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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 50-year-old female presented to us with a 3-week history of rapidly progressive neck swelling associated with mild neck discomfort. She did not complain of any respiratory difficulty, voice change, haemoptysis, dysphagia, weight loss, loss of appetite or fever. She had no previous history of radiation exposure, no significant medical illness or surgery, and no family history of thyroid malignancy. Her physical examination revealed a large hard nodule on right side of thyroid with relative fixity to surrounding structures along with palpable right cervical lymph nodes. Her thyroid function tests were within normal limits. Ultrasonography of the neck revealed dominant solid hypoechoic nodule in right lobe of thyroid measuring 5.9 × 5.2 × 2.8 cm with internal vascularity and without any cystic changes. Computed tomography (CT) in the neck revealed heterogeneously enhancing lesion with areas of necrosis and calcification involving the right lobe and isthmus with ill-defined margins in places and mildly enlarged, necrotic level 3, 4 and 5 lymph nodes (). Ultrasonography-guided fine needle aspiration cytology was suggestive for malignancy, possibly medullary carcinoma/poorly differentiated carcinoma (Bethesda category VI). Her serum calcitonin and urine catecholamines levels were within normal limits.\nAfter detailed discussion and consent she underwent total thyroidectomy and bilateral neck node clearance. Intraoperative clinical findings of the tumour in right lobe of the thyroid showed desmoplastic reaction in surrounding soft tissues with no local invasion on the right side. The left lobe was apparently uninvolved and the postoperative course was uneventful.\nHistopathology showed spindle cells with high-grade sarcoma of the thyroid origin, likely LMS (). Eight out of 41 lymph nodes sent for biopsy were tumour positive. Immunohistochemistry showed that neoplastic spindle cells were positive for smooth muscle actin (), vimentin, caldesmon and had Ki-67 index of 60%; while no reactivity was reported for thyroglobulin, calcitonin, thyroid transcription factor-1, S-100, CD45, CD 34, paired box gene-8 (PAX-8), chromogranin and cytokeratins.\nA positron emission tomography scan conducted postoperatively and showed no evidence of residual tissue and distant metastasis. The patient was then planned for radiotherapy after stabilisation; however, within 1 month of the waiting period, she developed local recurrence. The oncology team were consulted and she received 35 cycles of radiotherapy and paclitaxel and carboplatin-based chemotherapy. During last follow-up telephonically 5 months post-surgery she was alive; however, in a very poor general condition. |
A 63-year-old right hand dominant female sustained a fall injuring her left shoulder. She was initially seen in a local emergency room (ER) on the day of the injury. An anteroposterior radiograph was performed in the ER and the diagnosis of a lateral clavicle fracture was made with sling and swathe as the treatment. Orthopedically, the patient was seen in follow-up two weeks thereafter in clinic. On physical inspection, a bony prominence was noted in the region of the supraspinatus muscle, seemly protruding through the trapezius muscle (Figure\n). The patient was neurovascularly intact with full motor function and normal sensation to light touch over the left upper extremity. Both anteroposterior and the axillary radiographs were obtained. The anteroposterior radiograph (Figure\n) of her shoulder revealed a Type V fracture of the lateral clavicle in what appeared to be in acceptable position, however, the axillary projection showed the distal end of the proximal clavicular segment to be significantly posteriorly displaced (Figure\n).\nThe patient was subsequently taken to the operating room at which time the lateral clavicle, AC joint, and acromial areas were surgically exposed. The proximal clavicular segment was indeed posteriorly displaced and buttonholed through the trapezius muscle (Figure\n). The segment was extricated from the muscle and reduced relative to the distal clavicular segment. The inferior bone fragment of the fracture was still attached to both the conoid and trapezoid (CC) ligaments. Internal fixation was provided by two smooth K-wires. One was passed through the proximal aspect of the acromial process and into the intramedullary canal of the proximal segment exiting through its superior cortex. The 2nd K-wire was passed through the distal aspect of the acromial process across the AC joint, and down the intramedullary canal of the distal and proximal clavicular segments exiting out the posterior cortex of the proximal segment. Number 5 Ethibond sutures were then passed around each K-wire in a figure-of-eight fashion to create tension band constructs and secure fixation. The lateral ends of the K-wires were bent to prevent migration and left beneath the skin. The wound was closed with xeroform dressing wrapped around each K-wire. Figure\n shows the postoperative AP and axillary radiographs. At 6 weeks the K-wires were removed, functional use of the shoulder was encouraged, and physical therapy was begun and designed to regain maximal shoulder range of motion and strength. At the final follow up appointment (1 year post-op), the patient was doing well and had returned to full activity. On physical examination, she had 165 degrees of forward flexion, 45 degrees of external rotation, 110 degrees of abduction and internal rotation to the high part of her back, as well as active elevation of her arm to well above the horizontal. These measurements were very similar to the contralateral upper extremity. A six-month postoperative radiograph is seen in Figure\n. |
A 38-year-old female with no significant medical history presented with severe headaches and nausea that was exacerbated by activities such as bending over and lifting. The patient was previously seen by other neurosurgeons outside of the United States and had undergone an attempted biopsy for a pineal region lesion through a frontal endoscopic approach. This patient developed Parinaud syndrome, diplopia, and gait imbalance which lasted approximately 1 month. Later, they had a second stereotactic biopsy that revealed a WHO Grade 1 meningothelial/fibroblastic meningioma. The patient was neurologically intact by the time of our initial evaluation. Imaging revealed a 3.5 cm × 3.1 cm × 3 cm large pineal region enhancing lesion with brainstem (midbrain in the tectal plate) and cerebellar compression [-].\nThe lesion was resected through bilateral parietal-occipital stereotactic craniotomy (larger on the right side) utilizing a posterior right occipital interhemispheric and transtentorial approach as described in the previous case. Given the steep tentorial angle, prior surgical treatments this patient was subjected previously with subsequent morbidity, and goal to reach the most ventral aspect of the tumor without cerebellar retraction, the right occipital interhemispheric and transtentorial approach were selected over the supracerebellar-infratentorial option. The lesion was highly vascular, and we performed gradual central debulking with an ultrasonic aspirator with careful microdissection of the tumor capsule from vascular structures. A subtotal resection was performed, leaving the superior and left the aspect of the capsule, which was attached strongly to the Vein of Galen, bilateral veins of Rosenthal, and also toward the tectal plate of the midbrain. A right frontal ventriculostomy was placed preoperatively and removed 2 days after surgery.\nOn discharge, the patient’s baseline symptoms resolved and she was neurologically intact. Pathology confirmed a WHO Grade I fibrous meningioma. Follow-up magnetic resonance imaging (MRI) 8 months after surgery revealed the minimal residual presence and no signs of progression [-]. |
A 41 years old male presented with chief complaint of gradual onset breathlessness on exertion New York Heart Association (NYHA) II and pain in right lower chest. There was no history of haemoptysis, orthopnea or paroxysmal nocturnal dyspnea. Pain in chest was dull in nature, non-radiating and relieved by taking medication. There was no history of hypertension, diabetes mellitus or any other prolonged illness.\nThere was no history of significant loss of weight and appetite.\nOn examination patient was conscious, cooperative and average built. His pulse rate was 78 per minute and blood pressure was 120/70 mmHg.\nThere was no significant respiratory distress at that time. Systemic examination revealed decreased air entry on auscultation at the lung bases.\nOther systems were within normal limits. Routine blood investigations including complete blood count (CBC), renal and liver function tests were within normal limits. His coagulation profile including platelet count, prothrombin and activated partial thromboplastin time were also within normal limits.\nChest radiograph revealed bilateral pleural effusion that was later confirmed by ultrasonography of the chest.\nEchocardiography revealed localized pericardial effusion adjacent to right atrium (RA) with multiple echodense nodular masses seen within pericardial cavity with constriction pathology.\nThere were significant respiratory variations across mitral and tricuspid valve. Inferior venacava (IVC) was dilated and collapsing less than 50% with inspiration. There was mild dilation of RA with no regional wall motion abnormality (RWMA). Computerized tomography (CT) scan thorax revealed large eccentric sarcomatous mass lesion, aneurysmal dilatation of RA and bilateral pleural effusion. Based on these results pleural tapping was done which revealed haemorrhagic fluid. Fibreoptic bronchoscopy was also done. Fine needle aspiration cytology (FNAC) and pleural cytology revealed no active malignant or inflammatory cells.\nA provisional diagnosis of anterior mediastinal tumor was made and exploratory biopsy via midsternotomy was planned to confirm the diagnosis and informed consent was obtained for the same.\nPatient was premedicated with lorazepam 2mg and pantoprazole 40 mg a night before the surgery. On arrival in the operating room, pulse oximetry revealed oxygen saturation of 96% on air. After securing venous access, radial artery cannulation was performed. Internal jugular vein cannulation was performed with trilumen catheter (7F). Anaesthesia was induced with sodium thiopentone (3 mg/kg) and fentanyl citrate (5 mcg/kg). Vecuronium bromide (0.15 mg/kg) was administered intravenously to facilitate tracheal intubation. Anaesthesia was maintained with intermittent doses of fentanyl citrate (1 mcg/kg), vecuronium bromide (0.02 mg/kg), and isoflurane in 50% oxygen in air.\nIntraoperative monitoring included EKG with ST segment analysis, oxygen saturation, end tidal CO2, temperature and urine output, direct arterial blood pressure, central venous pressures, cardiac output and derived parameters, arterial blood gas analysis, and activated coagulation time (ACT). Transesophageal echocardiography (TEE) revealed large cavity around RA with communication with it and there was homogenous opacity inside the cavity.\nHeparin sulphate 4 mg/kg was administered to maintain ACT more than 450 seconds before initiating cardiopulmonary bypass (CPB). Moderate hypothermia with cooling upto 28 °C was maintained. Total bypass time was 98 minutes. Aortic crossclamping was not done during dissection of the mass. A large necrotic vascular mass was seen in the anterior pericardium with invasion of RA and right ventricle. During dissection of the mass a rent in RA occurred which was repaired with bovine pericardial patch. Weaning from CPB was uneventful. Heparin was reversed with protamine monitoring the ACT.\nA total of three units of packed red blood cells (PRBC) and four units of platelet concentrates (PC) and fresh frozen plasma (FFP) were transfused intraoperatively. Haemostasis was done however; patient was bleeding from the rough surgical surfaces. Tranexamic acid 10 mg/kg as bolus and 1 mg/kg/hr infusion was also started. Thereafter, mediastinum was packed with sponges and only skin layer was closed. Patient was shifted to the postoperative recovery room for mechanical ventilation and monitoring of haemodynamics and bleeding from chest drains.\nIn the recovery room patient was haemodynamically stable however constant bleeding was there from the mediastinum. He bled about 600ml in first and about 300 ml in next 24 hours. Echocardiography did not reveal any evidence of cardiac tamponade at that moment. He was planned for sternal closure on the second postoperative day after stabilisation of bleeding. However, during reopening significant oozing was still there from the surgical sites. Mediastinum was again packed with only skin closure and patient was shifted to the recovery room.\nHistopathology of the mediastinal sample gave impression of angiosracoma with microscopic findings of highly vascular mitotically active spindle cell neoplasm composed of freely anastomosing vascular channels lined up by atypical cells.\nHe was haemodynamically stable in the postoperative period. However, constant bleeding from the chest drain was still there at 150-200 ml per day for next three days. On sixth postoperative day drainage was 40 ml in 24 hrs. Therefore, he was planned for removal of pack and secondary closure of the chest. During secondary closure, significant bleeding was still there from the raw surgical surface that was not controlled even by adequate haemostasis and electrocautry.\nThromboelastography (TEG) was within normal limits. A total of nine packed red blood cells, four units of fresh frozen plasma and four units of platelet concentrate and apheresis were transfused in the recovery room. Keeping in view of the intractable bleeding, massive transfusion and second reopening it was decided to administer rFVIIa (Novoseven, Novo Nordisk India Ltd) in the doses of 90 mcg/kg to this patient.\nAfter administering recombinant factor seven oozing from the surgical surface settled down. Secondary closure of the chest was done and patient was shifted to the recovery room. Trachea was extubated after four hours and rest of the postoperative course was uneventful. After stabilization and discharge from the hospital patient was referred to oncology unit for further management and he is on a regular follow up for radiation therapy. |
The second case is a 56-year old Lebanese male, smoker, that was found in April 2016 to have multiple soft tissue nodules in the left lung on CT scan, the largest of which measuring 1.5 cm. Bronchial biopsy showed a metastatic epithelium of poorly differentiated adenocarcinoma (TTF1 and p63 positive) with rare carcinomatous non-small cells in bronchial wash. PET CT scan in April 2016 showed left upper lobe active mass with small active left hilar lymph node and centimetric adenopathy in the left para-aortic region, the latest were noted as inflammatory or secondary by the radiologist. Injected total body CT scan later on in April showed a 2-cm spiculated nodule in postero-apical segment of left lobe with intimate contact with left pulmonary artery (staged as T4) along with infiltration around the left main bronchus, 3 cm above the carina with no other associated abnormalities. Patient was then classified as IIIA (T4N1M0) inoperable NSCLC according to the eighth AJCC classification. 21 days cycles with Gemcitabine 1,300 mg day 1 and 8 and carboplatin 300 mg day 1 were started and given for 7 cycles. After that, a chest CT scan was done in October 2016 and showed persistence of the previously noted inoperable spiculated mass in the left upper lobe with newly appearing hilar adenopathies with posterior extension reaching lung fissure along with new appearing 7 mm opacity in the right lung at the postero-basal segment. In front of this progression, PD-L1 test was done using IHC staining for Abcam clone on the initial lung tissue and showed 80% PD-L1 expression. So the decision was to start second line therapy in November 2016 with pembrolizumab 200 mg IV administered every 3 weeks. A control CT scan in February 2017 after 4 doses of pembrolizumab were given showed a good response to therapy with regression of the left previously spiculated left lung mass. PET CT scan later on in April 2017 showed stable nonactive lung nodule at left apex with good response to therapy in superior lobe of left lung, 17 mm in diameter with SUV of 4.1 against 23 mm diameter and SUV of 5.1 previously. Injected brain MRI was negative. Immunotherapy was continued and reevaluation was done in august 2017 and disease was stable on CT scan. So decision was also to continue immunotherapy till November 2017 when a PET-CT scan showed a 16 mm opacity with new nodule in posterior segment of upper lobe suggestive of recurrence with active left hilar adenopathy. An MRI of mediastinum was done and showed a 12-mm mass compatible with the known neoplastic process separated this time from left pulmonary artery and aortic arch, in contrast to initial imaging, with presence of left hilar adenopathy. In front of these new findings and the anatomical separation, decision was to do left lobectomy and patient was operated in January 2018. The final pathology report came positive for a moderately differentiated infiltrating lung adenocarcinoma of acinar and papillary types of 1.7 cm in diameter with one positive node out of 4 in aorto-pulmonary window and negative other adenopathies in left bronchus and 3 negative interlobular and hilar nodes. PD-L1 on post-operative tissue was negative using the same Abcam clone. In April 2018, the patient was still off therapy and a total body CT scan done for diffuse whole body pain reported by the patient showed no evidence of recurrent disease. He was diagnosed with a left hip fracture, operated in June 2018, with no evidence of malignancy on bone specimen. |
A 65-year-old diabetic male was referred to our burns centre from the accident and emergency department of a peripheral hospital with a full-thickness burn to his longstanding, irreducible umbilical hernia. This injury was sustained whilst at work where he operated as a metal drop forger, shaping metal bars under intense furnace heat. When placing metal bars into the furnace he was exposed to temperatures exceeding 1350°C for 20 to 30 seconds a time approximately 48 times a day giving him a total of 24 minutes exposure in an eight-hour shift.\nHealth and safety regulations on site stipulate a heat-resistant apron must be worn which the patient was wearing at the time of injury. However due to his umbilical hernia he also normally wore a waist support belt which temporarily flattened the hernia and reduced the risk of this protruding aspect of the abdomen getting injured. On the day of the incident, the patient was not wearing this protective belt. There was a delay in the patient presenting to the hospital as he was initially unaware that the injury had occurred, presenting on day 3 post-injury when he noticed a discharge from this area of skin was making the front of his shirt wet.\nAt the presentation in the burns centre, the patient was systemically well with no abdominal symptoms and normal bowel motions. On examination, he had a 0.25% full-thickness burn (Figure and Figure ) with surrounding cellulitis supported by raised biochemical markers of inflammation; a C-reactive protein of 21mg/L and WCC of 11x109/L. Wound swabs from the burn site later confirmed the presence of Staphylococcus aureus sensitive to flucloxacillin. Empirical therapy of flucloxacillin was already commenced intravenously at the time of presentation; 2g four times a day. Subsequent repeat blood tests showed a response to antibiotic therapy with a reduced C-reactive protein of 14mg/L and WCC of 8.6x109/L on day 6 of admission.\nThe abdomen was soft and non-tender with bowel sounds present throughout. The general surgical team was asked to review the patient on day one of the presentation and a decision was made to perform a CT abdomen to determine both the underlying anatomy of the hernia sac, its contents and whether the full thickness burn was communicating with any loops of bowel. The CT scan demonstrated an umbilical hernia with a neck of 2.3cm. There was a breach in the hernia fascia, but no communication between bowel and the burn. The general surgical opinion was to aim for definitive surgical repair of the hernia with a mesh.\nOptimal management of a full-thickness burn would typically be surgical debridement of the area to remove burnt and infected tissue. However in this case there was concern that surgical excision could potentially lead to the spread of infection deeper into the peritoneal cavity. The active infection would also be a contraindication for the use of mesh to repair the hernia.\nAfter extensive discussion between the plastic surgery and general surgery teams, it was agreed upon to manage the burn non-operatively and allow the cellulitis to resolve before considering hernia repair. The full-thickness burn was managed with daily flamazine, jelonet, gauze and hypafix dressings. This dressing allowed the eschar of the burn to gently lift off and aid healing underneath with further epithelialisation. The patient was trained by the nurse specialists as to how to change his dressings himself at home. He was discharged with follow-up outpatient consultations to review the wound three times per week for the next two weeks.\nA few weeks later, the patient was reviewed by the general surgery team. The decision at this outpatient appointment was to proceed with elective hernia repair with a mesh as his burn had reduced in size and the acute infection had been treated. |
A 42 year-old man with a 2-year history of persistent dry cough was referred for the diagnosis of cause. He coughed all the day, severely at postprandial time or when going to bed. An occasional regurgitation was described but without heartburn or the sensation of postnasal drip (Table\n). The patient was a lifetime nonsmoker, and had no history of chronic rhinitis or occupational exposure to pollutants. Six months ago, he visited to another respiratory clinic, where chest X-ray and lung function examinations were within the normal limits, and bronchial challenge confirmed airway hyperresponsiveness, as shown by 3.0 μmol of the cumulative provocative dose of histamine causing a 20% fall in FEV1. Then, the diagnosis of cough variant asthma was considered. However, the anti-asthma therapy including the initial inhalation of salmeterol/fluticasone 50/250μg, twice a day for one month and the subsequent trials with oral prednisone and montelukast failed to relieve the cough.\nPhysical examination was normal. The repeated bronchial provocation with histamine demonstrated the recovery of airway responsiveness. Cell examination in the induced sputum showed 82.5% of macrophages,12.0% of lymphocytes and 5.5% of neutrophils. MII-pH study was performed which revealed a slight abnormal acid reflux and the marginal level of symptom association probability (Table\n).\nThe initial trial with omeprazole plus domperidone was unsuccessful. Thus, baclofen was added to the therapy while domperidone was withdrawn. Cough significantly improved within the 4 weeks and completely resolved within the two months. The effectiveness was also verified by the decreases in cough symptom score and cough reflex sensitivity to capsaicin (Figure\n). The dosage of baclofen was maintained for two months after the resolution of cough, and then began to reduce in a speed of 10mg a week. Since cough did not relapse with the minimum dose of 20 mg daily, baclofen was stopped two months later. No obvious adverse effect except for a slight sleepiness was reported during the treatment with baclofen. |
A 30-year-old female reported to Department of Oral Medicine and Radiology with a chief complaint of swelling in left lower back tooth region since 1-year. History revealed that patient had undergone extraction of mobile teeth in the same region, 1½ years back. After 5–6 months of extraction, she noticed a swelling in the same region. The swelling progressively increased in size and was associated with mild intermittent pain in left ear. There was no history of pain or any pus discharge in the associated area. Her past medical and family history was noncontributory. Extra-orally, mild facial asymmetry was noticed on the left side of face near the angle and ramus of the mandible. On palpation, a hard nontender swelling measuring approximately 2 cm × 2 cm was evident on left lower third of face extending from mid body of the mandible to 1 cm anterior to angle of the mandible. On intraoral examination, lower left molars were missing. Mucosa over the alveolar ridge was normal in color and texture. On palpation, 3 cm × 2 cm hard, nontender expansion of buccal and lingual cortices was evident extending from region distal to lower left second premolar up to the anterior border of ramus of mandible with obliteration of the lower buccal sulcus [].\nPanoramic radiograph presented a multilocular radiolucency involving the left side of the mandible []. Computed tomography (CT) revealed a well-defined expansile lytic lesion involving left angle and ramus of mandible with associated thinning of the cortices and breach in the lingual cortex of left ramus of the mandible [Figures and ]. Contrast magnetic resonance imaging (MRI) showed that lesion was heterogeneously hyperintense on T2-weighted images, isointense on T1-weighted images with heterogeneous enhancement on contrast. Minimal soft tissue extension was noted along the floor of the mouth abutting the left mylohyoid muscle. The left inferior alveolar nerve (IAN) in the mandibular canal was also seen to be displaced by the lesion [].\nBased on the painless and slow growing nature of the swelling, located in posterior mandible and existing radiographic appearance, the following conditions were considered in our differential diagnosis such as benign tumors of odontogenic origin such as ameloblastoma, ameloblastic fibroma, odontogenic myxoma, and keratocystic odontogenic tumor. Nonodontogenic tumors such as ossifying fibroma, osteoblastoma, desmoplastic fibroma, intraosseous mucoepidermoid carcinoma, and BFH were considered because of the predilection for occurrence in the mandible with a similar appearance.\nThe patient was operated under general anesthesia, and segmental mandibulectomy was performed using intraoral approach. Gross examination of the tumor revealed well encapsulated tumor mass with areas of firm but unmineralized yellow tan tissue and partially hemorrhagic red brown tissue []. The microscopic examination revealed an admixture of spindle-shaped cells (fibroblasts) and epithelioid cells (histiocytes) in a fascicular and storiform pattern. Multinucleated cells and scattered lymphocytes were also seen. The stroma consisted of large areas of foamy cells interspersed with fibrovascular septations. No mitotic activity, cellular atypia or pleomorphism of tumor cells was evident. Vascularity of tumor varied from relatively inconspicuous vessels to exceedingly prominent vascularity with striking hyalinization [Figure –]. The histopathological features were suggestive of BFH. Immunohistochemical staining showed positive CD68 cells in the lesional tissue [].\nThe patient has been followed up periodically, but no recurrence or other changes were noted for 2 years. |
A 65-year-old woman was referred to a gynaecologist for a urethral mass that on gross examination appeared to be a caruncle. Upon examination, a 3-cm wide, pedunculated, black pigmented, friable, haemorrhagic polyp was found at the posterior wall of the urethral meatus. The tumour was removed by local excision. A histopathologic analysis partially revealed a polypoid tumour with superficial ulceration that comprised squamous and transitional epithelia (). The tumour was composed of loosely cohesive nests of atypical epithelioid and spindle-shaped melanocytes that showed diffuse and nested growth patterns. The neoplastic cells had an abundant eosinophilic cytoplasm, large hyperchromatic nuclei with prominent nucleoli, and brisk mitotic activity. Most of the tumour cells contained coarsely granular melanocytic pigment (). The depth of tumour invasion, as measured by a digital microscopic camera (Olympus DP10), was 3.57 mm. No vascular/lymphatic invasion was observed histologically. Immunohistochemically, the tumour cells showed strong cytoplasmic reactivity for HMB-45 and S100 (). Three months after the initial surgery, a resection of the distal third of the urethra showed no evidence of disseminated disease. The initial staging, which included computerised tomography (CT) of the chest, abdomen, and pelvis, revealed no evidence of disseminated disease. A repeat CT of the chest, abdomen, and pelvis two years after the initial diagnosis revealed two enlarged right parailiac lymph nodes, which remained unchanged at two subsequent CT assessments that were performed four and six years after the primary diagnosis. The patient showed no evidence of disease during a six year follow-up period.\nIt has been suggested that vulvar cancer exists as two separate diseases. The first type involves human papillomavirus (HPV) infection, which leads to vulvar intraepithelial neoplasia (VIN), a predisposing factor for vulvar cancer. An estimated 80% of untreated women suffering from VIN III develop invasive disease []. This type of vulvar cancer often develops in younger patients, and a recent review noted that approximately 15% of all vulvar cancers occur in women under age 40 []. Other predisposing factors, for example, a history of condylomata or sexually transmitted diseases (STD), low-economic status or nicotine abuse, have also been found [].\nThe second type of vulvar cancer involves vulvar nonneoplastic epithelial disorders (VNED) and advanced age, which lead to cellular atypia and eventually to cancer [].\nMalignant melanoma of the vulva is a tumour of elderly women 65 to 75 []. Other factors, such as parity, genetic factors, or hormonal influences, do not appear to be related to either the occurrence or extent of vulvar melanoma. The relationship between ultraviolet radiation exposure and the risk for malignant melanoma has been known for some time; however, the knowledge of this correlation has not helped to clarify the aetiology underlying vulvar melanoma. It has been posited that UV light may be directly involved in the pathogenesis of this condition by causing a cell-mediated, systemic alteration of the immune response, which then increases the risk for vulvar melanoma []. Melanoma is a tumour originating from the neuroectoderm. Vulvar melanoma may develop from preexisting junctional or compound nevi as well as de novo from the melanocytes resting in the basal layer of squamous epithelium [].\nThe most frequently reported symptom of vulvar cancer is a long history of pruritus. Less commonly presenting symptoms include vulvar bleeding, discharge, dysuria, and pain. The most obvious presenting sign of vulvar cancer is a vulvar lump or mass, which may appear ulcerated, leukoplakic, fleshy, or warty.\nIn contrast, patients with malignant melanomas arising from the distal part of the urethra suffer from rapidly developing, nondistinct symptoms. These signs and symptoms include a urethral mass, nonspecific perineal pain, dysuria, incontinence, haematuria or local bleeding, and pruritus. The melanoma is usually pigmented and varies in colour from black to blue or light brownish; the lesions are firm, nodular, and often ulcerated.\nVulvar cancer is staged using the TNM classification system. Staging reflects the characteristics of vulvar cancer growth, which occurs in the following manner: first by direct extension into the adjacent organs (the vagina, urethra, and anus), followed by lymphatic metastasis to the local lymph nodes (from the inguinal to the femoral to the pelvic lymph nodes) and finally by haematogenous spread to distant sites (liver, lungs, and bones) [].\nIn malignant melanoma, there are several systems to describe the extent of lesions in addition to the TNM staging system.The Clark-Level [] delineates five levels of tumour invasion in cutaneous melanoma based on the penetration of dermal connective tissue planes and their correlation with prognosis, irrespective of the volume or the superficial tumour spread. The Breslow-Index, levels I–IV, [] uses tumour thickness as the most significant measurement of size and correlation with prognosis. The Chung-Levels, levels I–V, [] also describe the depth of tumour invasion and tumour thickness, while taking the lack of papillary dermis in the mucous membranes of the labia into account. This classification is often used to estimate the risk of regional and distant metastases [].\nAlthough it is primarily used for squamous cell carcinomas of the vulva, most investigators have found the FIGO classification to be of minimal prognostic value with respect to vulvar melanomas [].\nIn cases of suspicious vulvar lesions, even if the patient is asymptomatic, a biopsy must be performed down to the dermis for a histological diagnosis and to measure the tumour thickness. The affected area must be examined immunohistopathologically (Vimentin, S-100-Protein, HMB45) for further classification [].\nOther diagnoses that may present without any evidence of tumour cells, such as melanosis vulvae, melanocytic naevi, pigmented VIN, seborrhoeic keratosis, or angiokeratoma, should be excluded by biopsy as well.\nSurgical resection is the gold treatment standard in patients with vulvar cancer. Tumours <1 mm should be removed with a 1 cm margin, whereas radical vulvectomy is recommended if the tumour is >1 mm. Surgery should completely remove the cancer and identify the extent of disease to determine the stage of the lesion and the need for further therapy. The extent of disease determines the amount of surgery needed [].\nRadical vulvectomy with bilateral dissection of the inguinal and pelvic nodes was initially recommended as the standard treatment for most patients. The current recommendation is that a more individualised and conservative approach be used to treat such patients. Depending on the tumour's localisation, the extent of stromal invasion, and the general staging, it may be possible to choose radical local excision for treatment []. In cases of a malignant melanoma of the vulva located at the distal part of the urethra, treatment includes a partial or total urethrectomy (depending on the level of invasion) [].\nFor vulvar cancer, the need for a lymphadenectomy depends on the stromal invasion. Stromal invasion of <1 mm is not associated with inguinal node metastases, whereas a patient with a tumour thickness >1 mm must be treated using inguinal-femoral lymphadenectomy, or at least sentinel lymph node biopsy in cases of inconspicuous groins []. If three or more inguinal-femoral lymph nodes are positive or if there is a macrometastasis (>10 mm), pelvic lymphadenectomy is recommended [].\nBecause of the small number of cases, the treatment of vulvar malignant melanoma is similar to that of cutaneous malignant melanoma. Melanomas with <1 mm of dermal invasion must be removed with a 1 cm margin, whereas melanomas with >1 mm of dermal invasion should be surged with a 2-3 cm margin. In cases where there is adverse localisation (paraurethral), a radical vulvectomy with a partial urethrectomy and colpectomy should be discussed [].\nSentinel lymph node biopsy (SLN) is also being recommended in selected patients who have early stage vulvar cancer to avoid the operative morbidity that is associated with inguinofemoral lymphadenectomy, such as wound complications or lower extremity oedema [, ]. Sentinel lymph node mapping was initially used to identify regional lymph node metastases in breast cancer and cutaneous melanoma and has now been evaluated in patients with early stage vulvar cancer, as well as malignant melanoma of the vulva with a tumour thickness <1 mm [–]. The sentinel lymph node can be detected using injected radio colloid 99mTc and isosulfan blue, which are preoperatively injected around the lesion. A hand-held gamma detection device is used to identify the sentinel lymph node(s) []. It is estimated that only 25–30% of patients with early stage vulvar cancer have lymph node metastases []. A patient with a positive sentinel node should undergo a full inguinofemoral lymphadenectomy followed by postoperative radiation therapy to the involved groin and pelvis. If the sentinel lymph nodes identified by mapping are histologically negative, no further treatment is indicated. The SLN may be used if (clinically and sonographically) there are no suspicious inguinal-femoral lymph nodes and if the tumour is not thicker than 1 mm (T1) []. Because of the rare incidence of malignant melanoma of the vulva, no SLN standards have been published; however, there are several studies that have shown the benefit of SLN, such as the smaller number of postoperative complications (e.g., lymphedema, deep venous thrombosis, and groin wound infections) [].\nPrimary radiotherapy or radiochemotherapy, which is sometimes used as the sole treatment, is recommended for cases of more advanced disease in which surgical resection (with the aim of an R0-situation) is not possible. If there are more than three positive inguinal lymph nodes or if the vulvar cancer is not completely removed (R1 or <1 cm margin without the possibility of follow-up resection), adjuvant radiotherapy is sometimes recommended. Chemotherapy has poor response rates and is ineffective in treating recurrent disease after surgical resection [, ]. Similar to cutaneous melanoma, adjuvant therapy with interferon-alpha should be discussed for malignant melanoma of the vulva >1.5 mm. Interferon-alpha supports the immune system in eradicating solitary tumour cells after surgery and is associated with a longer disease-free survival time [].\nThose patients with a previously treated metastatic melanoma also benefit from ipilimumab, a new antibody therapy. This fully human monoclonal antibody binds to CTLA-4 (cytotoxic T lymphocyte-associated antigen 4), a molecule on cytotoxic T lymphocytes that is believed to play a critical role in regulating natural immune responses []. Ipilimumab is designed to block the activity of CTLA-4, thereby sustaining an active immune response to cancer cells [].\nThe prognosis of patients with vulvar cancer is generally good when appropriate treatment is provided in a timely manner. The overall five-year survival rate () is 70% and correlates with the disease stage and lymph node status. The number of positive inguinal lymph nodes is the most important prognostic factor [].\nIn contrast to squamous cell carcinoma, there are limited reliable data concerning prognostic factors for nonsquamous cell vulvar malignancies. The thickness and depth of invasion of vulvar melanoma (as described by Breslow and Clark), as well as the regional lymph node metastasis, has been shown to correlate with the pattern of spread and prognosis of vulvar melanoma [, ]. Breslow's classification describes tumour thickness (mm), as measured by the distance between stratum granulosum and the deepest tumour cells [].\nThe depth of invasion classified by Clark describes tumour invasion in relation to the layer of skin (e.g., tumour restricted to the epidermis = level I; infiltrating the stratum papillare = level II) [].\nUlceration in melanomas presumably reflects very aggressive tumour growth that infiltrates and destroys the mucosal membrane. In general, vulvar melanoma carries a poor prognosis and has a tendency to recur locally as well as to develop distant metastases [].\nVulvar melanoma has a local recurrence rate between 30 and 51%. The preferred site of recurrence is the groin, followed by the perineum, the rectum, the vagina, the urethra, and the cervix. In patients who develop a recurrence, the disease-free period averages one year and ranges from one month to 14 years []. |
