaecological history, it emerged that the patient had a Lippes loop inserted 25 years previously. She was not counselled regarding the importance of re-attending to have the coil checked or told that she would need to have it removed at some point. In addition to this, the patient was not aware that she needed to have regular cervical smear tests and was then unfortunately lost to follow up. Closer examination revealed a tender 24 weeks size uterus.\nShe was taken to theatre, where 1600 ml of pus was drained from the uterus and the coil removed. However, at operation the cervix was noted to be suspicious. Biopsies were taken and further examination under anaesthesia clinically confirmed cervical cancer.\nAfter drainage of the pyometra and administration of appropriate antibiotics, the patient made a good recovery from her septic state. Histology later confirmed a stage 1b poorly differentiated squamous cell carcinoma of the cervix. She was referred to the oncologists and is due to have a radical hysterectomy. |
A 43-year-old man with a history of chronic alcoholism presented with abdominal distension. The previous day, the patient had presented to a local hospital with anal bleeding and abdominal pain after an incidental insertion of barbecue skewer per anus in the drunken state; subsequently, he had undergone sigmoid loop colostomy for rectal perforation. However, after the operation, the patient had become hemodynamically unstable. At presentation, his systolic blood pressure was 90 mmHg and the pulse rate was 135 beats/min. Although there was no gross rectal bleeding, the digital rectal examination revealed a penny-sized anterior rectal wall defect 6 cm from the anal verge (AV). Computed tomography (CT) revealed a hematoma (12 × 10 × 15 cm) with active bleeding in the pelvic cavity and a pseudoaneurysm in the anterior wall of the rectum (). Since the patient was hemodynamically unstable, an emergency operation was performed. During the operation, a massive subperitoneal hematoma in the rectovesical pouch and large amount of blood in the peritoneal cavity were found. After evacuation of the hematoma and blood, oozing continued in the rectovesical pouch (). Thus, compression with gauze was performed for 30 min until the oozing stopped. The Hartmann procedure was performed with the suspected bleeding focus included, but the perforation site was not included.\nAlthough the postoperative course was uneventful and there was no evidence of recurrent bleeding on the follow-up CT on the 7th postoperative day (POD), a focal enhancing lesion in the anterior wall of the rectum indicating a residual pseudoaneurysm was noted (). On the 11th day POD, his hemoglobin decreased from 11.6 g/dL to 7.9 g/dL, and the follow-up CT revealed recurrent hematoma (6.0 × 4.2 cm) in the pelvic cavity and the residual pseudoaneurysm (). Following the diagnosis of recurrent bleeding from the residual pseudoaneurysm, an angiography was performed. However, the angiography failed to localize the pseudoaneurysm, and definite signs of extravasation could not be ascertained. Thus, prophylactic gelfoam embolization at the anterior branch of both the internal iliac arteries was performed (). The subsequent hospital course was uneventful, and the patient was discharged on the 25th POD. After 3 months, the previous rectal lesion (AV: 6 cm) healed, and colostomy reversal was performed without morbidity. |
The patient was a 25-year-old female patient who was admitted to hospital on May 28, 2018 for “9 years of frequent urination before and after menstruation and 1 year of dyspareunia.” When the patient was four years old, she had uncontrollable and persistent urine leakage, for which she had been diagnosed with “congenital bladder valgus and urethral mucosal prolapse” and underwent surgery (a urogenital tract malformation repair operation). After the surgery, her persistent urine leakage remained uncontrolled. The patient gained control over her urination approximately 2 to 3 years after the surgery, when her urine overflowed slowly without spray urination. Occasionally, the patient presented with spray urination during urinary urgencies; however, no involuntary urine overflow was noted. Since the patient's menarche at 17 years of age, the number of urinations had increased 4 to 5 days before and after menstruation. The urinary volume was small, and sometimes her urinations occurred as frequently as once per hour. Five years ago, the patient presented with repeated urinary tract infections that usually lasted for 6 months before they were resolved by anti-infection treatment. A year ago, when the patient started having sex with her boyfriend, the man's penis bled due to abrasion, and the patient palpated a hard lump in her vagina. The patient visited a local hospital, and a B-ultrasound examination showed a 59 × 55 × 23 mm fusiform region of increased signal intensity in the vagina followed by a sound shadow. There were no obvious abnormalities in the uterine attachments (Fig. ).\nA physical examination after admission revealed that her vulva shape was normal. There was an old longitudinal surgical scar in the middle of her pubic symphysis, and its lower end reached the left side of her clitoris. A urethral catheter was inserted into the urethra through the opening for approximately 70 mm, and no urine flow was observed. The long diameter of the vaginal opening was 20 mm. A brown substance seen in her vagina had a hard texture, felt like a stone, was visible on direct observation, and measured approximately 60 × 50 mm in size. There was urine outflow during the palpation. The urinary fistula and the cervix could not be seen. After admission, while under anesthesia, vaginal stone removal and cystoscopy were performed. The tissues around the fistula were carefully separated, and the stone was completely removed (Fig. ). At 3 months after the stone was removed, we repaired the urethrovaginal fistula. An examination revealed that the cervix was normal. The cystoscope was inserted into the urethra from the urethral opening for 20 mm and revealed that the broken end of the urethra was connected to the vagina. The proximal broken end of the urethra was 20 mm from the distal end. The surface of the fistula was mucosal, and the bladder was seen after the cystoscope entered the broken end of the urethra. No obvious abnormality was observed in the bladder cavity, and urine ejection was observed at the opening of the bilateral ureters. The patient was diagnosed with a vaginal stone and urethrovaginal fistula. The patient had recovered well at 1 month after the operation. Urine frequency symptoms and pain during sexual intercourse were relieved. A follow-up was conducted 1 month after the operation and then every 3 months.\nBecause this report only reviewed previous data and did not involve any human trials, there was no need to conduct special ethical review, and ethical approval was not necessary. Written informed consent was obtained from the patient for the publication of this case report and its accompanying images. |
A 36-year-old man was referred to our clinic from his family dentist with a complaint of pain around the anterior maxillary region on the right side. The patient’s medical history was non-contributory. Intraoral examination identified a firm, non-fluctuant mass with no ulceration in the vestibular region between the right upper central incisor and canine (Figure ). The right upper central and lateral incisors were missing. A computed tomography (CT) showed severe bony resorption between the right upper second premolar and the left upper lateral incisor (Figure ). Incisional biopsy was performed via vestibular approach. The specimen of the tumor was extirpated with overlying mucosa. During the biopsy, an intact cortex was not observed. The histological diagnosis was moderately differentiated SCC. The connection between the tumor and the overlying mucosa was not observed. Contrast-enhanced computed tomography (CE-CT) revealed a destructive tumor with no cystic lesion, >50 mm in diameter. The tumor pressed the skin around the nasal ala and the nostril of the right side, but no direct connection between the skin and tumor was observed. Although the maxillary sinus and nasal cavity were deformed by the tumor, no abnormal findings were seen for the mucosa of the nasal cavity and maxillary sinus (Figure ). One swollen and enhanced lymph node was recognized in the submandibular area of the right side. Positron emission tomography (PET) revealed the enhanced lesion in the maxilla and the right submandibular area. There was no evidence of metastatic disease on chest radiography, upper gastric endoscopy or PET. Based on these findings, solid type PIOSCC was diagnosed.\nThe patient underwent tumor ablative surgery. After bilateral supraomohyoid neck dissection, the primary tumor was radically excised. The surgical specimen comprised the anterior two-thirds of the hard palate and nasal septum, bilateral inferior nasal conchae and the skin around the nasal ala and nostril of the right side. The surgical defect was reconstructed using a partially double-folded free radial forearm flap and prefabricated denture-based surgical obturator. Microscopic examination of the surgical specimen revealed SCC without cystic component in the maxillary bone. The islands of the tumor cells extended into the bone (Figure ) with no dysplasia or carcinoma in the skin and mucosa of the nasal cavity and maxillary sinus. The dissected surgical specimen of the neck showed one lymph node metastasis of the ipsilateral submandibular region. Although adjuvant radiotherapy was recommended, the patient declined additional treatment. The postoperative course was quite good. Neither recurrence nor metastasis had been found as of 3 years and 1 month postoperatively. |
Upon assurance of patient's consent to participate in the study by signing the written consent form, from 8 months before the investigation, a 34-year-old married female patient (a medical staff) with a history of BN and weight and height of 52 kg and 164 cm, respectively, had been initially treated with slow-release bupropion at an initial dose of 75 mg due to sadness and impatience (mostly in the mornings) as well as hypersomnia and overeating. The mentioned dose reached 300 mg within 2 weeks. The patient was simultaneously taking 300 mg of bupropion with 20 mg of propranolol and 10 mg of chlordiazepoxide 10 at noon. The patient stated that her appetite would be decreased only in case of taking the three pills simultaneously. During the 8 months, the patient lost 6 kg of weight. Accordingly, after a while, she gradually increased the dose of slow-release bupropion to 450 mg/day due to the persistence of weight. Following such an increase in bupropion dose from 300 mg to 450 mg, after 3 days, she was suffering from painful muscular contractions in the legs, thighs, respiration, and swallowing and also abdominal pain, drooling, and suffocation feeling. She then referred the emergency department and received 10 g of diazepam intramuscularly. However, no change in symptoms made her suspected to be with acute dystonia. To this end, she was treated with 2 mg of biperiden intravenously, and then symptoms were resolved shortly. After 3 days, with the beginning use of 450 mg of bupropion again, the symptoms of acute dystonia begin to recur, which is ameliorated by the injection of biperiden. Two days later, the patient took 300 mg of bupropion again, which also led to acute dystonia symptoms.\nThe patient stated a history of BN disorder since the age of 15, during which she had used different calorie diets, fluoxetine tablets, ritalin tablets, diethylpropion hydrochloride tablets, laxatives, and bisacodyl suppositories as well as the intragastric balloon method. Besides, the patient stated no history of head trauma, convulsions, fever, and drug use. There was also no history of psychiatric disorders and physical diseases in her family.\nNormal complete blood cell counts, calcium and magnesium levels, vitamin B12 levels, folic acid levels, and ferritin levels were reported. The patient's brain magnetic resonance imaging (MRI) also reported normal conditions. Accordingly, following diagnosis of bupropion-induced acute dystonia, the patient was advised to discontinue bupropion intake. In the follow-ups done 2 weeks later, 3 months later, and 6 months later, no signs of acute dystonia was observed. |
This patient—a police officer, 35 years old at the time of his initial visit in October 1995—reported he had had a round, uniformly red rash on his upper inner right leg just below the knee, roughly the size of a lemon, some time 5–10 years earlier. It was thought to be a ‘spider bite’ and no treatment was given. He developed hip pain in 1992 with anxiety, panic attacks, and palpitations. In summer 1994, extreme fatigue developed, and by fall 1994 he was feverishness, with malaise and flu-like symptoms, chills, mild unrelenting headache, and a stiff neck. In December 1994, disorientation, memory and speech difficulties developed.\nHe was active out of doors for work and recreation in Rockland County, New York. He hiked extensively in the lower Hudson River Valley and had an indoor/outdoor dog.\nA physician treated him with amoxicillin for two weeks with improvement, but symptoms recurred following cessation of the drug. A second course of amoxicillin of four weeks duration likewise improved his symptoms, but they relapsed following cessation of the agent. The treating physician advised the patient that he suspected him to have Lyme disease and he was referred to an infectious diseases specialist, who did not agree with the diagnosis, as serologic tests were negative. Neurological consultation was obtained. An MRI of the brain with and without gadolinium was normal in May 1995, as was spinal fluid. Although Lyme disease was not able to be confirmed by laboratory methods, the neurologist advised the patient that it had not been ruled out. In June 1995 he was treated for six weeks with IV ceftriaxone, with initial intensification of joint symptoms, but with subsequent marked overall improvement. By fall of 1995 he experienced a relapse of symptoms, feeling ‘hung over’ and also a sense of breathlessness. A pulmonary evaluation was obtained. The angiotensin converting enzyme was elevated but pulmonary evaluation did not sustain a diagnosis of sarcoidosis.\nThe brain single photon emission computed tomography SPECT at Columbia Presbyterian Medical Center in November 1995 showed diffuse heterogeneous cerebral hypoperfusion, with decreased up-take in the white matter. A Lyme ELISA at the State University of New York at Stony Brook was negative, and a Western blot showed no bands on IgM but 39 and 41 kiloDalton (kDa) bands on IgG [].\nHe experienced multi-system symptoms, including headaches, stiff neck, chills, photosensitivity, swollen glands that accompanied his cycling symptoms, mood swings, sleep disturbance, joint pain, debilitating fatigue, cognitive and balance difficulties, and diminished libido, among others.\nAware of his cognitive difficulties, the patient often found himself in situations that could involve split-second decisions on resorting to deadly force. He sought and received medical leave from duty in the fall of 1995.\nFormal detailed neuropsychological testing in May 1996 demonstrated impairments in practical reasoning, social judgment, auditory processing speed, primary visuospatial processing, and visual memory—areas of particular premorbid strength. These objective findings corroborated the patient’s subjective sense of cognitive difficulties and buttressed the administrative decision to grant medical leave.\nFrom November 1995 to February 1996 he was treated with high dose amoxicillin (maximum dose was 3.5 g of amoxicillin Q 8 h, along with probenicid) aimed at CNS Lyme disease, achieving a peak amoxicillin blood level of 20.6 mcg/mL (therapeutic for CNS = 15–25 mcg/mL) []. Treatment was combined with clarithromycin in doses as high as 1.5 g PO Q 12 h. Despite this, he was getting lost while driving in familiar areas, had difficulty expressing himself, and experienced intolerable anxiety. Minocycline was substituted for clarithromycin.\nIn January 1996, a Lyme Western blot at the State University of New York at Stony Brook showed 30, 39, and 93 kiloDalton bands on IgM and 41, 45, and 58 kDa bands on IgG [,].\nIn March 1996, oral amoxicillin was discontinued, and IV ceftriaxone (2 g/day) was initiated and continued, with some hiatuses, until October 1996, when it was suspended due to the development of asymptomatic pseudocholelithiasis. Imipenem/cilastatin (1 g IV Q 8 h) was substituted until December 1996, and then cefotaxime (2 g IV Q 8 h) was administered until February 1997, when this drug was discontinued due to leukopenia. Oral minocycline was continued until October 1996, when clarithromycin was resumed and minocycline stopped. Altogether, treatment with IV antibiotics had continued for the bulk of the eleven months from March 1996 to February 1997, with occasional hiatuses.\nDuring this treatment period, the patient reported improved mental sharpness, with easier word retrieval and facility of speech, diminished depression and anxiety, and improved sleep quality and libido.