The first patient is a 70-year-old man, diagnosed with HIV in 2004 and started on stavudine, lamivudine, and nevirapine in 2007. In September 2009, his antiretroviral regimen was switched to lamivudine, zidovudine, and nevirapine as stavudine was phased out of the treatments available from the national program. His viral load in February 2018 was 1168 copies/ml on this regimen. In March 2013, he was diagnosed with an unprovoked DVT with HIV being his only notable risk factor and started on warfarin with the aim of achieving an INR of 2-3. A therapeutic INR of 2.7 was achieved in September 2014 at a warfarin dose of 63 mg per week (9 mg once daily). Two lower limb Doppler ultrasounds completed 6 months and 1 year after initiation of warfarin showed chronic DVT and led to the decision to place the patient on long-term anticoagulation. During subsequent clinic visits between 2014 and 2018, his INR remained within the therapeutic range of 2-3 at doses between 63 mg and 70 mg per week. By February 2018, his viral load was undetectable, and he was switched to lamivudine, tenofovir, and dolutegravir in December 2018 as part of the phasing out of old regimens. His viral load, as of June 2019, remained undetectable on the new regimen. During the next anticoagulation clinic visit, however, his INR was supratherapeutic at 6.5 without any report of bleeding. Four doses of warfarin were held, after which he continued on his normal dose. A week later, his INR was 5.5, and warfarin was subsequently held for a week. After holding warfarin, his INR dropped to 1.4, and warfarin was restarted at a dose of 56 mg per week, representing a 10% reduction in his previously stable weekly dose of 69 mg warfarin prior to starting dolutegravir. Over the next 2 weeks, his INR was 2.4 and 3.2, respectively, and he was asked to return in 1 month. After returning in 1 month, his INR had risen to 6.5 again and necessitated holding four doses of warfarin with the weekly dose being reduced to 35 mg per week. In the subsequent two weeks, his INR was 1.4 and 1.7, respectively. The weekly dose was increased to 42 mg, and a therapeutic INR of 2.3 was achieved. His INR remained therapeutic at 2.2 two weeks later, and his weekly dose of 42 mg was maintained. shows the changes in INR and warfarin dose that have been described above. |
A 72-year-old male presented to our institution for orthopedic consultation following a visit to his primary care physician with a complaint of approximately five weeks of progressive bilateral lower back pain without a history of trauma. The pain was described as sharp with movement and constantly dull at rest with a rating of 4/10 on the VAS (Visual Analog Scale) pain scale. On physical examination, there was tenderness over L1 and L2, but no deficits in lower extremity sensation, motor strength, or reflexes. Computed tomography (CT) of the lumbar spine without contrast was performed and mild compression fractures of L2 and L3 were identified, likely subacute and non-healed (Figure ). No soft tissue masses were noted. Abnormal marrow signal intensity was also seen compatible with an infiltrative marrow process such as MM. One week later, a follow-up magnetic resonance imaging (MRI) of the thoracic spine with and without contrast showed a 1.5 cm enhancing lesion in the right pedicle of T8, as well as a diffuse infiltrative process of the marrow. These results prompted a referral to hematology to confirm the diagnosis of MM.\nLab tests were performed by the hematology consultant to evaluate for the presence of MM. A serum protein electrophoresis showed gamma globulins reduced to 0.9% with an abnormal protein of 0.4 g%. The immunoglobulin studies showed a 0.34 g% monoclonal component of lambda light chains. Two samples of kappa and lambda light chains were elevated. One sample showed elevated lambda light chains to 335 mg/dL, giving a ratio of 0.6. The other sample showed elevated lambda light chains to 11,463 mg/dL. Chemistries were abnormal, with an elevated BUN (blood urea nitrogen) at 42 and creatinine at 1.98. Quantitative immunoglobulin studies were normal.\nDue to the concerning results of the blood work, a radiographic bone survey was performed. It revealed innumerable small lytic lesions scattered throughout the axial and appendicular skeleton, all likely secondary to MM. The patient was subsequently put on a combination of bortezomib (Velcade) and dexamethasone for treatment, which worked well, decreasing lambda light chains from 11,463 mg/dL to 49.16 mg/dL on repeat lab work approximately 10 weeks later.\nThe patient was admitted approximately three months after the myeloma diagnosis for extreme right hip pain and CT scan of the right hip, abdomen, and pelvis was performed. The study showed a right total hip arthroplasty and an osseous mass extending from the right acetabulum and right iliac crest. The mass had a “sunburst” pattern (Figure ). The mass was partially ossified with soft tissue components (Figure -).\nOver the next few weeks, in addition to persistent severe right hip pain, the patient developed progressive shortness of breath, cough, and fever. Multiple CT scans of the chest over several weeks showed progressively worsening infiltrates, effusions, and mid and lower lung nodules, with most nodules showing calcifications (Figures -). Metastatic OS from the right pelvis was suspected. Wedge resections of the left lower lobe and left upper lobe were obtained and histopathologic assessment was performed on the obtained tissue.\nThe resected tumors revealed identical histopathology. Figure demonstrates areas of hemorrhagic necrosis and sheets of viable tumor cells that display irregularly shaped, variably sized, hyperchromatic, and sometimes vesicular nuclei. Varying amounts of pale eosinophilic cytoplasm without brown granular pigment, mucinous vacuoles, or cytoplasmic keratinization are also visualized (Figure ). Architecturally, the tumor cells do not form glands, cysts, papillary fronds or any formed patterns. There are also curved or irregularly shaped deposits of eosinophilic extracellular matrix consistent with malignant osteoid (Figure ). Immunohistochemical stains showed no immunoreactivity to four keratin markers (AE1, Cam 5.2, 34betaE12, CK5/6), S100, SOX10, calretinin, Wilms tumor-1, carcinoembryonic antigen (CEA), thyroid transcription factor-1 (TTF-1), or MOC-31. These overall findings were diagnostic of metastatic osteogenic sarcoma. |
A 42-year-old man presented with a 3-months history of right neck pain that worsened on turning to the left. He did not complain of any other symptoms such as globus sensation or odynophagia. Physical examination was negative. Palpation of the tonsils did not worsen the pain. 3D-CT reconstructed images showed a longer left process than the right. Quantitative measurements showed the left styloid process was abnormally long (44 mm), while the right was normal (25 mm, Figure \n). In view of the neck pain, the patient was diagnosed with Eagle's syndrome. The condition was explained to the patient and operative treatment was recommended. Surgical correction was made via the transoral approach. On the first postoperative visit, rotation of the head did not elicit any pain.\nThe operation was performed under general anesthesia while the patient was in supine position with neck extension. An endoscope was inserted through the mouth, and the magnified surgical field allowed identification of the small vessels and nerves and allowed the surgeon to avoid injuries. Unilateral tonsillectomy was performed first, the tonsillar bed was palpated and the tip of the styloid process was identified. The mucosa was separated longitudinally at the point of palpation of the process in the tonsillar fossa. The tissue of the parapharyngeal space was carefully separated by q-tips to avoid vascular injury. Palpation was occasionally used during surgery to confirm the location of the styloid process. After exposure of the styloid process, the periosteum was incised at the tip of the process, then stripped from the tip to the base. The styloid process was excised with a bone nibbling rongeur. The styloid process must be penetrated before its removal. If the styloid process fractures accidentally before it is completely dissected, the fractured piece(s) can be pulled down through the tonsillar bed after diverging the muscles attached to it, a step that might reveal the broken piece(s). After resection of the process, the tonsillar bed was sutured with absorbable sutures to protect the parapharyngeal space from sputum at the pharynx. Oral feeding was possible the next day. Intravenous cefalotin (1 g) was injected just before the start of surgery. Follow-up examination showed uneventful recovery and no complications. |
A 45-year old man presented with sudden onset palpitations whilst exercising. There was no associated hemodynamic compromise. Electrocardiogram (ECG) showed monomorphic ventricular tachycardia (VT) with a right bundle branch block pattern and a superior axis ().\nHe gave a history of 3 prior episodes over the preceding 20 years and had seen an electrophysiologist previously. There was no history of syncope and no family history of sudden cardiac death. Resting ECG was within normal limits () and echocardiography demonstrated a structurally normal heart. At that time, a diagnosis of idiopathic fascicular VT was made and after discussion the patient elected not to undergo an electrophysiology study (EPS).\nOn this occasion he was treated in the Emergency Department with IV flecainide at the instruction of his treating electrophysiologist. This resulted in termination of tachycardia with restoration of sinus rhythm but unmasked a latent type 1 Brugada ECG pattern (), not seen on his resting ECG (). In view of his history of ventricular arrhythmia and a type I Brugada ECG pattern he was referred by the admitting team for consideration for an implantable defibrillator (ICD).\nHowever, following discussion with the electrophysiology service, a diagnostic EPS with a view to ablation of his idiopathic fascicular VT was recommended. Following administration of intravenous isoproterenol, monomorphic VT (CL 260ms) was easily induced on multiple occasions with programmed ventricular stimulation. This VT had an identical morphology to that seen on arrival at the Emergency Department. An externally irrigated ablation catheter (Cool Path Duo, St Jude Medical, St Paul, MN, USA) was advanced retrogradely to the left ventricle and a 3 dimensional (3D) left ventricular geometry and activation map was created using Ensite Velocity (St Jude Medical, St Paul, MN, USA) ().\nEarliest activation of the ventricle was seen at the insertion of the posterior fascicle with centrifugal activation of the rest of the chamber. The catheter was drawn back along the inferior septum to the region of the posterior fascicle until a discrete Purkinje potential could be identified preceding each ventricular electrogram (Figure 1D). Ablation was performed (maximum 40W) in the region of the posterior fascicle (). Following ablation no polymorphic or monomorphic VT or ventricular fibrillation was inducible with programmed stimulation and up to 3 extra beats either in the baseline state or with isoproterenol. |
A 37-year-old woman was noted to have pancytopenia on routine blood screening during the first trimester of pregnancy. Complete blood count demonstrated a platelet count of 85 × 109/L, white cell count of 2.3 × 109/L (of which neutrophils were 1.3 × 109/L and monocytes were 0.2 × 109/L), and hemoglobin 10.6 g/dL. She reported occasional easy bruising in the last two months with no other bleeding issues, lymphadenopathy, or splenomegaly. The patient had one previous uncomplicated pregnancy delivered thirteen months previously with normal platelet counts throughout and had been in good health prior to this pregnancy. She had a personal history of equivocal hypothyroidism, had a family history of hypothyroidism and systemic lupus erythematosus, was on no regular medications, and had not commenced any new medications in pregnancy. Thyroid function tests, immunoglobulins, and folate and vitamin B12 levels were within normal range with a negative antinuclear antibody test. As a low serum ferritin level was noted, she commenced on iron supplementation. The patient was given a presumptive diagnosis of immune thrombocytopenia in pregnancy. During pregnancy, fatigue was her only symptom and she had no bleeding complications with her platelet count remaining static. There were no clinical findings to suggest preeclampsia or HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Blood film examination was performed routinely throughout pregnancy with no morphological abnormalities observed. At 38-week gestation (platelets 61 × 109/L), she was commenced on prednisolone 20 mg oral daily, but the platelet count showed no response (platelets 65 × 109/L) prompting immunoglobulin therapy at 1 g/Kg of booking weight (70 g once-only dose), but this again had no effect on the platelet count (platelets 52 × 109/L). Soon after, she presented to a maternity hospital in spontaneous labor and had a normal vaginal delivery at full term with minor bleeding and no postpartum hemorrhage. The platelet count was 52 × 109/L at the time of delivery, and no therapy was given. Her male infant had a normal platelet count at birth.\nAt review five weeks postpartum, the patient had developed a pustular skin rash on her left upper arm, lower abdomen, and lower back. A skin biopsy demonstrated dermal neutrophils producing upper dermal edema and a subepidermal blister consistent with bullous Sweet's syndrome (acute febrile neutrophilic dermatosis) (). Her hematological indices displayed persistent cytopenias with a hemoglobin of 13.0 g/dL, a white cell count of 1.7 × 109/L (of which neutrophils were 0.5 × 109/L and monocytes were 0.1 × 109/L), and a platelet count of 49 × 109/L. Occasional hairy cells were noted for the first time on blood film examination. Immunophenotyping demonstrated a population of B lymphocytes that represented 5% of non-erythroid cells. These B cells were kappa-restricted and positive for CD10, CD11c, CD19, CD20, CD25, and CD103. The bone marrow aspirate was normocellular for age and showed 23% lymphocytes with 10% having hairy projections. Trephine biopsy demonstrated an infiltration of hairy cells with typical morphology () with immunohistochemistry of these hairy cells demonstrating positivity for CD10, CD20, CD25, CD72, and BCL2 (). The BRAF c.1799T > A; p.(Val600Glu) mutation was detected in genomic DNA from the bone marrow by a next-generation sequencing approach; all of which were consistent with a diagnosis of HCL.\nFollowing this diagnosis of HCL, the patient received a single five-day course of cladribine at a dose of 0.1 mg/kg/day by continuous intravenous infusion at nine weeks postpartum. This was complicated by an episode of febrile neutropenia and a diffuse erythematous rash secondary to allopurinol and co-trimoxazole which resolved following cessation of these medications. At the last follow-up seven months after cladribine treatment, the patient is well with hemoglobin of 13.1 g/dL, a white cell count of 4.2 × 109/L (of which neutrophils were 2.9 × 109/L and monocytes were 0.3 × 109/L), and platelet count of 207 × 109/L with resolution of Sweet's syndrome. At this center, in such a case of favorable dermatological and hematological response, repeat bone marrow analysis to assess immunophenotypic or molecular residual disease is not routinely performed unless clinically indicated. |
After due patient consent and approval from the institutional ethical committee, we present the following report:\nA 63-year-old old woman, menopausal since 20 years presented at the Gynecology OPD at All India Institute of Medical Sciences, Raipur, with a chief complaint of discharge per vaginum since 6 months. Per speculum examination revealed a cervix flushed with vagina and friable tissue at the external os. There was a 2.5 cm × 2 cm firm mass in the posterior fornix confirmed on per rectal examination. Bilateral parametria and rectal mucosa were free from tumor. Pap smear and biopsy revealed a squamous cell carcinoma. Ultrasound reported a uterus of size 6.8 × 3.2 cm. The endometrial cavity was distended with echogenic content and minimal fluid. Both ovaries were normal suggesting a diagnosis of “asymmetrical hetero echoic thickening of the endometrium with collection in the endometrial cavity, suspected carcinoma endometrium.” Magnetic resonance imaging (MRI) abdomen and pelvis [] revealed a fluid-filled endometrial cavity, an irregular, circumferentially thick, intermediate to high signal intensity endometrium surrounded by thin hypointense rim seen in the fundus, body, and lower uterine segment. The endo-myometrial junction was mildly obscured, and the visualized myometrium was normal in signal intensity. The lesion was seen extending into the cervical canal reaching just proximal to the external os, and the internal os was seen mildly widened by the lesion. A small nodule was noted in the stroma of the anterior cervical wall with signal characteristics similar to the endometrial lesion, and it was suggested to represent local spread. The uterine serosa, parametrium, mesorectal fascia, urinary bladder, and lymph nodes were not involved. Bilateral adnexa was reported normal.\nThere was a “diagnostic dilemma” given findings of cervical malignancy on biopsy but predominant endometrial lesion on imaging studies.\nA Type III Radical Hysterectomy with bilateral pelvic lymphadenectomy was performed keeping a clinical diagnosis of carcinoma cervix stage 1B2 (revised FIGO staging). On the gross cut section, the entire endometrium was filled with friable tissue admixed with gelatinous mucoid material. A small friable mass was seen in the endocervical canal []. Histopathology revealed a squamous cell carcinoma, nonkeratinizing, moderately differentiated, grade 2, depth of invasion 16 mm, closest resected margins uninvolved, all lymph nodes were negative, parametrium and paracervical tissue uninvolved, and the tumor had spread superficially to the endometrium. Bilaterally, the tubes and ovaries were uninvolved. Human papillomavirus (HPV) DNA was positive for serotype 16. At follow-up, the patient was alive at 6 months after surgery. |
The patient was a 39-year-old Japanese man with a history of treatment at the age of 20 years for Hodgkin's lymphoma that underwent complete remission with no recurrence. From February 2013, he developed a dry cough and sputum production. Afterwards, he occasionally had bloody sputum and dyspnea and was referred to our hospital in June 2013. A chest X-ray on admission showed left pneumothorax (). He underwent drainage of the pleural space, but lung expansion was poor, and the air leakage was not improved. On the 11th hospital day, a contrast-enhanced computed tomography (CT) scan revealed intrapulmonary infiltration in the mediastinal side of the left upper lobe (). There was no swelling of the hilar or mediastinal lymph nodes, and no emphysematous changes were seen. Therefore, he underwent surgery on the 13th hospital day. Although we were not able to confirm the site of the air leakage clearly, we recognized a high amount of sphacelus tissue adhering to neighboring tissue in the left upper lobe (S1+2) and lower lobe (S6). We therefore considered these lesions to be the cause of the air leakage, and we resected the left upper lobe and S6. Histological findings of these lesions showed prominent proliferation of elements of malignant giant cells and spindle cells with small foci of adenocarcinoma, and a pathological diagnosis of primary pulmonary pleomorphic carcinoma was made, with the margin described as positive at the edge of the bronchus (). After the operation, the patient received chemotherapy (1st line: carboplatin and paclitaxel, 2nd line: docetaxel, and 3rd line: pemetrexed). However, he repeatedly complained of bloody sputum, and chest CT revealed multiple cystic metastases in the lung. Moreover, during chemotherapy, pneumothorax occurred three times after the direct rupture into the pleural cavity of developing cystic lesions in the pulmonary metastatic tissue (). His condition deteriorated and he died 10 months after surgery. |
A 28-year-old, active-duty military male with relapsed, refractory, metastatic, malignant, giant-cell-rich, primary bone sarcoma presented to the Emergency Department (ED) by cardiology instruction for evaluation of a large pericardial effusion with possible early tamponade physiology that was identified on an outpatient transthoracic echocardiogram (TTE). The echocardiogram was performed to further evaluate findings from a positron emission tomography (PET) scan completed three days prior that demonstrated findings concerning for cardiac metastasis to the interventricular septum with extension into the right ventricular outflow tract, along with an interval increase in the size of a pericardial effusion when compared to a PET scan from five months prior (Figure ). At the time of initial evaluation in the ED, the patient was asymptomatic from a cardiopulmonary standpoint, specifically denying chest pain or dyspnea, though endorsing chronic superficial abdominal tenderness in multiple abdominal regions. Vital signs were normal and physical examination was significant only for mild epigastric and right upper quadrant tenderness. An ECG was obtained that demonstrated sinus rhythm, low voltage, and anteroseptal myocardial injury (Figure ).\nIn the setting of ST-segment elevation, additional concern was raised by the discovery of hypokinesis of the septal myocardium on bedside echocardiogram, though it should be noted that this area of myocardium was being impinged upon by a 47 mm × 23 mm mass in the interventricular septum (Figure ).\nHowever, given the lack of cardiopulmonary symptoms, the patient was treated conservatively with serial ECGs and cardiac enzymes. Initial troponin was 0.14 ng/mL (99th percentile for assay being 0.03 ng/mL) with stable values on repeat assays performed at six-hour intervals. The patient was then admitted to the Coronary Care Unit and monitored closely for signs of cardiac tamponade (i.e., pulsus paradoxus). He subsequently required pericardiocentesis for a large pericardial effusion concerning for tamponade physiology based on mitral inflow velocities. Based on cytology with negative infectious labs, the effusion was deemed secondary to his malignancy. Following a seven-day hospitalization and extensive multidisciplinary discussions with the patient and his family regarding the prognosis, the patient elected to forgo any further cardiac imaging and was discharged home with hospice, where he died shortly after discharge. |
A previously healthy 48-year-old woman living in rural Maryland presented in early June with a two-week history of neck pain, fatigue, anorexia, nausea, and intermittent fevers and chills. She was concerned that she might have Lyme disease because of tick bites in the past and ongoing recreational exposure to ticks but did not recall a tick bite in the weeks preceding the onset of her illness. A tentative diagnosis of a viral illness, possibly mononucleosis, was made during an initial visit to her primary care physician. She presented to the emergency room the following day with worsening neck pain, stiffness, and myalgia and was admitted for dehydration and further evaluation. Physical exam showed a temperature of 101.1°F, pulse 101 beats per minute, and a new oval nonpainful red rash on her left hip which was attributed to a drug reaction (). Laboratory evaluation of complete blood count (CBC) showed a total white blood cell (WBC) count of 4.9·103 U/L with 8% lymphocytes and a low absolute lymphocyte count of 400 U/L. A comprehensive metabolic panel (CMP) showed an abnormal AST of 245 U/L, ALT of 218 U/L, and alkaline phosphatase of 215 U/L. A CT scan of the head without contrast was negative. A lumbar puncture showed one white blood cell, normal protein, a normal glucose level, negative bacterial culture, and negative Lyme antibodies in the CSF. Her blood cultures, hepatitis serology, and influenza A and B were also negative. The Epstein-Barr virus serology was interpreted as consistent with either late primary infection or reactivation of mononucleosis with a (+) IgG antibody, (−) IgM, and (+) EBNA. She was discharged with a diagnosis of mononucleosis due to EBV and recommendations for symptomatic care. A Lyme serology was sent with pending results at the time of discharge that subsequently showed no ELISA done, (+) IgM Western blot, and (−) IgG Western blot.\nOver the next 4 weeks, she had waxing and waning symptoms of fever, fatigue, arthralgia, and anorexia. She developed new complaints of difficulty finding words and trouble concentrating, especially with reading. The original skin lesion on her hip enlarged, and new skin lesions appeared on her left lower leg and stomach. She was treated with Keflex for a presumed bacterial cellulitis. The rash did not respond to the antibiotics but eventually resolved over a period of weeks.\nOver the next week, she developed increasing exertional dyspnea when climbing stairs, anxiety, and chest heaviness. The patient also noted an irregular heart beat and that, on one occasion, her pulse rate was 31 bpm. She felt like she was going to pass out and returned to the emergency department 6 weeks after her initial visit for further evaluation. There, she was noted to be bradycardic with a pulse rate in the 40's. The admitting EKG showed sinus tachycardia with new onset second-degree AV block with 2 : 1 conduction (). The chest X-ray was normal, and a transthoracic echocardiogram did not show any valvular or structural abnormalities, with an ejection fraction of sixty percent. Her repeat Lyme ELISA was positive, and WB came back with all three IgM bands positive, and IgG was positive for eight out of ten bands, confirming the diagnosis of Lyme disease with carditis. The AST and ALT were normal at 29 U/L and 44 U/L, respectively. Following treatment with three doses of ceftriaxone, the AV block resolved while in the hospital, and she was discharged on a 3-week course of doxycycline.\nAfter treatment with 21 days of doxycycline, the patient had resolution of her shortness of breath and palpitations and had gradual improvement in her symptoms of fever, headache, and anorexia. She remained fatigued and had increasing problems with insomnia. There was also ongoing trouble with word finding, focusing, and concentrating. A stress test showed above-average exercise capacity with no arrhythmia. She developed new symptoms of bursitis in hips, coldness in hands, shooting pains in arms, and numbness in hands and feet. She stated that she “feels like a 90-year-old woman” with hip and knee pain and remained out of work from July to September. Ten weeks after completion of her doxycycline, an infectious disease consultation was obtained for evaluation of her ongoing fatigue, hip pain, paresthesias, and difficulty with concentration. The consult felt as though the original EBV serologies were consistent with remote exposure and not an acute infection. Additionally, the patient's ongoing symptoms following successful treatment of her Lyme carditis were due to slowly resolving symptoms from her previously treated Lyme disease. A repeat convalescent Lyme serology performed by Quest diagnostics was negative, with a positive ELISA and IgM western blot, but a negative IgG western blot with four bands. The CBC, CMP, sedimentation rate, and C-reactive protein were normal.\nSeven months after completion of the antibiotic treatment for her Lyme carditis, the patient sought a second opinion for her continued symptoms and resulting substantial reduction in her ability to function at work and home. The patient had no evidence of other tick-borne infection or other medical causes of her fatigue and musculoskeletal pain. Based on the Infectious Diseases Society of America case definition, she was diagnosed with posttreatment Lyme disease syndrome. Eighteen months after resolution of her Lyme carditis, she is working full time and continues to report her pain and fatigue levels as a 4/10, which she rates as mildly disrupting her work, social, and family life. |
A 66-year-old female patient with a history of bilateral lower limb lymphedema reported the aggravation of the condition over the years, reaching stage III (elephantiasis). The patient was sent to the Godoy Clinic and reported having undergone several treatments throughout her life as well as several episodes of erysipelas. She did not marry due to the lymphedema and complained of the frequent occurrence of strangers staring at her leg, which upset her. The physical examination confirmed elephantiasis. The circumference of the left lower limb was 106 cm. Her body weight was 106 kilograms, height was 160 cm, and the body mass index (BMI) was 41.6 kg/m2 ().\nThe patient was submitted to intensive treatment for three weeks, which led to a 21 kg reduction in weight and 66 cm reduction in leg circumference ().\nIntensive treatment with the Godoy Method consisted of eight hours per day of mechanical lymphatic drainage, 15 minutes of simultaneous cervical lymphatic therapy, and hand-crafted compression stockings made from grosgrain fabric. Mechanical lymphatic therapy consisted of an electromechanical device that performs plantar flexion and extension. After three weeks of intensive therapy, the patient continued treatment at home using the compression stockings. At the follow-up evaluation, the patient was submitted to electrical bioimpedance analysis as well as circumference measurements and volumetry. The bioimpedance analysis revealed a pattern of normality, with the reduction in lymphedema. Ten years after treatment, the patient has maintained the results with the compression stockings. In occupational therapy throughout this period, the patient has been encouraged to perform activities that she has always wanted to do to improve her wellbeing. She took up belly dancing, followed by tap dancing. She reports that these activities changed her life and she is very happy for being able to realize her dream of dancing, which is an activity that she began at the age of 76 years. The study was approving Ethical Committee of Medicine School of Sao Jose do Rio Preto# 2.929.115. |
The patient is a 33-year-old African American male with a past medical history of hypertension who was brought to the emergency department for the evaluation of inability to speak. As per family, symptoms started two days before the presentation and progressed over time. He is noncompliant with his blood pressure medications. Blood pressure on arrival was 189/137. Physical examination showed a muscle strength of 5/5 in all four extremities, intact sensations, and normal deep tendon reflexes. There was no appreciable nystagmus. Speech evaluation revealed significant anomia, word-finding difficulty, nonfluency in speech, appropriate comprehension, and slightly reduced attention, requiring occasional repetition of prompts. Obvious limitation of expressive language skills was noted. The patient was only able to reply to questions few hours after presentation with "YES" or "NO." At this point, he denied any recent episodes of headache, nausea, balance problems, falls, or weakness. Initial computed tomography (CT) scan of the head and CT angiography of the head and neck were unremarkable. While waiting for the MRI of the brain, patient was admitted in intensive care unit for neurological examination every hour and management of possible hypertensive emergency/cerebrovascular accident (CVA) for which he was started on nicardipine drip for targeted blood pressure goal, together with aspirin and atorvastatin. He was not a candidate of fibrinolytic therapy (tissue plasminogen activator).\nMRI of the brain with contrast showed patchy areas of T2 hyperintense signal within the central aspect of pons and throughout the supratentorial white matter in a periventricular and subcortical distribution (Figures , ). There was also a mild T2 hyperintense signal along the undersurface of the corpus callosum.\nMRI spine was negative for any demyelinating plaques. The findings were discussed with the patient and his family and the past history of any neurologic problems was asked. The patient’s mother reported that the patient has been following up with an ophthalmologist for “blurry vision from the left eye” that started in 2019 and has improved significantly since then. He uses contact lenses. A provisional diagnosis of MS was made based on revised McDonald’s criteria (two clinical attacks and clinical evidence of lesions), so we deferred lumbar puncture at this time. The patient was started on methylprednisolone 1 g intravenous infusion daily for five days. Physical, occupational, and speech therapies were initiated. The symptoms improved significantly over the course of four days. He was able to articulate without difficulty and word-finding pauses disappeared over time and he was discharged home on day 5 of admission to the hospital. Follow-up care was established at St. Barnabas Medical Center Immunotherapy Clinic. |
A 71-year-old male with diabetes, hypertension, and hyperlipidemia underwent coronary bypass surgery (CABG) for 3-vessel coronary artery disease in 2005. He presented 11 years later with chest pain, weight loss, night sweats, and vision changes. He had recently completed treatment for locally advanced prostate cancer with prostatectomy in addition to hormone and radiation therapy. Computed tomography angiography (CTA) of his chest was performed to evaluate for pulmonary embolism and instead revealed an ascending aortic pseudoaneurysm measuring up to 4 cm in diameter (). One year prior, he had undergone a cardiac catheterization at another facility, and no pseudoaneurysm was appreciated at that time.\nThe patient had no leukocytosis or bandemia on presentation nor during his hospitalization, but blood cultures and inflammatory markers were ordered to evaluate for potential infectious etiology. Blood cultures were negative. C-reactive protein was elevated at 4.3 mg/dL. His diabetes was well controlled; HbA1c was 6.5% on admission.\nDuring his workup, both transthoracic and transesophageal echocardiograms were utilized to evaluate the pseudoaneurysm and baseline cardiac function. His left ventricular ejection fraction was 55%. Aneurysmal dimensions measured by echocardiogram were 4.2 × 6.9 cm with extension into the aortic root. Foreign material was floating within the lumen, concerning for an infected pseudoaneurysm. No valvular vegetations were seen. The aortic valve had moderate to severe insufficiency.\nSurgical intervention was planned for both the aortic pseudoaneurysm as well as the occluded bypass grafts that had been identified on the recent cardiac catheterization. He was taken to the operating room and the right axillary artery and right femoral vein were cannulated for cardiopulmonary bypass prior to redo-sternotomy. Aorta was cross-clamped distal to pseudoaneurysm and cardioplegia was administered.\nContrary to preoperative imaging, the ascending aorta actually had 3 separate pseudoaneurysms. One pseudoaneurysm originated from the aortic root; it had eroded and destroyed the noncoronary aortic valve commissure. The other 2 pseudoaneurysms involved the proximal anastomoses of the coronary bypass grafts. These were both multilobulated and contained several vegetations. Tissue culture was sent from these vegetations, aortic valve leaflets, and the pseudoaneurysm itself. All specimens demonstrated invasive fungal infection with Aspergillus fumigatus ().\nThe ascending aorta and aortic root was resected. A 24-mm aortic homograft was selected for aortic root replacement. Due to severe inflammation, the left coronary button was unable to be mobilized and instead was reimplanted using the inclusion technique. The right coronary button was 100% occluded and oversewn. Proximal anastomosis for posterior descending coronary artery and obtuse marginal bypass grafts were implanted in the ascending aortic graft. Mediastinal drains were placed. Axillary and femoral cannulas were removed. Cardiopulmonary bypass time was 320 minutes, and aortic cross-clamp time was 247 minutes.\nThe morning of surgery, the patient complained of new-onset headaches. Shortly after the procedure, he developed confusion and seizures. CT head without contrast identified a new infarct in the right temporoparietal region. Neurology and infectious disease specialists were consulted due to concern for mycotic embolism. Broad-spectrum antimicrobial therapy was initiated and eventually narrowed to voriconazole after finalized tissue culture results confirmed Aspergillus fumigatus infection. The patient’s confusion, short-term memory difficulty, and dysphagia all improved prior to discharge, and he had no long-term neurologic deficits.\nThe patient was discharged on postoperative day 8 to a long-term care facility and eventually returned home. Per infectious disease recommendations, he was treated with long-term voriconazole. Follow-up CTA chest was performed at 1 and 6 months and 1 year postoperatively; no recurrence of pseudoaneurysm was seen (). One year after aortic root replacement, he has remained free of recurrent infection and is maintained on voriconazole. The interventional cardiologist who performed the cardiac catheterization prior to the onset of the patient’s symptoms was notified of our findings of a possible procedure-related infection. |
A 37-year old female was referred to gastroenterology department for an incidentally detected gastric mass. The reason for her hospitalization was per vaginal bleeding and lower abdominal discomfort since last two weeks. She was asymptomatic for gastric lesion and physical examination of the abdomen revealed no signs of tenderness or of a palpable mass. Her medical history was non-significant. She had history of previous two caesarean sections and umbilical hernia.\nRoutine blood investigations were unremarkable. She had undergone contrast-enhanced CT of abdomen and found to have complex bilateral ovarian cysts with focal nodular mildly enhancing mass lesion arising from fundus and body of stomach about 3.8 cm, indenting the left lobe of liver without obvious infiltration. Upper gastrointestinal scopy showed a large submucosal mass of approximately 4-5 cm in the fundus and body of stomach along greater curvature with ulcer at the summit of mass (). Biopsy specimens obtained at the endoscopy yielded nonspecific signs of mild inactive chronic inflammation and gastric ulcer without evidence of a dysplasia or malignancy. Patient underwent CT guided biopsy of gastric mass at our centre. Biopsy specimen showed spindle cell neoplasm. Patient was counseled for surgical options considering GIST as a provisional diagnosis and offered an elective laparotomy. After an informed consent, patient had undergone exploratory laparotomy for total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy and 5x5 cm gastric tumor resection and umbilical hernia repair.\nMacroscopically, the nodular gastric mass covered by mucosa measured 5×5×4 cm with cut surface shows firm lobulated yellowish white tumor abutting the serosa (). Microscopically, submucosal tumor composed of spindle cells arranged in interlacing fascicles separated by myxoid or hyalinised stroma. Neoplastic cells contain ill-defined eosinophilic cytoplasm and slender elongated often wavy, bland appearing nuclei (). The neoplastic cells were immunoreactive with S-100 protein and vimentin (), but lacked immunoreactivity with CD-117, DOG-1, CD-34, smooth-muscle actin and desmin. The histopathologic features and immunohistochemical staining pattern were consistent with gastric schwannoma. Bilateral adnexae had showed serous borderline cystic tumor (low malignant potential) with micropapillary pattern.\nThe postoperative period was uneventful and one-month follow-up was unremarkable. |
Patient is a 73 year-old right hand dominant female who initially presented to the office complaining of atraumatic right shoulder pain with activity and limited range of motion of longstanding duration. On physical exam, she was found to have significantly limited active range of motion of the right shoulder and clinical signs of impingement. Radiographs at that time demonstrated superior escape of the humeral head with impingement of the greater tuberosity on the acromion and early acetabularization of the acromion (Fig. ). MRI findings were consistent with her x-ray and also demonstrated a lack of contiguous supraspinatus or infraspinatus tendon. At this juncture, the patient was diagnosed with rotator cuff arthropathy and elected to proceed with reverse total shoulder arthroplasty.\nThe patient was brought to the operating room and placed in the beach chair position. An incision was made from just lateral to the coracoid to the medial border of the proximal humeral shaft in line with the axillary fold. Subcutaneous tissue was dissected and the cephalic vein was identified. As the cephalic vein was mobilized and the clavipectoral fascia was incised, a discrete, branching, fascicular nerve was identified lateral and deep to the cephalic vein within the deltopectoral groove (Fig. ). The nerve was further dissected and traced both proximally and distally. Distally, the nerve and all branches were found to be diving into the anterior deltoid muscle. Proximally, it was found to run deep to the conjoined tendon, towards the brachial plexus. The nerve was freed from the deltoid muscle belly, allowing enough excursion to access the glenohumeral joint via a small deltoid window. The remainder of the operation concluded without complication and the wound was closed primarily (Fig. ). The patient was neurovascularly intact post-operatively with intact sensation in the axillary nerve distribution and able to fire her deltoid muscle. She healed without complications. At 4-month follow-up, she was doing well and able to actively abduct and forward flex her right shoulder to approximately 120 degrees (Fig. ). |
A 40-year-old Hispanic man with a past medical history of human immunodeficiency virus (HIV) was brought to the emergency department complaining of right upper extremity (RUE) weakness and numbness for four days with associated bitemporal headache and generalized fatigue. The patient reported first time use of intranasal cocaine and heroin, after which he lost consciousness and woke up approximately four hours later with new onset RUE and headache. His cluster of differentiation 4 (CD-4) count was reported above 500 cells/mm3 and viral load (VL) was undetectable. The patient did not have any known CNS complications in the past.\nOn physical examination, his blood pressure was 151/97 mm Hg and pulse was 82 and regular. He was alert and cooperative. His cranial nerves were intact. His motor exam, however, was abnormal in the RUE with 3/5 arm strength and wrist drop; the strength and tone of the other extremities were normal throughout. Deep tendon reflexes were normal bilaterally, but his gait could not be evaluated. His sensory function decreased to pin sensation at the RUE and normal sensation was noted in the rest of the extremities and face. Laboratory testing was normal except for an elevated creatinine of 6.9 mg/dl, creatine phosphokinase (CPK) of 7855 IU/l, alanine transaminase (ALT) of 139 IU/l, and aspartate transaminase (AST) of 109 IU/l. Urine toxicology was positive for metabolites of cocaine and heroin. Magnetic resonance imaging (MRI) of the brain was done and it revealed two areas of increased T2/FLAIR signal within the medial aspect of both basal ganglia, measuring 16 mm in the right and 12 mm on the left involving each globus pallidus and the genu of the internal capsule, as can be seen in Figures -. His chest radiography was normal, computerized tomography (CT) of the brain, as can be seen in Figure , and cervical spine were normal. His electrocardiogram was normal.\nIn the subsequent days, his kidney function and rhabdomyolysis improved. The patient remained fully awake, alert and oriented, but the weakness of his RUE persisted. The patient decided to leave against medical advice despite full explanation of the risk of leaving.\nThe patient was contacted over the phone and he informed us that he followed up with his primary care physician and reported improvement of the weakness. He received physical therapy and was independent in all activities of daily living and functional mobility. His only limitation was a moderate decrease in fine motor coordination of the RUE. |
A 23-year-old Hindu female, married for 5 years, living with her husband, a 4-year-old son, and a 20-weeks-old daughter, and in-laws in a joint family, belonging to the middle socioeconomic strata, came to psychiatry OPD with complaints of having thoughts which were extremely distressing to her. The thoughts were of her performing sexual acts with Lord Hanuman and also of her children sharing incestuous relations. The thoughts first began 2–3 days after delivery of her daughter, 5 months ago. They would initially occur only when she would happen to view her newborn daughter's genitals, whereas bathing her or cleaning her, when she would happen to see Lord Hanuman's image on her daughter's genitals. This persisted for around a month. After that, she began having thoughts of her son having sexual relations with her daughter. This would earlier occur only when her two children were sitting or playing together but eventually began occupying her thoughts throughout the day. Alongside, the thoughts of her performing sexual acts with gods also began increasing. The thoughts were also associated with vivid visualization of the act as if occurring in front of her eyes and continuing to be seen by her even when she would shut her eyes in an attempt to stop the images. She would try her best to suppress such thoughts by diverting her mind to other thoughts or by keeping herself occupied with work, which, albeit effective in the beginning, was not helping her. These thoughts grossly violated her moral and religious ethics and were causing her immense distress. She took cognizance that the thoughts were her own, and that they were unreal and impossible. She would feel apprehensive with palpitations, tightness in chest, and restlessness. She also began reporting no interest in the world around her and her daily activities. She completely stopped breastfeeding her daughter, and vehemently refused to have any sexual relations with her husband. She would have multiple episodes of inconsolable crying in the day. She no longer felt she deserved to live. She was barely eating and not able to sleep for more than 10–15 min a day. Desperate for a remedy, she came to her sister-in-law in Pune, as her in-laws were not very understanding of her illness, and came to psychiatry OPD with her sister-in-law.\nThe patient had similar symptoms 1-month after the delivery of her first child, 4 years ago. After her son became a month old, her parents took her to a temple for a religious ritual. Over there, as she was praying in front of the idol of a Goddess, she first experienced an intrusive, distressing, unwanted thought, defying her principles: of the goddess sharing a sexual relationship with the priest in the temple. She got anxious at that thought and left the temple immediately. However, the thoughts kept recurring whenever she saw an idol of a god or a goddess, imagining herself having sexual relations with the gods, and her husband having sexual relations with the goddesses. The thoughts kept worsening, and she was unable to stop them despite trying to. They persisted for a year, but resolved spontaneously upon the death of her husband's paternal grandmother, due to being extremely caught up in the rituals and rites for 17 days following her death. After that, they never recurred or distressed her, until the current episode. Premorbidly, she held a firm belief in religion and was a very dedicated devotee of Lord Hanuman. She would pray for 30 min every morning. She would regularly observe fasts and would spearhead all the religious functions at home with great vigor. She would often narrate stories from the Ramayana and Mahabharata to her children, and would regularly read religious scriptures at home. Mental status examination revealed a young lady hiding her face with her saree. Conveying mood as self-contemptuous, having a reactive, and dysphoric affect. Preoccupying thoughts of obsessive nature were present, not associated with any compulsion. The patient had an intellectual insight. Y-BOCS score was 31, corresponding to moderate-severe symptoms. The patient was started on capsule fluoxetine 40 mg OD and tablet clonazepam 0.5 mg and was advised to review back after 5 days, to which she did not comply and was eventually lost to follow-up. |
A 28-year-old woman initially presented with blurry vision that developed over the span of approximately one month. The blurry vision was initially most prevalent on horizontal gaze but progressed to include vertical gaze. It resolved with closure of one eye. She reported a history of gradually worsening headache over the past several years. Her headaches both worsened in intensity and increased in frequency, until it was quite debilitating and occurred daily. She described the headache as an intense pressure in both the front and back of her head. She also noted a “whooshing” sound in her right ear. She denied any nausea or vomiting and had not had any syncope, numbness, weakness, facial droop or slurred speech. Furthermore, she had no history of bladder or bowel dysfunction.\nHer medical history was pertinent only for obesity with a body mass index (BMI) of 39. On physical exam she was noted to have papilledema. Her neurological exam was unrevealing with the exception of a subtle sixth cranial palsy.\nA magnetic resonance image (MRI) was obtained which showed a T1 hypointense and T2 hyperintense cystic lesion arising from the pineal gland measuring 2.0 x 1.1 cm in the sagittal plane with mild mass effect on the tectum and partial effacement of the cerebral aqueduct (Figures , ). The lesion demonstrated a thin rind of contrast enhancement and had thin enhancing internal septations. The lateral ventricles were mildly enlarged. There was no restricted diffusion and no loss of gray white differentiation. Cine flow study noted cerebral spinal fluid (CSF) flow through the cerebral aqueduct. Based on the radiographic images, the most likely diagnosis was an atypical pineal cyst.\nGiven the rapidity of the vision changes, the decision was made to pursue surgical intervention. An endoscopic third ventriculostomy (ETV) with pineal cyst fenestration was performed without complication. A computed tomography (CT) scan obtained post-operatively noted questionable decompression of the lateral ventricles but the patient reported no improvement in symptoms (Figure ). Ophthalmologic evaluation noted worsened papilledema. At this time the patient underwent a lumbar puncture, which noted an opening pressure of 32 cm H2O. Subsequent catheter venography noted severe stenosis of the right transverse sinus associated with a 9 mm Hg trans-stenosis gradient (Figure ). Placement of a venous sinus stent obliterated the pressure gradient (Figure ).\nAt six-month follow-up, her blurred vision, headaches and papilledema had all resolved. She reported complete resolution of her symptoms and plans were made for continued annual follow-up to monitor symptoms and ensure patency of the stent. |
A 27-year-old female patient presented with spontaneous and continuous pain in tooth #46. Clinically, there was an unsatisfactory glass ionomer restoration, which was radiographically assessed to extend deep pulpally in tooth #46, with a normal periodontal pocket and no missing teeth. Clinical tests indicated negative palpation and percussion tests, and a positive vitality test with slow pain decline in tooth #46, indicating a diagnosis of irreversible pulpitis for which endodontic treatment was necessary. Considering the presence of canine guidance and absence of parafunction (), endodontic treatment followed by permanent indirect restoration with the aid of CAD/CAM in a single clinical session was planned. The patient's consent was obtained.\nTo commence the treatment, the antagonist and maximum intercuspation hemi-arch intraoral scans were obtained and analyzed to determine the intermaxillary relationships and for shade and ceramic block selection (). Subsequently, the protocol followed was as described in for case 1. Initially, inferior alveolar nerve block was performed, followed by rubber dam isolation, surgical access, root canal localization, cervical preparation, pulp removal, and irrigation until cessation of bleeding (). Removal of the previous restoration and preparation of the cavity with capping of the mesiobuccal, mesiolingual, and distolingual cusps to receive an onlay restoration followed. As in case 1, an interim layer of 3 mm of resin was inserted over the root canal orifices and pulp chamber to enable the preparation to be scanned without undercuts, allowing fabrication of the permanent ceramic onlay during the root canal biomechanical preparation and obturation procedures. Subsequently, scanning (), design, and milling of the onlay were performed. The provisional resin was removed and the endodontic treatment was performed (), followed by placement of a 3-mm resin layer over the root canal orifices and pulp chamber. The restoration fit, seating, marginal adaptation, and proximal contacts were assessed clinically, and without removing the isolation, the restoration was cemented (). After removal of the isolation, occlusal contact was verified, followed by finishing and polishing of the margins and final radiography ( and ). At 2 years of follow-up, the restoration was esthetically and functionally satisfactory, without complications ( and ). |
A 64-year-old man was referred to our hospital and presented with dysuria for 2 months. Since the onset of the disease, there had been no incidences of low fever or night sweat and no significant changes in weight. Nevertheless, he complained about a decline in physical strength since the onset of the disease. He had a history of hypertension and diabetes for more than 20 years and had a cerebral infarction 8 years ago. On physical examination, a grade 3 enlarged prostate was found by digital rectal examination, with a hard texture, rough surface, and poor mobility. On laboratory examination, all other routine blood indexes were within the normal limits. The prostate-specific antigen (PSA) value was 0.09 ng/mL, which was in the normal range (<4.0 ng/mL), and other tumor markers, including carcinoembryonic antigen and glucosaminidase, were also within the normal limits. Urinalysis showed that all indicators were in the normal range. Based on the details above, we performed transabdominal contrast-enhanced ultrasonography and transrectal prostate biopsy.\nTransrectal ultrasound showed that the appearance of the prostate was enlarged, the size was 91 mm × 63 mm × 90 mm, the outline was irregular, the internal echo was not uniform, and a necrotic liquefying area in the center was seen, with a range of approximately 78 mm × 48 mm; furthermore, color Doppler flow imaging (CDFI) revealed rich blood supply signals from the surrounding soft tissue (Fig. A), RI: 0.89. CEUS of the abdomen showed that the peritumoral parenchyma began to enhance rapidly in 12 seconds (Fig. B), the large blood vessels in the necrotic liquefying area of the tumor center enhanced synchronously with the surrounding tissues, and the enhancement was most significant at 21 seconds (Fig. C). The necrotic area shown by contrast-enhanced ultrasonography was much larger than that by two-dimensional ultrasonography. According to the enhanced area as shown by CEUS, a biopsy was performed (Fig. D), and the tissue strip was sent to pathology. The patient was pathologically proven to have prostatic stromal sarcoma.\nThe pathological and immunohistochemical results showed that the patient had stromal sarcoma of the prostate. He underwent radical prostatectomy because of a lack of obvious metastasis, which was supported by PET/CT. After the operation, he had a catheter and abdominal drainage tube installed. One month later, the amount of drainage fluid increased. Urinalysis showed that the white blood cell, red blood cell, epithelial cell, and bacterial counts in the urine had increased. After nutritional support and symptomatic treatment, the patient's condition improved, and chemotherapy combined with local radiotherapy was gradually administered. However, the patient died 1.5 years after surgery. |
A 4-year-old girl was referred to the Pediatric Dentistry Department of Tehran University of Medical Sciences with the chief complaint of upper anterior teeth missing and esthetics problem.\nHer parents stated that her teeth have never erupted and when their daughter was 8–9-months-old she fell from the stairs and after that her upper lip was swollen.\nIQ of the patient was normal and the family and medical histories were not remarkable.\nIn the intraoral examination, the child’s occlusion was mesial step and there were some carious teeth in the oral cavity.\nThe maxillary incisors were absent and gingival tissues in this region had a normal color and seemed bulgy without any pain in palpation ().\nRadiographic examinations showed inverted incisors with open apexes and incomplete roots and odontoma-like particles were seen near the permanent maxillary incisors ().\nBecause the child was not cooperative and she needed other dental procedures, surgical removal of the teeth under general anesthesia was decided.\nThis condition was explained to the parents and informed consent was taken. First all carious teeth were restored.\nThen after elevation of the mucoperiosteal flap in the anterior sextant of the maxilla, the teeth were removed. The teeth were rotated 180° and the palatal and incisal sides were located in the buccal and apical, respectively.\nTeeth had short roots with open apexes ().\nAfter suturing the impression was made. The molds were then used to create Nance appliance because the child was not cooperative and fix appliance was chosen ().\nAfter soft tissue healing, the Nance appliance was delivered to the child and oral hygiene instructions were explained to the parents and the child.\nMoreover, follow-up visits were scheduled for monitoring of the patient ().\nIn the follow-up visit, the Nance appliance was removed and the abutment teeth were cleaned and fluoride therapy was done, then the Nance appliance was recemented in her mouth. |
A 26-year-old male presented to Accident and Emergency following assault with a baseball bat. His past medical history consisted of longstanding Hepatitis C infection only, he was on no regular medications nor had any known allergies.\nOn admission, the patient had complained of bilateral pleuritic pain on inspiration with visible, tender bruising to the posterior thoracic region bilaterally. There were no urinary, bowel or neurological symptoms reported and no open wounds were visible on examination. The anterior abdomen was soft and non-tender to palpation.\nA CT scan of chest (performed at 30s post i.v. injection) showed acute fractures of left 10th/11thand right 8th/9th ribs, but no evidence of pneumothorax or lung contusion. A splenic laceration was not suspected clinically and the timing of the scan, optimized for the chest, only showed subtle splenic changes which were not identified by the original reporter ().\nLater that day, the patient complained that their pain had spread from the chest down to the right and left flanks. This prompted a CT scan of abdomen/pelvis (performed at 70 s), which demonstrated a contained splenic laceration with no evidence of capsular breach (). Both the rib fractures and splenic injury were treated conservatively with bed rest and analgesia.\nThe patient abruptly deteriorated becoming hypotensive and tachycardic in keeping with hypovolaemic shock. A further CT scan showed splenic capsular rupture, active bleeding and large-volume intraperitoneal haemorrhage ().\nThis was managed urgently with endovascular embolisation. While the patient gave informed consent for the procedure he indicated that he was unlikely to be compliant with some aspects of his aftercare and likely to self-discharge very soon after completion of the procedure.\nFollowing local anaesthesia an ultrasound guided puncture of the right common femoral artery was performed and the splenic artery selectively catheterized with a Sim 1 catheter (a reverse angled catheter). Angiography revealed three pseudoaneurysms, two arising from a second order upper pole branch and one from a third order equatorial branch (). These were then superselectively catheterized and embolized with a series of 3, 4 and 5 mm microcoils (Nester & Vortex, Nester = Cook Medical, Bloomington, IN, USA, Vortex = Boston Scientific, Watertown, MA, USA), delivered through a microcatheter. This abolished filling of the pseudoaneurysms but there was more devascularisation of the upper half of the spleen than envisaged when the decision to perform superselective embolisation was made. The right common femoral artery was then closed with a Starclose vascular closure device (Abbott Medical).\nThe decision to perform superselective embolisation was made with the intention of achieving definitive haemostasis in a patient who was likely to leave hospital at the earliest possible opportunity and not comply well with the procedural aftercare (). There was no further bleeding.\nDay 1 post-procedure, the patient developed a post-embolisation syndrome (PES) characterized by fever, nausea and left abdominal pain. A fourth CT scan was performed. This showed splenic necrosis, parenchymal and intravascular gas, which is an expected appearance post-embolisation. No fluid collection was demonstrated. Conservative resuscitation was initiated with i.v. fluid and antibiotic administration.\nThere was no indication for surgical drainage at this point as the CT scan showed only splenic necrosis, with no evidence of abscess formation. The patient’s temperature and clinical features were also settling, further reassuring stabilisation of his condition ().\nThe patient recovered from this episode a few days later, and deemed medically fit, was subsequently discharged home.\n3 weeks later, the patient presented to Accident & Emergency once again; this time with episodes of frank haemoptysis. He had an associated fever, and left upper quadrant (LUQ) pain.\nBlood tests showed elevated inflammatory markers, suggesting an infection, thought to be of likely respiratory origin at this time. |
A 62-year-old male without known coronary artery disease, recently diagnosed with rheumatoid arthritis (RA) treated with methotrexate for 5 days, was admitted to our emergency department with acute and typical chest pain for an hour. On ECG, ST-segment elevation myocardial infarction (STEMI) in anterior and inferior leads were noted. There were hypertension and hyperlipidemia as risk factors of coronary artery disease. After the patient was treated with 300 mg of aspirin, 600 mg of clopidogrel, as well as a weight-adjusted (70 IU/kg) bolus of unfractionated heparin, coronary angiography was performed showing an ostial left main coronary artery (LMCA) thrombus with 90% obstruction (, ), a total occlusion of the mid left anterior descending artery (LAD) () and a mid right coronary artery (RCA) thrombus with 80% obstruction (, ). From this point on we had three possible strategies. The first one was the interventional strategy (stenting/aspiration with high risk of cerebral and peripheral embolism, which would have exposed the patient to short and long-term risks). The second one was the coronary artery bypass graft surgery (the coronary artery bypass grafting may represent a high risk of early occlusion because of normal native coronary flow after dissolving the coronary thrombus with medical therapy) and the third one was pharmacological strategy (thrombolytic therapy), eventually followed by the pharmacological strategy. Thus, based on the STEMI guideline of European Society of Cardiology; alteplase (rt-PA) 15 mg was injected intravenosus as a bolus dose and subsequently 50 mg alteplase was infused for 30 minutes (0.75 mg/min) and 35 mg was infused for 60 minutes. After the fibrinolytic therapy, clinical and electrocardiographic success criteria were obtained. Repeated coronary angiography was performed 24 hours later, revealing that the large thrombus in ostial LMCA () and mid portion of RCA () was totally dissolved but only the apical portion of the LAD was occluded (). In addition, laboratory workup for hypercoagulability was negative. |
We report the case of a 15-year-old girl with Cystic Fibrosis (dF508/dF508), with first Pseudomonas aeruginosa infection at 1 year of age, and chronic colonization at the age of 5 years. The standard first line therapy and in consequence eradication of the bacteria was impeded by a hyperresponsiveness of the bronchial airway to nebulized tobramycin and colomycin, regardless of pre-medication with bronchodilator and corticosteroids. The patient was treated regularly with oral antibiotic courses (ciprofloxacin) and the attempts for aerosol administration of anti-pseudomonas antibiotics were repeated. The initial clinical course was stable, with no acute exacerbation. However, the lung function was deteriorating continuously with time. At the age of 12 years the girl was experiencing deterioration of clinical condition with increased need for oral antimicrobials and acute exacerbation resulting in hospital admissions for I.V. treatment. During one of the hospitalizations, intravenous antibiotic therapy with tobramycin and ceftazidime was initiated. Due to new microbiological findings revealing resistance of Pseudomonas aeruginosa to tobramycin, the antibiotic therapy was switched to ceftazidime, flucloxacillin and colomycin, all given intravenously. Minutes after the initiation of the colomycin infusion the girl reacted with generalized urticaria, dyspnea, tachycardia and hypotension. The infusion was stopped and steroids and antihistamines were applied. The patient recovered and the antibiotic therapy was changed and well-tolerated. The colomycin hypersensitivity was confirmed with positive prick-test (7 mm) and clinical significant FEV1 deterioration (>20 %) after inhalation with colomycin. Since then, colomycin was avoided. Further clinical course showed progressive lung deterioration. The microbiological findings revealed repeated detection of Pseudomonas aeruginosa up to 108 cells/ml. The patient was treated regularly with oral antibiotics. Attempts for nebulized tobramycin therapy were tried again, but was not tolerated by the patient. Repeated IV therapies were resulting only in temporary clinical improvement. At the age of 15 years, due to clinical signs of pulmonary exacerbation, the girl was again hospitalized. The lung function revealed FEV1 54% predicted, FVC 75% predicted, the microbiological findings showed two strains of Pseudomonas aeruginosa with resistance to tobramycin and sensitive to colomycin. Due to lack of alternative therapy, colomycin desensitization was initiated. The patient and her legal representatives gave an informed consent for desensitization procedure. At first, a 4-step intravenous desensitization protocol established in our center was applied (). The target dose of colomycin was 1,000,000 U. Pre-medication with methylprednisolone 1 mg/kg was used. During the desensitization procedure mild symptoms of a rush and restlessness were observed. The symptoms improved without medication and the procedure was continued without modification. After successful desensitization, the intravenous course of colomycin 3 × 1,000,000 IU/day was continued for 14 days without any adverse events. The clinical condition of the patient and the lung function improved significantly. On day 15 the therapy was switched to nebulized colomycin 2 × 1,000,000 IU and was continued for the next 2 days in inpatient setting. The inhalation treatment was well-tolerated, and thus the girl was discharged with the recommendation for long-term nebulized therapy in home care (). The patient and her parents were instructed on the necessity of strict and regular administration of the inhaled drug and the possible consequences of discontinuation. Anaphylaxis-rescue medication was prescribed and management training was performed. This therapy was successfully continued for a period of 10 years without any relevant complication. The clinical condition improved significantly, the lung function trend was stable, the microbiological findings of Pseudomonas aeruginosa were reduced to 103 cells/ml, also the number of oral and intravenous antibiotic courses could be reduced as well as the number of hospital admissions due to pulmonary exacerbations (). |