\nRepeat brain SPECTs at Columbia Presbyterian Medical Center in October 1996 and April 1997 showed interval improvements, with progressive improvement in cortical heterogeneous and white matter hypoperfusion, however the pattern did not return to normality.\nOral antimicrobial therapy, with high dose amoxicillin and probenicid, combined with clarithromycin, was resumed in March 1997.\nIn April 1997, the patient was able to resume work on a limited schedule. The patient felt he had improved to about 65–70% of his normal ‘pre-morbid’ status and felt he was still making gains on oral antimicrobial therapy, although he experienced stiffness about his hips and deep bone pain in his thighs. Oral antibiotics were changed to cefuroxime (1 g) and azithromycin (0.5 g PO Q 12 h).\nIn May 1997 at the State University of New York at Stony Brook, a Western blot showed 37 and 41 kDa bands in IgM and 18, 39, 41, and 93 kDa bands on IgG [,,].\nIn late May 1997, the patient’s psychiatrist added donepezil, which resulted in improved word-finding and confidence with speech. The patient felt psychologically improved, and felt his condition was ‘holding’ on oral antimicrobial therapy.\nIn July 1997, on his own advice, he adopted a ‘pulse’ approach to oral antibiotic usage, partly due to loose bowels when on intensive treatment, despite ample probiotics. Diarrhea remitted. He felt at “75%” of his pre-morbid status and was able to resume full-time police work by fall 1997. Oral azithromcyin was discontinued and instead cefuroxime (2 g PO Q 12 h) was combined with doxycycline (150 mg PO Q 12 h) []. In December 1997, monotherapy with azithromycin (500 mg PO Q 12 h) was begun, to which cefuroxime was added in January 1998. He was maintaining his ground on oral antimicrobial therapy.\nIn February 1998, the patient sustained an Achilles tendon rupture while playing sports, and surgical repair was undertaken. He had never received any treatment with quinolone antimicrobials.\nIn July 1998, the patient was doing well on a rotating schedule of single antimicrobial agents, which included cefuroxime, doxycycline, amoxicillin, and clarithromycin, sequentially. In October 1998 the patient was feeling well and he suspended all antibiotic treatment. In April 1999, he was working hard, full time, occasionally doing ‘double shifts’. In July 1999, he noted spells of feeling ‘sick’ with malaise, sinusitis-like symptoms, stiffness about his hips, and some decrease in articulateness. In November 1999, he took a two-week course of left-over cefuroxime (1 g Q 12 h). In an exam in December 1999 he was noted to exhibit mild ataxia on tandem gait with eyes closed. Over the prior spring, summer, and fall, he had been hiking, including ‘bushwacking’, through dense brush in Harriman State Park and the Shawangunk Mountains—heavily tick-infested areas—but he was unaware of any known tick attachments.\nIn February 2000, he reported experiencing monthly flares of symptoms, which had been absent since the prior spring but had begun to resurface in the summer and fall of 1999. High dose amoxicillin was resumed but then discontinued from March to August of 2000. Modest doses of amoxicillin were resumed until October 2000, then discontinued, as the patient was unclear as to the cause of his symptoms or the efficacy of amoxicillin. He modified his habits, with more rest, and avoidance of alcohol and caffeine. Nonetheless, right hip pain severe enough to interfere with sleep, dull head pressure, anxiety and panic attacks, and feeling ‘at a loss’ for words recurred. He suspected that Lyme disease was responsible. In January 2001 he resumed left-over doxycycline, using up to 400 mg/day, and noted that his hip pain resolved within a few days []. Further doxycycline was prescribed for a few months.\nIn September 2001, he developed fatigue so severe he could barely get out of bed, along with muscle and joint pain and stiffness, and he resumed left-over doxycycline. By November 2001, he was feeling relatively well once again. He reported hiking in Harriman State Park several times per month until the fall of 2001, again, without known tick attachments.\nIn November 2001, a Western blot at the State University of New York at Stony Brook showed band 23 on IgM and bands 23 and 41 on IgG.\nDoxycycline was continued until January 2002, when it was discontinued by the patient, who was feeling relatively well. Within one month of discontinuing the drug, muscle and joint stiffness recurred. Doxycycline was resumed but after one additional month it did not seem to be conferring benefit. He had a deep aching in his thigh muscles ‘close to the bone’, diminished stamina, bilateral Achilles tendon pain, and recurrent cognitive symptoms. Treatment was changed to cefuroxime, combined with clarithromycin, and by June 2002 the patient was feeling remarkably better.\nA decision was made to treat ‘episodically’ as seemed necessary, and discontinue when a satisfactory status was achieved, with the option to resume treatment if needed.\nThe patient remained relatively well off antibiotic therapy from June 2002 until January 2004, when sinusitis-like symptoms occurred. He was treated for sinusitis by a physician, with amoxicillin/clavulanate for 10 days, and felt better for several weeks. Sinusitis-like symptoms recurred, along with fatigue, leg pain, heaviness, stiffness, and weakness. He reconsulted the physician he had seen and was re-treated with the same regimen. All symptoms responded. The patient began to suspect Lyme disease once again because of the recurrence of this familiar constellation of symptoms. He used a friend’s left-over cefuroxime (1 g Q 12) for some three months and felt better.\nHe was feeling fairly well and able to function. He had had no known tick attachments but had been in the woods a lot with his dog at Harriman State Park, in June 2004. After the outing, he removed some 20 ticks from the animal.\nIn late August 2004, off antimicrobials, a dry cough developed that lasted six weeks, which was resolved with cefuroxime but recurred 3 weeks later. This resolved with amoxicillin, which he acquired from a friend.\nHe commented that sinusitis had been a prominent symptom with the onset of his illness in 1994, and that an annoying ‘dry’ cough was a frequent symptom that responded to antibiotic therapy. He averred that on antibiotic therapy he generally would feel well, his anxiety would decrease, and his libido would improve.\nWith recurring symptoms of Lyme disease, and ongoing exposure risk, a laboratory re-assessment for tick-borne infection was undertaken in December 2004, which revealed a positive Lyme ELISA at the State University of New York at Stony Brook, with an optical density of +0.206 and a positive cut-off of 0.152, but a concurrent Western blot showed only band 41 on IgM and IgG. Empiric re-treatment with cefuroxime and clarithromycin was instituted and continued until February 2005. With this, he felt better and suspended further treatment.\nIn March 2005 right elbow pain and right knee pain developed, which required three–four weeks to resolve, and which flared again in early June 2005, along with a dry cough and some anxiety. He resumed left-over cefuroxime and clarithromycin—the joint pain improved within several days of resumption of antibiotics and the cough and anxiety resolved.\nIn February 2006, he returned for care, reporting significant stiffness since the prior November, interfering with his functioning and quality of life. He was also experiencing loss of words and, once again, difficulty with his sense of direction, and expressed that he was living a ‘diminished’ life. Oral amoxicillin was prescribed at 3500 mg PO Q 12 h, and he remained on this between February and October 2006, feeling relatively well.\nHe retired from the police department after 20 years of service in August 2006.\nIn late February 2007, he developed a mild fever and chills, worse with exertion, which lasted 10 days. Once again, he began to consider Lyme disease. During 2007, he resorted to short courses of azithromycin, doxycycline, or amoxicillin when symptoms which he interpreted as denoting Lyme disease (sinusitis, right knee and left Achilles tendon pain, anxiety) recurred, achieving temporary surcease and returning to a sense of relative well-being.\nIn late 2007 and early 2008, the patient experienced periodic ‘flares’ of symptoms he attributed to Lyme disease, with sinusitis-like symptoms, excessive somnolence, headache, feverishness, and a stiff neck. In May 2008, minocycline was prescribed.\nHis psychiatrist prescribed duloxetine, which was tried without benefit.\nA Lyme ELISA at the State University of New York at Stony Brook, March 2008 was positive, with an optical density of 0.239 and a positive cut-off of 0.140, with a 41 kDa band on the IgM Western blot but no bands on the IgG blot. A Western blot at IGeneX in May 2008 showed the presence of 31, 34, and 41 kDa bands on the IgM Western blot [].\nA brain SPECT in April 2008 at Columbia Presbyterian Medical Center showed moderate global cortical hypoperfusion and heterogeneity, which had worsened compared to the prior study of April 1997.\nIn May 2008, otolaryngology was consulted in view of recurring sinusitis-like symptoms. An MRI of the sinuses was clear of any anatomical features typical of sinusitis, despite the patient’s symptoms.\nIn September 2008, despite treatment with minocycline, the patient reported a worsened cognitive status, with poor comprehension and sense of direction and an inability to read, which he usually enjoyed.\nIV ceftriaxone was instituted in April 2009 and continued, with some hiatuses related to difficulties with vascular access during the spring and summer of 2009, for a total of 28 administered doses. Parenteral treatment was combined with oral clarithromycin.\nAround this time, serologies returned positive for exposure to Babesia duncani from several different laboratories, including the Public Health Laboratory of the County of Sonoma, California. Treatment aimed at babesia piroplasms was instituted for the first time, using atovaquone/proguanil.\nParenteral and oral treatment was discontinued in September 2009, with greatly improved symptoms, including the resolution of cognitive difficulties, with the patient feeling generally well.\nIn November 2009, following a hike in Harriman State Park, he removed a fully engorged tick from his skin. A 50 cent piece-sized area of inflammation developed around the tick bite site, and a one-month course of doxycycline was taken.\nIn December 2009, chills, fatigue, and difficulty regulating his body temperature occurred, and the patient took left over amoxicillin, up to 5.25 g PO Q 12 h, with no impact on his chills. Ceftriaxone was resumed until March 2010 with atovaquone/proguanil, chills subsided, and he was feeling well.\nIn May 2010, he was on azithromycin and atovaquone liquid and feeling well with no further chills or sense of ‘air hunger’. Artemesinin was added (150 mg PO BID), and by September 2010 he reported feeling the best he had in a year [].\nFrom November 2011 to April 2014, the patient was under the care of a practitioner who utilized benzathine penicillin, atovaquone, clarithromycin, and sulfamethoxazole/trimethoprim for his treatment.\nIn November 2013, he had sustained a new, engorged deer tick attachment, for which doxycycline was prescribed for 21 days.\nIn December 2013, serology for exposure to Babesia duncani was reactive from one commercial reference lab, and in March 2014 this was confirmed at a second commercial lab.\nA Lyme PCR in whole blood was positive for detection of the OspA plasmid target, at IGeneX, April 2014, confirmed by southern dot blot. A Lyme Western blot at the same lab showed the presence of a 39 kDa band and faint bands at 31 and 41 kDa on IgM and 41 and 58 kDa bands on IgG, with a faint band at 23 kDa.\nBenzathine penicillin was discontinued in May 2014, and the patient was treated with intramuscular ceftriaxone, initially at 1 g in a regimen of two days on and one day off (per the patient’s preference), along with a regimen of oral treatment mostly consisting of azithromycin (0.5–1 g PO QD), atovaquone (250 mg) and proguanil (100 mg PO X 2 BID), as well as liquid atovaquone (ranging from 750–2250, mg twice daily), but orals were usually limited to five consecutive days per week (again, per the patient’s preference).\nDue to inadequate control of symptoms, ceftriaxone was increased to 2 X 1 g IM, two days on and one day off, September 2014.\nTreatment was changed to IV ceftriaxone between December 2015 and February 2016. At that point, an attempt to transition from parenteral ceftriaxone with oral azithromycin and atovaquone to oral minocycline or doxycycline with artemether/lumefantrine proved unsuccessful. All treatment was suspended in March 2016, but a return of the symptoms of tick-borne infection required reinstitution of atovaquone with azithromycin []. A fully implanted vascular access device was placed, and the patient was treated with IV ceftriaxone (2 g/day) from May 2016 until August 2016. From August 2016 to January 2018, the patient was treated with IM ceftriaxone (1 g X 2), two days on and one day off. Attempts at cessation of treatment with azithromcyin and atovaquone resulted in the onset of drenching night sweats.\nThe patient was advised of the impact of treatment with disulfiram in the patients described in Cases 1 and 2, and after due consideration, including a discussion of its potential risks and its uncertain utility in the treatment of human Lyme disease, he requested a trial of treatment with that agent. Correspondence was held with his treating psychiatrist before treatment was initiated.\nDisulfiram (250 mg tablets, 1–2 per day) was prescribed, and the patient initiated treatment in mid-January 2018, discontinuing all other antimicrobial treatment.\nDisulfiram resulted in profound fatigue that interfered with functioning, and initially the patient was unable to tolerate more than 125 mg of disulfiram every other day. The dose was gradually increased from January to April, when he was able to tolerate 500 mg/day for about the last two months of treatment (ending in late May 2018). Periodic surveillance laboratory testing was satisfactory. Notable to the patient was that, despite the discontinuance of azithromycin and atovaquone in mid-January of 2018, he experienced no recurrence of night sweats, even shortly after initiating only low dose disulfiram. He remained clinically well on no antimicrobial treatment until December 2018. He noted improved libido following the course of disulfiram. In retrospect, he opined that the debilitating initial effects of disulfiram seemed most consistent with Jarisch–Herxheimer-like effects, which he had experienced with the application of conventional antibiotics during his course of care.\nIn November 2018, he requested interval testing for tick-borne diseases stating, however, that he remained clinically well. Surprisingly, Lyme PCR for detection of the plasmid target in the serum returned positive. Babesia duncani antibodies at the County of Sonoma Public Health Laboratory also returned reactive, at 1:256. Babesia microti antibodies were negative, as were direct detection for B. microti and B. duncani by PCR and F.I.S.H at IGeneX.\nDuring December 2018 and January and February of 2019, the patient experienced left Achilles tendon and right hip pain, the latter interfering with sleep and reminiscent of past relapses of Lyme disease. On his own advice, he administered a five-day course of IM ceftriaxone, with left-over supplies that he had on hand. The course, from 12 February 2019 to 16 February 2019, reduced musculoskeletal pain by some 60%, by his estimation.\nA second course of disulfiram was initiated on 19 February 2019, starting slowly with 0.5 X 250 mg every other day, with the intention to ramp the dose up over several weeks to 750 mg/day, remain at that dose for 90 days, and then discontinue the agent for a period of observation. |