Two brothers presented to us, both with similar symptoms. Our first patient was an eight-year-old male who presented with an inability to stand or walk since the past two months, along with bilateral foot deformities. According to his father, the patient had developed a difficulty in walking and in climbing stairs, accompanied by frequent falls - about six months back. Gradually, he had lost the ability to walk even with support and was mainly confined to his bed-although he could sit up and crawl.\nThe patient’s intelligence was unaffected by the illness; he had no history of trauma, fever, fits, incontinence, or syncope and did not display vision, speech, or hearing abnormalities. A detailed review of the gastro-intestinal, genitourinary, respiratory, and cardiovascular systems showed no abnormality. The patients’ parents were first cousins, albeit unaffected by the disease themselves. However, out of five siblings, two of the patient’s sisters (12 and 14 years of age) and one brother (five years old) were affected by a similar illness. The patient had had an unremarkable birth history, had reached all the relevant milestones timely and was said to be taking a nutritionally adequate diet. As per the parents, all his vaccinations were complete and the past medical history was clear.\nOn general examination, the patient was well oriented in time, place, and person with his vitals, height, and weight all within the normal ranges. Regarding system-wise examination, the central nervous system examination showed no signs of wasting or abnormal tone in the upper limbs, the power in both the upper limbs was 4/5, and the deep tendon reflexes were normal when elicited. However, the bulk of both the lower limbs was decreased, with the right lower limb being slightly more wasted than the left. The tone was decreased as well and power in both the lower limbs was 2/5. The deep tendon reflexes of the lower limbs were absent. On further examination, Babinski sign was negative and the pupils were bilaterally and equally, reactive to light. The gait of the patient could not be assessed as he could not stand. However, there were no signs pointing towards cerebellar or cranial nerve dysfunction and mental functions and speech proved to be intact. On sensory examination, a higher threshold to pain and temperature was noted.\nOn examination of the musculoskeletal system, the patient had marked wasting in the anterior compartments of both legs. He demonstrated a bilateral foot drop with pes cavus (Figure )-more pronounced on the left side - and his feet were kept in a plantar, fixed position in the relaxed state. Contractures on the knees and Achilles tendon were seen. The upper limbs did not show any marked abnormality other than contractures over the interphalangeal joints of the fingers, with the skin prominently thicker there. The examinations of all the other systems were unremarkable.\nAs per laboratory investigations: the complete blood count, electrolytes, and creatine-phosphokinase levels were all within the normal ranges. The nerve conduction velocities were markedly decreased. A sural nerve biopsy was carried out and a subtotal reduction in myelin fibers was noted, along with focal endo-neuronal edema. No granuloma formation or inflammatory component could be identified. Based on the clinical, hereditary, and investigative findings, the patient was diagnosed with CMT disease, type 2.\nIn terms of management, no specific medical treatment is available, but the patient was referred to the orthopedic and rehabilitation departments to manage the foot deformity.\nOur second patient, brother of the first patient, was a five-year-old male who presented with difficulty in walking and frequent falls since the past two months. Gradually, his condition had progressed and his distal weakness had worsened, due to which he had been unable to walk without support since the past two weeks. Unlike his brother, the patient could still walk with support, although by dragging his feet. There was no significant difference in the history and examinations of this patient when compared to his brother and similar investigations were carried out, wherein the sural nerve biopsy showed nerve bundles with adequate myelin. Based on the above information, a diagnosis of CMT disease was made here as well and the child was similarly referred to the orthopedic and rehabilitation departments.\nConcerning the apparent autosomal recessive mode of inheritance, the parents of the boys were counseled accordingly regarding the implications of having more children in future and the manner in which their children could further propagate the condition. Additionally, they were counseled to bring in their affected daughters with reportedly similar symptoms for an evaluation and any possible rehabilitation as well, even though the parents described their status as nonambulatory. |
A 59-year-old European man crashed his car into a concrete dam (Fig. ). Bystanders attending to the accident found him in cardiac arrest and started cardiopulmonary resuscitation (CPR) immediately. Sufficient CPR efforts were continued until the emergency services had arrived. The first recorded heart rhythm was ventricular fibrillation (VF). On inspection, no signs of injury were immediately visible and no skid marks were found. CPR was continued by physician-staffed emergency medical services (EMS) according to the current advanced life support (ALS) guidelines []. Return of spontaneous circulation (ROSC) was achieved after 30 minutes. He remained unconscious without any sign of muscular activity. He was intubated, mechanically ventilated, and treated with catecholamines during and post CPR.\nAlthough the car was severely damaged, the prehospital physician deemed a traumatic cause for out-of-hospital cardiac arrest (OHCA) unlikely. Based on findings indicative of myocardial ischemia in a post-ROSC electrocardiogram (ECG), acute coronary syndrome was suspected as the etiology of cardiac arrest. After telephone consultation with the trauma leader of the regional trauma center, the patient was transported to the trauma center with percutaneous coronary intervention (PCI)-capability primarily within 120 minutes of the accident.\nOn arrival at the trauma center, the patient appeared clinically stable. His heart rate was 65 per minute, systolic blood pressure was 150 mmHg, oxygen saturation measured by pulse oximetry was 94%, and body temperature was 34.2 °C. Signs of myocardial ischemia were found in the ECG (Fig. ). His pupils were found to be equal, round, and reactive to light.\nAfter primary evaluation in the emergency room a whole-body CT scan revealed findings listed in Table . An MRI scan (Fig. ) of his head and neck was obtained immediately due to the severity of the CT findings. Additional findings in the MRI scan are summarized in Table .\nThe medical and social history of our patient were provided by his family. Subjective overall health assessment found the married man, who was a father and grandfather, to be in good health. He had suffered a fall leading to a fractured scapula 8 years before this accident, which was treated non-operatively. Two years ago, he was assessed for suspected coronary heart disease by a specialist in cardiology, who could not substantiate this suspicion.\nHe was transferred to the intensive care unit (ICU) for further treatment. Halo fixation was installed because only ligamentous structures were disrupted in this case. This procedure is common and adequate in AOD when no cervical spine fractures are present [].\nDue to several episodes of severe bradycardia, transient transvenous pacing was conducted. Cardiac diagnostics showed an ischemic cardiomyopathy with recurrent episodes of ventricular tachycardia. Assessment via echocardiography was performed in the trauma room, 3 weeks and 2 months after the accident and revealed akinesia of the left anterior descending coronary artery (LAD) region and hypokinesia of the inferior wall after a suspected myocardial infarction and VF. Early coronary angiography could not be performed due to severe brain injuries.\nAlthough he was initially assessed to have a poor neurological prognosis from the perspective of the neurologists and neurosurgeons because of his severe brain injuries, he could be discharged from the ICU after 23 days; he was responding to verbal contact and was able to move all his extremities.\nAfter 23 days of treatment at the trauma center he was transferred to a hospital close to his home. Further in-patient treatment was continued by local protocol for further 33 days (timeline in Table ).\nHe was discharged to a neurological rehabilitation facility, where care and rehabilitation efforts were continued with great success. Three months after the incident the tracheostomy was surgically closed.\nCoronary angiography was performed 4 months after the primary event and revealed no coronary artery disease. Subsequently, he had to wear a life vest due to arrhythmia. He was defibrillated once by the LifeVest® 3 months after the trauma during his stay at the neurological rehabilitation facility. Finally, 6 months after wearing the life vest an implantable cardioverter-defibrillator (ICD) was installed.\nSix months after the trauma, he was fully conscious, spontaneously breathing, independent of help in everyday life, and mobile with walking crutches. However, he was unable to swallow granular feed due incomplete bilateral paresis of the hypoglossal nerve. His neurologic status is continuously improving; treating neurologists attested a high potential of restitution. |
A 44-year-old female diagnosed with carcinoma of the left breast was posted for modified radical mastectomy with axillary clearance. On preanesthetic evaluation, no positive history or findings were elicited and baseline investigations were within normal limits. ECG was normal without any electrical alternates, and her two-dimensional echocardiogram showed normal left ventricular function with an ejection fraction of 60%. Her effort tolerance was >4 METs. Preoperative instructions were given for fasting for 8 h for oily solid food and to have clear liquids up to 2 h before surgery.\nHowever, she had fasted for 16½ h when she was received in the operating room. General anesthetic was planned. ECG, noninvasive blood pressure (NIBP), and pulse oximetry (SpO2) monitors were attached and a 22 G cannula was secured. At this time, variation in the R wave amplitude was noticed in Lead II on ECG. It correlated with variations in the plethysmograph []. The patient's heart rate was 98 beats/min and blood pressure was 112/86 mm of Hg. Since the patient was asymptomatic and hemodynamically stable, it was decided to proceed with the surgical procedure. Lactated ringer solution was started before induction of anesthesia and after 500 ml of fluid administration, variation of R wave amplitude started reducing but plethysmography variation persisted []. Perfusion numeric (perf) on monitor was 0.3 [] before giving fluids, which improved to 1.3 [] after giving fluid. General anesthesia was induced with sevoflurane along with 50 mg propofol, 100 mcg fentanyl, and 25 mg atracurium. Variation in R wave amplitude further decreased and eventually became normal after 1 L of lactated ringer solution was administered. Variations in plethysmography also became normal after 1 L of fluid administration. The patient was hemodynamically stable throughout surgery. The intraoperative blood loss was 200 ml. The procedure was uneventful and the patient was shifted to the postanesthesia care unit (PACU) for postoperative care where ECG, NIBP, and SpO2 were monitored continuously. R wave amplitude variation and plethysmography variations were not observed, and the patient was started on clear liquids once wide awake. The patient was discharged from PACU after 2 h and shifted to postoperative ward. The patient was discharged from hospital on the next day. |
A 27-year-old pale Caucasian male was admitted into the emergency room of our tertiary care hospital with the diagnosis of uremia. Upon arrival the overweight patient (BMI 32.7 kg/m²) had a blood pressure of 164/91 mmHg and a heart rate of 110/min. He presented with nausea and diarrhea, but was alert and awake. The remainder of the physical examination showed pericardial friction rub as well as a uremic foetor. His mucus membranes were dry and pale. His past medical history was significant for a living related kidney transplantation in 2008 for chronic interstitial nephritis and reflux nephropathy.\nAbout ten weeks prior to the admission he stopped taking the immunosuppressive medication which consisted of tacrolimus, prednisolone and mycophenolat-mofetil. The main reason for discontinuation was allegedly his financial situation after a job loss, which did not allow him to pay the necessary co-payment for the immunosuppressive drugs. He did neither seek help from the hospital nor from the welfare authorities/social services. Laboratory analysis was remarkable for a serum creatinine of 2443 μmol/l a serum urea of 67.6 mmol/l and a serum phosphorous level of 2.69 mmol/l. His venous blood gas analysis showed a metabolic acidosis (pH 7.27, bicarbonate 14 mmol/l). He was anemic with a hemoglobin level of 6.9 g/dl.) Renal biopsy showed a severe irreversible damage of the graft due to chronic humural rejection. After transfer to our unit hemodialysis was initiated. The patient also received two units of packed red blood cells, which lead to an increase in hemoglobin level to 8.8 g/dl. Peak creatinine declined under the initiation of dialysis. Due to an ongoing research project on the evaluation of cognitive function in dialysis patients we were able to use a battery of established test for neuro-cognitive function for frequent monitoring. The patient consented to take part in frequent psychological tests consisting of computer-based attention tasks, memory tasks (short-term and long-term memory) and also several tasks to examine executive functions. The full test battery consisted of Wechsler Digit Span, Stories of Rivermead Behavioral Memory Test (RBMT), TMT A and B, Alertness and Go/NoGo subtest of Testbatterie zur Aufmerksamkeitsprüfung (TAP), Key Search and Zoo Map of Behavioral Assessment of the Dysexecutive Syndrome (BADS), Regensburger Wortflüssigkeitstest (RWT) as well as Rey Complex Figures.\nThe neuropsychological assessments were conducted by trained personal in a quiet environment. Tests were all performed between 08:00 and 10:00 AM to minimize the effect of circadian changes. To retain and optimize the patients´ compliance and motivation as well as to avert effects of interference between the several domains, the tests alternate in the order related to their verbal and visuo-constructive characteristic. Initial practice trails were administered for the following tests (TMT A, TMT B and TAP) to make sure that the task was understood by the patient. Tests with known training effects were not regularly repeated. The test battery with no or minimal influence by learning effects consisted of Wechsler Digit Span, Stories of RBMT, TMT A and B, Alertness and Go/NoGo subtest of TAP as well as Rey Complex Figures [].\nIn parallel to the correction of uremia, indicated by the marker substances creatinine, urea we saw general improvement of cognitive function (Figure a, , ). However, after the first hemodialysis the patient showed the lowest performance, possibly due to a slight disequilibrium syndrome despite the fact that the first hemodialysis lasted only 120 minutes. The disequilibrium syndrome is caused by rapid removal urea resulting in an increased osmolality in the brain tissue (as compared to the serum) leading to water flux in the brain. i.e. cerebral edema. Indeed, in our patient there was a small but steady decrease in the calculated serum osmolality (Table ). The neuropsychological performance is reported in the Table . Scores on Wechsler Digit Span (forward) increased with ongoing testing. Further verbal memory performance, tested with Stories of RBMT, also increased. But in RBMT we found a performance loss on the second testing. All in all the short-term memory performance (on Wechsler Digit Span and RBMT) improved on the last testing compared with the first testing. Figural memory performance in Rey Complex Figures was consistently in an average range, (Table and Figure ), except for the second testing, were we found a marked decrease in performance (Figure ). Patient´s memory span plus standardized scores of verbal and figural memory showed no evidence of neuropsychological deficits for the patient compared to established control ranges. Interestingly, in the computer-based alertness attention task, we also found an improvement concomitant with the improvement of uremia. The assessment of executive functions after admission showed a marked underperformance even after correcting for the patients´ age. Concomitant with the initiation of dialysis therapy the patient considerably improved. Performance on the TMT A increased in the first three tests (Figure ). During the whole test period we observed an improvement from underperformance to average performance. Accordingly to TMT B we found a salient increase not until the fourth testing. TMT A and B performance was perfectly correlated with the correction of the serum creatinine level (Spearman's rank correlation: rs=1.0, p <.05). Nevertheless the performance on TMT B was clearly below-average, thereby indicating a continuous deficit in cognitive function, which is only “average” in the last of the all tests. The correction of uremia only slightly improved verbal fluency tasks (RWT). |
A 68-year-old man with no significant past medical history had suffered from paresthesia in his right lower leg. The area of paresthesia slowly extended rostrally up to the umbilicus in 6 months. At 5 months after the onset, he could not walk without cane and at 6 months, vesicorectal dysfunction appeared. On physical examination upon admission at 9 months after the onset, a sensory disturbance below the level of T12, impaired vesicorectal function, and spastic gait were observed. Manual muscle testing showed no weakness in the lower extremities, but he could not walk without cane due to the spasticity and impaired deep sense in lower legs. The deep tendon reflex was promoted in the lower extremities. Blood cell and chemistry examinations revealed no abnormalities. Cerebrospinal fluid test was normal (cell 2/3, protein 21 mg/dl, glucose 65 mg/dl).\nMagnetic resonance imaging (MRI) showed an intramedullary T2 hyperintensity and swelling of the spinal cord extending from the level of T5 to T7 []. And the lesion showed faint and partial Gadolinium enhancement. Findings of flow void or dilated pial venous plexus around the spinal cord were not observed. The spinal cord of this patient was about 45° rotated to the right in axial sections of the thoracic MRI, even though there was no rotation of the vertebral column.\nBased on his progressing deterioration and radiological findings, the differential diagnosis included demyelinization disease, transverse myelitis, sarcoidosis, Lyme's disease, spinal cord infarction, and spinal cord tumor. The patient was first admitted to the neurology department and completely examined. He had no history or finding of being bitten by a tick. Vitamin B12 and angiotensin converting enzyme was normal. HIV, syphilis, and hepatitis were negative. Immunoglobulin was also within normal level. The ophthalmological examination showed no abnormal finding. Chest computed tomography (CT) scan showed no bilateral hilar lymphadenopathy. A trial of steroid-pulse therapy (i.v. methylprednisolone 1000 mg for 3 days) resulted in no clinical or radiological improvement. Based on the no efficacy of steroid-pulse therapy in addition to the slowly progressing clinical course transverse myelitis or demyelinization disease was less likely suggested. It was thus suspected that the patient had a spinal cord tumor, and he was referred to our department for histological diagnosis.\nWe performed a midline myelotomy following T5 to T8 laminectomy. Because the spinal cord was rotated, it was difficult to find its midline. Thus we used dorsal column mapping at the midline myelotomy. A biopsy specimen was obtained at the T7 level on the enhanced area of the MRI. No dilated or tortuous vessels suggestive of vascular malformation were observed on the surface of the spinal cord or the dural sac [].\nThe biopsy specimen showed an increased number of small hyalinized vessels, gliosis, vascular thrombosis, and hemosiderin deposition. Neither tumor cells nor signs of active inflammation were observed []. There were no signs of demyelinization by either myelin basic protein or Klüver-Barrera stain. These histological findings were compatible with a venous spinal infarction caused by congestive myelopathy.\nOne week-postoperative MRI showed a marked decrease of the T2 hyperintensity area, spinal cord swelling and lesion enhancement []. Clinically, his gait disturbance became worse transiently, presumably due to the damage to the dorsal funiculus, but it improved with rehabilitation. The sensory disturbance was stable. No clinical and radiological recurrence was observed at one year after the operation. |
We report a case of a 57-year-old Indian man, who had a 2-year history of a cervical lymphadenopathy, followed by the Department of Pneumology. He lived in Italy since a few years, had no relevant medical history and didn’t take any drugs. In February 2018 he performed a cervical lymph node biopsy. The histopathologic findings showed extensive lymphohistiocytic inflammatory reaction with a central abscess and a peripheral granulomatous reaction, the PCR amplification was positive for atypical mycobacteria. QuantiFERON-TB Gold (QFT-G) test was borderline. The patient refused the purposed antibiotic treatment because of non-compliance. In January 2020 he was admitted to our dermatological department with a 5-month history of cervical wound healing disorder. On physical examination, he had mild, tender left anterior cervical lymphadenopathy with two hard nodular erythematous formations associated with a violaceous plaque with satellite papules and ulceration in the area of the manubrium of sternum ().\nOne of the cervical lymph nodes ulcerated after 1 week and evolved with the persistence of a secreting fistula with drainage of serous-purulent content. We performed a blood test, a smear from the ulcerated area of the neck and a skin biopsy from the plaque. In our skin biopsy, the histopathologic findings showed a dermal infiltrate consisting of epithelioid granulomas without necrosis surrounded by lymphocytes and multinucleate giant cells ().\nThe PCR amplification was negative for mycobacteria. The histologic features were compatible with lupus vulgaris. The culture of material obtained from the cervical draining lesion was positive for Mycobacterium tuberculosis. QuantiFERON-TB Gold test was positive, laboratory findings were within normal range, HIV test was negative. We also performed a chest radiography and a chest, neck and abdomen computed tomography which excluded visceral tuberculosis. A diagnosis of scrofuloderma associated with lupus vulgaris was made. For the planning of the correct treatment and management, the pneumologists were involved. We started an antituberculous quadruple therapy combining rifampicin 600 mg/daily, isoniazid 300 mg/daily, ethambutol 1.2 g/daily, and pyrazinamide 2 g/daily. At the dermatologic follow up visit, after 2 months of therapy, cutaneous lesions were regressed, leaving hyperpigmented patches. The treatment is still ongoing with only rifampicin 600 mg/daily and isoniazid 300 mg/daily. |
The second patient was a 70-year-old man with complex left leg swelling and pain. He had a Charcot left foot deformity, foot ulcers, and peripheral neuropathy secondary to diabetes mellitus. He wore a leg brace and an orthotic boot on his left leg. A right diabetic foot infection led to a right below-knee amputation. He had multiple reasons for lower-extremity edema, including a significant cardiac history, renal failure, chronic venous insufficiency, and liver cirrhosis. The patient presented with a 1-month history of left lower leg edema below the knee. Consequently, he was no longer able to fit into his left leg brace and orthotic boot, and he developed significant pain that limited his ability to ambulate and elevate his leg for prolonged periods. The swelling in his left lower leg was maximal in the ankle and foot (). Because of failure of traditional management, we proceeded with a trial of the geko device to treat his leg swelling.\nInitial placement of the geko device over its intended site did not stimulate the calf muscles. We suspected that this was a consequence of diabetic peripheral neuropathy of his common peroneal nerve. Instead, the geko device stimulated the short head of the biceps femoris muscle. Following 4 weeks of daily geko use, there was almost complete resolution of the left lower leg swelling, with the majority of improvement occurring during the fourth week of therapy (). During this time, the device produced minimal discomfort for the patient. The initial left calf circumference decreased from 29 to 23 cm (21% reduction). The patient was ambulating, had significant improvement in leg pain, and was able to fit into his orthotic boot. Given the improvement in leg swelling and pain, the geko device was discontinued, and the patient was instructed to wear his orthotic boot when ambulatory and to resume leg elevation and exercise. |
A 25-year-old Sudanese male, working in the military, presented in May 2018 to the emergency department with headache, dizziness, and vomiting. The patient has on and off headache for the past 2 weeks, mainly in the occipital region associated with multiple episodes of vomiting. There was no previous history of direct head trauma or any coagulation disorders.\nGeneral physical examination revealed normal vital signs. The neurological examination revealed an alert, oriented patient with no focal neurological deficit. Routine blood investigations, including complete blood count, electrolytes, renal, and coagulation profiles, were within normal values. Brain computed tomography scan revealed a 4-mm thick right parietal acute SDH []. A subsequent brain and spine magnetic resonance imaging (MRI), on the 4th day, was negative for subarachnoid hemorrhage but indicated the right front parietal subacute SDH with marginal blooming at the interface of subdural collection with the cerebral cortex, whereas spinal axis MRI was unremarkable. Conventional cerebral angiograms (digital subtraction angiography) revealed a 1.5-mm diameter aneurysm in the distal perirolandic cortical branch of the right MCA [Figure and ]. The remaining intracranial arteries appeared unremarkable.\nDue to small size of the parent artery and distal location, the patient was ineligible for the conventional therapy as it carried a high risk of rerupture and rebleeding. Endovascular embolization using Onyx was considered a viable treatment option. The procedure was discussed with the patient and his family, and an informed consent was obtained.\nUnder general anesthesia and local anesthesia using 1% lidocaine, cerebral angiography was performed through the right common femoral artery. A 6-French Arrow guide catheter was navigated in the right internal carotid artery (ICA), and an intermediate catheter was advanced with Sonic microcatheter to the cavernous part of the ICA in a coaxial fashion. Thereafter, the Sonic microcatheter was navigated into the cortical branch (M4) of the right MCA and positioned close to the microaneurysm. Onyx 18 was injected and noted to be migrating distal to the aneurysm bulb []. Complete occlusion of the aneurysm with Onyx 18 was successfully achieved. This also resulted in the cessation of flow to the distal segment of the aneurysm. A postprocedure cerebral angiogram confirmed the complete obliteration of the microaneurysm as well as the distal part of the parent artery [Figure and ].\nPostprocedure period was uneventful; the anesthesia was reversed and the patient was noted to be alert with no neurological deficit. There was no evidence of postendovascular complications on the follow-up MRI and magnetic resonance angiogram in the next day. An area of high intensity consistent with early subacute infarct in the right frontal region was noted []. Furthermore, there was no significant interval change as compared to the last MRI. Transthoracic echocardiography revealed no intracardiac mass, thrombi, or vegetation. Therefore, the patient was discharged with analgesics. The patient had no neurological signs and symptoms 2 weeks after discharge. |
A 47-year-old male patient presented to the surgical consultation with recurrent attacks of epigastric pain, melena, and epigastric fullness.\nUpper and lower GIT endoscopies were performed during the first admission which showed no abnormal findings.\nDuring the last presentation, the patient was admitted to the surgical unit.\nThe patients had negative drug history. The family history was negative for any genetic diseases, and the psychosocial history was negative.\nThe general examination revealed pale conjunctivae, palms and oral mucosa. There were no cyanosis or jaundice.\nThe blood pressure was 100/60 mmHg on admission, the pulse rate was 95 beats/minute, the temperature was normal and the body mass index was 38.\nThe abdomen was soft with no areas of tenderness. There were no dilated veins over the abdomen with no stigmata of chronic liver diseases.\nInvestigations were performed for the patient. The hemoglobin level was 8 gm/dl with normal serum electrolytes, normal liver and renal function tests. The patients received 2 units of compatible blood and arrangement was done to perform another endoscopy.\nThe endoscopy this time revealed a large pedunculated polyp with ulceration in the second part of the duodenum just distal to ampulla of Vater. .\nCT-scan of the abdomen was normal with no abnormal findings.\nArrangement was done to perform surgical intervention, during surgery we adopt an upper midline incision, exploration of the abdominal cavity showed normal liver, spleen, and omentum, with no ascites and no dilated veins.\nMobilization (kocherization) of the duodenum was performed, the anterior wall of duodenum was opened, and a pedunculated polyp (about 4 cm in size) was found which was arising from the posterior wall of the duodenum just distal to the ampulla of Vater. , .\nExcision of the polyp with its base was performed and was sent for frozen section examination during surgery which revealed a benign lesion with no signs of malignant activity.\nThe posterior and the anterior duodenal walls were sutured transversely in two layers by a slowly absorbable 3/0 suture material (polydioxanone). The inner layer with continuous full thickness suturing and the outer layer with interrupted seromuscular suturing.\nThe procedure was performed by two general surgeons who are specialized in the field of general surgery.\nThe final histopathology examination revealed a well-defined mass in the submucosa of the duodenum that showed tri-phasic endocrine components; i.e. carcinoid like cells in compact nests and trabeculae, spindle cells (Schwan cells), and ganglion type cells of varying proportions. The immunohistochemical analysis of the polyp showed that all cells stained positive for synaptophysin, Schwan cells stained positive for S100 marker, with focal expression of pankerain in the endocrine cells. The mucosa and the margins were free from the tumor. These features were consistent with the diagnosis of duodenal Gangliocytic paraganglioma. , .\nThe patient was admitted to the hospital for 5 days with smooth post-operative recovery and no postoperative complications.\nNo specific postoperative considerations were undertaken. |
The patient was a Caucasian male aged 39, who reported to a neurologist because of his throat’s discomfort and foreign body sensation. The throat discomfort had been occurring periodically for many years. The throat discomfort and the foreign body sensation intensified following an episode of vomiting several weeks prior to the visit. The vomiting episode was related to a dietary error. He denied odynophagia, dysphagia, tinnitus, vision disturbances, headaches, and relevant risk factors or medication. He had an otherwise healthy life before the event, reporting no invasive procedures at the area of the throat and larynx, cervical part of spine as well as cranial area. In his childhood, he was only diagnosed with thyroid nontoxic goiter.\nOn physical examination, head movements or opening the mouth do not cause pain, but currently, there is pressure soreness in the submandibular area on the left side. There were no neurological deficits.\nThe neck ultrasound did not show any enlarged lymph nodes or nodal packages.\nThe computed tomography (CT) scan showed a bilaterally elongated styloid process: 57–58 mm on the left side, and the right side was 41–42 mm (). Below the top of the right styloid process, there was placed a well-calcified shadow about 15–16 mm long, which could correspond to its continuation, without a clear connection with the top of the process (). In addition, the study showed adenoid tissue overgrowth of Waldeyer’s lymph ring with the presence of minor polycyclic calcifications, abolition of physiological cervical lordosis and degenerative changes in the cervical spine.\nEventually, the diagnosis of the classic type of Eagle’s syndrome (ES) was made; on the left side, so called elongated type, and on the right-side the segmented type (consisting of an uninterrupted segments of mineralized ligament). The patient underwent laryngological and surgical consultation. Due to the lack of symptoms related to the compression of the carotid arteries, no surgery was recommended. Pharmacological treatment was prescribed—first of all, painkillers. In the event of persistent symptoms, it was recommended to consider the inclusion of steroid and antiepileptic drugs in the treatment. The patient is under follow-up. An evaluation of the effectiveness of treatment with painkillers has been planned. |
A 28-year-old primigravida, at 31+3 weeks of gestation and no other co-morbidity, had an acute onset of upper abdominal pain and recurrent vomiting for one day. Her blood investigations were normal, except for a raised total leukocyte count (16,800/μL). She was managed symptomatically for the next 4 days. On day 5, she developed a sudden onset of breathlessness. Her chest X-ray revealed left hydropneumothorax with mediastinal shift to the right []. She was having hypoxemia, dyspnoea, tachycardia, even on rest, with aggravation in supine position. Intercostal drain (ICD) was placed in the left 5th intercostals space, in mid-axillary line []. ICD showed no air or fluid drainage. Patient was shifted to intensive care unit for further management. Thoracic surgeon was consulted and fetal well-being and fetal maturity were assessed. CT scan and MRI could not be done as patient was not able to lie down and the family expressed unwillingness to expose the patient to further radiations. Differential diagnosis of left pulmonary bulla, left pulmonary cyst, and tension gastrothorax were made. Multiple attempts for placement of nasogastric tube were made but failed, as the patient was very uncooperative and did not tolerate removal of the oxygen supply. She was becoming hypoxemic within seconds after removal of the oxygen flow. Patient and family were counselled about the need for urgent delivery of the baby by caesarean section. She was given two doses of betamethasone 12 mg, 24 hours apart. Patient was not allowed to take food through the mouth and was feed through the parenteral route. Lower segment caesarean section (LSCS) was carried out under general anaesthesia. All preparations were made for thoracotomy. Patient was intubated with single lumen endotracheal tube after rapid sequence induction and ventilated with pressure control mode. After closure of uterus and good uterine contraction, abdominal cavity was explored along with the diaphragm. There was herniation of stomach, spleen, and part of colon into the left hemithorax, due to rupture of left hemidiaphragm. Thoracotomy was performed with reduction of herniated contents and repair of the diaphragm. Left lung was fully inflated and an implantable cardioverter defibrillator (ICD) placed before closure of the thorax. She was extubated after surgery. Post-operative period was uneventful. ICD was removed after 2 days. Patient was shifted to the ward in a stable condition on the fourth day after the operation. Patient and her baby were discharged on 21st day after admission. |