A 63-year-old man with a history of hypertension presented to our emergency department with a 3-day history of a high fever (39°C) and abdominal pain. Initial laboratory results and computed tomography (CT) images suggested the presence of acute cholecystitis with a perforated gallbladder, which was initially treated with percutaneous drainage of the gallbladder and administration of intravenous antibiotics. However, the CT images also revealed a complex AAA comprising two separate aneurysms: a 3.7 cm saccular aneurysm at the suprarenal level, and a 6.6 cm fusiform aneurysm above the iliac bifurcation. The suprarenal aneurysm began at the level of the SMA, and exhibited a saccular shape, protruding from behind the right renal artery (). The maximum diameter measured 3.7 cm, and the aneurysm showed partial involvement of the right renal artery. The distal flow of these arteries and each of their corresponding organ perfusions remained intact.\nThe second aneurysm was an infrarenal dumbbell-shaped fusiform aneurysm starting 4.8 cm inferior to the level of the renal artery (). The superior dumbbell portion was the larger of the two, and was 6.6 cm in maximum diameter. The inferior dumbbell measured 5.2 cm in maximum diameter. The aneurysm did not involve the common iliac arteries, and extended just above the iliac bifurcation.\nEndovascular aneurysm repair (EVAR) was not considered a first treatment option because of the unfavorable anatomy of the proximal neck. Instead, we utilized a hybrid technique in which an open approach was used to clear the main branching arteries adjacent to the first aneurysm, followed by correction of the remaining aneurysms with an endovascular stent covering both aneurysms ().\nFor the antegrade debranching method, we used a bifurcated Dacron Y-graft to reconstruct the vasculature. Using a midline incision, sufficient aortic exposure was achieved by dissecting the diaphragmatic crura, and creating downward traction on the thoracic aorta. After proximal and distal (infrarenal) clamping, proximal end-to-side anastomosis was performed on the supraceliac aorta on the anterior surface. A smooth take-off angle was ensured when performing the proximal anastomosis. The graft was positioned retropancreatically, and the distal anastomoses involved the SMA and renal arteries. The left limb was anastomosed in an end-to-end fashion to the SMA, and the right limb was simultaneously anastomosed to both renal arteries; the right renal artery in an end-to-side fashion, and the left renal artery in an end-to-end fashion.\nAfter completion of the antegrade debranching technique and closure of the midline incision, endovascular stenting was performed in the traditional manner. The stent was deployed just below the celiac axis to the common iliac arteries, covering both the saccular and fusiform aneurysms. After stent deployment, a final angiographic examination was performed to confirm the distal flow of the graft segment and to ensure that there were no endoleaks. The final angiographic images confirmed patent vessels () and no endoleaks.\nThe surgery lasted 10 hours and 20 minutes, including anesthesia and application of intraoperative monitoring devices. Aorta clamping was performed 2 hours into the surgery, and the total clamping time was 67 minutes. The open approach lasted 360 minutes, including abdominal wall closure, and the EVAR procedure lasted 200 minutes. Estimated blood loss was 2,700 mL, and intraoperative transfusions were performed with 5 units of red blood cells and 2 units of fresh frozen plasma. After the surgery, the patient was admitted to the intensive care unit for close monitoring, where he stayed overnight. The next day, he was transferred to the general ward and was discharged on postoperative day 14. Antiplatelets (aspirin, clopidogrel) were administered as soon as the patient could take oral medication. After confirmation of total recovery from surgery, the patient underwent elective laparoscopic cho-lecystectomy 3 months after the procedure. The patient is currently alive and well, and most recently had his 6-month follow-up visit. CT images at the 6-month follow-up () showed patency in the debranched graft, no change in the saccular aneurysm, and regression of the fusiform aneurysm to 5.7 cm (from 6.4 cm). |
We present a 48 years old woman who was following up with the diagnosis of stage 5 hydatic cyst. The serological test is performed for hydatic cyst (indirect hemagglutination test) and the result was 1/640. In her routine examinations alpha feta protein was 3900 n/mL. Abdominal Computed Tomography revealed 65 × 55 mm stage 5 Hydatic cyst in the right hepatic lobe and a 30 × 28 mm in the left hepatic lobe (). There was not another pathology detected in abdominal magnetic resonance imaging. In FDG18 Positron Emition Tomography there was a 4 × 5 cm mass in the liver. It was near the right lobe cyst and it was reached to the diaphragmatic crura. This mass revealed a high activity of FDG (suvmax 80) (). The patient was operated with the diagnosis of hepatic mass. There was a hydatic cyst in the 6-7th segments of the liver and on the posterior of the cyst there was a 4 × 5 cm mass which was invaded to diaphragmatic crura. Segment 6-7 hepatectomy and diaphragmatic resection were performed. The adrenal glands and paraganglia were normal. The postoperative course was uneventful and the patient was discharged on the fifth day after the surgery. On the hystopatological examination, the tumor was composed of medium-sized polygonal cells with granular eosinophilic cytoplasm resembling to hepatocellular carcinoma. There was not any tumour cells in the liver parenchyma close to the tumour. Therefore immunohistochemical stains were performed to differentiate the origin of the tumor. These tests are used to understand whether the tumor was a real hepatocellular carcinoma or a hepatoid adenocarcinoma ant to differentiate whether the tumor is originated from the liver or from the adrenal gland. The stains showed diffuse +AFP, Glipan, and CK8; +Heppar in the solid areas; +CK17 and 19 in the glandular areas; −chromogranin, CD20, ER, PR, and GCDFP15; +luminal/focal polygonal CEA (Figures and ). It was a trabecular and sinusoidal structure with CD34 and reticulin. Sinaptofisin and inhibin-A were focally positive (Figures and ). Finally the morphologic and immunohistochemical features of the tumour suggested a hepatoid adenocarcinoma originated from adrenal gland. Adjuvant therapy with 5-flourousil and gemcitabine for 6 months and radiotherapy with the dose of 4500 cGy for 5 were decided to be given to the patient. |
A 39-year-old woman complained of neck pain and left scapular pain in January 2006. The symptoms gradually increased and she was eventually admitted to our hospital. Physical examination revealed deep tendon hyper-reflexia of the upper and lower extremities. Motor palsy was not evident and no sensory disturbance was found in the upper or lower extremities or in the trunk. The plain lateral radiography did not show any abnormality. The antero-posterior radiography showed enlargement of the left C3-4 foramen (). Cervical magnetic resonance imaging demonstrated a dumbbell type tumor compressing the spinal cord at the C3-4 levels (). The tumor was located on the left side of the spinal cord. The tumor showed low intensity on the T1-weighted image and iso-high or slightly high intensity on the T2-weighted image. Gadolinium enhancement was noted in the tumor ().\nThe patient underwent an operation in April 2006. Cervical laminectomy at C3-4 was carried out and total resection of the cervical tumor was performed. The tumor was encapsulated () and it involved the posterior ramus of the C4 spinal nerve, and so the nerve was resected. The dura mater was opened to identify the presence of any intradural tumor invasion, but no intradural invasion was observed. Thereafter, the dura matter was tightly sutured. The round-shaped dark red tumor mass was covered by a thin membrane. The size of the tumor was 2.6 × 2.4 × 1.3 cm. The histological examination revealed a schwanoma with both Antoni type A and type B areas. No malignant characteristics were found for the pathology.\nThe surgical drain was removed 2 days after surgery. The patient had a slight fever. On the 9th postoperative day, she displayed a high grade fever (> 39.0℃) and vomiting. The Kernig sign was observed. She had fluctuation near the surgical wound, and so a spinal cord puncture was carried out; 25 ml of turbid CSF was obtained near the surgical wound. Culture of the CSF revealed MRSE. We diagnosed that she was suffering with postoperative meningitis. The patient also had liquorrhoea. Debridement was performed and the laceration of dura matter was sutured on the 18th day after the first surgery. The size of laceration was 1 mm in diameter at the site where the posterior ramus of the C4 spinal nerve was resected (). She had drainage from the lumbar spine for 1 week after the second surgery. As for chemotherapy, she received intravenous administration of 600 mg of LZD twice a day from the 11th day to the 20th day after the first surgery, and the same dose of LZD was given from the 5th day to the 12th day after the second surgery. These treatment methods, including the second surgery, spinal drainage and chemotherapy, were very effective. The patient did not have fever thereafter and the infection healed. She was discharged on the 18th day after second surgery (). Thereafter, she has had an excellent postoperative course and no recurrence of infection or tumor was observed for the following 3 years. |
The case of a 56-year-old male patient, aged 56, polyallergic, known with high blood pressure, admitted in our clinic for a crush injury of the anterolateral aspect of the right calf and subsequent haematoma, is presented. The patient had been complaining for a discomfort to the anterior right calf during effort for the past 10 years. Four years ago, the discomfort became pain, accompanied by muscle weakness, and the patient noticed a slowly growing 5-6 cm soft tissue mass on the inferior pole of the tibialis anterior muscle. He seeked for medical attention, underwent a plain MRI investigation of the right lower limb that revealed a deep haematoma of the external aspect of the right calf 16.5/3.8/6.8cm that pierced the fascia entering the anterior compartment, with associated muscle atrophy, but without muscle invasion. The attending general surgeon diagnosed him with chronic venous insufficiency and prescribed medical treatment. After an accidental crush injury to the affected region, the patient was referred to our clinic. No medical documents were presented at admission.\nPhysical examination showed a global enlargement of the right calf, more significant on the anterolateral compartment, measuring 27/ 10 cm, tender, with associated muscle weakness (M3), fixed to adjacent structures. After a blood test, EKG and a cardiologic evaluation, the patient was operated on. The haematoma was evacuated through an S-shaped incision on the external aspect of the right calf. Because of the abnormal gross anatomy, the surgeon also performed an incisional biopsy. The wound was sutured, dressed and the patient discharged after 3 days.\nThe pathologic examination described a low-grade myxoid liposarcoma showing multiple transition areas to round cell liposarcoma G1 with positive margins and subsequent muscle fiber invasion ().\nThe patient was referred to an oncologist and radiation therapy specialist. The imagistic evaluation of the tumor was recommended. Gadolinum enhanced magnetic resonance imaging (MRI) of the lower right limb showed a soft tissue mass, deep in the anterior compartment of 28.6/9.4/6.1cm, with increased intake, increased vascularisation, of lipomatous signal and central necrosis, invading the tibialis posterior muscle, and compressing the fibula ().\nComputer tomography (CT) scan of the thorax, abdomen and pelvis revealed 2 right pulmonary nodules of 5/ 4mm in the superior right lobe and 8/ 6 mm in the inferior right lobe, without distant metastases. A benign nodule of the latissimus dorsi of 14/ 7 mm was discovered ().\nWhole body positron emission computer tomography (PET-CT) confirmed the presence of a benign nodule of the latissimus dorsi and increased FDG intake in the upper two-thirds of the right calf on the anterolateral compartment (standardized uptake value SUV=2,60) and subsequent skin ulceration (SUV 5,23) ().\nWhole body bone scan excluded distant metastasis, showing a soft tissue mass with increased intake and high vascularisation ().\nAfter a complete evaluation, the tumor was staged according to the American Joint Committee on Cancer (AJCC) system stage T2bN0M0 G1, stage IIB disease. Due to posterior and anterolateral compartment involvement, limb-sparing surgery could not provide the necessary margins of safety. The radical surgical procedure was imposed by the tumor’s large dimension, muscle invasion and invasion of vascular and nervous bundle. Amputation was proposed, but refused by the patient. In order to perform the radical excision of the tumor, radiation therapy was necessary. Neoadjuvant external beam radiation therapy was administered using a regimen of 2Gy in 25 fractions in 30 days. The MRI examination showed a significant decrease in tumor size to 18/7,5/5.6 cm, with no posterior compartment invasion. Unfortunately, fibular invasion and involvement of the tibialis posterior artery and were noted. An area of skin invasion of 2.5 cm was visible ().\nThe patient was admitted in our Clinic, and after a preoperatory workup, an S-shaped incision was placed on the external aspect of the right calf, en bloc excision of the primary tumor, drainage, suture and splinting were performed (). The resected piece, consisted in a 6/ 2 cm skin paddle, 12 cm of fibula, tibialis anterior, extensor hallucis, extensor digitorum, peronier, tibialis posterior and external half of the soleus muscles were sent for pathologic examination (). All the margins were positive.\nThe patient was discharged after 3 days and developed a 5/ 4cm necrosis on the anterolateral aspect of the right calf, that was surgically removed, en-bloc with a 3 cm mass of soleus muscle (). The excision margins were positive.\nThe patient was referred to the oncologist, and is currently undergoing a chemotherapy regimen with Ifosfamide 5 g/ m2 and Doxorubicin 50 mg/ m2 once a week and a pulse of 20Gy of radiation therapy to the tumor bed. The disease specific survival probability at 5 years, in this case, is 80%, and 63% for 12 years. Clinical and imagistic evaluation at each 3, 6, 12, 18, 24, 36 months postoperatory is mandatory, then yearly the next 2 years. |
A 64-year-old man presented to his primary care provider with the chief complaint of passing small blood clots with urinary voiding. He stated that he had urinary frequency for three to four months before the hematuria, and he was also only able to void small volumes. He denied flank pain but endorsed a history of chronic lower back pain, attributed to the nature of his occupation as a general contractor. The patient additionally denied any personal history of kidney stones or family history of kidney, bladder, or prostate cancer. His mother died from complications of metastatic breast cancer (subtype unknown). He quit smoking 25 years prior to the presentation but was previously a one-pack-per-day smoker for 20-25 years. His urinalysis at presentation revealed >100 RBCs and trace leukocyte esterase with a negative corresponding culture result. His blood urea nitrogen (BUN) and creatinine levels were 27 and 1.93 mg/dL, respectively. His PSA was measured to be 4.32 ng/mL. These laboratory values in conjunction with the patient’s symptoms prompted urology referral.\nPending input from the urology team, a renal/bladder ultrasound (US) was ordered by the primary care provider in lieu of a CT with contrast due to the elevated kidney function tests. The US revealed a simple cyst of the right kidney and a large bladder mass measuring 8.4 × 3.1 × 5.4 cm3 with the observance of blood flow, which was suspicious for bladder carcinoma. The mass is shown in Figure . Bilateral hydronephrosis and a post-void bladder volume of 95 mL were additional concerning findings. After consultation from the urology team, the patient underwent a CT scan without contrast. The CT scan revealed left ureteral obstruction, a nodular outgrowth of the left aspect of the bladder with adjacent fat infiltration and nodularity. Left pelvic sidewall lymphadenopathy was also present. Figures and 3 reflect these findings. A biopsy and potential resection of the tumor were performed subsequently via a transurethral resection of bladder tumor (TURBT) procedure to establish the diagnosis. Resection was not attempted at the urologist’s discretion due to the friability of the tissue. Pathology results revealed primary SCCB as suggested by diffuse positive CD56, CK AE1/AE3, and synaptophysin tissue staining. The tissue was weakly positive for chromogranin and negative for GATA-3. These positive markers are indicative of a tumor with a cellular subtype of neuroendocrine origin. The staining results are shown in Figures -. Co-occurrence of UC in situ was also noted in the pathology report, further supporting the diagnosis of a primary SCCB. The two tumor subtypes are shown in Figures and 9.\nThe patient was ultimately referred to an outside facility after establishing the diagnosis of SCCB. At this facility, a PET scan revealed extensive nodal, hepatic, and osseous metastases. There was no lung involvement. His cancer staging was determined to be T4N2M1. The patient completed targeted palliative radiotherapy (30 Gy/10 fractions over two to three weeks), which resulted in temporarily decreased hematuria. The chemotherapy regimen consisted of cisplatin, etoposide, and atezolizumab. These agents are commonly used in treating small cell carcinoma of the lung. The combination of these drugs and pain control with opiate medications resulted in several episodes of acute kidney injury and constipation. The patient also underwent the placement of bilateral nephrostomy tubes. After considering the nature of the disease, he discussed his advanced directives and underwent hospice care. The patient died six months after the initial presentation. |