A 68-year-old woman (166 cm, 67 kg) with chief complaints of hoarseness, frequent coughing, and mild orthopnea was diagnosed with a recurrent vocal papilloma. The patient provided written informed consent for publication of her information and images. Approval by an ethics committee was unnecessary because of the nature of this study (case report). The tumor had been first recognized 16 years ago, and she underwent two surgical resections in 1996 and 2002. In 2002, a 6.0-mm internal diameter cuffed oral preformed tube was used to intubate the patient without problems. At the current presentation, however, the recurrent mass had grown to approximately 1 × 2 cm, which was larger than that during the previous surgeries. The pedunculated papilloma seemed to originate in the left vocal cord and extended to the subglottis, occupying most of the glottic airway (). During the preoperative visit, the patient’s airway was classified as Mallampati class 2 and her neck extension was slightly stiff. Her lung sounds were clear, but she constantly coughed while sitting upright and expressed that her breathing was uncomfortable while in the supine position. The anesthetic plan regarding the use of a flexible fiberoptic bronchoscope and McGrath® videolaryngoscope and the possibility of awake intubation were explained to the patient. The patient understood and agreed to undergo the procedure.\nThe patient was premedicated with intramuscular injections of 0.5 mg atropine and 1.5 mg midazolam. On arrival in the operating room, the patient was sufficiently preoxygenated and subject to monitoring. Simple bipolar electrocardiographic monitoring and a finger pulse oximeter were applied, while noninvasive blood pressure was measured with a cuff on her upper arm. Her body temperature was monitored with an axillary temperature probe. In the operating room, the patient was still intermittently coughing and seemed to struggle with secretions. To stabilize the patient and obtain a better view of the airway, an intravenous bolus injection of 0.2 mg glycopyrrolate and an infusion of remifentanil were given. A commercial 10% lidocaine oral spray was applied for oropharyngeal topical anesthesia. Right and left superior laryngeal nerve blocks and translaryngeal nerve blocks for the recurrent laryngeal nerve were performed, each with an injection of 2 mL of 2% lidocaine. Supplemental oxygen was administered by a nasal cannula throughout the nerve block procedure.\nTo visualize the actual mass and plan the most appropriate intubation technique, a flexible fiberoptic bronchoscope (LF-GP; Olympus, Tokyo, Japan) was carefully inserted into the patient’s oropharynx. The endoscopic view of the huge vocal cord mass nearly obstructing the airway indicated that the size of the glottic opening was too small for even a 6.0-mm tube. Furthermore, advancing the endotracheal tube (ETT) through the fiberoptic bronchoscope could not show whether the tube itself was damaging the tumor or pushing tumor particles deeper into the airway, which could cause major problems such as endobronchial occlusion. Thus, the McGrath® videolaryngoscope and a smaller 5.5-mm internal diameter ETT were used. With a clear view of the vocal cord area, the 5.5-mm ETT was advanced safely through the space between the vocal papilloma and the glottis. The tumor was not damaged, and no bleeding occurred. As soon as successful intubation was confirmed by capnography, general anesthesia was induced with propofol, sevoflurane, and rocuronium. Pulse oximetry did not show a decrease in the patient’s oxygen saturation at any point during the intubation procedure.\nThe surgeon removed the vocal papilloma using the Jackson laryngoscope for visualization and microscissors for resection (). Bleeding was well controlled with epinephrine-impregnated cotton balls and electrocauterization. The operation time was 35 minutes, and the total anesthetic time was 80 minutes. A safe reversal agent (2 mg/kg of sugammadex) was administered, and the extubation was uneventful. Pathological examination confirmed that the mass was a squamous papilloma. There was no recurrence at the 2-month follow-up visit (). |
A 14-year-old female attended the Orthopedic Oncology Clinic at The First Affiliated Hospital of Nanchang University (Nanchang, China) for a follow-up. She underwent two operations successively due to pathological fractures in the left femur and tibia in a local hospital about 1 year ago. Then, she was diagnosed with FD according to the postoperative pathological examinations. Recently, she had no obvious symptoms and discomfort. Physical examinations showed nothing remarkable, but a little swelling in her left distal thigh. She had a negative family history of bone tumor. There was no fever or respiratory embarrassment accompanying the swelling. No history of weight loss or exposure to tuberculosis was mentioned.\nIn a further examination, physical examination showed no palpable head, neck, supraclavicular, axillary, or epitrochlear lymph nodes. Inflammatory markers were within the normal limits. Plain radiographs indicated an expansile osteolytic lesion with marginal sclerosis and a matrix with a ground glass appearance and without periosteal reaction of the left distal femur, which was consistent with FD (). Then, a diagnosis of recurrence of FD in the left femur was made given the medical history of FD, symptoms, and imaging findings.\nBased on the exclusion of surgical contraindications, professional surgeons, who specialized in treating bone tumors, performed the surgery of curettage and grafting in the left femur. Histologic analysis of hematoxylin and eosin-stained specimens showed irregularly shaped spicules of immature bone without osteoblastic rimming and fibrous stroma with few mitotic activities (). Immunohistochemical analysis showed positive for CD68 and Vim but negative for bcl-2, CK, CD34, CD99, EMA, SMA, and S100. According to the above findings, the diagnosis of the recurrence of FD of the left femur was finally confirmed.\nNevertheless, she presented to our clinic again with a chief complaint of pain and swelling in her left tibia and calcaneus 4 months later. Radiographic review showed a prominent osteolytic lesion located in the distal portion of the tibia and calcaneus with cortical destruction (). The patient underwent surgery with the removal of the tumor tissue and reconstruction with allogenous bone graft. According to the histological and immunohistochemical findings, the diagnosis of PMH secondary to FD was confirmed by an expert pathology consultant.\nFinally, the patient had to undergo an amputation of the left thigh. The tumor consisted of densely distributed pleomorphic cells, ranging in configuration from small spindled elements to large plump epithelioid variants with prominent pink cytoplasm. Scattered cells with vacuolated cytoplasm were also present. Prominent pleomorphic nuclei contained optically empty centers with peripheral marginalization of the chromatin, in addition to conspicuous nucleoli. Multinucleated cells were also present. Vasoformative elements, such as multi-cellular vascular channels or intracytoplasmic vacuoles, were identified (). Immunohistochemical analysis showed positive for CK, CD31 (), but negative for s-100 and CD34. According to the above findings, the diagnosis of PMH secondary to FD of the left lower limb was finally confirmed.\nThe patient was discharged without any complications 1 week after the amputation. At the time of the 3-month follow-up, the patient reported no pain or discomfort in her left lower extremity. No evidence of recurrence or distal metastasis was noted during the 3 months after surgery. However, it is necessary to perform continuous observations of the patient because of a high rate of recurrence and metastasis.\nPseudomyogenic hemangioendothelioma is a rare soft tissue tumor—distinct from epithelioid hemangioendothelioma []—originally described as a “fibroma-like variant of epithelioid sarcoma” []; it has been recently concluded that the “epithelioid sarcoma-like hemangioendothelioma” is essentially the same pathological entity []. PMH affects predominantly young males. The tumor is multifocal, involving different tissue planes and, although, it shares some histopathologic features with epithelioid sarcoma, it has a different, spindle cell morphology with common positivity for CD31, lack of CD34 reactivity, and intact INI-1 immunoreactivity [, ].\nBy contrast, fibrous dysplasia (FD) is a common tumor-like lesion characterized by solitary or multifocal polyostotic intramedullary lesions []. It has a frequency of 2.5 % for all bone lesions and 7 % for benign bone tumors []. While any bone can be affected by FD, the most common sites of the disease are the femur, the tibia, the ribs, the skull, the facial bones, the humerus, and the pelvis. Although many bones can be affected at once, FD is not a disease that spreads from one bone to another. Multiple affected bones are often found unilaterally [].\nHowever, malignant transformation of FD is rare and occurs in less than 1 % of the cases [, ]. The most commonly observed malignant transformations are osteosarcoma, fibrosarcoma, and chondrosarcoma []. As far as we can see, the current case is the first case reported ever of PMH secondary to FD, which is extremely rare and worthy of special remark.\nFD can be divided into two major types: monostotic and polyostotic []. According to the largest series, malignant changes seem more likely to occur in polyostotic than in monostotic FD [, ]. It is consistent that the current research described a malignant change in a polyostotic FD. Although it is important to recognize malignant transformation as early as possible, it may not be easy as in our case. Suspecting from malignant transformation can be extremely difficult especially in cases with monostotic disease having either subtle symptoms or none. In the reported cases up to date, the special symptoms of the malignant change were mainly pain, swelling, and late appearance of a bony mass [, ]. But given the fact that the pain is a nonspecial finding and is the most common complaint followed by pathological fracture in FD [], great care should be taken when evaluating this symptom. Pain which is rapidly becoming worse over a relatively short period without trauma should be considered alarming [].\nPMH is extremely difficult to diagnose because of no morphological evidence suggestive of endothelial differentiation is present to confirm a radiological pattern of vascular neoplasm (multiple well-limited purely lytic lesions) []. The tumor is composed of large spindle cells, arranged in sheets or fascicles. Tumor cells with epithelioid cytomorphology are also often present. In this case, the tumor consists of spindle cells, and round epithelioid cells exist. The cells are large and have abundant eosinophilic cytoplasm, mimicking a myoid tumor or epithelioid carcinoma cells. The tumor cells are plump but show no apparent pleomorphism. The nuclei of the cells have small nucleoli without notable atypia, and the mitotic activity is scarce.\nBased on previous reports in other locations, PMH has a more indolent clinical course with a small risk of metastasis []. Therefore, complete macroscopic excision is the treatment of choice. Local recurrence must be considered, even with complete, gross surgical resection; close follow-up and adjuvant therapy are warranted. |
A 44-year-old man with several tumors in a left solitary kidney was referred to our team. He was healthy, with no weight loss. Laboratory tests showed serum creatinine 1.30mg/dl, elevated serum C-reactive protein, and erythrocyte sedimentation. Reported one sister and tree uncles operated with renal tumor, two of them with bilateral tumors.\nHe had a right open radical nephrectomy four months before for a big Kidney tumor. Pathology reported a pT2bN0Mx multifocal Papillary Renal Carcinoma with 20.2 x 11.6cm and invasion of the capsule and collector system.\nUltrasound (US), computed tomography (CT) and MRI showed in the solitary left kidney at least eleven renal tumors, the largest entirely exophytic with 4.2cm at the upper pole and 4.7cm and 3.7cm at the lower pole. No central tumors or involvement of the renal sinus, renal vein or cava vein. No lymphadenomegaly or metastases in abdomen or thorax ( ).\nWe discussed with the patient the options: a definitive laparoscopic radical nephrectomy, hemodialysis and kidney graft several years later or a planned conservative surgical procedure. He decided for the conservative approach.\nA planned left transperitoneal laparoscopic radical nephroureterectomy was performed. After complete release of the kidney, the artery and the vein were proximal doubled clipped by polymer clips and cut off. The ureter was cut at crossing iliac vessels level. The kidney was withdrawn with an iliac fossa incision, was kept in ice slush and perfused with a continuous cold storage transplant solution. With ultrasonography aid, a bench nephron-sparing surgery was performed and all tumors resected or enucleated to maximally preserve the normal parenchyma. The incised calyxes were repaired and a meticulous reconstruction of the kidney with absorbable sutures and hemostatic booster interposition made. It was spent 105 minutes to resect and to reconstruct ( ). During the bench surgery the patient was repositioned in the dorsal decubitus and a hemodialysis catheter was inserted in the subclavian right vein. With a Gibson right incision an autotransplantation was performed. Reconstructed kidney was autotransplanted in the retroperitoneum and the vascular axis was anastomosed in the external iliac vein and artery. The ureter was implanted in the bladder by an extravesical Lich-Gregoir antireflux procedure with a double J stent. A Penrose drain was left in place.\nHemodialysis was necessary daily in the first week. On the 9th post-op a great urinoma collection forced a reoperation and a suction silicon drain was left. The patient was discharged on the 19th post-op with a high debit urinary fistula. He was in good physical and health condition. The fistula closed spontaneously on the 40th post-op and the double J stent was withdrawn.\nThirteen portions of the kidney were resected or enucleated. The anathomopathological report a multifocal Papillary Renal Carcinoma types 1 and 2: Cromophilus ( ).\nThe serum creatinine was monitored and dropped down progressively. It was 2.76mg/dl in the 30th post-op and 1.74mg/dl in the 60th post-op. No other complications occurred. No relapse tumors in abdomen MRI made on the 4th, 8th and 12th months post-op were observed. On the 13th month post-op he has good health, takes a normal life, and the creatinine is 1.69mg/dl. |
A twenty year old male patient presented with headache and vomiting since 3–4 months. The patient was fully conscious with fundus examination showing papilledema. Cranial nerves were normal on testing. Magnetic resonance imaging (MRI) revealed a large extra-axial tumor in the middle cranial fossa which was hypointense on T1, hyperintense on T2 with contrast enhancement [Figures –]. The base of the tumor was on the petrous temporal bone. A diagnosis of meningioma was made preoperatively, and the patient was taken up for surgery.\nThe patient was operated in supine position under general anesthesia. The right side temporal craniotomy was made. The tumor was firm grayish red in color. The tumor was completely removed by standard microsurgical technique of internal debulking of the tumor followed by capsular dissection. There was bleeding at the base at the petrous temporal bone which was cauterized. After securing hemostasis and dural closure, drain was kept in the subgaleal space, and the incision was closed in a standard fashion.\nIn the immediate postoperative period, total right-sided facial palsy was noted. The patient was observed in the intensive care unit for 2 days. The patient remained fully conscious and was discharged on the 7th postoperative day. Histopathological examination revealed biomorphic tumor composed of sheets and cords of epithelioid cells and bundles of spindle cells []. The epithelioid component showed features of nuclear anaplasia along with frequent mitosis [].\nImmunohistochemistry (IHC) revealed immunopositivity for vimentin, confirming the mesenchymal differentiation of the tumor []. The tumor cells showed patchy nuclear and cytoplasmic immunostaining for S-100, confirming the neural differentiation [Figures and ].\nGlial fibrillary acidic protein, smooth muscle actin, desmin, HMB-45, epithelial membrane antigen, and cytokeratin were negative.\nMRI 1 month after the surgery confirmed the total excision of the lesion []. The patient was referred for radiotherapy. At 6 months, there was an improvement in the right-sided facial palsy []. |
A 51-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cancer when she was 48 years old, and lymphedema developed in the left leg soon after the operation. She had one episode of cellulitis. Despite wearing elastic stockings, lymphedema worsened, and she visited our institution at the age of 49 years. She was diagnosed with lymphedema based on lymphoscintigraphic finding. There was a development of collateral lymphatic vessels and dermal backflow in bilateral lower leg (See figure, Supplemental Digital Content 1, which displays lymphoscintigraphic findings. Collateral lymphatic vessels were observed in the bilateral lower legs. Lymphatic function in the left thigh was impaired, ). She had no allergies or other pertinent medical histories.\nLVA was performed at the age of 50 years. The postoperative course was uneventful, and lymphedema improved. However, lymphedema worsened again at 1 year postoperatively after taking a long flight, although she wears elastic stockings daily. A second LVA was planned (Fig. ).\nPreoperative indocyanine green (ICG) lymphography showed a linear pattern in the right leg. Dermal backflow was observed in the left thigh and lower leg. There was no linear pattern in the area (left lower leg) where the lymphatic thrombus was found afterward. Preoperative echography showed 2 hypoechoic circles measuring about 0.5 mm in diameter that did not collapse with pressure from the probe, although the veins collapsed with pressure (Fig. ). Compared with lymphatic vessels, veins usually collapse more easily under pressure, because the inner pressure of the lymphatic vessels is higher than that of the veins. In this case, the 2 circles did not collapse under pressure, and we surmised that the inner pressure prevented collapse. The hypoechoic circles extended proximal and distally and did not have flow with on color Doppler mode.\nDuring LVA, we identified 2 parallel white vessels beneath the superficial fascia. Two vessels were in close contact. We diagnosed these as lymphatic vessels because of the location and appearance and the fact that they ran in parallel, which is not usually observed with other vessels or nerves. When we incised the vessels, white material was extruded (Fig. ). A diagnosis of lymphatic thrombosis was made, and we concluded that the vessels did not collapse with pressure from the probe during echography because of thrombus. Intraoperative echography revealed the same findings, that is, a hypoplastic circle without collapse by probe pressure, 15 cm distal from the incision, which indicated that there was lymphatic thrombus in at least 15 cm. We ligated the lymphatic vessels, closed the wound at this site, and performed LVA at other sites (4 sites in the left and 1 site in the right leg). Though the postoperative course was uneventful, the patient’s lymphedema did not improve postoperatively. This may be partially because the patient gained weight after LVA, and there is another possibility that postoperative thrombus formed within the anastomosis site had harmful effect for lymphedema, although it is difficult to confirm.\nHistopathological examination showed a thickened smooth muscle layer (tunica media) in the lymphatic vessels (See figure, Supplemental Digital Content 2, which displays histopathological findings of the lymphatic vessel and the lymphatic thrombus. (a) The lymphatic vessel (a) and the lymphatic thrombus (b) (×20, H&E). Thickened smooth muscle layer (tunica media) in the lymphatic vessels is observed. (c) The lymphatic vessel (×100, D2-40). The endothelial cells of the vessel were negative for D2-40, ). Fibrous thickening of the tunica intima was observed, and the inner lumen was narrow. The inner layer of the vessel was negative for D2-40, which is a marker to stain the lymphatic endothelial cells. In the thrombus, hyperplasty of fibroblasts and organization were found (Fig. ). We did not observe hyperplasty of the lymphatic endothelial cells, which are positive for D2-40 within the thrombus. |
A 4-month-old male infant presented to the emergency department with a 3-day history of vomiting, diarrhea and difficulty breathing. Excessive and rapid weight gain was noted by the parents since the age of 2 months. The baby was previously healthy and born at term with a birth weight of 3.62 kg; the pregnancy and delivery were uncomplicated. There was no family history of consanguinity, malignancies, unexplained childhood deaths or stillbirth.\nOn a detailed physical exam, the baby was noted to have Cushingoid facies, poor muscle tone and excessive fat pad on the back and arms (). Notably, his length was 54 cm (below the 1st percentile) and the weight was disproportionately elevated at 6.7 kg (50th percentile). He was obese with weight for length ratio more than 99th percentile for the comparable age and sex. His blood pressure was elevated for age, ranging from 93 - 121 to 40 - 73 mm Hg. A large abdominal mass was palpable on the left lower quadrant and the borders were difficult to define. He had a prepubertal genital examination with no overt virilization; his phallus was typical in size but buried in the suprapubic fat pad. Neither acne, adult body odor, nor axillary hair was noted on examination.\nIn light of the respiratory distress, a chest radiograph was obtained and demonstrated bilateral upper lobe infiltrates, and henceforth he was admitted with a working diagnosis of pneumonia.\nWhile being treated in the hospital for pneumonia, an evaluation was launched to delineate the etiology of the excessive weight gain, growth arrest, hypotonia and the abdominal mass. Laboratory evaluation revealed consistently elevated serum cortisol levels, irrespective of the time of the day with values of 73.0 μg/dL at 5:08 am and 68.6 μg/dL at 12:50 pm (normal range 4 - 22 μg/dL). Serum adrenocorticotropic hormone (ACTH) was suppressed at 7 pg/mL, at 5:16 am (normal range 0 - 60 pg/mL), suggestive of a primary adrenal cortisol hypersecretion. Dehydroepiandrosterone sulfate (DHEA-S) was mildly elevated at 115 μg/dL (normal range 16 - 96 μg/dL), but clinical features of hyperandrogenism were absent. The aldosterone level was normal at 23 ng/dL with a slightly elevated renin of 43.51 ng/mL/h. There was a slight increase in urine vanillylmandelic acid (VMA) and metanephrines, excluding a pheochromocytoma. An ultrasound demonstrated a 6.8 × 7.7 cm size solid mass at the upper pole of left kidney. A subsequent computerized tomography scan confirmed a large left-sided soft tissue mass with internal necrosis and a well-defined capsule abutting the left kidney with non-visualization of the left adrenal gland (). Henceforth, presumptive diagnosis of a functional cortisol producing adrenal tumor was made.\nThe baby underwent exploratory laparotomy and a left adrenalectomy was performed. Intraoperative findings revealed a 9.0 × 7.0 × 5.0 cm (315 cm3), well-encapsulated tumor weighing 180 g without breach of the capsule. The tumor was resected completely. There were no other signs of tumor spread including no liver metastasis or suspicious periaortic lymph nodes. Histological classification confirmed a high-grade adrenocortical carcinoma (24 mitotic figures/50 HPF) with a few pleomorphic nuclei (). The tumor was finally classified an adrenocortical carcinoma (ACC), stage 2 (based on combined American Joint Committee on Cancer (AJCC)/International Union Against Cancer (UICC) classification). During the perioperative period, the baby was treated with stress dosing of hydrocortisone and had an uneventful recovery. He continued to receive maintenance doses of hydrocortisone which were subsequently weaned. No adjuvant treatment with mitotane was done. During the recovery period, his linear growth velocity improved and his weight normalized (). Yearly follow-ups for the next 4 years and then most recently at 7 years and 10 months after treatment indicate continued health and successful recovery (). |
PCM, 18 years old, female, from the Gorj County, was admitted on 21 September 2019 to the Infectious Diseases Department with high fever (39–40 °C), chills, intense sore throat and headache. The onset of the disease was two weeks earlier with fever and sore throat. She had consulted the general practitioner who established the diagnosis of exudative tonsillitis and recommended treatment with 625 mg amoxicillin/clavulanate three times per day and 200 mg ibuprofen two times per day. The patient followed the treatment for 12 days, but the condition worsened, the fever gradually went up and a bulge appeared on the left side of the neck.\nHer past medical history was inconspicuous; the patient denied consumption of tobacco, alcohol, recreational drugs or oral birth control drugs. She had received all the mandatory immunization (according to the Romanian national program).\nThere was no contact with other sick persons with similar symptoms.\nWhen she was admitted to the hospital, her general condition was consistent with moderate sepsis; she had fever (38.9 °C), elevated pulse rate (137 bpm), normal blood pressure (116/74 mm Hg) and normal oxygen saturation (97%). A significant lymph nodes enlargement (about 6 cm in diameter) was noted on the left side of the neck, in front and behind the sternocleidomastoid muscle, and left supraclavicular adenopathy; there was limited movement of the enlarged lymph nodes and they were hard on palpation. Both tonsils showed white exudate on their surface. There was no abnormal lung or heart sounds, no liver or spleen enlargement and no neurological abnormalities. The urine output was normal.\nThe initial laboratory tests are shown in .\nThe chest X-ray showed consolidation on the lower right lobe. An ultrasound of the left side of the neck highlighted the lymph nodes enlargement, intense vascularization and a deep edema. A computed tomography (CT) of the neck (see and ) and the upper thorax showed segmental partial thrombosis of the left internal jugular vein. The Ear-Nose-Throat (ENT) examination established the diagnosis of cryptic tonsillitis and cervical adenitis.\nThe throat swab culture on blood agar media and blood culture using BACTEC vials for aerobic germs were negative. An enzyme-linked immunosorbent assay (ELISA) test for HIV was also negative.\nThe diagnosis of Lemierre’s syndrome was established and the initial prognosis was reserved (the patient met the old criteria for sepsis); she started antimicrobial treatment with penicillin G, 4 international megaunits (MUI)/day, intravenous (i.v.), and metronidazole, 1.5 g/day, per os (p.o.). She also received enoxaparin, 0.6 mL twice a day, s.c., and diclofenac, 100 mg/day, p.o.\nThe fever gradually decreased, the patient returned to normal temperature after 6 days of treatment, and the general condition improved; however, the lymph nodes remained enlarged throughout the period of hospital admission.\nThe follow-up laboratory tests are shown in .\nThe patient was discharged after 16 days; she received a prescription for metronidazole, 1.5 g/day, p.o., for another 14 days and for rivaroxaban, 20 mg/day, p.o., for 30 days; she continued with this regimen at home as recommended.\nA month later, she was seen in our department: she was in good condition, there were no signs of infection, but the ultrasound still showed signs of thrombosis of the jugular vein. She refused the surgical intervention to remove the thrombus. |
A 74-year-old Caucasian man, who was a regular drinker and heavy cigarette smoker, presented to a rheumatologist for pain at motion of his left thumb. The physician diagnosed a tendonitis, and the patient was prescribed anti-inflammatory drugs, which allowed for a moderate improvement in the pain.\nThe patient presented one month later to our clinic complaining of persistent pain in his left hand, even at rest. A physical examination revealed a severe tenderness of the first metacarpophalangeal joint and, though less severe, of the lateral carpal region. Pain increased with the mobilization of the wrist and the thumb.\nThe patient was prescribed plain radiographs of his left hand which showed diffuse osteoporosis and signs of osteoarthritis of the trapeziometacarpal joint (Figure ). A diagnosis of thumb carpometacarpal osteoarthritis was made. A hand surgeon prescribed a specific brace.\nWe had no contact with the patient after his follow-up, which was held two months after his presentation to our clinic, until he was admitted to the internal medicine department of our hospital three months later for severe asthenia and a weight loss of 10kg over the previous five months. The patient declared to have been eating normally until the last month when his appetite was reduced. The patient was examined by one of the authors of this report for an enormous mass that involved his whole left hand (Figure ). Severe pain discouraged the patient from using his left hand in daily activities. Pain was even present on raising the hand from a table. Physical examination showed a large mass of his left hand, deep tenderness, an increase in local temperature and edema. Simply touching the hand caused excruciating pain. No palpable lymph nodes were present. The results of laboratory tests showed: an alkaline phosphatase of 408mg/dL, erythrocyte sedimentation rate, leukocytes, C-reactive protein, antistreptolysin O, rheumatoid factor, immunoglobulin G (IgG), IgA, IgM, C3, C4, circulating immune complexes, antinuclear antibodies, antimitochondrial antibodies, carcinoembryonic antigens, α-fetoprotein and prostate-specific antigen were normal. Calcemia was also in the limits of normality. An ecography of the hand revealed ‘synovial hypertrophy, with rich vascularisation’. The patient received a new plain X-ray of his hand, which revealed a diffuse osteoporosis of the whole hand, with resorption of almost the whole trapezium, about 50% of the trapezoid, and erosion of the distal part of the scaphoid. The metacarpal and phalangeal bones were severely osteoporotic (Figure ). A chest X-ray induced the suspicion of a malignant lesion in the superior lobe of the patient’s left lung. A subsequent computed tomography (CT) scan of the chest showed a large mass in that area (Figure ).\nAt this point, the patient received a total-body bone scan with Tc99, which revealed the presence of a unique hot area corresponding to the left hand (Figure ). A total-body CT scan was negative for other locations.\nThe patient underwent an open biopsy. Histology revealed an undifferentiated large-cell carcinoma (Figure ). Immunohistochemistry was thyroid transcription factor-1 positive, which was a confirmation of the lung origin (Figures and ). The diagnosis was metastasis of undifferentiated large-cell lung carcinoma. Magnetic resonance imaging (MRI) was attempted twice without success because the patient could not tolerate to keep his left hand steady.\nWe performed an amputation at the distal third of the forearm.\nAt six-month follow-up, the amputated forearm was in perfect condition. The patient was submitted to chemotherapy but, at present, his general condition is worsening because of respiratory problems. |
A 55-year-old female with a history of renal cell carcinoma of the left kidney metastatic to the bony pelvis, lungs, mediastinum, and spleen presented to the emergency department with shortness of breath, pleuritic chest pain, and left scapular pain. She presented to the same emergency department one week prior with pleuritic chest pain but was discharged home after pulmonary embolism was ruled out.\nShe was diagnosed with renal cell carcinoma of the left kidney five years prior after presenting with gross hematuria. At that time, she underwent left radical nephrectomy. One year later, she developed a metastatic lesion in the bony pelvis for which she underwent radiation therapy. She as treated with pazopanib for two years with stable disease but stopped due to gastro-intestinal toxicity. Therapy was switched to nivolumab, which was discontinued after six months due to grade four pancreatitis and grade two rash. Eight months prior to her current presentation, she underwent radiation treatment to metastatic lesions in the left pubic symphysis and spleen. The patient initiated therapy with cabozantinib, a tyrosine-kinase-inhibitor used to treat renal cell carcinoma, three months prior to her current presentation.\nOn physical examination, she was wheezing in all lung fields and hypoxemic requiring supplemental oxygen. She had prior 12-pack-year smoking history but no formal diagnosis of chronic obstructive pulmonary disease (COPD). A chest x-ray revealed a small left pleural effusion and left basilar atelectasis. Laboratory workup, including complete blood count, renal and hepatic panels, and troponin, was unremarkable. An electrocardiogram (ECG) revealed sinus tachycardia without signs of ischemia. CT was not repeated due to her negative CT angiogram one-week prior. Given radicular and left scapular pain, an MRI of the spine was done, which revealed no pathologic metastases in the thoracic or lumbar spine but did reveal a new sacral lesion. Given her progressive stridor, she underwent laryngoscopy, which revealed a normal upper airway. A bronchoscopy showed significant trachea-bronchomalacia and thick purulent secretions in the left upper lobe, lingula, and right upper lobe.\nTwo days after admission, repeat chest X-ray revealed near complete opacification of left lung and large pleural effusion, a remarkably different radiograph from admission (Figure ).\nSubsequent CT chest revealed a large left pleural effusion with partial loculation as well as partial atelectasis of the left upper lobe and complete atelectasis of the left lower lobe. A right perihilar metastasis and perisplenic metastases were reported. The study was negative for pulmonary thromboembolism.\nThoracentesis revealed cloudy straw colored exudative effusion. A four French pigtail catheter was placed. Approximately 400 milliliters of yellow-green fluid was immediately drained. Pleural fluid studies revealed a white blood cell count of 33,000/μL (97% neutrophils), pH of 6.44, LDH of 4760 U/L, and an amylase of 394 U/L. She was started on vancomycin, cefepime, and metronidazole for presumed empyema. Pleural fluid cultures showed heavy growth of lactobacillus species, heavy growth of anaerobic gram negative cocci, and moderate growth of Candida krusei. Antimicrobial therapy was subsequently narrowed to ertapenem and anidulafungin. Given lack of improvement and continued significant chest tube output over the following week, further CT imaging was obtained, revealing a gastro-pleural fistula (via the left diaphragm and superior posterolateral stomach) with associated complex pleural effusion containing contrast material and gas (Figure ). This process abutted the known splenic metastases.\nAn esophagogastroduodenoscopy (EGD) revealed a 1.5-cm fistula in the posterolateral stomach that opened to the pleural space (Figure ).\nEndoscopic suturing was attempted to close the fistula with limited success (partial closure noted on imaging, with methylene blue dye taken via mouth visualized in the chest tube drainage catheter on water seal; Figure ).\nFor complete closure, the authors attempted a novel approach utilizing a venting gastrostomy tube and chest tube to water seal to facilitate closure of the fistula over the ensuing six weeks. Enteral feeding via jejunostomy tube to aid closure of the fistula was employed. The patient was continued on ertapenem and anidulafungin. She was also initiated on a proton pump inhibitor. She was discharged to a rehabilitation facility with plans to repeat imaging and methylene blue swallow in six weeks.\nUnfortunately, CT scans after six weeks showed that the fistula remained patent. A second attempt was made at endoscopic closure, which was again unsuccessful. One month later, during a hospitalization for electrolyte abnormalities, the patient decided to pursue elective surgical repair of the fistula in hopes of regaining the ability to resume normal oral intake. Four months after her initial presentation, she underwent laparoscopic surgery for fistula repair. The surgeon visualized extensive radiation fibrosis involving the stomach, spleen and retro-peritoneum. Given these findings and to avoid splenic bleeding, they pursued a conservative surgery whereby they stapled the stomach to ligate the gastro-pleural fistula anatomically. This approach is novel and was successful in our patient. A fluoroscopic upper GI series with oral contrast three days after surgery demonstrated no leakage of contrast outside of the GI tract or into the pleural space, and CT five days after surgery revealed no evidence of communication between the stomach and pleural space (Figure ).\nShe tolerated an oral diet. Gastrostomy tube, jejunostomy tube, and chest tube were removed without complication. |
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 73-year old male patient with pain in the right shoulder joint and acromioclavicular joint was admitted via this hospital's outpatient rehabilitation clinic. He had received spinal fusion of L5 and S1 vertebrae 10 years ago for herniation of an intervertebral disc, and was on medication for hypertension diagnosed 10 years ago. He had no history of other medical conditions such as diabetes, tuberculosis, and hepatitis. Further, he had no family history of these particular findings and he had no history of a recent injury. He was a farmer.\nA year before the visit to this hospital, he had received several sessions of acupuncture treatment for pain in his right shoulder joint at an oriental medicine hospital. As the pain persisted despite the treatment, he received several sessions of physical therapy, and drug therapy including injections for several months at a nearby orthopedic hospital. For two weeks before the visit to this hospital, pyrexia, skin flare, and edema developed in his right shoulder joint and acromioclavicular joint area with pain radiating from the right shoulder joint to the acromioclavicular joint. Upon presentation to this hospital, his vital signs were stable. However, during a physical examination, a tender point was detected by palpation in the right shoulder joint and acromioclavicular joint.\nThe patient had a pain score of 9 out of 10 on the visual analogue scale, and the pain worsened through the night. The movement range of the right shoulder joint was limited to 120° (flexion), 90° (abduction), 40° (external rotation), and 45° (internal rotation), but the range was normal for extension and adduction. Obtaining an accurate assessment of the manual muscle test was impossible due to the pain; however, the results of the sensory function and deep tendon tests were normal. The results of Lhermitte and Spurling tests were negative, and the empty-can test, lift-off test, and Hawkins-Kennedy tests could not be performed due to the pain.\nAt a routine blood test performed upon presentation to this hospital, the white blood cell level was 6.62×103/µl indicating normal differentiation. Red blood cell sedimentation rate and C-reactive protein level were both high (52 mm/h, and 1.2 mg/dl, respectively). No abnormal findings were observed in the other biochemical tests. The results of the ANA, anti-dsDNA, VDRL, RA factor, HLA-B27, and HIV tests, together with the sputum culture test, acid-fast bacillus stain, acid-fast bacillus culture test, nontuberculous mycobacterial test, and mycobacterium tuberculosis polymerase chain reaction test were all negative.\nA simple radiography of the right shoulder joint showed a narrowed acromion, sclerotic changes, and bony erosion of the acromioclavicular joint (). A bone scan using 99mTc DPD showed an increased uptake of radioactive isotope in the humeral head and acromial area of the right shoulder joint ().\nUltrasonography showed the shadow of a large amount of heterogeneous cystic tissue extending from the right subacromial bursa to the right humeral head area (). On ultrasonography, the right supraspinatus muscle and biceps brachii muscle were not observed. To decompress the enlarged cystoma, 33 cc of cystic fluid was suctioned using a 50 cc syringe under the guidance of ultrasonography.\nThe cystoma fluid was a red mucus containing yellow precipitate. Cytology showed that the red blood cell level was 43,200/ul, white blood cells was 22,000/dl, multinuclear white blood cells were 55%, and lymphocytes were 45%. Candida parapsilosis was detected in the culture test.\nMagnetic resonance imaging of the right shoulder joint showed a large amount of effusion, diffuse synovial hypertrophy, contrast enhancement in the shoulder joint, a change in signal intensity, and contrast enhancement in the bone marrow of the acromioclavicular joint (). The supraspinatus muscle was not observed in the subacromial space; muscles and ligaments of the long head of the biceps brachii muscle were not observed in the humeral head area. Local change with high signal intensity in the bone marrow and contrast enhancement were observed in the humeral head ().\nAt day 17 of admission, the patient was referred to the orthopedic department where he received arthroscopic irrigation and debridement of the right shoulder joint. During surgery, rupture and pus were found in the biceps brachii muscle and supraspinatus muscle. PCR for Mycobacterium tuberculosis was negative but Candida parapsilosis was detected in the pus culture test.\nAfter intravenous administration of fluconazole 400 mg, the symptoms improved. Three weeks after administration, the VAS score was 1. A biochemical blood test performed one month after the admission showed that the red blood cell sedimentation rate and the C-reactive protein level had decreased to 32 mm/h and 0.2 mg/dl, respectively, without particular symptoms or pain. The patient is now on follow-up. |
A 53-year-old woman referred to our department with the complaint of clear watery discharge from the right nostril. She gave a history of head trauma due to car accident 6 years ago that underwent surgery for the evacuation of right temporal intraparenchymal hematoma.\nShe suffered from intermittent rhinorrhea starting 5 years after trauma which had lasted for 1 year and had been continuous for the previous 3 months. She had two bouts of meningitis after rhinorrhea that was treated conservatively in a different hospital.\nShe had no anosmia, and other neurological examinations were normal.\nRoutine biochemical and hematological investigations were within the normal range.\nThe image findings of axial brain and coronal sinus computed tomography (CT) scans were evidence of previous right temporal craniotomy and adjacent parenchymal changes. CT cisternography, after intrathecal injection of 20cc Visipaque (VISIPAQUE Injection 270 mgI/ml, 20 ml, GE Healthcare, Norway), did not show bony defect on the anterior cranial fossa or detectable contrast leakage into the paranasal sinuses and nasal cavity (not shown).\nBeside the mentioned findings, coronal T2-weighted magnetic resonance images depicted the high signal intensity area in favor of encephalomalacia in the left inferior temporal region associated with fluid signal in the left tympanic cavity and mastoid air cells []. It was the only clue to reassess the axial brain CT scan which revealed partial opacity of left mastoid air cells [], and further evaluation with coronal images of the petrous bone which depicted large bony defect of the left tegmen tympani, tegmen mastoideum associated with opacity in the middle ear cavity, and lateral displacement of the ossicles [].\nThe patient suspected to have paradoxical CSF rhinorrhea through eustachian tube from the defect of left temporal bone.\nFor further documentation, she underwent endoscopic transnasal examination after intrathecal injection of fluorescein dyes, which showed leakage of fluorescein, from left eustachian tube to the nasopharynx.\nThe patient underwent surgical repair of leakage through transmastoid approach.\nThe patient is placed in a lateral decubitus position, and a curve line incision behind the mastoid was performed. A wide mastoidectomy is performed and repair of the floor of middle fossa with fascia and autograft bone, and eustachian tube closure was done extradural. |
A 45-year-old gentleman with a known case of diabetes mellitus, hypertension and dyslipidemia was admitted with complaints of fever, headache, cough and dyspnea. Nasal and oropharyngeal swab for the real-time polymerase chain reaction (RT-PCR) test for SARS-CoV-2 came out positive. Chest X-ray showed accentuated bronchovascular and perihilar marking with multifocal patchy ground-glass opacities in the bilateral lung field. He was hospitalized with severe COVID-19 pneumonia; his respiratory status deteriorated rapidly for 3 days since admission and required high oxygen and intubated ventilation due to respiratory distress. During his ICU course he developed sepsis and required broad-spectrum antibiotics. He had acute kidney injury for which he required one session of dialysis and improved with medical treatment. He started per rectal bleeding and was managed conservatively with input from the gastroenterology team. He had high troponin and the cardiology team was consulted and their opinion was of type 2 myocardial infarction secondary to sepsis and managed conservatively. Echocardiography showed a normal ejection fraction of 56% without any left ventricle clot. He was successfully extubated after 14 days. He developed critical illness myopathy after the prolonged ICU course.\nAfter one month's stay in the intensive care unit (ICU) with recovering from critical COVID-19 infection, the patient developed of sudden onset of bilateral painless loss of vision for 1 day. On examination right eye had perception of light only and the left eye with the counting of fingers at 1 meter with normal intraocular pressure. Right fundus showed severe non-proliferative diabetic retinopathy (NPDR), Weiss ring floaters, peripheral flat retinal break and peripheral degeneration whereas the left eye with severe NPDR, peripheral degeneration which could not explain his sudden vision loss. MRI head diffusion-weighted image (DWI) showed a tiny right deep frontal acute ischemic stroke (as shown in Figure ). Magnetic resonance angiography (MRA) head and neck vessels with gadolinium showed tapering of the proximal segment of the left internal carotid artery after carotid bifurcation followed by distal occlusion with absent flow and refilling of the terminal segment through reflex circulation (as shown in Figure ).\nMRI orbits with gadolinium axial view of T1-weighted image (T1WI), T2-weighted image (T2WI), T1WI with gadolinium showed distal intra-orbital optic nerve sheath faint enhancement (as shown by red arrows in Figure ), however, no extension into pre-chiasmatic or proximal intra-orbital segments and normal appearance of the uveoscleral coat and normal muscle cone with no intra- or extra-conal abnormality (as shown by red arrows in Figure ).\nMRI orbits fat-suppressed T1-weighted image with gadolinium coronal view showed distal intra-orbital optic nerve sheath faint enhancement and appearance of doughnuts like shape and normal appearance of the uveoscleral coat and extraocular muscles (as shown by red arrows in Figure ).\nMRI cervical and thoracic spinal cord with gadolinium was unremarkable for any detectable intermedullary signal abnormality. Doppler ultrasound carotid artery showed (0.4x 0.2 cm) calcified thrombus in the proximal right internal carotid artery just at the level of the bifurcation with turbulent flow with no significant stenosis and good distal run-off. In the left proximal internal carotid artery (ICA), an echogenic thrombus (0.4 x 0.2 cm) with no significant stenosis was detected and normal arterial flow with good distal run-off.\nRoutine laboratory results showed high ferritin, CRP, lactate dehydrogenase (LDH), IL-6, urea, creatinine, troponin- T, N-terminal pro B-type natriuretic peptide (NT-pro BNP), high creatine kinase (CK), alanine transaminase (ALT), aspartate transaminase (AST), high glycated hemoglobin (HbA1c), normal thyroid-stimulating hormone (TSH), vitamin B12, negative antinuclear antibody (ANA), negative antineutrophil cytoplasmic autoantibody (ANCA) and negative anticyclic citrullinated peptide antibody. Lumbar puncture showed slightly high protein with normal white cell count. Patient had negative CSF oligoclonal bands, negative for myelin oligodendrocyte glycoprotein (MOG-IgG) antibody. Viral meningitis PCR panel including herpes simplex virus (HSV) 1, 2 was negative and no growth of CSF cultures and autoimmune vasculitis panel were negative (as shown in Table ).\nAfter excluding secondary etiologies, finally, the patient was diagnosed to have bilateral optic nerve perineuritis probably due to COVID-19-related inflammatory changes. He was started on IV methylprednisolone pulse therapy for 3 days and he showed dramatic response within a week. His vision improved to counting fingers at 1 meter on the right eye and complete recovery of the left eye. Hence, he continued a steroid course orally of 60mg daily for 2 weeks and after slowly tapered weekly with a 10mg dose. He was transferred to the rehabilitation center for the critical illness myopathy physical therapy and follow-up neuro-ophthalmology clinic after 3 months. |
We present the case of a 48-year-old male, who was evaluated by the medical genetics service because he had noticed weakening of his voice with a high pitch since age 35, associated with premature graying since his 30s and skin lesions since about the age of 40. At the age of 32, bilateral cataracts were diagnosed and at 44 he was diagnosed with diabetes mellitus, currently on oral hypoglycemic agents. Additionally, he has hypothyroidism and hypertriglyceridemia in management and calcification of the Achilles tendon. Patient endorses lack of an early adolescent growth spurt; however, final stature is similar to his other 3 siblings (164 cm). Patient reports he had no child by choice.\nPatient is product of the union of consanguineous parents (second cousins) and has a 49-year-old brother with similar clinical characteristics, including voice changes since the age of 28, bilateral cataracts at age 29 (subsequently presents complications from corneal ulceration and is currently legally blind), and premature graying since age 33, moreover, scleroderma-like skin changes since his 30s and diagnosis of type 2 diabetes mellitus at age 35. His brother also endorses no child by choice. No other complications such as atherosclerosis, dyslipidemia, hypertension, osteoporosis, or tumors were reported.\nUnfortunately, patient's brother and parents declined genetic testing. There are no other relatives with clinical suspicion of WS.\nPatient states maternal aunt has unspecified type leukemia and father with a history of acute myocardial infarction at age 65 and a diagnosis of melanoma at age 85. Maternal uncle diagnosed with lung cancer at age 72 and maternal grandfather with prostate cancer diagnosed at age 73.\nOn initial physical examination, he appeared much older than his age with “bird-like” facial appearance, beak-shaped nose, and bilateral cataracts, his voice was high-pitched and his hair and eyebrows were scarce and markedly gray. He had thin upper limbs with decreased subcutaneous fat and truncal obesity (). Moreover, we found short stature, hypogenitalism, lower limbs with markedly atrophied skin and subcutaneous fat, abnormal pigmentation of the skin and hyperkeratosis, and flat feet (Figures and ).\nWRN gene sequencing identified the homozygous variant NM_00553.4: c.2581C>T (NP_000544.2: pGln861Ter). WRN gene sequencing report can be found in Supplementary . This variant generates a stop codon at position 861 and has been classified as pathogenic and previously described in homozygous status in a Caucasian patient from the United States in 2006 [].\nLaboratory findings included normal renal function, high blood glucose (164 mg/dl), elevated glycosylated hemoglobin (9.4%), and elevated triglycerides (324.6 mg/dl) with normal cholesterol (162.4 mg/dl). EKG showed an elevation of the J point by early repolarization. Abdominopelvic CT-scan showed bilateral renal cysts, small umbilical hernia, and no fatty liver. Testicular ultrasound showed decreased bilateral testicular volume mainly left side.\nRegular screening for malignancies is recommended for patients with WS, due to the high risk of early-onset neoplasms. Also, it is very important to rule out cardiovascular and metabolic diseases during the follow-up of these patients. Our patient is still under periodic clinical observation and follow-up. Currently, he is on treatment with oral hypoglycemic agents for DM2 with adequate glucose control and in treatment of hypertriglyceridemia. Until now no signs of atherosclerosis or cardiovascular disease have been detected. However, he was recently diagnosed with refractory cytopenia with multilineage dysplasia, a form of myelodysplastic syndrome, which has required multiple transfusions.\nAccording to a clinical history, the patient's brother is being monitored for inadequate control of diabetes mellitus and severe skin lesions that have been difficult to treat, but no cancer has been documented. |
A 38-year-old Caucasian woman, 35 weeks into her first pregnancy, presented to the emergency department for acute right-sided hip pain which precluded weight-bearing. Her right leg was shortened and externally rotated - there was no bruising or evidence of trauma.\nThe patient’s history was significant for hereditary thrombophilia (Factor V Leiden) and secondary anemia. Hip radiography revealed an unstable, displaced, right-sided femoral neck fracture with no evidence of osteonecrosis (Figure ). The decision to administer radiography, in this case, was based on the American College of Radiology guidelines, which cite an absence of in-utero deterministic effects of ionizing radiation effects after 27 weeks of gestation. Unfortunately, it was not possible to evaluate the symptoms of the patient with MRI at this time due to the coronavirus disease pandemic-induced stress on the healthcare system of our country.\nThe patient denied falls or trauma during the pregnancy, nor was there any history of smoking, alcohol abuse, use of glucocorticoids, or presence of rheumatologic/oncologic disease. Additionally, the patient was not malnourished, she underwent routine antenatal care, and took multivitamins. Serologic tests for inflammatory markers, as lab tests for serum calcium, phosphate, alkaline phosphatase, parathyroid hormone, vitamin D, and D-dimer returned normal.\nDuring multidisciplinary rounds, it was decided that delaying surgery was the best course of action out of fear of causing either mechanical or fluoroscopy-induced damage to the fetus during total hip arthroplasty. Five days later the patient experienced premature rupture of membranes, which was managed with emergency cesarean section (C-section) - no complications were encountered and a healthy 2300 g female was successfully delivered. Three days later the patient was transferred to our orthopedic surgery department for the treatment of the fracture. The significant degree of displacement (grade IV) of the fracture lasting over one week precluded open reduction with internal fixation due to fears of femoral head necrosis. During our literature review, we encountered a similar case of femoral neck fracture with grade IV displacement that was treated with open reduction internal fixation - despite restoration of blood flow to the femoral head within 15 hours, the authors still encountered femoral head necrosis with collapse six months later []. Given the considerable delay between symptom presentation and treatment, we decided the case warrants total hip arthroplasty instead of native hip salvage. Hemiarthroplasty was considered but was ultimately discarded as the conversion rate to total hip arthroplasty in young patients remain relatively high and the fracture was subsequently treated with a total uncemented prosthesis (Figure ), consisting of a 50 mm cup with 32 mm ultra-high-molecular-weight polyethylene insert and a 32 mm head with a 4 mm ceramic insert (Link Inc., Hamburg, Germany). Postoperative radiography confirmed prosthesis placement (Figure ); antibiotic and anticoagulant prophylaxis was initiated with ampicillin/sulbactam and enoxaparin sodium, respectively.\nThree days after the intervention the patient developed moderate abdominal pain without fever and accelerated intestinal transit, which both worsened over the next four days. Given the clinical presentation, there was a high index of suspicion for infection with Clostridium difficile (C. diff), although the diagnosis was ambiguous as the enzyme immunoassay (EIA) for the C. diff-specific antigen glutamate dehydrogenase was positive, while EIA for exotoxin A and B were negative. Due to exacerbation of symptoms and development of moderate hypokalemia (2.9 mEq/L), empiric treatment with metronidazole was began and marked rapid improvement. The patient was discharged five days later. |
In 2018, a Caucasian 54-year-old woman presented to our institution of Nuclear Medicine for a breast cancer with bone metastases. She reported fulvestrant therapy and ten cycles of radiotherapy and she also reported the beginning of zoledronate therapy (i.e., zometa) in June 2015, which was suspended in June 2017 due to the appearance of bone exposure, after the dental extraction of the upper right first molar.\nThe intraoral clinical examination showed an area of exposed necrotic bone, with oroantral communication associated with suppuration and pain. The computed tomography (CT) scan showed how ONJ process involved the entire upper right maxilla, and the ONJ was classified as Stage 3 (SICMF-SIPMO staging system). The patient underwent medical treatment based on the administration of ampicillin and sulbactam (1 g i. m. 2 × daily for 7 days) and metronidazole (250 mg per os 2 × daily for 7 days) as well as the use of antiseptic mouthwashes (chlorhexidine 0.2%, 30 ml swished up to 60 sec, 3 × daily for 7 days). Initial antibiotic therapy for ONJ is an ineffective conservative treatment because ONJ was classified as Stage 3.\nAt Stage 3 of the disease, most antibiotic regimens fail without appropriate surgical treatment.\nIn February 12, 2018, a bone scan with 99mTc-methylene diphosphonate confirmed neoplastic bone lesions. The technetium polyphosphate 99mTc scan demonstrated increased 99mTc accumulation in areas of increased blood flow and new bone formation secondary to the infection [].\nSince the patient refused any treatment and taking into account the bone disease, our multidisciplinary team evaluated a supplementary strategy with a possible bone-targeted agent targeting bone metastasis with Ra223. Additional goal was to select a therapy aiming to maintain the quality of life to avoid a new refuse of the patient for the therapy proposed. Based on Phase 2 clinical data, we decided to propose the off-label use of the radiopharmaceutical Ra223.[] The patient was instructed about the risks (as expected adverse events) and potential benefits of the therapy, the off-label use, as well as required precautions to be taken after Ra223 administration. A complete blood count and chemistry profile ensured that the patient was eligible for Ra223 therapy; subsequently, the agreement on informed consent for the off-label use was obtained. Hospital administration approved the authorization for the off-label use of Ra223 at September 2017. A total of six treatments were planned with a dose of 55 KBq/kg every 4 weeks according to Phase 2 data; the first administration of 3300 KBq of Ra223 was given on March 13, 2018, and a deposit area of radium 223 was detected in ONJ [].\nOn September 29, 2017, CT scan shows evidence of necrosis [].\nOn May 3, 2018, the patient presented for examination with a self-extracted necrotic bone fragment []. Oroantral communication remained in the absence of algic symptomatology or suppuration.\nThe weather radium 233 causing to self-bone extraction is about 50 days, after two administrations of Ra223. |
A 59-year-old female patient presented to a plastic surgery clinic with a lump on her right medial thigh. She noticed the lump eight months ago and it was gradually increasing in size. It was painless initially; however, the lump gradually became tender. The patient was otherwise fit and well with no co-existing morbidities. Closer examination of the right thigh revealed a high consistency mass, with reduced mobility. There was no right inguinal lymphadenopathy or lymph nodes enlargement.\nLaboratory blood tests were unremarkable. An MRI scan was requested to further assess the mass, which was irregular but well-defined in the medial compartment of the thigh, measuring 8.2 x 6.6 x 4.3 cm in size. The mass showed an intermediate signal on both T1 and T2-weighted imaging, appearing hyperintense compared to the adjacent muscular tissue, which also persisted on fat-suppressed imaging. The mass invaded the intramuscular fat planes and caused compression and displacement of the adductor muscles. There was no evidence of intra-muscular invasion. Normal cortical outline and medullary signal intensity of the femur were seen in the right thigh. There was no evidence of bone contusion, marrow oedema, fracture line, or cortical discontinuity. Other muscles of the thigh had a normal outline and signal intensity with no evidence of focal or diffuse oedema. Neurovascular structures were unremarkable. Overall, MRI findings were suggestive of soft tissue neoplasm, with a possibility of a neurogenic tumour. A wide excisional biopsy was then performed to further assess the tissue histologically by the pathologist and confirm the diagnosis. The removed mass measuring 8.5 x 6.5 x 5.4 cm and is shown in Figure . The mass had a smooth outer surface and a soft consistency. The cut surface showed a homogenous tan-white appearance with some slit-like spaces and occasional haemorrhagic spots. Multiple sections were processed for histopathological examination from different planes.\nHistopathology of the mass revealed a tumour arranged in sheets and fascicles composed of round to oval cells along with variably sized adipocytes (Figure ). Numerous interspersed lipoblasts with indented nuclei were also seen. Moreover, severe nuclear atypia was noted including bizarre cells (Figure and Figure ). An area of necrosis was seen with a few areas, which showed malignant and fibrous histiocytoma-like features. Furthermore, a few thin-walled dilated and congested blood vessels were also noted. Also, brisk mitosis was noted (35-38 per 10 HPF) (Figure ). Immunohistochemical staining (IHC) was also performed and the tumour cells were diffusely positive for vimentin and focally positive for S-100; while negative for creatine kinase (CK), smooth muscle antigen (SMA), desmin, CD34 and MyoD1. Overall, the pathological findings are all suggestive features of a high-grade sarcoma, favouring the epithelioid variant of PLS.\nThe patient was also referred to the Department of Nuclear Medicine & Molecular Imaging for post-surgical restaging and assessment for possible metastases using 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) scan. The abdominopelvic section showed the liver measuring within normal limits; however, the scan revealed an ill-defined hypermetabolic hypodense lesion seen involving segment VI measuring approximately 2.8 x 1.8 cm suggestive of hepatic metastases with concurrent fatty liver. The scan was also remarkable for the musculoskeletal system, revealing multiple hypermetabolic osseous lesions involving the skull base, right iliac bone, and few vertebrae (predominantly T7-L1); which was suggestive of skeletal metastases. A non-hypermetabolic pulmonary nodule in the right lung field was also detected; it was considered to be likely benign, but interval scanning is suggested to monitor further progress. Finally, there was also evidence of diffuse subtle hypermetabolism at the site of surgery which is suggestive of postsurgical inflammatory sequelae; however, microscopic residual disease cannot be completely ruled out. These multi-organ lesions were due to be biopsied to examine their immunohistochemical findings to confirm metastatic lesions of primary pleiomorphic liposarcoma. However, the patient did not attend the follow-up appointments. |
This study discusses the case of an 86-year-old man who was admitted to hospital for a three-week history of increased blurring of vision, vertigo on head movement and worsening cognition. On admission, his Montreal Cognitive Assessment score was 13/30 which was abnormal for him, as there was no previous history of significant cognitive impairment. Examination of the cranial nerves revealed horizontal nystagmus upon left lateral gaze as well as horizontal binocular diplopia, and incomplete abduction of his left eye. Initial visual field assessment showed a left inferior quadrantanopia. There were no motor or sensory deficit across all four limbs; however, the patient was noted to have left upper limb dysdiadochokinesia with past-pointing, pronator drift, and an ataxic gait. There was no reported headache or loss of consciousness.\nThe patient reported no history of recent illnesses. His past medical history included ischaemic heart disease, mild vascular dementia, hypertension and gastro-oesophageal reflux disease. The patient had no significant family history, minimal alcohol consumption and was an ex-smoker several decades ago. There was no history of foreign travel to indicate an infective cause for symptoms. His pre-admission medications were aspirin, amlodipine, atorvastatin and isosorbide mononitrate.\nFull blood count, and urea and electrolytes were found to be unremarkable. Liver function tests and serum ammonia were also within normal limits. Clinically, this patient was diagnosed with a posterior circulation stroke and as such, his antihypertensives were reduced to avoid hypoperfusion during his stroke disease.\nHe subsequently received a CT of the head that revealed no acute intracranial infarction or haemorrhage; however, the occipital lobes showed diffuse and symmetrical low attenuation changes predominantly affecting the white matter (Figure ). This imaging was able to provide a provisional diagnosis; however, it was later confirmed with MRI. MRI returned high signal on T2 and fluid-attenuated inversion recovery (FLAIR) sequences, which extensively and bilaterally affected the grey and white matter from the trigones of the lateral ventricles to the posterior occipital lobes (Figure ). This represented widespread posteriorly located oedematous changes. Numerous hyperintensities were visible in the deep white matter representing established small vessel ischaemic changes (Figure ).\nTwo days post-admission, the patient described progressively worsening visual symptoms and was found to have developed a left superior quadrantanopia whilst complaining of visual hallucinations. The patient described seeing hair on the bed and holes in the wall; however, fundoscopy did not reveal any intraocular pathology. His blood pressure was mildly elevated at 150 mmHg systolic on average, with periodic increases in excess of 180 mmHg systolic during his admission.\nAs the patient’s blood pressure was only mildly elevated despite withholding antihypertensives, they were not recommenced. However, despite further investigations and lack of evidence to support any precipitating cause for his development of PRES, mild hypertension was isolated as the sole potential cause of his symptoms. Subsequently, the patient received increasing titrations of amlodipine and additional ramipril with aim to reduce the blood pressure and alleviate his symptoms.\nFollowing the increased dose of antihypertensives, his symptoms started to resolve and following a short admission, in which he also received rehabilitation from allied health professionals, he was discharged. He was subsequently maintained on a higher dose regimen of antihypertensives for life to avoid future complications. Two weeks post-discharge, his symptoms had resolved and his blood pressure had stabilised at an average 119/68 mmHg. |
A 66-year-old man with a past medical history significant for GERD, hypothyroidism, previous head and neck cancer status, post-resection history chemotherapy, and radiation presented to the hospital for evaluation of headaches. The patient reported waking from sleep with a pounding headache two to three times per month over a 6-month period. The headaches were associated with palpitations, dizziness, and diaphoresis. He denied any complaints of blurred vision, slurred speech, weakness, numbness, or tingling of his extremities, nausea, and vomiting. He reported no complaints of shortness of breath, cough, nasal congestion, fatigue, or diarrhea. His pain was reportedly resolved by two to three tablets of ibuprofen 200 mg. While his headaches did not significantly impact his activities of daily living, they did prompt discussion with his primary care physician. An outpatient MRI brain was performed, which revealed findings suspicious of CVST. Thus, he was sent to the emergency department for further evaluation.\nThree months prior to admission, the patient was tested for SARS-CoV-2 virus infection from a potential exposure at work. He tested positive and exhibited symptoms of cough, headache, and nasal congestion. He never exhibited significant shortness of breath or pleuritic chest pain. His symptoms improved with basic medical management. On his current admission, he was again tested for COVID-19 and tested positive. He denied a history of smoking, alcohol abuse, or recreational drug use. He had a past medical history of cancer on the base of the left tongue involving the left tonsils and left neck lymph nodes which was treated by surgery, radiation, and chemotherapy 11 years prior. He never presented any signs or symptoms of recurrence. Family history was not significant. His body mass index at the time of admission was 28 kg/m2, vital signs were within normal limits, and he was saturating well on room air. Physical examination including complete neurological examination was within normal limits. CBC revealed hemoglobin of 12.3, BMP revealed mild elevation of potassium was 5.2 mg/dl, TSH was within normal limits. His IgG, IgM, and IgA beta-2 glycoprotein antibodies were all within normal limits. IgG, IgA cardiolipin antibodies were within normal limits, but IgM was mildly positive at 21. Factor V Leiden and prothrombin gene mutations were negative. On telemetry, the patient had a pattern of bigeminy and trigeminy. He had no complaints of chest discomfort; there were no signs of ischemia, and a subsequent two-dimensional echo was within normal limits. CT brain imaging without contrast revealed no acute intracranial hemorrhage, midline shift, or mass effect but showed parenchymal volume loss, which was a probable sequelae of chronic small vessel ischemic change, as expected for age of the patient. There was no evidence of recurrence of his known cancer. MRI brain findings showed an abnormal FLAIR signal with loss of flow void in the distal left transverse sinus, sigmoid sinus, and jugular bulb (). This was followed by a CT venogram, which revealed a filling defect in the left sigmoid sinus, jugular bulb, and visualized left internal jugular vein compatible with dural venous sinus thrombosis (). He was started on intravenous heparin for dural sinus venous thrombosis. Prior to discharge, he has transitioned to Rivaroxaban 20 mg with anticipated treatment lasting 6 months. His symptoms had resolved at the follow-up appointment 3 months later. |