We describe the case of a 34-year-old gravida II para l woman, with a gestational age of 26 + 3 weeks at admission, who had a relatively healthy 4-year-old child with her 40-year-old husband of non-consanguineous marriage. She had been on injectable contraception for 2 years and had regular menses for 6 months before the pregnancy. She had antenatal care at a local health center and was vaccinated with tetanus toxoid once and supplemented with iron for 3 months. She was screened for retroviral infection, hepatitis, and syphilis and it was documented nonreactive. She had no anatomic scan at early gestation. She came to Felege Hiwot Referral Hospital with the chief complaint of severe and persistent headache of a day’s duration which was occipital in location associated with blurred vision and generalized body swelling of 1 week’s duration. She had no other danger signs in pregnancy. Her past gynecologic history, medical history, and surgical history were uneventful. She is Amhara by ethnicity. She had no known family history of hereditary or chromosomal disorders.\nHer blood pressure at admission was 180/120 mmHg and pulse rate was 84 beats per minute; her respiratory rate was 22 breaths per minute and she was afebrile. She had pink conjunctiva and non icteric sclera, 24 weeks-sized gravid uterus, no abdominal tenderness, no organomegaly, no sign of fluid collection in her abdomen, and the fetal heart beat was positive. She had no vaginal bleeding or discharge. She had pedal and pretibial edema. She was conscious and oriented to person, place, and time. Her deep tendon reflex was +2 and her motor and sensory examinations showed no motor or sensory problems. Other parts of systemic examinations were normal.\nHer hypertension was controlled with intravenously administered hydralazine 5 mg two doses at our emergency department. In her complete blood count her white blood cells were 7300 cells/micL, hemoglobin of 13.4 g/dl, and platelet count was 169,000 cells/micL. Urine protein dipstick was +2, and liver and renal function tests were done: serum glutamic pyruvic transaminase (SGPT) 89 IU/L (elevated), serum glutamic oxaloacetic transaminase (SGOT) 102 IU/L (elevated), alkaline phosphatase (ALP) 229 IU/L, and lactate dehydrogenase (LDH) 288 IU/L. Total bilirubin was 0.24 mg/dl, albumin was 3.49 g/dl, blood urea and nitrogen was 12 mg/dl, serum creatinine was 0.69 mg/dl, and oral glucose tolerance test was in the normal range. Obstetric ultrasound showed a singleton, alive, intrauterine pregnancy with average gestational age of 26 weeks, there was a single large ventricle with partially formed midline structure (see Fig. ), amniotic fluid index was 13.4 cm, placenta was located anteriorly at the body of the uterus, and the presentation was breech; the fetus had normal four chambers of heart with normal outflow tract.\nAfter blood pressure was controlled (it took 2 hours), she was admitted with the diagnosis of late second trimester pregnancy and preeclampsia with severity feature plus semilobar HPE. Seizure prophylaxis for preeclampsia was given (magnesium sulfate according to World Health Organization guideline), methyldopa 500 mg orally every 8 hours was added, and she was counselled about options of management; the high incidence of associated anomalies, severe morbidities of survivors, and poor prognosis were discussed. Termination was decided and done with misoprostol 100 microgram every 3 hours at the third dose with outcome of 1.1 kg male, alive neonate. On examination of the neonate, there was cebocephaly, hypotelorism, single patent nostril which enabled nasogastric tube 6F, micropenis (8 mm), and unilateral right hand polydactyly with agenesis of middle phalanges of the fifth finger. There was rigidity involving all extremities which resisted extension and flexion (see Figs. , and ).\nAfter basic neonatal care was given (cord tied, airway cleaned, and newborn dried), he was transferred to our neonatal intensive care unit (NICU) but he died 20 minutes after admission to NICU. Immediate cause of death was not known. Following his death, further investigations were not possible for cultural reasons. At third postpartum day, maternal blood pressure was 130/90 mmHg, pulse rate was 78 beats per minute, and respiratory rate was 20 breaths per minute. Her complete blood count showed white blood cells of 12,000 cells/micL, hemoglobin was 11 g/dl, and platelet count was 122,000 cells/micL. Liver function tests showed SGPT of 35 IU/L, SGOT of 12 IU/L, ALP of 359 IU/L, and LDH of 254 IU/L; total bilirubin was 0.56 mg/dl, blood urea and nitrogen was 22 mg/dl, and serum creatinine was 0.8 mg/dl. After she was counselled to have preconception care and prenatal screening in next pregnancy, she was sent home relatively healthy. She was well at postpartum visits and methyldopa was discontinued at seventh postpartum day. |
A 22-year-old male patient complaining of a pulsatile mass on the left side of his neck was referred to our hospital. The patient had received a knife wound while serving in the military a year prior. The pulsatile cystic mass was consistent with a 3 cm pseudoaneurysm on a color Doppler sonography originating from the cervical segment of the left vertebral artery with a to-and-fro type flow on the pulsed mode. Other imaging studies, including a computerized tomography, magnetic resonance imaging and magnetic resonance angiography were performed which revealed, in consensus, a vascular mass originating from the left vertebral artery which caused a compression on the hypopharynx.\nThe patient was thoroughly informed of the lesion as well as the outcomes and possible complications of the various available treatment methods. After informed consent was obtained for the stent-graft procedure and other endovascular occlusive treatments, a digital subtraction angiography (DSA) (Multistar Plus/T.O.P., Siemens AG, Forchheim, Germany) was performed using Five French catheters (Weinberg, Digiflex, Boston Scientific Corporation, Watertown, MA), which were introduced into the vertebral arteries via the femoral approach. The selective views of the vertebral arteries were taken by injecting 6 ml of nonionic contrast agent (Iomeron 400 mgI/ml, Bracco SpA, Milan, Italy). The angiography confirmed a 3 cm pseudoaneurysm at the cervical segment of the vertebral artery in addition to multiple arterivenous fistulas (, ). The right vertebral artery was patent with adequate basilar artery flow in addition to the cross-filling of the left vertebral artery from the right vertebral artery with retrograde opacification of the fistulas. The markings on the catheter served for sizing the diameter of the vertebral artery as well as the careful measure of the pseudoaneurysm. In addition, special care was taken to identify the exact origin and level of the arteriovenous fistulas via multiple injections and meticulous analysis of the angiographic sequences.\nBy gentle methodical probing of the left vertebral artery, a 7-Fr guiding catheter (Jomed GmBH, Coronary Guiding Catheter, JR 4.0 SH, Germany) was put in. Next, a 300-cm long, by 0.014 diameter stiff guide wire (Urolix, Urtech, GmbH, Germany) was advanced into the distal portion of the vertebral artery. In addition, a polytetrafluoroethylene (PTFE)-covered stent-graft (19-mm long by 4.0 mm diameter, Jostent Coronary Stent-graft Supreme System; Jomed GmBH, Rangendingen, Germany) was advanced until both the pseudoaneurysm ostium and A-V fistula ostia were covered. The oversize rate was approximately 0.4 mm, as a 4 mm stent-graft was used in a 3.6 mm vertebral artery. With a few remaining fistulas apparent on the angiogram caudal to the initial stent graft, a second PTFE-covered stent-graft was deployed with a slight inferior overlap with the first PTFE-covered stent-graft. After each deployment, the balloon expansion of each stent was performed (5.0 mm diameter - 20 mm length Smash Peripheral PTA Balloon Catheter, Boston Scientific Corporation Ireland LTD., Galway, Ireland) up to 18 atm. During deployment of the stent-graft, the patient felt pain on the left side of his neck, probably due to stretching of the vessel. This pain was relieved in a few minutes after deployment. The angiograms revealed that the pseudoaneurysm and all of the arteriovenous fistulas were completely sealed and that the distal flow was improved (). The recovery was uneventful and the patient was discharged with a prescription of 75 mg clopidogrel per day. The patient was informed of the possible hematologic complications of this medication. Despite the disclaimer, the patient remained on the medication based on agreeable patient compliance and close surveillance of the hematologic parameters.\nAfter 18 months, a follow-up angiogram showed stenosis within the vertebral stent (). A balloon angioplasty was undertaken over a guide wire (0.014 in by 180-cm guide wire, Wizdom, Cordis Corporation, Miami, FL) using a balloon catheter (4.0 mm diameter - 20.0 mm length Orix Coronary Dilatation Catheter, Modular Stent Systems, Inc., Anaheim, CA). After completing the procedure, better luminal filling was observed on the angiogram (). |
A 48-year-old male patient presenting with sudden-onset symptoms of sweating, dizziness and falling to the ground, with loss of consciousness, and spontaneous recovery. After observing electrocardiographic changes, he was hospitalized with suspected acute coronary syndrome. An angio-computed tomography (CT) was performed, resulting in a diagnosis of pulmonary thromboembolism, with no improvement after anticoagulant therapy. The patient was transferred to our hospital, where a repeat angio-CT was performed, revealing a filling defect in the pulmonary artery trunk, extending from the subvalvular area to the origin of the right pulmonary artery, with no change in size with respect to the previous angio-CT (Figure ). The patient was operated on for a suspected primary tumor of the pulmonary artery, resulting in with complete resection of the mass, whose pathological result was an intermediate-grade malignant tumor suggestive of PAIS (Figure ). Following an extension study, CT showed only a nonspecific pulmonary nodule of 4 mm in diameter in the right upper lobe. No adjuvant treatment was given. Three months later, a positron emission tomography-computed tomography (PET-CT) found that the previously mentioned nodule measured 6 mm, suggestive of metastasis. Another 4 mm de novo nodule was found and two more of 2-3 mm in size, possibly granulomas, all without an increase in metabolic activity. The patient underwent surgery where 4 bilateral pulmonary lesions, compatible with metastasis, were resected. Fifteen months later, a new PET-CT reveals a subpleural nodule in the left upper lobe, again suspicious of metastasis (Figure ), as well as several millimeter-size nodules reported in the previous CT, not metabolically characterizable. After discussion in a multidisciplinary committee, the patient was given treatment with SBRT (12 Gy × 5 fractions), with excellent control. Six months later, growth of the two new pulmonary lesions noted in the previous CT was observed (Figure and C). Chemotherapy was prescribed, which was rejected by the patient, and a second course of SBRT was given on both pulmonary lesions. Nine months after SBRT, the patient is disease-free by PET-CT. |
A 23-year-old male was feeding his horse, and while stroking the horse's hair, the animal chewed the fourth finger of his left hand causing violent pain and total functional impotence of the finger. Both the patient and the horse were up to date on their required vaccinations at the time of the incident. The patient was transferred to an emergency department and was admitted six hours after the incident. He was conscious, in good general condition, and apyretic. An examination revealed a crush injury of the fourth finger with tendons and bone exposed ().\nCopious irrigation with normal saline (2 liters) at the injury site was performed along with injection of 0.5 ml tetanus toxoid and 500 IU of human tetanus immunoglobulin. Postexposure rabies prophylaxis (rabies immune globulin human 20 IU/kg) with the first-dose rabies vaccine was injected into the depth of the wound as well as around the wound. The remaining rabies immune globulin was injected into the deltoid muscle. The patient was also treated with prophylactic antibiotic therapy with intravenous amoxicillin-clavulanate, gentamicin, and metronidazole.\nAfter this initial treatment, radiography revealed a fracture dislocation of the proximal interphalangeal joint of the fourth finger with a third fragment (), prompting the patient to undergo surgery. Surgical exploration under locoregional anesthesia found that the ulnar digital pedicle was sectioned and thrombosed, the radial digital pedicle was intact, the flexor and extensor tendons were sectioned and shredded, and the skin was irreparably shredded ().\nSurgical procedures included removal of foreign bodies and excisional debridement of devitalized tissue, collection of bacteriological samples, copious irrigation with saline serum (3 liters), tendon striping, and finger amputation with coverage of the bone by the radial digital flap using separate stitches (). The surgery was followed by careful clinical and biological monitoring.\nA clinical assessment of the patient 1 day postoperatively showed that he was apyretic with no necrosis of the flap and no purulent discharge. A neurovascular examination was normal. Biological findings at this time showed a C-reactive protein level of 30 mg/dL and a white blood cell count of 11000/μL.\nThree days postoperatively, bacteriological samples found an evidence of Pasteurella species and Staphylococcus sensitive to amoxicillin + clavulanic acid with C-reactive protein levels of less than 10 mg/dL and a white blood cell count of 7000/μL. At this time, the patient received the second dose of rabies vaccine.\nOne week postoperatively, the patient was discharged with a prescription for a course of 10 days amoxicillin-clavulanic acid treatment to be reviewed weekly. At a three-month follow-up (Figures and ), the patient showed no sign of infection; he returned to his usual activities and was discharged from care. |
An 82-year-old male was admitted to the emergency department for worsening shortness of breath and hypoxia. He was admitted a week after he was diagnosed with a left ninth rib fracture secondary to a fall. He had long-standing history of chronic obstructive pulmonary disease, coronary artery disease, and peripheral vascular disease. Chest radiographs revealed a left pleural effusion and possible infiltrate. The patient was initially treated with a nebulizer, prednisone, and empiric antibiotic coverage with ceftriaxone and azithromycin. The patient failed to improve with the medical interventions and a therapeutic thoracentesis was performed. The thoracentesis was completed with ultrasound guidance, with the puncture made above the 11th rib at mid chest on the left. The pleural effusion was found to be frank blood. No immediate complications were noted, and the patient was taken to recovery. The next day the patient was found to be in respiratory distress. A chest x-ray revealed an opaque left hemithorax that was likely rapid accumulation of pleural fluid (Fig. ). A follow-up contrast-enhanced computed tomography (CT) of the chest performed during the arterial phase revealed a left intercostal pseudoaneurysm with hemothorax and adjacent compressive atelectasis (Fig. ). Ultrasound of the left chest wall was performed (Fig. ) directly over the thoracentesis site and doppler flow revealed bidirectional fluid flow, indicating the presence of a large pseudoaneurysm (Fig. ).\nFollowing identification of the left intercostal pseudoaneurysm, the patient underwent a thoracic aortogram and multiple-level left intercostal angiogram (Fig. ) under IV conscious sedation. Selective catheterization of the T5, T6, and T7 intercostal arteries was unsuccessful in identifying the pseudoaneurysm. Selective catheterization of T10 and T11 intercostal arteries was performed with a C2 Cobra catheter, following multiple catheter exchanges due to the patient’s atherosclerotic vessels. The pseudoaneurysm was ultimately found to have a left T10 origin and the C2 Cobra catheter was exchanged for a microcatheter. Once access was gained, coil embolization of the pseudoaneurysm was performed with a series of 15 Axium micro coils. Significant room was left on both sides of the pseudoaneurysm and a follow-up angiogram was performed via the microcatheter, then a 5-French Cobra catheter. The follow-up angiogram demonstrated no further filling of the pseudoaneurysm (Fig. ). The catheter was removed and a Perclose device was placed in the left groin for hemostasis. Following completion of the procedure, the patient was taken to recovery. The patient proceeded to return to his baseline following medical management during the remainder of his hospital stay and was discharged home after 5 days. |