The patient is a 34-year-old G5P3 woman who had gynecologic assessment for an enlarged right ovarian cyst suspected to be a dermoid, measuring 10.3 cm × 10.1 cm × 9.3 cm in size on ultrasound. Surgical management of the cyst was chosen, and workup included a negative serum beta-human chorionic gonadotropin, and her Ca-125, Ca-19, carcinoembryonic antigen, alpha-fetoprotein (AFP), and lactate dehydrogenase (LD) were all within normal boundaries. The patient had a history of a previous incomplete laparoscopic ovarian cystectomy that was stopped prematurely due to surgical complications, although records of this could not be found. She was an occasional smoker. The patient was otherwise healthy.\nThe patient underwent her initial operation which was a laparoscopic bilateral salpingectomy and cystectomy of a large right dermoid cyst, and a left cystectomy of a small simple cyst. Upon examination of the pelvis, a 2 cm cyst was noticed on the left ovary, and on the right ovary a 12 cm dermoid-appearing cyst was seen sitting within the cul-de-sac. The uterus, fallopian tubes, and remaining pelvis were otherwise normal in appearance. The cystectomy began on the left side, and the small cyst was excised without complication. On subsequent dissection of the right ovary away from the cyst capsule, there was rupture with intra-abdominal sebaceous spill. After this took place, another dermoid cyst could be appreciated and was excised before managing the spill. The abdomen was then copiously irrigated using approximately 25 L of normal saline, with the patient in both regular and reverse Trendelenburg positions for complete lavage of all sebaceous material. Ovarian cyst walls and both tubes were sent for pathological analysis. Proper hemostasis of the right ovary was obtained and the blood loss was minimal. The patient tolerated the procedure well, was sent stable to recovery and was discharged home the same day.\nTwo days later, the patient re-presented with concerns surrounding her incisions. On inspection, her laparoscopic sites were healing well, and hemostasis was noted. However, the patient was experiencing significant pain out of keeping for her postoperative course. She was passing flatus, with no nausea/vomiting or abdominal distention. A pelvic ultrasound showed an 8.0 cm × 7.0 cm × 4.7 cm complex mass-like lesion in the right adnexa, initially suspicious for hematoma within the ovary as a result of surgery. The patient was admitted overnight for pain management and monitoring. The pain resolved and the patient was discharged home the following day with a plan for outpatient follow-up and reimaging.\nThe patient then presented again 1 week later with worsening de novo right lower quadrant pain. Doppler ultrasound showed an enlarged right ovary (7.2 cm × 5.2 cm × 6.2 cm) as well as a swirling vessel sign, suggestive of OT. Upon consideration of the patient's clinical status, she was taken back to the operating room (OR) for urgent laparoscopy. Examination revealed numerous filmy adhesions between the greater omentum and rectosigmoid colon, consistent with remnants of the sebaceous material from the prior ruptured dermoid cyst. Inspection also revealed a torsed and necrotic right ovary that did not appear salvageable by detorsion. After a repeated round of irrigation for the removal of residual sebaceous material, the surgeons proceeded with a right oophorectomy. There were no complications and there was good hemostasis. Of note during this procedure was a lengthy infundibulopelvic ligament on the right side. There were no other abnormalities in the abdomen. The patient was stable throughout and after surgery, was taken to the recovery room. She was discharged on postoperative day 1, as the patient was eating and voiding well, with pain well controlled on naproxen and acetaminophen with no need for narcotics. At her postoperative follow-up, the patient reported no concerns with no ongoing bleeding or pain. Her pathology results from her first operation were reviewed, which revealed a benign mature teratoma/dermoid. She did not require any further follow-up. |
A 64-year-old postmenopausal female, P5 L5A1, attended a gynecological clinic with a history of mucoid to foul-smelling white discharge per vagina for the past 6 months. Her previous menstrual cycles were regular, the perimenopausal transition was smooth, and she attained menopause 14 years ago. She was previously asymptomatic with no history of postmenopausal bleeding. She had five spontaneous vaginal deliveries and one termination of pregnancy (medical termination of pregnancy [MTP]). Her antepartum and postpartum periods were uneventful with five living issues and her last childbirth 35 years back. Thirty years back, she underwent MTP at 16 weeks by dilatation, evacuation, and history of excessive bleeding, requiring blood transfusion. No other history of past surgeries or tuberculosis. She is a well-controlled hypertensive on medication and coronary artery disease, for which she underwent percutaneous transluminal coronary angioplasty and stenting 5 years ago.\nOn examination, general physical examination was unremarkable. The abdomen was soft, with no mass or organomegaly. On gynecological examination, the vulva and vagina were atrophic. Per speculum revealed pus-like discharge with normal ectocervix. The uterus was enlarged to parous size, mobile with free fornices. Pap smear was inflammatory and negative for intraepithelial lesion or malignancy. Her ultrasonography revealed an enlarged uterus of 10.7 cm × 7.5 cm filled with linear, echogenic structure in the endometrial cavity up to the cervix with fluid around. The endometrial cavity showed evidence of pyometra. Differential diagnoses on ultrasound were suspected retained bones or dystrophic calcification. Magnetic resonance imaging done showed evidence of pyometra of 39 mm × 26 mm × 31 mm and bone-like echogenicity in the endometrial cavity. The patient underwent hysteroscopy, which revealed multiple pieces of shredded bony structures embedded in the endometrial cavity, all removed using forceps []. The endometrial cavity did not have any abnormal vascularity or hyperplastic changes. Endometrial sampling was done, and specimens were sent for histopathological examination.\nThe microscopic examination of the paraffin-embedded H and E sections showed woven bone in bony bits, and endometrial tissue showed foamy histiocytes with multiple tiny calcareous round basophilic bodies (Michaelis-Gutmann bodies) along with lymphocytes, hemorrhage, and fibrin []. Von Kossa and periodic acid Schiff (PAS) stain highlighted the basophilic bodies; Perl's stain highlighted the iron pigment []. Histopathological diagnosis of malakoplakia with osseous metaplasia was made. |
An otherwise healthy 15-year-old girl was referred by her clinician to our hospital for imaging of a mass in the right axilla. The mass was slowly growing over the prior 6 months. Physical examination revealed a 5 cm painless, nonmobile, palpable firm lesion in the right axilla. The girl denied tenderness, erythema, or fluctuance. The patient recalls no antecedent traumatic event and has no pertinent medical or surgical history. Her laboratory tests include white blood cell count, erythrocyte sedimentation rate, and C-reactive protein, all of which were normal. Frontal and lateral radiographic views of the chest were normal. MRI was subsequently requested to define the lesion location and to evaluate for any specific imaging characteristics that might propose a diagnosis. MRI showed a well-defined, well-circumscribed, 4 × 5 × 6 cm in diameter solid mass within the subcutaneous fat of the right axilla. The mass was slightly hyperintense to muscle on T1-weighted images () and homogeneously hyperintense to muscle on T2-weighted images (). There was marked diffuse homogeneous enhancement throughout the lesion following the administration of intravenous contrast (). The remaining subcutaneous fat and adjacent muscle were normal, without surrounding inflammatory changes. Although adjacent to the chest wall, the adjacent ribs were considered normal in morphology and signal intensity. Few normal-sized scattered lymph nodes were present in the axilla, but no lymphadenopathy was noted. The imaging characteristics of the mass were deemed nonspecific, and, because malignancy could not be excluded, tissue sampling was recommended.\nFollowing biopsy and frozen section analysis, the patient underwent full excision of the lesion. The excised mass was well demarcated, multilobulated, measuring 7 × 6.5 × 4.2 cm and weighing 75.9 grams. The cut surface was firm, grayish white, with focal gelatinous areas (). Upon microscopic examination, the mass was surrounded entirely by a fibrous capsule. The lesion was composed of islands and elongated lobules of mature benign hyaline cartilage with well-vascularized fibrous stroma (). The cartilage consisted of bland appearing chondrocytes in lacunae () with a hypercellular zone at the periphery of the nodules. There was no substantial cytologic atypia, mitosis, or necrosis. No calcification or ossification was present throughout the mass. Histopathologic findings were consistent with an extraskeletal or soft tissue chondroma. |
A 45-year-old Canadian man had been followed regularly at the local hospital because of a liver mass detected on abdominal ultrasonography and computed tomography (CT) scan four years previously. At the time of the first diagnosis, the liver had hypervascular features with a lobular and cystic portion seen on CT scanning, and which was regarded as a cavernous liver hemangioma. Three years later, he was transferred to our institute for further evaluation of his right upper quadrant pain. On the physical examination there was neither hepatomegaly nor a palpable abdominal mass. Routine laboratory tests including CBC, liver battery, and coagulation tests were within normal ranges. A serologic test was negative for both the hepatitis B virus surface antigen and hepatitis C virus antibody. Tumor markers such as α-fetoprotein, protein induced by vitamin K absence-II (PIVKA-II), carcinoembryonic antigen (CEA), and carbohydrate antigen 19-9 (CA 19-9) were also within a normal range.\nOn liver dynamic CT, the mass in segments IV and VIII, and measuring 6.5×5.3 cm was hypervascular and showed intratumoral cystic change and calcification. There was another enhanced mass measuring 2.3 cm in the right hepatic dome and that was thought to be a hepatic hemangioma. The large hepatic mass was enhanced on arterial phase imaging of liver dynamic CT scan, but was not washed out in either early portal phase or delayed phase imaging (). The liver mass was a well-defined, hypointense, solid mass on T1-weighted imaging and was hyperintense on T2-weighted imaging of MRI scan; there were multi-lobulated, cystic areas within the mass seen on MRI. Following enhancement with gadolinium-EOB-DTPA, the mass appeared hypervascular on the arterial phase and showed decreased enhancement on the delayed phase (). Besides, there had been a renal cortical cyst which size was increased from 1.4 cm to 3.0 cm during 4 years of followup, but it was regarded as a simple benign cyst by imaging study.\nA preoperative liver core biopsy was performed, because a definitive diagnosis was not made. Since a malignancy could not be completely excluded and the patient had presented with right upper quadrant pain, partial hepatectomy of segments IV and VIII was performed. The patient made an uneventful recovery.\nPreoperative liver biopsy showed multifocal proliferation of clear, cuboidal cells in a tubular and cystic structure and highly vascular edematous stroma. Because the clear cells were positive for cytokeratin and vimentin, metatstatic renal cell carcinoma was considered, although the tumor cells were negative for CD10 that is used as a marker for clear cell, renal cell carcinoma. The resected liver showed a solid mass measuring 7.5×6.5×3.7 cm and with a well-circumscribed border that was continuous with the Glisson's capsule and pushing the hepatic parenchyma. The mass was mostly solid, partly myxoid and hemorrhagic, and partly cystic with multiple, various sized cysts (). The cut surface of the solid portion of the mass was mostly whitish-yellow, partly reddish, smooth, and glistening. The cysts were filled with serous fluid (). Microscopically, the tumor showed proliferation of flattened or cuboidal epithelioid cells in tubular or glandular structures as well as highly vascular stroma with thin-walled blood vessels (). The epithelioid cells were diffusely positive for cytokeratin (AE1/AE3), vimentin, calretinin, and cytokeratin 5/6, were focally positive for CD10, and were negative for WT1 and CD34 (). These findings support the mesothelial differentiation of the tumor cells. Apart from this mass, there was an hepatic mass, measuring 2 cm in diameter and located in the right hepatic dome; the mass was a typical, cavernous hemangioma (). |
In December 2007, a 57-year-old female patient was attended in the clinic with alterations in behavior, lack of self-control, depression and anxiety. These symptoms had appeared 12 months previously. The patient, had already been evaluated in a psychiatric institution, had been diagnosed as having a mood disorder.\nOn clinical examination she was apathetic and little collaborative with pale skin. On abdominal palpation there was no evidence of visceromegalies. Her medical history included type 2 diabetes mellitus diagnosed in 2000 and controlled with oral hypoglycemic agents. Laboratory findings are shown in .\nA percutaneous needle liver biopsy to measure liver copper showed no accumulation of this element but showed marked iron overload; urinary copper excretion in 24 hours was normal. Hereditary hemochromatosis related to the HFE gene was ruled out because of the absence of the C282Y/H63D mutation.\nBrain magnetic resonance imaging (MRI) showed bilateral low signal on T2 at the basal ganglia probably due to iron deposits; similarly, MRI of the liver showed a significant iron deposit in the parenchyma.\nWhen the diagnosis of hereditary aceruloplasminemia was established, treatment was initiated with 20 mg/kg/day deferasirox for 10 months, during which time there was an improvement in the psychotic symptoms and a significant reduction in ferritin levels (from 1114 ng/mL to 457 ng/mL). The dose of deferasirox was reduced to 10 mg/kg/day and after six months the ferritin level had dropped to 107 ng/mL. A liver MRI in that period showed a reduction in iron deposits (). However, the brain MRI showed that the iron deposit in the basal ganglia remained almost unchanged compared to the first MRI performed before the initiation of treatment. Currently the patient is asymptomatic but taking 5 mg/kg deferasirox daily.\nThe second case, the 61-year-old sister of the previous patient was asymptomatic until that age and was also attended in the clinic for hematological screening.\nOn clinical examination, she reported suffering from cardiac arrhythmia and hypertension, both compensated and treated with amiodarone and valsartan, respectively. Abdominal palpation did not identify visceromegalies. Her laboratory findings are shown in .\nA percutaneous needle liver biopsy to measure liver copper showed no accumulation of this element however it revealed marked iron overload. The urinary 24-hour copper excretion was normal. The brain and liver MRIs were similar to those of the first patient.\nAfter diagnosis of aceruloplasminemia, treatment was initiated with 20 mg/kg/day deferasirox for 3 months with a notable decrease in ferritin levels (891 ng/mL to 410 ng/mL). The dose of deferasirox was reduced to 10 mg/kg/day for 6 months with further decreases in ferritin. Current treatment is with 5 mg/kg/day of deferasirox and the ferritin level is at 118 ng/mL. A liver MRI in this period, as opposed to the brain MRI (), showed reductions in iron deposits.\nFasting glucose remained at 100 mg/dL throughout the follow-up and the patient is so far asymptomatic. |
A 63-year-old woman presented with a 4-year history of myalgia related to statin therapy. Myalgia persisted more than 1 year after statin discontinuation in 2010. Muscle testing showed mild proximal weakness, and laboratory studies found elevated serum creatine kinase (1,400 IU/l, normal values <170 IU/l). An electromyography (EMG) identified a myogenic pattern, and magnetic resonance imaging (MRI) revealed inflammation disseminated in the gluteal and adductor muscles. A muscle biopsy did not show any particular signs, and capillaroscopy results were normal.\nIn January 2011, a diagnosis of polymyositis was made. Initially, a treatment with methotrexate (15 mg daily) and corticoids (30 mg daily) was started, and then the dose was progressively reduced. A transient clinical and biological improvement was observed. Recurrence of myalgia was reported in May 2011, with an increase in creatine kinase levels in August 2011. These circumstances led to the introduction of the IVIg treatment. The patient received a 6-month therapy of IVIg at 60 g monthly. A partial decrease in pain without significant clinical improvement was noticed.\nIn November 2011, the patient was referred to our clinic where a diagnosis of IBM was confirmed by muscle biopsy. The patient was able to move almost normally; however, precise movements were difficult to perform without help (i.e. to open a bottle). She had a poor appetite and had lost 4-5 kg during the last months. She was exercising daily to increase muscle strength, despite an increased asthenia and difficulties in climbing and descending stairs, and she had some problems in swallowing. She reported a diffuse arthralgia affecting particularly the joints in the hands and knees. The EMG confirmed a persistent myogenic pattern. MRI demonstrated an extensive high short-tau inversion recovery signal abnormality in the musculature of the adductor and fasci with fat replacement and amyotrophia. The muscle strength score was 65/88 (88 points corresponds to normal muscle power) and the creatine phosphokinase (CPK) level was elevated (216 IU/l).\nUpon the diagnosis of IBM, the patient was initiated on SCIg (2 g/kg/month) in March 2012. She received 18 infusions during 4.5 months (2 infusions/week). CPK levels remained above the standard levels (378 IU/l after 4 months of treatment). She reported mild headaches during the SCIg treatment, but the treatment was generally well tolerated. Despite an improvement in muscle strength (muscle strength score from 65 to 70/88), the treatment with SCIg was discontinued because of reimbursement refusal, and IVIg was thus reintroduced. |
On 16th March 2012, a 49-year-old female who experienced symptoms of exertional dyspnea and increased fatigue for 15 months presented at the Department of Cardiac Surgery, Xinqiao Hospital (Chongqing, China). The patient reported dyspnea, which developed following activity and sleeping in the supine position, which gradually worsened, without chest pain, heavy sweating and palpitations. The patient additionally reported poor sleep and a loss of appetite, although no marked weight loss. Two days prior to the the patient presenting at the hospital, the described symptoms worsened and the patient experienced bilateral edema in the lower limbs. Physical examination revealed cyanosis, an enlarged heart and a weakened cardiac sound. A transesophageal echocardiography demonstrated a right atrial mass originating in the inferior vena cava (IVC; size, 14×8 cm) that caused a tricuspid inflow obstruction and the abdomen ultrasonic examination demonstrated a mass in the lower right kidney. An emergency resection of the intra-atrial tumor thrombus was performed. Frozen sections obtained from the intra-atrial surgery revealed abundant necrosis with heterotypic cells. The intra-atrial tumor thrombus was tightly attached to the abdominal aorta, therefore, only a partial resection could be performed to relieve the tricuspid obstruction. Histological examination confirmed the diagnosis of a clear cell renal cell carcinoma, however, the patient’s symptoms had partially subsided following a month of recovery. The patient refused a right nephrectomy, and interleukin-2 and interferon-α therapy, as well as additional types of targeted therapy. The patient underwent intensity-modulated radiation therapy (56 Gy/24 fractions of one fraction per day, five days a week; from the IVC, where the mass initiated, to the iliac vein, which included the mass on the upper kidney). Following this, the patient refused further therapy. Six months following the radiation therapy, the symptoms had markedly improved, thus enhancing the patient’s quality of life; furthermore, the edema in the lower limbs had disappeared. On the 9th November 2012 and the 22nd February 2013, computed tomography (CT) indicated that the tumors on the kidney and IVC were stable (), however, additional metastatic lesions were identified in both lungs. Patient provided written informed consent. |
A 20-year-old female patient from the Middle East was admitted to our institute with a chief complaint of worsening dyspnea for the last 3 months (New York Heart Association class III). She had an episode of syncope with bilateral upper limb numbness a month ago. History was significant for surgical removal of left atrial myxoma in 2011 and 2015. Medical records of the previous cardiac surgeries were not available. Family history was negative for such illness in the siblings or parents. Her physical examination was unremarkable except for the presence of a previous sternotomy scar. Routine laboratory parameters and endocrine hormone levels such as thyroid hormones, adrenocorticotropic hormone, and growth hormone were within normal limits. Whole-body computed tomography (CT) scan was performed to exclude any other primary tumor. Non-contrast CT scan of the brain showed an old wedge-shaped infarct in the right temporoparietal region with gliotic changes. 2-D transthoracic echocardiography (TTE) showed a 20 × 19 mm hyperechoic mass in the left ventricular outflow tract (LVOT) adherent to a ventricular aspect of the anterior mitral leaflet (AML). The mass swung back and forth with each beat into the LVOT cavity. There was no LVOT obstruction, no aortic regurgitation, trace mitral regurgitation with normal ventricular function. The cardiac MRI reported an oval lesion in LVOT measuring 21 × 18 mm, suggestive of myxoma [].\nThe patient was taken up for surgical resection of the mass with a high-risk consent. Considering a re-redo surgery, necessary precautions were taken including the application of external defibrillator pads, availability of blood, and looping of the femoral vessel for emergent cardiopulmonary bypass institution, etc. Intraoperative transesophageal echocardiography (TEE) confirmed the findings of TTE. The mass was seen adherent to AML [] and was swinging back and forth into the LVOT cavity []. After sternotomy, aorto-bicaval cannulation, normothermic antegrade cardioplegia, a transverse aortotomy was performed. A translucent jelly-like myxomatous mass became visible through the aortic cusps. The pedicle of the mass was seen attached to the junction of anterolateral papillary muscle and its chordae. En-masse removal of the tumor was done carefully [] without causing its fragmentation, or any damage to the mitral valve apparatus. TEE evaluation confirmed the complete removal of the mass and trace mitral regurgitation. Histopathology examination of the specimen confirmed the diagnosis of myxoma. The postoperative course was uneventful, and a 6-month follow-up echocardiogram did not show recurrence of the tumor. |
A 67-year-old female with history of chronic tobacco use, chronic obstructive pulmonary disease, hypertension, and hyperlipidemia, presented to the ED with symptoms of TIA. The patient described the acute onset of left-sided facial weakness that waxed and waned, recurring several times throughout the day, and lasting 2–3 minutes at a time. The left facial weakness was also associated with mild, left-arm weakness and “clumsiness” involving fine motor function of her left hand. She noted lightheadedness but denied leg weakness, headache, visual changes, chest pain or shortness of breath. She also noted that symptoms were brought on by use of her upper extremities and when she changed her body position from lying to sitting. She denied any similar symptoms previously or stroke history. Of note, she noticed a rapid improvement in her symptoms to resolution just prior to ED presentation.\nOn examination, her blood pressure (BP) was 183/86 millimeters of mercury (mmHg). She was awake, alert, oriented, and able to describe a detailed history. Her cranial nerves were intact, motor strength was 5/5 bilaterally, and fine motor movements in both her hands were normal. There was no ataxia, extraocular muscle dysfunction, or indication of posterior circulation involvement.\nJust after her initial asymptomatic presentation to the ED, her symptoms recurred when her systolic BP dropped by 20 mmHg upon standing from a supine position. Emergent computed tomography angiogram (CTA) of the head and neck demonstrated a severe flow-limiting lesion of the innominate artery (). Further investigation with magnetic resonance imaging demonstrated decreased signal intensity within the right internal carotid artery at the cavernous sinus and petrous segments, a finding that potentially represented slow flow ().\nThe patient subsequently underwent emergent cerebral angiogram, which demonstrated occlusion of the proximal innominate artery () at the aortic arch with resultant left to right vertebral artery steal phenomenon supplying the right subclavian artery (). The distal brachiocephalic artery flow was reconstituted via the subclavian artery and secondary steal phenomenon occurred into the right common carotid artery, causing delayed flow to the right cerebral hemisphere ().\nThe patient was maintained on a norepinephrine bitartrate infusion to increase BP, and her symptoms subsequently resolved. The symptoms recurred when she was positioned supine, but upon being placed in the Trendelenburg position her symptoms again resolved. The patient was therefore maintained with systolic BP goals between 160 and 210 mmHg. She remained asymptomatic during this period of elevated BP management. For definitive care, she underwent elective left carotid to right carotid “necklace” bypass surgery with complete and permanent resolution of her symptoms. |
A 17-year-old female was admitted to the hospital due to severe suicidality. At the time of admission she complained about an irritating feeling in her nose, which made her constantly grimace in the area around the nose. She was excessively worried about having a serious illness of her nose (secondary hypochondriacal delusions) and was suicidal as a consequence. Her belief persisted even after any underlying medical condition of the nose has been ruled out by extensive medical examinations. She also presented with disorganized behavior, stereotypical movements, emotional instability and lability, and a below average level of intelligence during hospitalization. On the PANSS, her symptoms scored 29/23/70 (for the Psychotic, Negative and General Psychopathology Scale, respectively). Brief neurological examination revealed no abnormal neurological signs. As ascertained by the history taken from the patient and her mother, she had a history of school phobia that began at the age of 12 years, emotional disorders, normal cognitive and physical development, and a three-year history of chronic headache. She managed to complete primary and secondary education with the help of school counseling services given to her on account of school phobia. She had not received any psychiatric care before the described admission. A diagnostic evaluation for chronic headache at the University Children’s Hospital was undertaken a year before admission.\nCalcium, phosphate and parathyroid hormone blood levels were normal. Vitamin D levels were decreased with decreased calcium levels in the urine. No signs of calcium depositions in organs other than the described brain regions were determined by ultrasound. Ophthalmological, ear-nose-and-throat examination and electroencephalography were also normal.\nDetailed neurological examination revealed dysfunction of pursuit eye movement, dystonic positioning of both arms when stretched ahead, discrete ataxia of the arms and legs, and a pathological extensor response of the left big toe.\nBilateral symmetrical calcification in head, body and tail of the caudate nucleus and ventral part of the thalamus were determined by computerized tomography (Figs. and ). MRI was preformed twice over a two-year period using the same protocol. Corresponding MR T1 sequences showed hyperintense calcifications in the same regions as those found on CT examination (Figs. and ). Both MR examinations showed a high signal of calcified areas on T1 weighted sequences due to the surface area of calcium crystals. The same areas had an isointense signal on T2 weighted sequences. No other abnormalities of the brain were detected on the MRI.\nDuring psychometric evaluation, the patient’s cognitive abilities were assessed with RPM, TOL-II, d2, CTMT and Stroop tests. The patient performed significantly worse than her normative age group in terms of general cognitive abilities, coming in below the 10th percentile. She was unable to perform problem-solving operations that require abstract thinking. Assessment of her attention performance showed below-average results in scanning and alternating attention. She also showed below-average performance in her sustained and divided attention, with her concentration performance in the 8th percentile. Her planning abilities were significantly worse in comparison with her normative group, where she was unable to construct a problem-solving strategy. Her approach was a trial-and-error strategy and she failed to solve the problem within the time limit. The patient had no significant difficulties with inhibition of dominant response, reaching a borderline average result. We performed a retest after a year of treatment and the results showed no significant changes, although she was slightly better, but unfortunately without significant improvements, in her planning abilities and in her sustained attention.\nA whole blood EDTA sample was used for extraction of genomic DNA according to established laboratory protocols using the FlexiGene DNA isolation kit (Qiagen, Germany). Whole exome sequencing a trio (index patient and her parents) was performed in collaboration with NovoGene Corp. Inc. (Davis, CA, USA) using an Agilent Sure Select Human All Exon V6, 5191–4004 kit for whole exome enrichment preparation together with an Illumina Platform PE150 (Illumina, San Diego, USA) to perform the whole exome sequencing. Genetic variants with coverage >15x were analyzed using Variant Studio 3.0 software (Illumina). Evaluation of variants was firstly restricted to those located in eight genes related to Fahr’s disease (SLC20A2, PDGFRB, PDGFB, XPR1, KRIT1, SLC19A3, TREX1, MYORG). We reached 99.9% with at least 10X coverage for the patient. A search tool for the retrieval of interacting genes/proteins (STRING, ) was used to construct the protein-protein interactions that are involved downstream and upstream in Fahr’s syndrome (GNAS, ERCC8, PDGFB, CYP2U1, GNA11, SLC20A2, IFIH1, PSMB8, PDGFRB, CA2, ERCC6, SAMHD1, TREX1, CASR, TREM2, TYROBP, GJA1, ERCC3, FAM111A, RNASEH2B, SLC46A1, SLC7A7, ATP13A2, PARK7, HMBS, KRIT1). No causative mutations were found in the selected genes in the patient.\nComputerized tomography scans of the heads of the patient’s parents were normal.\nThe patient was treated symptomatically with quetiapine sustained release (initially 200 mg and gradually increasing to 900 mg daily) and sertraline (150 mg daily, gradually increasing to 200 mg daily). We did not observe any side effects with the use of quetiapine, although special attention was given to the possible exacerbation of extrapyramidal symptoms. Psychotic (PANSS scores at discharge were 8/13/27, PANSS scores after two years were 9/16/34 for the Psychotic, Negative and General Psychopathology Scale, respectively), affective and behavioral symptoms were improved; she was no longer suicidal and remained stable on gradually increasing doses of antipsychotic medication within two years of treatment, however, her intellectual abilities were not improved. Even though the patient completed secondary professional education and intense professional rehabilitation efforts were made, she has not been able to start working, mainly due to emotional instability. The patient was transferred to adult psychiatric services at the age of 21. |
A 39-year-old male and otherwise healthy patient presented in the emergency department with a history of 6 weeks of headache radiating to the neck and spine. The patient felt repeatedly hot flashes without reaching febrile temperature levels. On admission he also reported nausea without vomiting. The patient had no known allergies, no history of insect or animal bites, he had travelled to northern Italy 2 weeks before onset of the symptoms. One week before onset he had recieved surgical treatment of an axillary abscess under local anaesthesia. In the first two weeks of subfebrile temperatures and headache, he consulted his family doctor. Laboratory testing at his visit showed normal results for white blood-cell count and differential cell count, C-reactive protein, and serological tests for HIV, Borrelia, Yersinia, Chlamydia and Campylobacter. Results of testing for antinuclear antibodies [ANA], antineutrophil cytoplasmic antibodies [ANCA] and urinanalysis were normal. Magnetic resonance imaging [MRI] of the brain with administration of contrast material revealed no signs of inflammation, malignancies or vascular complications. The patient received an eight day therapy of steroids - 70 mg prednisolone per day – resulting in a transient improvement of his condition. However, 10 days before admission to this hospital his headache worsened again.\nOn admission to our hospital the temperature was 38.5°C, stable vital signs, blood pressure 140/75 mmHg, heart rate 70 beats per minute, oxygen saturation 97% while breathing ambient air. The weight was 79.5 kg. The skin was warm and dry. The abdomen was soft, normal bowel sounds, without distention, tenderness or masses. Neurological examination revealed positive meningeal signs, the remainder of the examination was normal. Echocardiography points to a mild pericardial effusion, while the electrocardiogram was normal - apart from nonspecific ST-segment changes. Standard laboratory tests were repeated, without significant findings [negative procalcitonin, negative C-reactive protein, normal white cell count]. On the second hospital day, the patient developed a right sided peripheral palsy of the VII. cranial nerve. An emergency MRI of the brain was performed, but did not show any significant pathological intracranial findings. Cerebrospinal fluid [CSF] analysis showed 280 cells, predominantly granulocytes [70%] and lymphocytes [20%], lactate and CSF total-protein levels were elevated, microscopic examination and gram staining revealed no bacteria in CSF [Table\n.]. Treatment with acyclovir [5 × 800 mg /d], ceftriaxon [2 × 2 g /d] and ampicillin [3 × 5 g /d] was promptly initiated.\nThe nuclear acid testing [PCR] for HSV1, HSV2 and VZV in CSF was negative, there was no detection of enterovirus nuclear acid in the patient plasma, no clinical and laboratory signs of CNS HIV or Treponema pallidum infection were observed. CMV and EBV serology showed positive IgG titers, whereas IgM titers were negative. Additional screening for Coxiella burnetii, Brucellosis and Tuberculosis [IGRA, CSF microscopy and CSF PCR] remained negative. Borrelia serology was not done regarding predominant granulocytes in the CSF. To rule out neurosarcoidosis or systemic sarcoidosis a total body Computed Tomography [CT] was performed, but without any significant findings, in particular neopterin, soluble IL-2 receptor [sIL-2r] and angiotensin converting enzyme [ACE] were not suggestive for sarcoidosis.\nThe follow-up lumbar puncture [see Table\n] noted pleocytosis with now predominantly lymphocytes. Total CSF-protein levels decreased from 2470 mg/l to 1510 mg/l. The total CSF cell count showed an increase to 360 cells – Fluorescence Activated Cell Sorting [FACS] analysis was used to ruled out clonal expansion of hematological cells in the CSF and cytologic analysis did not detect any malignant cell formations.\nIn view of lymphocytic CSF pleocytosis and facial palsy, Borrelia serology was repeated. Now the Borrelia specific CSF/serum IgG-antibody index (AI) was clearly positive, valued 20.6. The intrathecal Borrelia specific antibody production was further confirmed by IgG- and IgM-adjusted Immunoblots based on recombinant antigens (recomBlot, Mikrogen, Martinsried, Germany). Here, for both, IgG and IgM, clearly stronger reactive bands were visible in the CSF compared to serum (Figure\n). Routine PCR from CSF targeting ospA of B. burgdorferi was negative. All three criteria required in current guidelines for the definite diagnosis of LNB were met [see Table\n.]: I. neurological symptoms suggestive for LNB, II. CSF pleocytosis and III. B. burgdorferi specific intrathecal antibody production\n[]. At this point antiinfective treatment was adjustet to i.v. monotherapy with ceftriaxone 2 g q24 for 21 days.\nFor an additional diagnostic workup, specimens of the first two lumbar punctures and a third follow-up CSF sample were further investigated at the German National Reference Centre for Borrelia. Levels for CXCL13 were markedly elevated in the first two CSF samples, i.e. 5000 pg/ml and 2000 pg/ml. The cut-off range of the assay used is set at 100 - 250 pg/ml according to recent studies that reported sensitivities between 88% and 98% and specificities between 89% and 98%\n[,]. In the third liquor sample, after the patient received appropriate antbiotic therapy, CXCL13 levels have already been decreased to 450 pg/ml [see Table\n and CSF cell count dropped to 190 cells. However, cranial nerve palsy was persisting, without signs of clinical improvement. Advanced in-house OspA and p41 PCR diagnostics with several highly specific primers were positive in the first CSF specimen. Amplification and sequencing of the complete OspA gene identified B. garinii OspA-type 7, an overall rarely detected subtype, as the causative agent.\nImmediately after the completion of antibiotic treatment the patient felt good without meningeal signs and the peripheral facial palsy was slowly improving. Two month after being discharged, a follow up lumbar puncture [see Table\n.], revealed slowly decreasing levels of intrathecal Borrelia-specific antibody production and a CXCL13 at 52.8 pg/ml, now clearly below the threshold for LNB. There were no clinical signs of cranial nerve palsy left. |