A 55-year-old non-diabetic, non-hypertensive male with a history of recurrent colicky left lumbar pain presented with acute urinary retention. Catheterization was attempted; however, it was unsuccessful. Ultrasound revealed an overdistended urinary bladder with a normal-sized prostate and scarring and focal caliectasis in the left kidney (). The right kidney was normal and no calculi were seen on either side on ultrasound. Serum electrolyte, renal and liver functions were normal. The haemogram revealed neutrophilia. The prostate-specific antigen was within normal limits. A rigid urethroscopy was performed owing to suspicion of a left urethral calculus and a 11-mm size calculus was removed from the posterior urethra. The patient was discharged and had no difficulty with micturition for 2 weeks thereafter. The patient subsequently developed burning micturition with hesitancy and induration in the perineal region. The urine was turbid and microscopy revealed the presence of Escherichia coli. A perineal ultrasound revealed an abscess in the perineum, which extended to the proximal parts of the corpus spongiosum (). This abscess was drained under saddle block. The patient subsequently developed a discharging sinus at the operative site () leaking purulent fluid. A retrograde urography revealed periurethral extravasation of the injected contrast material with a lytic lesion in the left pubic ramus (). A repeat perineal ultrasound revealed a linear hypoechoic tract leading from the skin surface to the corpus spongiosum (). A retrograde CT urography was performed to look for the extent and ramifications of the abscess. On the non-contrast CT scan, a lytic lesion was seen involving the left inferior pubic ramus (). The pubic symphysis and bodies of both pubic bones were normal. A proximal femoral nail was noted in situ on the left side, which was inserted 10 years before for fracture of the proximal shaft of the femur owing to accidental trauma. On injecting iodinated contrast into the urethra, there was extravasation of the contrast in the periurethral region in the soft tissues surrounding the bulbar and the posterior penile urethra. An extension of the contrast through the external anal sphincter into the intersphincteric plane () with inflammatory stranding in the ischioanal fossae was seen. The contrast also extended into the lytic lesion present in the left inferior pubic ramus (). The contrast also extravasated through the cutaneous opening in the perineum and the natal cleft (). E. coli were isolated on pus culture. The patient was treated with intravenous antibiotics and suprapubic cystostomy was performed. The patient is presently being considered for elective urethroplasty. |
The patient is a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility to be evaluated for CNS vasculitis. She was admitted to this outside facility for a four-week period prior to being transferred to the primary facility for further evaluation over a subsequent 23-day period. Total duration of hospitalization at both the facilities was close to 7.5 weeks. Approximately one week into the initial four-week admission, her family found that she was very lethargic with diminished responsiveness and pronounced difficulty speaking. In the emergency room (ER), her blood pressure was measured at 243/129 mmHg with a blood glucose value greater than 400 mg/dL. She was started on aggressive antihypertensive therapy and underwent a series of diagnostic tests. Dual antiplatelet therapy (DAPT) consisting of aspirin and clopidogrel was initiated in combination with high-dose atorvastatin. With respect to her lethargy and fluctuating cognition, there was concern that she may be experiencing complex partial seizures, so lacosamide was also started.\nA baseline computed tomography (CT) scan of the head without contrast showed multiple indeterminate lacunar infarcts involving the head of the right caudate nucleus and left corona radiata. The same day, a magnetic resonance imaging (MRI) was performed and elicited similar findings with the addition of bilateral punctate infarcts of the left thalamus, right periventricular white matter, and right centrum semiovale. Magnetic resonance angiography (MRA) done on the following day showed high-grade stenosis of the left middle cerebral artery (MCA), in addition to markedly diminished caliber of the right MCA and high-grade stenosis involving the left posterior inferior cerebellar artery (PICA). Bilateral carotid ultrasounds showed very mild plaques. An angiogram exhibited an occluded left posterior cerebral artery (PCA) distally and was also suggestive of advanced intracranial atherosclerosis (more so than would be expected in CNS vasculitis). There was no evident change from day two to day six of this hospital course. A spinal tap performed at the end of the first week demonstrated elevated protein and IgG synthesis rate (16.4), which was concerning for CNS vasculitis. Appreciating the contrast between the imaging and spinal tap findings, CNS vasculitis could not be ruled out. The patient was started on intravenous (IV) corticosteroids briefly, however, the medication was discontinued due to worsening hyperglycemia that was progressively difficult to control. Near the end of the third week of hospitalization, a repeat MRI showed a new small stroke in the left subcortical parietal white matter.\nThe patient was transferred to the primary facility after this initial month of hospitalization, at which time the patient had a National Institutes of Health Stroke Score (NIHSS) of seven. She was alert and oriented to person only and able to follow simple commands. Significant findings on subsequent blood testing revealed leukocytosis (12.2), elevated absolute neutrophil count (ANC) at 11.3, hyperglycemia (314 mg/dL), HbA1c of 9.6%, mildly elevated erythrocyte sedimentation rate (ESR) at 36, positive herpes simplex virus type one (HSV1), and the presence of IgG and hepatitis B core antibody (HBcAb). Workup for hypercoagulable state was negative for Factor V Leiden and antithrombin deficiencies, though notably protein C was elevated. A repeat spinal tap on hospital day one showed elevated levels of protein (122), but also demonstrated an elevation in myelin basic protein (6.09). Otherwise, the patient was afebrile and hemodynamically stable on admission. On hospital day two, rheumatology was consulted. In order to confirm the suspected diagnosis of CNS vasculitis, the specialist recommended a leptomeningeal biopsy and IV corticosteroids in the interim. Although angiography is very sensitive, it is nonspecific as it cannot distinguish between vasculitis and reversible cerebral vasoconstriction syndrome (RCVS). Consequently, the angiography that the patient had undergone earlier in her hospital course could not provide us with a definitive diagnosis, thus warranting the biopsy of the brain.\nA baseline transthoracic echocardiogram (TTE) obtained on hospital day three revealed a left ventricular ejection fraction (LVEF) of 57 +/-5 percent with mild dilation of the left atrial cavity. Repeat imaging showed much of the same findings, however, radiology recommended further workup for underlying CNS vasculitis. Over hospital day four to six, the working diagnosis was “multifocal bihemispheric strokes with no clear etiology with an encephalopathic process.” The patient’s cardiovascular risk factors continued to be treated and monitored (lipids, blood pressure, and sugars), and a more extensive rheumatological workup was ordered. Continuous electroencephalogram (EEG) monitoring on hospital day eight also showed evidence of diffuse encephalopathy although there were no epileptiform changes or seizures recorded. Over the second week of hospitalization, a new left cerebellar infarct was detected on MRI, at which time steroids were tapered down and a transesophageal echocardiogram (TEE) was ordered. The results of this echocardiogram were unchanged in comparison to the baseline TTE. There was no thrombus detected in the left atrium, ruling out cardioembolic etiology of the new stroke. The CT studies of the chest and abdomen were negative for any findings pertinent to the patient’s chief complaint.\nDuring the third week of hospitalization, a repeat head CT without contrast revealed additional recent infarcts. A four-vessel angiogram showed 50% stenosis in the petrous and cavernous segments of the left internal carotid artery (ICA), a completely occluded M1 segment of the left MCA, and multiple alternating foci of narrowing within the M2 and M3 branches of the right MCA as well as the P2 and P3 branches of the PCA. In consideration of these findings and given the fact that there is a considerable overlap between the imaging appearance of vasculitis and atherosclerotic disease, neither diagnosis could be excluded. A second rheumatology consult recommended that a leptomeningeal biopsy be considered prior to starting cyclophosphamide, effectively ruling in CNS vasculitis versus ischemic stroke. The neurosurgery team agreed to conduct the biopsy of the meninges and brain. However, after discussing the details of the procedure with the patient's family, her family decided against her having the procedure due to the risks associated with brain surgery and the debilitating neurologic deficits already suffered by the patient. |
Abdominal computed tomography for regular monitoring of uterine fibrosis in a 49-year-old woman found a hypodense lesion 7 cm in diameter with a clear boundary near the gallbladder (Fig. a, b). No obvious change in size and internal density of uterine fibrosis had been observed during these 5 years (Fig. b). She had no previous history of alcohol or drug abuse. Ultrasound revealed a well-defined, non-calcified tumor between the gallbladder and liver (Fig. ). Magnetic resonance imaging demonstrated a hypointense tumor that compressed the gallbladder and liver on precontrast T1 mapping (Fig. a). T2-weighted images revealed hyperintense tumor with delayed enhancement on arterial phase and portal venous phase (Fig. b) followed by a delayed washout on the hepatocyte phase. Clinical evaluation and laboratory results were nonspecific, and serum tumor markers including carcinoembryonic antigen, cancer antigen (CA) 19-9, alpha-fetoprotein (AFP) and squamous cell carcinoma antigen were within their normal ranges. The findings were consistent with a gastrointestinal stromal tumor (GIST) or another benign tumor originating from the liver or gallbladder. Accordingly, we planned firstly laparoscopic partial hepatectomy concomitant with gallbladder resection. Lymphadenectomy among the hepatoduodenal ligament were also planned under the situations where malignant tumor was suspected intraoperatively. Under the laparoscopic view, a soft tumor had expansively progressed behind the gallbladder, with dorsal compression of the liver (Fig. ). The tumor was easily separated from Laennec’s capsule of the liver at the gallbladder neck and body without adhesion, which indicates the tumor is benign. Therefore, operative policy changed to tumor enucleation with cholecystectomy. Frozen sections including the surgical margin of the cystic duct were negative for tumor cells. As the tumor was moderately attached to the liver at the fundus of the gallbladder, the liver parenchyma was partially resected en bloc to ensure that the surgical margins were free of tumor tissue.\nThe resected specimen included soft and white tumor tissue with clear boundaries that was located between the liver and gallbladder (Fig. ). Pathological examination following Hematoxylin and Eosin and immunohistochemical staining of tumor specimens was consistent with SFT. The specimens included spindle-shaped tumor cells with elongated nuclei (Fig. a), present in both tangled and patterned arrangements in the subserosal layer of the gallbladder (Fig. b, c). The tumor tissue had a storiform pattern with alternating hypocellular and hypercellular areas with some showing myxoid degeneration. The tumor cells were positive for CD34, CD99 and B-cell lymphoma (BCL)-2 and negative for S100 and alpha smooth muscle antigen (αSMA) staining (Fig. d), and few cells were positive for the cell proliferation marker Ki-67/MIB-1. The tumor cells were negative for SMA, keratin, cytokeratin (AE1/AE3), CD117, epithelial membrane antigen, and desmin. The pathological diagnosis was SFT originating from the cystic plate.\nThe postoperative course was uneventful. She was discharged on postoperative day 9. Chemotherapy was not considered necessary, and postoperative follow up at 18 months found the patient well without any sign of recurrence. Periodical check-up for uterine fibrosis by gynecologist is also continued. |
A 77-year-old female with hypertension, untreated hyperlipidemia, hypothyroidism, but without prior history of CAD or angina symptoms was referred to a cardiologist's office for a treadmill exercise test secondary to new onset palpitations. She denied any chest pain or pressure, shortness of breath, exertional dyspnea, or leg swelling. She quit smoking 36 years ago and has no family history of early cardiovascular diseases. She has a very distant cardiac work-up years ago, including a stress test and an echocardiogram, which the patient reported were unremarkable. Vital signs prior to the test were a blood pressure of 140/78, heart rate of 80, and a respiratory rate of 14. Physical exam was unremarkable except for a systolic ejection murmur that was graded II/VI at the base. EKG was at baseline with a normal sinus rhythm, normal axis, and occasional premature ventricular complexes (PVCs).\nThe patient underwent an exercise stress test using the Bruce protocol and was able to complete stage 1 with exercise for three minutes at a speed of 1.7 mph and a 10% incline. The test was terminated due to dyspnea and fatigue without chest pain. She reached a heart rate of 141 beats per minute which was 98% of predicted for her age. She accomplished 4.5 metabolic equivalents of exertion. With exercise, she had occasional atrial premature complexes and PVCs with a ventricular couplet in recovery. She started to notice tightness in her chest. Her peak blood pressure at the time was 218/90.\nThe patient was transferred onto a stretcher, and an IV line was started. She was given sublingual nitroglycerin, 325 mg of aspirin to chew, and one 5 mg IV push of metoprolol tartrate. She then received nitroglycerin paste and metoprolol tartrate IV every 5 min for two more doses. At that time, her EKG on the stretcher showed ST elevations in leads I, aVL, V5, and V6 with ST depressions in leads III, aVF, and V1-V3 consistent with a lateral wall evolving myocardial infarction (). She was transferred urgently to our institution for cardiac catheterization.\nThe patient underwent an emergent cardiac catheterization with left ventriculography and intravascular ultrasound (IVUS) within 2 hours after onset of symptoms. Troponin-I levels prior to the catheterization increased to 11.17 (normal less than 0.05 ng/ml). The rest of the laboratories were within normal limits including a thyroid-stimulating hormone (TSH) level. Coronary angiography showed nonobstructive coronary artery disease (pLAD 40%) and highly tortuous coronary arteries. IVUS of the proximal LAD revealed a minimal lumen area of 5.2mm2, and no ruptured plaques. Left ventriculogram revealed a left ventricular ejection fraction (LVEF) of 20% and severe mid-cavitary hypokinesis with basal and apical hyperkinesis (Figures and ). To our knowledge, this is the first case of treadmill exercise testing-triggered mid-left ventricular ballooning variant of takotsubo cardiomyopathy, whereby obstructive epicardial CAD and ruptured plaques were excluded with angiography and IVUS, respectively.\nThe patient was started on medical management with standard therapy for heart failure. A follow-up echocardiogram was done two days after the event which redemonstrated mid-left ventricular ballooning, with an improved LVEF of 35%. The patient remained asymptomatic during the course of her hospitalization and troponin levels trended down from a postcardiac catheterization peak of 16.06 ng/ml. An echocardiogram was repeated during an outpatient follow-up two weeks later which showed resolution of wall motion abnormalities and an LVEF of 45-50%. |
A 52-year-old woman with no history of smoking or history of malignancy presented with bilateral breast tenderness and a palpable mass in her right outer breast. Screening mammograms performed 1.5 years prior to the development of symptoms were negative. Diagnostic mammograms were obtained to evaluate the breast symptomatology and demonstrated 2 new masses in the right breast and 1 in the left ( and ). Note that the largest/palpable mass appeared oval in shape with circumscribed margins. Since these masses were new from the prior study, further evaluation with ultrasound was performed. Targeted breast ultrasound confirmed indeterminate imaging features of the breast masses (), and findings were all classified as BI-RADS 4, suspicious. Ultrasound-guided core needle biopsies of all 3 breast masses were performed at an outside institution. Histopathologic examination of all 3 masses was consistent with high grade neuroendocrine neoplasm. No ductal carcinoma in situ was observed. Breast markers including ER, PR, and HER2 were negative. Synaptophysin and chromogranin were diffusely positive in the tumor cells, supportive of neuroendocrine origin. Immunohistochemistry was negative for pankeratin, cytokeratin 7, cytokeratin 20, GATA-3, and CD45 (LCA). TTF-1 positivity suggested the possibility of a lung primary, and absence of CDX-2 made a gastrointestinal primary less likely . Ki-67 expression was reported as high as 70%.\nCT of the chest, abdomen, and pelvis, PET/CT, and MRI of the brain were performed for initial staging. In addition to the biopsied bilateral breast masses, imaging demonstrated a 2.2 cm right lower lobe pulmonary nodule in a central location (), ipsilateral hilar and mediastinal lymphadenopathy (), a single 1.1 cm liver lesion (), and a nodule in the right temporal subcutaneous tissues (). No axillary lymphadenopathy was noted. Based on imaging and pathology, this was determined to be a primary lung small cell carcinoma with metastases, including bilateral breast metastases. This was staged as T1c N2 M1. |
A 45-day-old boy presented with progressively increasing neonatal obstructive jaundice and acholic stools. He was born by normal vaginal delivery at term with a birth weight of 2500 g and normal APGARs. The pregnancy was supervised and uneventful with two normal prenatal ultrasounds. There were no maternal risk factors identified. The neonate was well till day 3 of life when the parents noticed yellowish discoloration of the sclera followed by pale colored stools. He was evaluated and treated by several pediatricians in his hometown, without any avail, before being referred to us. There were no symptoms of bleeding diatheses. His general condition was well preserved but he was deeply icteric. He had a firm liver palpable 3 cm below the costal margin. Liver function tests (LFT) showed mildly deranged coagulation profile with a total bilirubin of 14.5 and direct bilirubin of 7.8mg/dl, elevated liver enzymes and normal proteins. The fasting ultrasonography revealed an enlarged liver with a normal gall bladder (GB) and common bile duct (CBD). There was no evidence of any subhepatic cyst but postprandial contraction of the GB could not be demonstrated. His TORCH titres were positive for Cytomegalovirus (CMV) IgM antibodies; however urine sample was negative for PCR detection of CMV, thus ruling out congenital CMV infection. A HIDA scan after 5 days of oral phenobarbitone revealed no excretion of radioisotope into the gut after 24hrs. Liver biopsy and MRCP were not done. Since the preoperative evaluation was highly suggestive of type 3 EHBA, he was taken up for laparotomy on day 54 of life without any further delay. At laparotomy, the liver was found to be cirrhotic. A normal looking GB, 2.7 cms long, was found to be communicating with a 4 x 4 cms cyst replacing the CBD (Fig. 1).\nNeedle aspiration did not reveal any bile. The above findings were confirmed by intra-operative cholangiography which demonstrated the cystic duct opening into a cystic structure similar to a CDC. The intra-hepatic ducts were not visualized and there was no dye in the duodenum (Fig. 2).\nThe cyst was unlikely to be a bile lake as it was in continuity with the GB and extra hepatic in location. On further exploration, the common hepatic duct (CHD) was found obliterated just at its origin and no bile flow was seen. The CDC was excised using Lilly's technique and Kasai's portoenterostomy (KPE) was done using a 20 cms long Roux loop of jejunum. Histopathology confirmed both EHBA and CDC with evidence of liver cirrhosis and periportal fibrosis. The atretic plate showed multiple bile ducts and the CDC had chronic inflammation of the cyst wall. The child recovered and was passing cholic stools from the 4th postoperative day with significant improvement in LFT (Total bilirubin of 7.5 and direct 4.5 mg/dl, alkaline phosphatase 190 IU/L). He was tolerating oral feeds well and was discharged on the 13th postoperative day on oral chemoprophylaxis, prednisolone, ursodeoxycholic acid and fat soluble vitamin supplements. He was followed up at 2 months with one episode of cholangitis which was treated conservatively with 4 weeks of 2nd line intravenous antibiotics. Within a month of discharge from hospital, he developed another episode of cholangitis and unfortunately died at home, which was 200 Kms away from our hospital, before treatment could be reinstated. |
A 38-year-old Greek woman was transferred to the Emergency Department of our hospital in a comatose state. After intubation, an urgent computed tomography (CT) scan of her brain was performed, which revealed a 7.8 × 3.3 × 5.0 cm intracerebral hematoma located at her left occipitoparietal area with surrounding edema and a midline shift.\nOur patient had a history of two pregnancies that were normal term Cesarean deliveries, the first four years ago and the second 10 months ago. The rest of her medical history was unremarkable.\nAn emergent craniotomy was performed along with evacuation of the hematoma. After surgery, she was transferred to the Intensive Care Unit (ICU). Our patient remained sedated and mechanically ventilated for neuroprotection, while an intraparenchymal monitoring device was inserted for continuous monitoring of her intracranial pressure. Intracranial hypertension was treated with osmotic diuretics, hyperventilation and hypertensive therapy, aiming to maintain a constant cerebral perfusion pressure higher than 60 mmHg.\nA laboratory workup, including liver and renal function tests, coagulation screening, fibrinogen and full blood count, were normal. Due to the enlargement of the right hilus found on a chest X-ray, extensive CT imaging studies were performed, which revealed a space occupying lesion at the inferior pole of her right kidney (approximately 3 cm), a solitary nodule at the IVa part of her liver consistent with a metastatic lesion, a soft tissue mass situated in the right hilus of her lung enhanced by intravenous contrast and diffusely distributed chest nodules without intrathoracic lymph nodes.\nHer serum level of β-chorionic gonadotropin (β-hCG) was over 200,000 mIU/mL and this value was repeatedly confirmed. Nonetheless, a pelvic examination was unremarkable and all the imaging studies (pelvic ultrasonography, including transvaginal ultrasonography and pelvic CT) revealed the normal appearance of her uterus and bilateral ovaries.\nDuring the next two weeks, our patient demonstrated gradual improvement of her general status and she was able to open her eyes spontaneously.\nMagnetic resonance imaging of her brain showed, apart from postsurgical lesions at the area of the hematoma, a metastatic lesion situated on her left temporal lobe, whereas a magnetic angiography did not reveal any vascular dysplasia.\nOn the 26th day of her ICU stay, our patient underwent an ultrasound guided fine needle aspiration of the hepatic lesion in order to establish a histological diagnosis for possible further treatment. Due to consequent intra-abdominal bleeding, she was given surgical radiofrequency ablation of the hepatic lesion. A right nephrectomy was also performed due to the size of the lesion with evidence of imminent bleeding.\nOn the 35th day she had a new episode of massive intracerebral hemorrhage, confirmed by CT, which was considered inoperable by our neurosurgeons. She died 10 days later, due to septic shock.\nMacroscopic examination following the right nephrectomy revealed the presence of a grey-red tan encapsulated tumor, which measured 6 × 3.5 × 3 cm, situated at the inferior pole, invading the renal pelvis-pelvicalyceal system and extending to the renal capsule. The histological examination of this mass showed the presence of a malignant neoplasm composed of syncytiotrophoblastic and cytotrophoblastic cells in an extensively hemorrhagic and necrotic background with subsequent cystic degeneration and numerous neoplasmatic vascular emboli (Figures and ). Immunohistochemical staining revealed intense expression of β-hCG (Figure ) and placental alkaline phosphatase, although it was negative for epithelial membrane antigen (EMA) and thyroid transcription factor-1 (TTF-1) expression. All above findings are indicative of a choriocarcinoma of the renal pelvis. The hepatic biopsy revealed neoplastic invasion of the liver by a tumor histologically identical to the choriocarcinoma of her renal pelvis. |
Patient CS, a single 60-year-old male presenting with a history of generalized anxiety with panic, major depressive disorder, and excessive guilt, was referred from a county hospital to a tertiary psychiatric facility for clarification of diagnosis and a more comprehensive assessment. His sister, and the family physician that had been following the patient for the past 4 years, helped provide collateral history. His family noted that he was born with a large head. He had a history of meningitis at the age of 9 or 10 after which it is thought that he developed a non-communicating hydrocephalus. His past psychiatric diagnoses included major depressive disorder, generalized anxiety disorder with panic, personality disorder, and “borderline intelligence.” He had several admissions to a psychiatric ward over the past 3 years for low mood and had been trialed on numerous psychotropic medications (citalopram, lithium carbonate, risperidone, olanzapine, quetiapine, paliperidone, clomipramine, clonazepam, lorazepam) with little effect or benefit. At the time of admission, he did not smoke, drink alcohol, or take illicit drugs. His past medical history was significant for hypothyroidism corrected with the use of thyroxine, bowel resections secondary to possible malignant changes, fatty liver with lobar resection secondary to liver cancer and nephrolithiasis.\nHe was born and raised in Europe until the age of 5, when he immigrated to Canada, and is bilingual. His family reported that he had always had a large head, micropenis, central obesity and short stature. He had a history of being bullied for “looking like a girl” and being different. At school his peers were physically aggressive, hitting him on his head. Born the youngest of seven siblings, he was raised by his parents and lived under their care into adulthood, until both parents passed away—his father had Diabetes Miletus and his mother had a brain tumor. Thereafter, he was taken care of by his sister. He had an older brother who also passed away secondary to a brain malignancy. One brother has dyslipidemia, and two sisters and one brother are healthy. He had no employment history and as a child had always struggled in school, completing a vocational stream of education until grade 10. Socially, he was active in a band for a few years (plays guitar well) and sang in a church choir. However, he never lived independently, and had no romantic relationships.\nInitial assessment revealed that he was a poor historian unable to give an accurate timeline of events. He often expressed fears that he was going to die. He suffered from delusions of guilt that he had caused the deaths of family members. His conversation was repetitive, he repeatedly asked the same questions and restated his fear of dying despite several reassurances. He had no history of self-harm or suicide attempts. On physical examination, he had a wide stance waddling gait, slow movements, limited arm swing and masked facies. He was noted to have enlarged head circumference (62.5 cm) and limited insight into his illness and the need for treatment. His clinical presentation prompted examination with magnetic resonance imagining (MRI) of the brain and formal neuropsychological testing.\nA sagittal T1, axial T2, axial T2 FLAIR and diffusion-weighted images were acquired throughout the brain. Findings indicated a long-standing overt ventriculomegaly, likely due to aqueductal stenosis, with bilateral gross dilation of the lateral and third ventricles, with a small aqueduct and fourth ventricle, with significant thinning of the corpus callosum and overlying cerebral cortex. Vascular flow-voids at the base of the brain were normal and there were no mass lesions, significant sulcal effacement, downward tonsillar herniation or restricted diffusion observed.\nManual segmentation of gray and white matter and cerebrospinal fluid (CSF; Figure ) of high-resolution T1 weighted MRI images was completed with Freeviewer in FSL (Jenkinson et al., ). Automatic segmentation of a comparison group of sex and age matched healthy controls (HCs; one aged 60, three aged 55 years, Table ) was completed with the FreeSurfer () recon tool. The participant’s volumes were converted to Z scores for comparison. Compared to similarly aged control participants, the patient had extremely large ventricular volume (821,452 mm3, Z = 161), reduced white (333,606 mm3, Z = −2.655) and gray (432,184 mm3, Z = −3.07) matter volume, and within normal range total intracranial volume (1,587,242 mm3, Z = 0.57) see Table and Figure .\nThe patient’s neurological exam was unremarkable.\nThe Wechsler Adult Intelligence Scale (WAIS-III; Wechsler, ) revealed a borderline IQ of 79, with a verbal IQ of 88, non-verbal performance IQ of 74, poor working memory IQ of 71, verbal comprehension IQ of 93, and visual-spatial IQ of 80. The patient had difficulty completing tasks requiring working memory, which was in the 3rd percentile, and processing speed was extremely slow (in the 1st percentile). Hopkins Auditory Verbal Learning Test (Brandt, ) indicated severe memory impairment, with initial memory for only a few items, no significant recall between administrations, and inability to recall any information after a brief delay. Rey-Osterrieth Complex Figure Task (Osterrieth, ; Rey et al., ) performance indicated impaired visual spatial and working memory abilities with more attention to small details, missing elements and less attention to the overall image. The Stroop test (Stroop, ) indicated impaired executive function, scoring below the 1st percentile, with a severe inability to suppress automatic responses. |
An inherently healthy 87-year-old man underwent first aid after a traffic accident. At the hospital, his general condition was stable and there were no abnormalities in his vital signs. Blood biochemistry tests showed an increased inflammation and a marked increase in pancreatic enzymes (AMY 818 U/L, P-AMY 705 U/L, and lipase 1191 U/L). Abdominal contrast-enhanced computed tomography showed fluid retention around the pancreas and wall thickening of the horizontal segment of the duodenum (Fig. a). The pancreatic head was severely injured, and laceration to the pancreatic head was suspected. The duodenum wall was enlarged, especially at the third part, and the horizontal segment was considerably large. Endoscopic retrograde cholangiopancreatography was performed to evaluate the presence of a main pancreatic duct injury. Pancreatography revealed a substantial leakage of the contrast medium from the main pancreatic duct at the pancreatic head (Fig. b). Endoscopic naso-pancreatic drainage (ENPD) was performed at the proximal side of the injury.\nFollowing the diagnosis of a pancreatic injury of grade IV or V, an emergency operation was performed. Intraoperative findings confirmed the severe pancreatic head injury (Fig. c). The pancreas was completely transected at the head of the pancreas, but the degree of disruption itself was not high. Based on these findings, the present case was classified as grade IV according to the AAST-OIS classification. In addition, the horizontal segment of the duodenum was widely swollen and its wall was thickened.\nThe ENPD tube could not be differentiated from the main pancreatic duct because the ENPD interposition was moved by the operative procedure. Our first treatment strategy was a primary repair by suturing the pancreatic laceration. However, the ENPD tube could not be detected at the main pancreatic duct, and the severe laceration would not heal by simple suturing. Although damage control surgery would have been another choice for this condition, our suspicions of a massive duodenum injury required us to remove this injured organ. We used PD-II as the reconstruction method. Pancreatojejunostomy was performed, with the Blumgart modification added for pancreatic duct-to-mucosa anastomosis. The pancreas was transected diagonally, and it was difficult to use the lacerated pancreas in anastomosis, so an anastomosis was performed by adding a pancreatic resection on the distal side. The operative time was 389 min, and blood loss was 527 mL. There were no postoperative events. No abnormal findings such as an abscess formation or pseudoaneurysm formation were found on computed tomography images 10 days after surgery (Fig. d). He was discharged on postoperative day 25. Examining the resected specimen of the pancreas, the pancreatic parenchyma revealed acute pancreatitis with an evidence of fat necrosis and neutrophil infiltration into the pancreatic parenchyma. The resected specimen of the duodenum also revealed that a mucosal enlargement and irregular bumps were found from the descending segment to the horizontal segment (Fig. a). Microscopic findings showed that atypical lymphocytes with a sickle-shaped nucleus and a nodular structure in the segment of the duodenum wall thickening (Fig. b) occurred. Immunohistochemical staining was positive for CD20 and bcl-2 and negative for CD5 (Fig. c). Duodenum follicular lymphoma (FL) was diagnosed. |
A 24-year old male with chronic rhinosinusitis was admitted for an elective Functional endoscopic sinus surgery (FESS) procedure under general anesthesia. During the operation, the surgeon applied several tampons soaked in 1:1000 dilution epinephrine to the nasal mucosa. Ninety minutes into the surgery, unexpectedly, blood pressure rose to 210/130 mmHg followed by pulseless electrical activity. CPR was initiated, with the administration of 2 mg of IV epinephrine in consecutive doses, leading to the return of spontaneous circulation. ECG showed sinus tachycardia and a prolonged QTc interval of 486 ms, Inverted T waves in leads I and aVL, and no signs of acute ischemic changes (Fig. ). A chest x-ray demonstrated pulmonary edema and a borderline enlarged cardiac silhouette (Fig. ).\nThe patient was placed on mechanical ventilation. Transthoracic echocardiogram showed a dilated left ventricle with an increased end-diastolic dimension (124% of normal value) with a mild reduction in LV mass, a severe reduction in systolic function, apical akinesis, and hyperdynamic base. The estimated systolic left ventricular ejection fraction was 30% (Fig. ). Cardiac troponin and CPK were elevated. NT or NT pro-BNP were not taken. A head CT was performed and demonstrated mild global cerebral edema, with multiple maxillary sinus fractures (Fig. ).\nMedical information gathered from family members indicated no family history of heart diseases. The patient did not smoke, use illegal drugs nor consumed alcohol on daily basis. The patient was diagnosed with dilated cardiomyopathy and admitted to the intensive cardiac care unit. Upon arrival, upload titration of ACE inhibitors, β-blockers, and diuretics initiated. The following ECG strips indicate normal sinus rhythm, QTc interval of 420 ms, and normal T wave in lateral leads. We decided not to perform a diagnostic coronary catheterization since the patient's risk profile for ischemic cardiomyopathy was low. Objectively, there were no ischemic changes on ECGs strips and no regional wall motional abnormalities were seen on TTE upon arrival.\nThree days later, a second echocardiogram was performed which showed a normal-sized left ventricle, with preserved systolic function (Fig. ).\nThe patient was discharged in full functional capacity. An ambulatory cardiac MRI performed two weeks after discharge revealed mildly dilated LV cavity, good global systolic function, and no signs of late gadolinium enhancement or edema. |
A 43-year-old man with a history of chronic alcoholism presented with abdominal distension. The previous day, the patient had presented to a local hospital with anal bleeding and abdominal pain after an incidental insertion of barbecue skewer per anus in the drunken state; subsequently, he had undergone sigmoid loop colostomy for rectal perforation. However, after the operation, the patient had become hemodynamically unstable. At presentation, his systolic blood pressure was 90 mmHg and the pulse rate was 135 beats/min. Although there was no gross rectal bleeding, the digital rectal examination revealed a penny-sized anterior rectal wall defect 6 cm from the anal verge (AV). Computed tomography (CT) revealed a hematoma (12 × 10 × 15 cm) with active bleeding in the pelvic cavity and a pseudoaneurysm in the anterior wall of the rectum (). Since the patient was hemodynamically unstable, an emergency operation was performed. During the operation, a massive subperitoneal hematoma in the rectovesical pouch and large amount of blood in the peritoneal cavity were found. After evacuation of the hematoma and blood, oozing continued in the rectovesical pouch (). Thus, compression with gauze was performed for 30 min until the oozing stopped. The Hartmann procedure was performed with the suspected bleeding focus included, but the perforation site was not included.\nAlthough the postoperative course was uneventful and there was no evidence of recurrent bleeding on the follow-up CT on the 7th postoperative day (POD), a focal enhancing lesion in the anterior wall of the rectum indicating a residual pseudoaneurysm was noted (). On the 11th day POD, his hemoglobin decreased from 11.6 g/dL to 7.9 g/dL, and the follow-up CT revealed recurrent hematoma (6.0 × 4.2 cm) in the pelvic cavity and the residual pseudoaneurysm (). Following the diagnosis of recurrent bleeding from the residual pseudoaneurysm, an angiography was performed. However, the angiography failed to localize the pseudoaneurysm, and definite signs of extravasation could not be ascertained. Thus, prophylactic gelfoam embolization at the anterior branch of both the internal iliac arteries was performed (). The subsequent hospital course was uneventful, and the patient was discharged on the 25th POD. After 3 months, the previous rectal lesion (AV: 6 cm) healed, and colostomy reversal was performed without morbidity. |
A 51-year-old man presented to the Neurologic Department because of right-sided weakness and recurrent left-sided paresthesias and bilateral visual disturbance for two weeks. He had a history of hypertension, diabetes mellitus, hyperlipidemia and smoking. He also had a history of myocardial infarction three years earlier.\nComputed tomography scan of the patient's brain demonstrated evidence of bioccipital ischemic infarct and multiple regions of lacunar infarction of basal ganglia and internal capsule. General hematological and biochemical tests were normal. To evaluate the source of emboli, Carotid Doppler measurements and transthoracic echocardiography was performed. Carotid Doppler measurements were normal. Transthoracic echocardiography showed mild left ventricular dilatation with moderate systolic dysfunction (ejection fraction 35-40%), akinesia of base of infeior segment andhypokinesia of lateral (base and mid) segment. There were two mobile masses in the left ventricle, one attached to the posterior wall of the left ventricle (2.5 × 1.8 cm) and another mass (2.8 × 2.2 cm) attached to the antrolateral wall of the left ventricle (mid part) which suggested cardiac tumor []. The patient was referred to our cardiology department for evaluation of cardiac masses. Transesophageal echocardiography was performed and confirmed TTE findings. Cardiac magnetic resonance imaging was not obtainable as a result of patient noncompliance. Due to our patient's regional wall motion abnormalities and history of myocardial infarction and diabetes, a coronary angiography was performed which showed severe three vessels disease. Our presumptive diagnosis was left ventricular myxoma. In view of these findings and the history of recurrent systemic embolism, the patient subsequently underwent open heart surgery for coronary artery bypass graft (CABG) and tumor resection. Trans-left atrial excision of the mass was performed through a median sternotomy with cardiopulmonary bypass. A reddish purple colored mass with an irregular surface (2.5 × 2 cm in diameter) was identified, that was attached to the posterior wall of the ventricle below the posterior mitral leaflet by a 2-3 mm tissue stalk about 1.5 cm under the mitral annulus. Another mass was not accessible trans-left atrium and mitral valve, thus a transverse aortotomy was performed. A reddish purple colored irregular surface mass (3 × 4 cm in diameter) that was attached to the antrolateral wall of the ventricle about 5 cm under the aortic annulus was excised. After resection of the masses CABG was performed. Postoperative recovery was uneventful. The histologic diagnosis revealed an organizing thrombus with no evidence of tumor cells [].\nThe patient was discharged with slight improvement of neurological deficit. Transthoracic echocardiogram showed no evidence of residual mass and ejection fraction had improved to 45%. |
A 21 year old woman with no evidence of structural heart disease referred to our center for evaluation of palpitation and dizziness. The structural heart disease was excluded by physical examination and transthoracic echocardiography. Transthoracic echocardiography showed normal cardiac chambers (including right ventricle), normal valvular function and ejection fraction (EF) without any wall motion abnormalities. During an episode of palpitation, the standard 12-lead electrocardiogram (ECG) showed documented wide complex tachycardia with a heart rate of 125 beats /min. The tachycardia was refractory to two intravenous antiarrhythmics (amiodarone, procainamide). The wide complex tachycardia had inferior axis and right bundle branch block morphology compatible with LVOT-tachycardia (). The baseline ECG showed no abnormality.\nAfter obtaining written informed consent, electrophysiologic study was done in the postabsorptive and nonsedated state. During programmed electrical stimulation from atrium and ventricle, dual AV nodal physiology with nonsustained AVNRT was induced. Then programmed ventricular stimulation was performed with standard protocol at three cycle length (600,500,400ms) and three extrastimuli up to coupling interval of 200 ms from two sites (RV apex, RVOT). No ventricular tachycardia was induced with and without isoproterenol infusion. Repeat programmed atrial stimulation resulted in induction of sustained AVNRT under isoproterenol infusion (). Radiofrequency catheter ablation of slow pathway was done at right posteroseptal area. Postablation programmed stimulation failed to induce any supraventricular tachycardia with and without isoproterenol infusion but a wide complex tachycardia (cycle length=480 ms) identical to clinically documented arrhythmia was induced by overdrive pacing from RVOT. Mapping of RVOT failed to show any early ventricular activation site, thus LVOT was mapped and tachycardia focus was localized in this area with 53 ms early ventricular activation relative to surface electrocardiogram () Radiofrequency energy delivery (50 W, 60°C) at this site resulted in termination of tachycardia (). Thirty minutes after ablation, no tachycardia was induced with and without isoproterenol infusion. During 12 month follow-up, she has been symptom free with no antiarrhythmic drugs. |
A 67-year-old woman with remote history of endocarditis s/p tricuspid valve repair and mechanical aortic valve replacement was referred for second opinion and management of new severe symptomatic tricuspid valve stenosis resulting in progressive debilitating congestive heart failure (HF). The patient was approved by the heart team to undergo redo open heart for surgical repair of the tricuspid valve. Intraoperative technical challenges were met to repair the tricuspid valve. In turn, the native valve was resected and a 33 mm On-X mechanical valve prosthesis (On-X Life Technologies, Austin, TX, USA) was implanted.\nThe patient’s post-operative course was complicated by recurrent haemoptysis related to endotracheal tube trauma, prolonged mechanical respiratory support, acute kidney injury, and cardiogenic shock. While unable to anticoagulate, increasing requirements of inotropic and vasopressor support were noted. On the fifth post-operative day, 2D examination revealed single leaflet fixation of the tricuspid mechanical prosthesis resulting in severe stenosis, along with moderate size iatrogenic ventricular septal defect (VSD) not previously seen and later confirmed on transesophageal echocardiography (TEE) (Figure ). The mechanism for the leaflet dysfunction remained unclear. There appeared to be no evidence of leaflet thrombosis. The left ventricle and mechanical aortic valve function remained preserved. Using a heart team approach, it was felt that surgical re-exploration to address the dysfunctional mechanical tricuspid valve and VSD would be prohibitive. A transcatheter assessment with ad hoc intervention was considered. Upon obtaining consent from the patient’s next of kin, the patient was emergently taken to the cath lab for further evaluation and management.\nThe patient was transferred to the cardiac catheterization laboratory in severe haemodynamic collapse. Initial fluoroscopic examination of the heart confirmed the echocardiographic results of an immobile septal leaflet of the recently implanted mechanical tricuspid valve (Figure ). A 9 Fr St. Jude Viewmate intracardiac echo-catheter was used to further assess the TV function and assist with transseptal puncture. Transseptal access allowed for LV and RV intracardiac pressure assessment across the VSD. Similarly, simultaneous right atrium (RA) and RV pressure gradients obtained confirmed the presence of severe TS with a mean gradient of 11 mmHg (Figure ).\nInitial attempts were made to force open the fixated tricuspid valve leaflet with a 6 Fr multiple purpose catheter from the right femoral vein (Figure ). Despite several attempts using different angles and techniques, it was unsuccessful. ‘Valvuloplasty’ was therefore pursued. A 0.035” Terumo angled-glidewire was carefully manoeuvered antegrade across a 6 Fr MPA catheter, between the aperture of two mechanical leaflets and into the pulmonary artery. The Multipurpose (MPA) catheter was then exchanged for an 8 × 40 mm Mustang OTW angioplasty balloon (Boston Scientific, Natick, MA, USA) was then advanced across the mechanical valve and inflated gradually at nominal pressure (8 ATM). A single inflation resulted in successful restoration of valve leaflet function (Figure ). Fluoroscopic examination with repeat haemodynamics confirmed successful procedural results with complete normalization in valve function and no residual stenosis (Figure ).\nPercutaneous closure of the VSD was subsequently pursued following balloon valvuloplasty. Significant temporary improvement in haemodynamic function ensued over the following days. Unfortunately, despite all heroic measures undertaken, the patient succumbed to her illness after multisystem organ failure 5 days later. |
A 59-year-old male patient visited emergency room of Soonchunhyang University Seoul Hospital for slight uneasiness in the chest and vertigo. He was fully conscious and no neurological findings were observed. The patient’s blood pressure was 77/66 mmHg, which was quite low; however, it recovered to 102/65 mmHg with a heart rate of 40 beats per minute with fluid therapy. Second-degree atrioventricular block was observed in the electrocardiogram. No abnormalities were observed in myocardial enzyme levels or in a standard blood examination. The department of emergency medicine collaborated with the department of cardiology to treat this patient. The patient was scheduled to be hospitalized in the cardiology ward for coronary angiography because Mobitz type II atrioventricular block and unstable angina were suspected.\nThe patient had a sudden convulsive seizure while waiting to be hospitalized. The seizure did not stop for more than 5 minutes, during which lorazepam was administered. The patient did not have a history of seizure, heavy drinking, or trauma. After the seizure, while in a semicoma, quadriplegia and the Babinski sign were observed. In order to determine whether the convulsive seizure was accompanied by an acute stroke, diffusion-weighted magnetic resonance imaging was performed. Multiple cerebral infarctions were found in both the frontal and temporal lobes (). Tissue plasminogen activator (t-PA) was administered promptly (within 90 minutes after symptom occurrence), and magnetic resonance angiography (MRA) was conducted while intravenous thrombolysis was performed. Forty minutes after t-PA administration, the common carotid arteries on bilateral sides showed no contrast on the MRA (i.e., the arteries were invisible on the scan), and aortic dissection was suspected ().\nStanford type A aortic dissection, which requires an immediate emergency operation, was observed on chest computed tomography (CT) (). However, the operation was postponed due to the high bleeding risk caused by the thrombolytic agent; the operation was finally conducted 13 hours later. The patient expired 4 hours after the termination of the operation due to excessive bleeding. |
C.M. is a 56-year-old man followed as an outpatient in mental health services for more than 20 years. Since adolescence, he was described as a shy, reserved person, and extremely sensitive to interpersonal interactions. He has always found himself at ease only with his family, having few friendships, however short-lived, and no significant romantic relationship. He reports that he has always found himself oscillating between the search of fulfilling a social relationship and his fear because he has always felt inadequate, and at fault when interacting with others. He always had a good academic performance, until he graduated in law.\nThe onset of the illness took place at the end of university when C.M. experienced a depressive episode. The environmental trigger seemed to have been a failed university course exam, after which the patient reported that he gradually developed the feeling that everyone was aware of his failure, and that they judged him for it. The extreme interpersonal sensitivity reached delusional characteristics during this period, structuring a real delusion of reference with persecutory aspects. C.M. described an experience of blame and shame, which he felt around him when he met people on the street, but also by what he saw on television or read in books. Ideas of guilt grew and worsened by the conviction that others could “read his mind” and therefore were aware of countless reasons to blame him. At that time, he reported the presence of derogatory auditory hallucinations blaming him. The episode, which lasted several months, resulted in a delay in achieving his degree, and then it resolved spontaneously and progressively without him seeing a psychiatrist. Although the episode was not accompanied by the execution of psychometric scales since the patient did not go to a psychiatrist, the characteristics of the episode satisfies the criteria for severe Major Depressive Episodes with Psychotic Characteristics, from the evaluation of the Structured Clinical Interview for DSM-5 Clinician Version (SCID-5-CV) regarding previous episodes (). Later, he described a period of increased energy, busyness, decreased need for sleep, and increased self-esteem, during which the patient enrolled in the faculty of political science, wanting to obtain a second degree. However, he stopped after taking a few exams, due to the recurrence of depressive symptoms.\nWhen he started treatment as an outpatient in psychiatry services, C.M., 36 years old, already had a long history of depression. He described symptomatology characterized by recurrent thoughts of guilt and inadequacy, mostly experienced in an ego-dystonic way, in which he patient complained of a sense of discomfort. There are also mental compulsions, consciously enacted in an attempt to ward off unpleasant thoughts. His symptoms are categorized as Obsessive-Compulsive Disorder, which leads to the commencement of psychopharmacological treatment. The prescription consisted of a high-dose antidepressant, sertraline up to 200 mg, and an antipsychotic, olanzapine up to 20 mg/day.\nOn regular basis, C.M. attends psychiatric appointments, and started attending rehabilitation activities, while retaining the tendency to socially isolate. Despite adherence to therapy, total remission of symptoms is never achieved. Over the years, his mood presents alternations between periods of depression, in which obsessive symptoms become more stubborn and disabling, and periods in which the patient feels “high,” active, less uncomfortable in social relationships, and less pervasively dominated by obsessive ideation. These activated phases never amount to real manic episodes but are characterized by increased energy, dysphoria, restlessness, and a greater drive to build social relationships. During mood episodes and intercritical periods, a marked interpersonal sensitivity with a tendency to misinterpret remains. The ideas of reference are experienced at times in an ego-dystonic way, as obsessive ideas, while at times in an ego-syntonic way, as authentic delusional ideas. In periods in which these ideas became more intense, haloperidol up to 2 mg/day is added to his prescriptions. The fluctuation of the thought content is congruent with the mood alternation, configuring prevailing experiences of inadequacy and guilt during the depressive phases and prevalent experiences of persecution during the activated phases.\nC.M. spends his life in an increasingly withdrawn way, struggling to interact with people also at work, therefore a disability pension is allocated to him and he continues only to carry out accounting work at his sister's office. He lives alone, in the same building where his mother also lives, with whom he spends most of the day. He still has very few social relationships and no romantic relationships.\nIn March 2020, his habitual stability was disrupted by the Covid-19 pandemic and the consequent lock-down. In addition to having to stop going to work, he was left to assist his mother almost alone, who had recently been diagnosed with Alzheimer's dementia. A depressive exacerbation began, and reached its peak in August. He was then voluntarily admitted to the psychiatry ward, sent by his psychiatrist, who found him completely blocked during an outpatient visit.\nUpon entering the ward, he appeared slowed down from a psychomotor point of view, gloomy, reticent, and distressed. After a few days, he reported that when admitted he felt as if he was in hell, and thought that the Day of Judgment would soon arrive. His thoughts appeared to be dominated by ruminations of guilt, experienced in an ego-syntonic way so much that C.M. appeared busy reviewing all his life looking for reasons for blame. He also reported that he took advantage of his family, but appeared confused and unable to explain how or when. Rating scales administered at admission show severe mental impairment: Brief Psychiatric Rating Scale (BPRS) = 69 (), Hamilton Rating Scale for Depression (HAM-D) = 22 (), Hamilton Anxiety Scale (HAM-A) = 19 (), Young Mania Rating Scale (YMRS) = 9 (), Positive And Negative Symptoms Scale (PANSS) = 87 (), Dimensional Yale-Brown Obsessive Compulsive Scale (DY-BOCS) = 55 (), and Global Assessment of Functioning (GAF) = 40 ().\nHis medication was changed by titrating clomipramine up to 125 mg and haloperidol up to 4.5 mg/day. Thus, we assisted with a rapid resolution of the psychomotor arrest, with greater accessibility for the patient to dialogue, remission of the delusional idea of guilt, toward which the patient showed progressively more insight and experienced it more as an obsessive doubt. No rituals or compulsions were highlighted. The mood remained depressed, even though the facial expressions were progressively more relaxed and he spent less time in bed. Despite the improvement, C.M. always felt he was getting worse, and he did not believe that he could overcome the current episode. Depressive symptoms are mainly characterized by anhedonia, hypersomnia, difficulty concentrating, feelings of guilt, and inadequacy.\nCognitive function was investigated, but the results of the neuropsychological tests (global cognitive function, selective attention and attention-executive skills, language, memory, logical-deductive reasoning, frontal efficiency tests) appeared to be normal. Brain Magnetic Resonance Imaging (MRI) did not show significant abnormalities. From a diagnostic point of view, the Structured Clinical Interview for DSM-5 Clinician Version (SCID-5-CV) () was administered, showing that the criteria for the diagnosis of Schizoaffective Disorder and Obsessive-Compulsive Disorder were met. Furthermore, the Structured Clinical Interview for DSM-5 Personality Disorders (SCID-5-PD) () highlights the coexistence of avoidant, obsessive-compulsive and paranoid personality disorders. These data will be confirmed, as we shall see, by a subsequent evaluation, once the acute aspects have been resolved.\nDespite the initial improvement, after 3 days there was a clinical flare-up. The patient appeared more confused, at times disorganized in behavior, again dominated by issues of guilt and unworthiness. Thoughts appeared accelerated, with elements of derailment, accompanied by motor restlessness and insomnia, effectively configuring a mood episode with mixed features. C.M. appeared distressed, with exacerbation of the delusional ideas of reference and persecution toward the other inpatients. Therapy was first modified by reducing clomipramine to 50 mg/day. The thoughts and behavioral disorganization were however worsening, configuring a picture of severe psychotic anxiety, with emergent suicidal ideation. Finally, it was decided to discontinue clomipramine and continue only with haloperidol therapy up to 6 mg. After a few days, we witnessed the remission of the most evident psychotic symptoms, with the persistence of persecutory ideas and guilt, lived at the time in an ego-syntonic and at times in an ego-dystonic way. C.M. was gradually less anxious, less restless, and less dominated by intrusive thoughts.\nOnce full remission of psychotic symptoms was achieved, we introduced a new antidepressant with a more sedative effect, which was trimipramine up to 80 mg/day. Agitation therefore reduced and, after 33 days of hospitalization, the patient was progressively less distressed and less gloomy. The mood seemed to improve both subjectively and objectively, in the absence of delusional ideation. The obsessive thoughts appeared less pervasive (although the interpersonal sensitivity and fleeting ideas of reference remained), the patient appeared to be less guarded with the medical staff. He was then discharged from our ward with a diagnosis of Major Depressive Episodes with Psychotic Behavior and Obsessive-Compulsive Disorder. We however suggested that the patient be admitted in a rehabilitation ward, in order to consolidate the achieved results. The patient accepted the suggestion. At the time of discharge, the scores of the rating scales showed significant improvement: BPRS = 29, HAM-D = 10, HAM-A = 8, YMRS = 5, PANSS = 44, DY-BOCS = 38, GAF = 65.\nThe patient follow up continued on an outpatient basis by psychiatric services, showing good compliance with the therapy and no further acute episodes at the one-year follow-up. Once the psychopathological compensation was reached and the acuity resolved, the patient was again subjected to personality assessment tests. The SCID-PD confirmed the diagnosis of avoidant, obsessive-compulsive, and paranoid personality disorder (). The patient was also subjected to further psychometric scales, such as the short version of the Temperament and Character Inventory (TCI-125) (), Magical Ideation Scale (MIS) (, ), Perceptual Aberration Scale (PAS) (), and Hypomanic personality scale (HPS) ().\nAs for the TCI-125 assessment, the results of the SCID-PD were in fact confirmed. The most significant traits appeared to be those relating to the Harm Avoidance dimension, with a score of 20, significantly higher than the average for age and sex (mean 8.05 ± 4.186). The scores of Novelty Seeking (2, mean 7.91 ± 3.244), Persistence (1, mean 3.21 ± 1.526), Self-Directeness (9, mean 18.92 ± 4.413), and Self- Transcendence (0, mean 5.96 ± 3.786) were instead lower than the average. Finally, the Cooperativeness score (18, mean 18.28 ± 3.470) and the Reward Dependence score (10, mean 7.68 ± 2.758) were slightly increased ().\nEvaluation with MIS demonstrated the presence of magical thinking, obtaining a score of 9/30, which appears higher than the Italian average for age and sex (mean 5.1 ± 4), testifying schizotypical traits (, ). The PAS showed a score of 13/35, which places the patient in a slightly above average position (mean 7.8 ± 5.77) (), highlighting the ease in incurring erroneous and abnormal perceptions of reality. The HPS evaluation instead showed a result clearly below average, with a score of 8/48 (mean 21.08 ± 8.19) ().\nThe re-evaluation of the patient at 1 year of follow-up also made it possible to evaluate the subjective experience of illness. The patient has good insight and good compliance with the proposed treatments, both pharmacological and psychological and rehabilitative, in the phases of clinical compensation. He appears to be very aware of his disorder, up to the point of identifying himself with the figure of “the patient,” who according to him allows him shelter from the world. Regarding his last hospitalization, C.M. shows a strong experience of shame, so he asks not to talk about it. |
A 16-year-old Asian American female; was admitted to the inpatient unit with depression and suicidal thoughts. The patient reports subsyndromal depressive symptoms for the two years, which have worsened in the last two months. Over the past several months, she started self-mutilating by cutting her arms and legs with a razor. She also had suicidal ideations with a plan to stab herself with a knife or get hit by a car. The precipitating event was being accused of cheating on a school test, and the fear of being expelled. The mother reported the patient was having increased sleep, decreased motivation, social isolation, and dropping school grades. Besides school stress, the patient reported conflict with her parents about the cultural differences. The patient stopped talking to her parents, siblings, and her teachers. She stopped doing homework or studying for tests; her grades dropped from A’s & B’s to C’s & D’s. She did admit to suicidal ideation for months, without a plan. The patient was born and raised in Asia until she was seven when her parents moved to the United States. Her parents expected her to follow their own culture and religion, which had posed many arguments in the household. The patient’s father worked at the same school, and she struggled between rigid rules of her parents’ traditions, and culture and finding a balance to maintain her own cultural identity.\nWhen the patient was in eighth grade, she was diagnosed with TS but was never disclosed to her. She was born after a full-term pregnancy with a caesarian section delivery and met all her milestones. The patient reported experiencing bullying since the fifth grade for being short. She started taking growth hormones in eighth grade but stopped one month ago when she became severely depressed. She continued to elaborate that she had low self-esteem because she felt that something was wrong with her. She described that her mother would occasionally meet with the doctor while she waited outside the room. She was told to have needed growth hormones because she was not growing appropriately. She was in 10th grade and had never repeated a grade. She reported that she was in the process of getting an Individual Educational Plan (IEP) evaluation as it took her extra time to finish papers, projects, and tests when compared to other students in her class. She denied any use of tobacco, alcohol, or illicit substances. She denied any history of physical or sexual abuse. She has been prescribed Sertraline 25mg after the consent of the mother and ascent of the patient. After one week, the Sertraline was titrated to 50mg and then to 75mg after the patient showed a minimal response. After week 2, the patient reported that she felt that the medication was slightly helpful, and she was more motivated about her future. However, there would be many times when she felt worthless. Sertraline was again increased to 100mg daily. At first, the patient had social difficulties with peers but reluctantly started to participate in group therapy. She reported having learned about herself in groups. After a few family sessions, the patient continued to feel rejected, misunderstood, and not ready to accept feedback. The day after this family session, the patient took a downturn and was punching walls and verbally aggressive. She also expressed that living with a family friend out of the United States might be an option as she did not want to return home. She agreed that listening to music and blogging on the computer were her coping skills at home and could add writing, drawing, and stretching to her list of other possibilities. Aripiprazole 2mg was added to her medication regime to augment the action of Sertraline. After a week on this combination regimen, she showed more improvement with her depressive symptoms. In the next family session, she agreed to step down to the partial hospital and continue to work on the family issues with her therapist. She was discharged back home in the care of her mother. |
A 36-year-old man presented to the Gastroenterology Outpatients Department with a four-month history of weight loss and iron-deficiency anaemia. He denied abdominal pain and described no change in bowel habit or rectal bleeding. He had previously been diagnosed with UC approximately 20 years ago and had undergone a colectomy with ileostomy two years later. 15 years ago, he had an ileoanal pouch formed.\nOesophagoduodenogastroscopy (OGD) and flexible pouchoscopy were unremarkable. VCE was performed; however the capsule was not passed (). Pouchoscopy/retrograde ileoscopy with a paediatric colonoscope showed a normal pouch with scattered aphthous ulcers in the ileum (). Two strictures were identified, the more significant 100 cm proximal to the pouch, which could not be traversed by the scope. Small bowel barium follow-through confirmed the endoscopy findings and showed the capsule was impacted but freely mobile proximal to this stricture.\nThe capsule was retained proximal to an ileal stricture. The findings of small bowel ulceration led to a diagnosis of small bowel CD being made. There was no history of nonsteroidal anti-inflammatory drug (NSAID) consumption, vasculitis, or connective tissue disease.\nA course of budesonide was given to resolve the inflammatory component of the stricture and the patient was followed up for two months with serial abdominal radiographs, but the capsule did not pass. At repeat ileoscopy, the dominant ileal stricture could not be traversed by a paediatric colonoscope even after gentle balloon dilatation. An attempt to insert a biodegradable stent was unsuccessful.\nAzathioprine and a tapering course of prednisolone were commenced but unfortunately the capsule was still retained. After eight weeks of immunosuppression therapy, colonoscopy showed that the ileal ulceration and inflammation had markedly improved. The dominant stricture was gently dilated to 12 mm using a controlled radial expansion balloon under fluoroscopy (), allowing passage of the scope passage and retrieval of the capsule with a Roth net (). |
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