A 38-year-old Caucasian woman, 35 weeks into her first pregnancy, presented to the emergency department for acute right-sided hip pain which precluded weight-bearing. Her right leg was shortened and externally rotated - there was no bruising or evidence of trauma.\nThe patient’s history was significant for hereditary thrombophilia (Factor V Leiden) and secondary anemia. Hip radiography revealed an unstable, displaced, right-sided femoral neck fracture with no evidence of osteonecrosis (Figure ). The decision to administer radiography, in this case, was based on the American College of Radiology guidelines, which cite an absence of in-utero deterministic effects of ionizing radiation effects after 27 weeks of gestation. Unfortunately, it was not possible to evaluate the symptoms of the patient with MRI at this time due to the coronavirus disease pandemic-induced stress on the healthcare system of our country.\nThe patient denied falls or trauma during the pregnancy, nor was there any history of smoking, alcohol abuse, use of glucocorticoids, or presence of rheumatologic/oncologic disease. Additionally, the patient was not malnourished, she underwent routine antenatal care, and took multivitamins. Serologic tests for inflammatory markers, as lab tests for serum calcium, phosphate, alkaline phosphatase, parathyroid hormone, vitamin D, and D-dimer returned normal.\nDuring multidisciplinary rounds, it was decided that delaying surgery was the best course of action out of fear of causing either mechanical or fluoroscopy-induced damage to the fetus during total hip arthroplasty. Five days later the patient experienced premature rupture of membranes, which was managed with emergency cesarean section (C-section) - no complications were encountered and a healthy 2300 g female was successfully delivered. Three days later the patient was transferred to our orthopedic surgery department for the treatment of the fracture. The significant degree of displacement (grade IV) of the fracture lasting over one week precluded open reduction with internal fixation due to fears of femoral head necrosis. During our literature review, we encountered a similar case of femoral neck fracture with grade IV displacement that was treated with open reduction internal fixation - despite restoration of blood flow to the femoral head within 15 hours, the authors still encountered femoral head necrosis with collapse six months later []. Given the considerable delay between symptom presentation and treatment, we decided the case warrants total hip arthroplasty instead of native hip salvage. Hemiarthroplasty was considered but was ultimately discarded as the conversion rate to total hip arthroplasty in young patients remain relatively high and the fracture was subsequently treated with a total uncemented prosthesis (Figure ), consisting of a 50 mm cup with 32 mm ultra-high-molecular-weight polyethylene insert and a 32 mm head with a 4 mm ceramic insert (Link Inc., Hamburg, Germany). Postoperative radiography confirmed prosthesis placement (Figure ); antibiotic and anticoagulant prophylaxis was initiated with ampicillin/sulbactam and enoxaparin sodium, respectively.\nThree days after the intervention the patient developed moderate abdominal pain without fever and accelerated intestinal transit, which both worsened over the next four days. Given the clinical presentation, there was a high index of suspicion for infection with Clostridium difficile (C. diff), although the diagnosis was ambiguous as the enzyme immunoassay (EIA) for the C. diff-specific antigen glutamate dehydrogenase was positive, while EIA for exotoxin A and B were negative. Due to exacerbation of symptoms and development of moderate hypokalemia (2.9 mEq/L), empiric treatment with metronidazole was began and marked rapid improvement. The patient was discharged five days later. |
A 63-year-old man had subtotal colectomy with liver metastasectomy in September 2012, according to the colon adenocarcinoma. In his surgery, 90% of colon was removed so he had a permanent ileocolostomy. The following couple of months after surgery, he had abdominal discomfort, diarrhea in his ostomy following the food intoxication, and acute kidney injury without any end-organ damage. While he had a regular diet and nutrition, in November 2013 he was admitted to the nephrology clinic with acute renal failure (serum creatinine 3.09 mg/dL, uric acid 13.5 mg/dL, and albumin 4.2 mg/dL with normal range of serum potassium, sodium, calcium, and phosphorus as well as serum hemoglobin and liver functions). Decreased skin turgor and dry oral mucosa were present on physical examination. Although he had the signs of dehydration, patient received 2 liters of water for each day and also he had at least 1 liter of urine output; he had no diarrhea and as stomal output. Intravenous hydration was started immediately and there was no acidosis on arterial blood gas analysis. 3000–4000 mL/day IV hydration improved the serum creatinine levels in 3 days (serum creatinine 1.3 mg/dL and uric acid 8 mg/dL), and changes of the laboratory parameters have been shown in . To exclude colorectal cancer recurrence, serum tumor markers such as CEA and CA 19-9 levels were measured and abdominal MRI and chest X-ray were performed. There were no findings of metastasis and recurrence on radiological and laboratory examinations. The patient was admitted to the nephrology clinic again for acute renal failure within 3 weeks of last admission to the hospital. He also denied the insufficient oral water intake and nutrition, but laboratory examination revealed acute renal failure (serum creatinine 5.0 mg/dL, uric acid 19.5 mg/dL, and albumin 4.0 mg/dL with normal range of serum potassium, sodium, calcium, and phosphor as well as serum hemoglobin and liver functions) and changes of the laboratory parameters have been shown in . Following the administration of intravenous fluid, serum creatinine and uric acid levels became in normal range within 4 days. After the second admission, we suspected for short bowel syndrome (SBS), and also radiological examinations demonstrated SBS (). Loperamide hydrochloride 10 mg/day was started in the hospital and he had no side effects and serum creatinine levels did not increase in 5 days. We then discharged the patient and followed him up for the next 2 months. He had no symptoms for acute renal failure and his last serum creatinine level was 1.05 mg/dL and uric acid level was 7 mg/dL. |
A 59-year-old female patient presented to a plastic surgery clinic with a lump on her right medial thigh. She noticed the lump eight months ago and it was gradually increasing in size. It was painless initially; however, the lump gradually became tender. The patient was otherwise fit and well with no co-existing morbidities. Closer examination of the right thigh revealed a high consistency mass, with reduced mobility. There was no right inguinal lymphadenopathy or lymph nodes enlargement.\nLaboratory blood tests were unremarkable. An MRI scan was requested to further assess the mass, which was irregular but well-defined in the medial compartment of the thigh, measuring 8.2 x 6.6 x 4.3 cm in size. The mass showed an intermediate signal on both T1 and T2-weighted imaging, appearing hyperintense compared to the adjacent muscular tissue, which also persisted on fat-suppressed imaging. The mass invaded the intramuscular fat planes and caused compression and displacement of the adductor muscles. There was no evidence of intra-muscular invasion. Normal cortical outline and medullary signal intensity of the femur were seen in the right thigh. There was no evidence of bone contusion, marrow oedema, fracture line, or cortical discontinuity. Other muscles of the thigh had a normal outline and signal intensity with no evidence of focal or diffuse oedema. Neurovascular structures were unremarkable. Overall, MRI findings were suggestive of soft tissue neoplasm, with a possibility of a neurogenic tumour. A wide excisional biopsy was then performed to further assess the tissue histologically by the pathologist and confirm the diagnosis. The removed mass measuring 8.5 x 6.5 x 5.4 cm and is shown in Figure . The mass had a smooth outer surface and a soft consistency. The cut surface showed a homogenous tan-white appearance with some slit-like spaces and occasional haemorrhagic spots. Multiple sections were processed for histopathological examination from different planes.\nHistopathology of the mass revealed a tumour arranged in sheets and fascicles composed of round to oval cells along with variably sized adipocytes (Figure ). Numerous interspersed lipoblasts with indented nuclei were also seen. Moreover, severe nuclear atypia was noted including bizarre cells (Figure and Figure ). An area of necrosis was seen with a few areas, which showed malignant and fibrous histiocytoma-like features. Furthermore, a few thin-walled dilated and congested blood vessels were also noted. Also, brisk mitosis was noted (35-38 per 10 HPF) (Figure ). Immunohistochemical staining (IHC) was also performed and the tumour cells were diffusely positive for vimentin and focally positive for S-100; while negative for creatine kinase (CK), smooth muscle antigen (SMA), desmin, CD34 and MyoD1. Overall, the pathological findings are all suggestive features of a high-grade sarcoma, favouring the epithelioid variant of PLS.\nThe patient was also referred to the Department of Nuclear Medicine & Molecular Imaging for post-surgical restaging and assessment for possible metastases using 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) scan. The abdominopelvic section showed the liver measuring within normal limits; however, the scan revealed an ill-defined hypermetabolic hypodense lesion seen involving segment VI measuring approximately 2.8 x 1.8 cm suggestive of hepatic metastases with concurrent fatty liver. The scan was also remarkable for the musculoskeletal system, revealing multiple hypermetabolic osseous lesions involving the skull base, right iliac bone, and few vertebrae (predominantly T7-L1); which was suggestive of skeletal metastases. A non-hypermetabolic pulmonary nodule in the right lung field was also detected; it was considered to be likely benign, but interval scanning is suggested to monitor further progress. Finally, there was also evidence of diffuse subtle hypermetabolism at the site of surgery which is suggestive of postsurgical inflammatory sequelae; however, microscopic residual disease cannot be completely ruled out. These multi-organ lesions were due to be biopsied to examine their immunohistochemical findings to confirm metastatic lesions of primary pleiomorphic liposarcoma. However, the patient did not attend the follow-up appointments. |
A 38-year-old man visited our hospital complaining of anterior chest pain. He had no significant medical or family history, and the vital signs were stable. Ischemic events were not observed in electrocardiography, but chest X-ray and computed tomography (CT) showed a cystic lesion (6.0 × 7.0 × 10.0 cm) in the anterior mediastinum (Fig. a). Although the cystic capsule demonstrated contrast enhancement, its fluid component had low radiation absorbance. Based on these findings, we suspected the mass to be a thymic cyst. Blood tests indicated the presence of inflammation (white blood cell count 11,200/μL and C-reactive protein 3.38 mg/dL).\nTwo days after hospitalization, the patient developed dyspnea and his chest pain worsened. Subsequent chest CT showed that the cystic lesion had become inhomogeneous and the radiation absorbance of the cyst’s fluid component had increased (Fig. b). The cyst wall became thickened, and bilateral effusion was observed. Blood tests indicated that hemoglobin levels had decreased from 15.8 to 12.8 g/dL, and levels of inflammatory markers had increased, with the fever exceeding 38.5 °C. Needle aspiration biopsy and tumor wall biopsy with a small skin incision were performed; however, we could not obtain a diagnosis. One week after admission, general condition and laboratory data of the patient gradually improved. A chest CT on day 13 showed that the tumor had become small in size with a thickened wall (Fig. c). The effusion on the right side had decreased and that on the left side had disappeared.\nThe patient had recovered enough to undergo surgery; the tumor was resected by sternotomy on day 18. The tumor was found to be encased in a smooth, yellow, and elastic coat. The tumor was densely adhered to the junction of the left brachiocephalic vein and superior vena cava, and it was required to detach the tumor from the dense adhesion site carefully. The right phrenic nerve was preserved, and the right pleural effusion was serous. The tumor and thymic tissue were resected en bloc. The operative time was 288 min, and the estimated blood loss was 521 mL. The resected tumor was covered with a thick, fibrous capsule, and the lumen was filled with necrotic tissue and hemorrhagic material (Fig. a, b). The postoperative course was uneventful, and he was discharged on day 26.\nThe pathological findings showed a fibrotic cyst wall; the cyst was filled with necrotic tissue. The slight proliferation of lymphocytes was confirmed in the necrotic tissue and around the cyst wall (Fig. a, b). The tumor was diagnosed as type B1 cystic thymoma (Fig. c). As the tumor did not appear to have spread beyond the capsule, it was determined to be at Masaoka stage I. Nevertheless, the dense adherence of the tumor to its surrounding tissue indicated the possibility of invasion, and postoperative radiotherapy (50 Gy) was administered.\nTwo years after the surgery, recurrent metastasis of the tumor was found on the right pleura and the left upper lobe of the lung. The patient was treated with chemotherapy, radiotherapy, and local resection. The patient remains alive 12 years after the first surgery. Following an analysis of the tissue obtained from the resected recurrent tumor, the pathological diagnosis was changed to type B3 thymoma. |
A 76-year-old male patient was referred to our hospital for the treatment of a saccular abdominal aortic aneurysm and a right common iliac artery aneurysm. The maximum diameters of the abdominal aortic aneurysm and the common iliac artery aneurysm were 35 and 27 mm, respectively. Because he had several comorbidities, including a cerebral arterial aneurysm and a history of cerebral infarction, EVAR was selected as the treatment approach. After embolizing the right internal iliac artery and the inferior mesenteric artery with coils, we placed an Excluder leg (W. L. Gore & Associates, Inc., Newark, DE, USA) spanning from the right common iliac artery to the right external iliac artery as the initial step. Because of a narrow and long proximal neck (diameter, 17 mm; length, 60 mm), it was assumed that the use of a modular device could cause compression and stenosis of the ipsilateral leg at the proximal neck. Therefore, we decided to use an AFX2 device as the main endograft. On completion aortography performed after deployment of the main AFX2 endograft, an endoleak was noted in the middle of the endograft. As it was considered to be a type Ia endoleak, we added two Excluder cuffs to the proximal neck. However, the endoleak persisted, and angiography with the tip of a Berenstein catheter (Merit Medical Inc., South Jordan, UT, USA) placed at the assumed endoleak point revealed that the endoleak originated from a graft hole, indicating a type IIIb endoleak (). Because no main endograft with an appropriate dimension was available and the reliability of the AFX2 device was unclear, we decided to perform parallel placement of Excluder legs. The required diameter of Excluder legs was calculated by the following formula:\nTo minimize gutter endoleaks that can occur between two Excluder legs, the diameter was increased by approximately 10%, and the final diameter was set at 23 mm. Two Excluder legs (bottom end diameter, 23 mm) were placed from immediately below the origin of the renal artery using the upside-down technique, which has been described in a previous article although it is an off-label technique. Briefly, an Excluder leg is removed from its delivery system, with the sleeve unopened. It is then inserted into a DrySeal sheath (W. L. Gore), which has been introduced to the top of the origin of the renal artery. After deploying the leg inside the DrySeal sheath, it is advanced to the planned position with a pusher created by cutting the edge of a dilator. It is then deployed in the landing zone by pulling the sheath while firmly holding the pusher. In our patient, two operators deployed two Excluder legs simultaneously to accomplish parallel placement. After deploying the Excluder legs on both sides with sufficient overlap to completely cover the previously placed endograft and expanding a balloon for touch-up, aortography and cone-beam computed tomography (CT) showed no endoleak or collapse of the Excluder legs. Contrast-enhanced CT performed 2 days after the EVAR showed no endoleak or obstruction of the Excluder legs (). Additionally, CT performed 6 months later revealed reduction of the abdominal aortic aneurysm to 31 mm, although the diameter of the right common iliac artery remained unchanged. |
A 21-year-male old with dissociative amnesia and approximate answers.
Mr S, a 21-year-old single mason, presented to us first in May 2012. As a child, he had a mild delay in all his milestones, was described as poor in academics. He had dropped out of school by 5th grade. However, he had no peri-natal complications or other neuro-psychiatric morbidity. There was no family history of neuro-psychiatric illness. He was described as capable of functioning as a mason with sufficient monitoring and in a structured workplace — clinically suggestive of a mild intellectual delay.
He presented with a 1-week history of a low-grade fever accompanied by signs of an upper respiratory tract infection, for which he was receiving treatment locally. On the 5th day of the fever, he was noticed to have become increasing aloof and irritable. The next day, he became violent suddenly without provocation and attacked his family with a heavy iron rod. Following this, he locked himself inside the house and attempted suicide by hanging. He was rescued by his family within 5 min and was resuscitated by a medical person on the scene. His vitals were stable, though he was unconscious, and he was rushed to a government hospital. After regaining consciousness, he continued to be violent and irritable and occasionally claimed that he could see a goddess coming to attack him.
Following discharge from the hospital, he was directly brought to our center. At the time of presentation, his vitals were stable. He was well-oriented to time, place, and person. He denied any knowledge of the incidents of the last 2 weeks or any psychotic phenomenon. No other possible effects of neural damage due to hanging were present.
Computed tomography scan of the brain and an EEG for Mr. S were normal. A significant finding was his constant approximate answers to simple questions, which was |
A 34-year-old woman (gravida 2, para 2) was referred to our hospital on account of persistent vaginal bleeding after a cesarean section she had undergone on the same day at a local obstetrics and gynecologic clinic. Her past medical history and family history were not significant. Her postpartum hemoglobin was 8.3 g/dL, which necessitated an emergency packed red blood cell transfusion. She had a blood pressure of 100/59 mmHg and a heart rate of 142/min. Angiography was performed as a primary diagnostic and therapeutic procedure. Contrary to expectations, both uterine arteries were not hypertrophied despite the recent postpartum state, and there was no evidence of vascular injuries such as active bleeding, pseudoaneurysm, or transection of blood vessel on both internal iliac artery angiographies (Fig. A and B ), and pelvic angiography. Considering the continuous vaginal bleeding and her unstable hemodynamic status, an emergency total abdominal hysterectomy with bilateral salpingectomy was performed right after the initial angiography, and vaginal laceration was confirmed during surgery. Pathological examination showed no abnormalities of the uterus, cervix, or fallopian tubes. Two days after the surgery, 2000 mL of blood was present in a drainage tube that had been inserted intraoperatively, and hemoglobin level declined from 8.3 to 5.2 g/dL. A dynamic CT scan performed revealed a tortuous, hypertrophied vascular structure at the right lateral aspect of the pelvic cavity (Fig. A) along with a large amount of hemoperitoneum (Fig. B). A repeat angiography of the right internal iliac artery demonstrated a complex tangling of vessels supplied by an enlarged feeding artery, and this was not seen on the first angiography (Fig. A). Moreover, superselective uterine artery angiography showed early venous drainage into hypertrophied veins, and stasis of contrast medium in the abnormal vascular structure, which indicated UVM (Fig. B). Therefore, embolization of the right uterine artery was performed using NBCA, and a post-embolization angiogram revealed successful hemostasis with disappearance of the UVM (Fig. C). After embolization, hemodynamic stability was achieved. A day after the embolization, hemoglobin was 10.2 g/dL, and the patient was discharged from the hospital 4 days thereafter. |
This case report has been written in accordance with the SCARE 2018 criteria [].
A 64-year-old female presented to the Emergency Department of our hospital with acute onset of a right-sided groin bulge that occurred earlier that day after doing heavy lifting. She had no significant past medical or surgical history. Her baseline functional status was exceedingly well. Her laboratory studies showed no leukocytosis or any electrolyte abnormalities. Initial radiologic studies included a transabdominal and transvaginal pelvic ultrasound, which showed a 2.4 × 2.6 cm fluid collection with some septations and absent visualized adnexal structures. A subsequent CT demonstrated a well-circumscribed 2.7 × 3.2 × 2.6 cm fluid collection containing echogenic debris. It did not have significant rim enhancement yet was noted to be most consistent with an abscess, complex seroma, or necrotic lymph node; its depth was amenable to percutaneous drainage. On further review with the radiologist, it was noted to contain the appendix, the tip of which was dilated and radiographically concerning for a cystic process, with mucocele remaining within the differential diagnosis.
The patient was kept nil per os, intravenous fluid resuscitation was initiated, and antibiotic therapy was begun. She was brought to the operating room for emergent surgical intervention. The patient underwent laparoscopic appendectomy with open femoral hernia repair. Intraoperatively, the appendiceal tip was incarcerated within the hernia sac (). Due to the preoperative radiographic appearance and the suspicion for mucocele, extreme caution was exercised to avoid rupture and not to provide excess tension in an attempt to reduce it laparoscopically. It was easily removed through the open inguinal incision after the appendix base was divided laparoscopically (). The femoral hernia was repaired in standard McVay fashion. Final pathology showed inflamed non-perforated acute appendicitis without evidence for neoplasm. The patient recovered well without notable complications and was discharged on postoperative day number two without antibiotics. |
A 37-year-old Bangladeshi male sustained a fall during work. He fell off an 8–10 m high scaffold on his left foot and then on his left side. Subsequently, a heavy object fell on his left foot. He was complaining of severe pain in the left foot and inability to bear weight on it. He was brought by the ambulance to the emergency department. On arrival, the patient was conscious, oriented with Glasgow Coma Scale 15/15. Physical examination showed severe tenderness over the left foot with deformity. Neurovascular status of the foot was intact. There was moderate-to-severe swelling with soft compartments of the left foot. He was cleared by the trauma team and investigation showed isolated left foot comminuted intra-articular calcaneal fracture along with Lisfranc fracture with dislocation of the tarsometatarsal joints. X-ray of the left foot is shown in . 1day after presentation to the emergency department, the patient under went open reduction and internal fixation (ORIF) of the left foot Lisfranc fracture and dislocation with cortical screws for the first to fourth metatarsal and 2 mm K-wire for the fifth metatarsal. A below the knee back slab was then applied and the patient was admitted to the orthopedics ward. The post-operative course of the patient was unremarkable except for swelling over the left foot. However, on the 4thpost-operative day, the patient developed clear skin blisters over the posterior aspect of the calcaneus. The skin condition of the patient deteriorated to skin necrosis as shown in . A computed tomography (CT) scan of the left foot was carried out in preparation for ORIF of the calcaneus. Cut sections of the CT scan demonstrating a fragment of the calcaneus pressing over the posterior soft tissue are shown along with other cuts in . The patient then underwent ORIF utilizing an extended lateral approach. Using a 3.5 mm K-wire inserted posteriorly, the posterior fragment and the articular surface were reduced under fluoroscopy supervision. A medium calcaneal plate was then applied and held in place with screws locking and non-locking. A back slab was applied and the patient was discharged 2days after the operation. Outpatient follow-up 4months postoperatively shows healing skin over the posterior aspect of the calcaneus (). At 1-year post-operative visit, the patient is back to complete daily activities with mild pain while running. Radiographs of the calcaneus and the Lisfranc fractures showed good fixation and healing ( and ). |
A 14-year-old Asian male presented to the psychiatric clinic with a one-month history of on and off left-sided headaches, photophobia, irritability, feeling sadness, and poor sleep. History did not reveal past history of other somatic or psychiatric illness and he was not taking any medication. He was not drug-addicted.\nHis physical examination was normal. Routine blood tests did not find abnormalities. His psychiatric assessment revealed that he was alert, well oriented with depressive signs. The patient denied suicidal thoughts. The neurological examination did not reveal focal neurological signs or cranial nerve deficits. According to the neuropsychological assessment, he had mild cognitive impairment, moderate to severe depressive episode, and mild anxiety. He was diagnosed to have an adolescent depressive episode, which was treated with Sertraline and Flunarizine. Two weeks later he presented to the emergency department with sudden onset of severe occipital headache and vomiting. There were no focal neurological deficits and Glasgow Coma Score was 15.\nThe non-contrast computed tomography (CT) of the brain was done to rule out acute brain insult. The CT brain demonstrated thick subarachnoid hemorrhage (Figure ). Subsequent contrast CT angiogram and digital subtraction angiogram (DSA) confirmed the diagnosis of a 5.5 × 9.2 mm2 saccular aneurysm originating from the bifurcation of the left internal carotid artery (Figures -). The neurosurgical team did the microsurgical clipping of the aneurysm. The immediate postoperative period was uncomplicated and the patient was discharged from the hospital seven days after the surgery. On psychiatric evaluation two weeks after the surgery, the patient showed improvement in sleep, less anxiety, and depressive symptoms. As the depressive symptoms were resolving, he had stopped taking Sertraline. On neurological assessment, he was alert and well oriented, without any neurological deficits. During the psychiatric assessment one month after the surgery, his depressive symptoms were resolved and he enjoyed his day-to-day activities. On psychological assessments, his cognitive functions were within normal limits, and we did not detect depressive symptoms or neurological deficits. |
A 49-year-old woman presented with a 3-day history of fever, generalized malaise, and worsening of severe, diffuse, acute-onset abdominal pain that was greatest felt in the left upper quadrant.\nShe had a history of three hospitalizations and one short observation stay for acute pancreatitis over a 4-month period. Her symptoms were classic for acute pancreatitis: abdominal pain with radiation to the back, elevated lipase level, and radiologic findings of acute pancreatitis involving the tail of the pancreas. She was treated conservatively following these events and recovered uneventfully, except for the persistence of intermittent left upper quadrant abdominal pain. The diagnosis was considered idiopathic, as she reported no significant alcohol consumption, trauma, new culprit medications, laboratory test result abnormalities, or imaging findings to suggest gallstone pancreatitis, hypercalcemia, or hypertriglyceridemia. On her second admission (approximately 8 weeks since the first admission), imaging revealed new peripancreatic (tail) fluid collection abutting the spleen and gastric fundus (). On the third admission (approximately 6 weeks since the second admission), imaging revealed new acute splenic vein thrombosis, gastric varices, and splenomegaly in addition to the known peripancreatic fluid collection (). The patient was started on anticoagulation with low-molecular-weight heparin bridged to a direct-acting oral anticoagulant (DOAC) after hematologist consultation. The international normalized ratio on DOACs ranged from 2.1 to 3.4. Diagnostic endoscopic ultrasound (3 weeks since the third admission and 3 days before the current admission) showed a 3-cm heterogeneous fluid collection in the pancreatic tail with an irregular ill-defined margin extending to the splenic hilum consistent with the known finding (). No mass lesion was identified. The pancreatic parenchyma in the head and body of the pancreas was within normal limits. The common bile duct and pancreatic duct were non-dilated with no filling defects. Several benign-appearing 1-cm lymph nodes were noted.\nOn arrival to the emergency department, the patient was hypotensive at a blood pressure of 75/45 mm Hg and tachycardic at a heart rate of 120 beats/min. The laboratory test results were remarkable for the lipase level (147 IU/L), white blood cell count (15×103 /μL), Hb level (10 g/dL), creatinine level (1.6 mg/dL), and beta-hemolytic non-group A or B streptococcus bacteremia presence. Abdominal and pelvic CT showed acute-onset chronic pancreatitis with fluid collection along the pancreatic tail (not organized) that extended into the postero-inferior aspect of the spleen and known thrombosis of the splenic vein with portosystemic collaterals, gastric varices, and mild splenomegaly. She was initially treated with fluid resuscitation and vasopressor and antibiotic administration with initial improvement in clinical status. However, she developed acute shortness of breath, worsening abdominal pain (progressed to diffuse, severe, and constant pain), and distension the following day. Repeat CT showed splenic rupture with moderate hemoperitoneum and perisplenic hematoma (). Emergency laparotomy with splenectomy was performed successfully with an uncomplicated recovery. Pathology revealed multiple lacerated spleen fragments. |
A 65-year-old healthy, non-smoking, male was referred to the plastic surgery department due to a growing mass on the scalp. The patient had first noticed the mass about 9 months earlier. He did not have any physical discomfort related to it but became worried because of the increasingly rapid growth. The patient did not report any symptoms of weight loss, fatigue or loss of appetite and had no history of previous malignancies.\nClinical examination revealed a 4 × 5 cm, erythematous tumor in the right occipital region (). On palpation the tumor appeared pulsating, soft and non-adherent to the underlying bone. There were no palpable lymph nodes regionally. Differential diagnoses considered were other vascular tumors such as pyogenic granuloma, Kaposi sarcoma and angiosarcoma.\nCT-angiography showed a richly vascularized, extracranial tumor with multiple large veins. The tumor was surgically excised under local anesthesia with a 5 mm margin and the defect reconstructed with a split skin graft.\nHistopathological examination revealed a well-defined mass in the deep dermis and subcutis, partly enclosed in a fibrous pseudocapsule. The process was formed by alveolar, tubular and tubulocystic formations of cells with clear cytoplasm and central, round nuclei (Figs. and ). Immunohistochemistry showed the lesion was positive for broad-spectrum cytokeratin, Vimentin and PAX8, and negative for S-100 and Leucocyte Common Antigen. The tumor was excised with radical margins and the morphology and immunophenotype were consistent with a metastasis from a clear cell renal cell carcinoma.\nThe patient was referred to the department of urology where a CT-scan and a PET-CT were obtained and revealed a 13 cm tumor in the left kidney, small lung metastases bilaterally and multiple suspicious mediastinal lymph nodes. The patient was discussed at a multidisciplinary team-conference and subsequently underwent a debulking laparoscopic nephrectomy, confirming a primary 10 cm clear cell renal cell carcinoma with vascular invasion (), pT3a. No systemic treatment was initiated. Follow-up CT-scan three months postoperatively showed that one of the lung metastases had grown 3 mm, but otherwise no progression in the disease. |
An 8-month-old Philippine female baby without other health problems presented with a palpable neck mass of 4 months’ duration. The patient did not suffer from dysphagia nor dysphonia. The past medical history was unremarkable. On physical examination, a midline anterior neck mass, tender and mobile when swallowing, contiguous to the hyoid bone was observed. No enlarged lymph nodes were detected in the lateral neck region. Laboratory values were in normal range. At US, a lesion largely hypoechoic measuring 20 x 14 mm in diameter, with internal heterogeneous echoes, with a well defined periphery (Figure\n) was detected and the thyroid gland did not show abnormalities. A thyroglossal duct cyst was suspected and the patient underwent surgical excision. Intraoperatively we couldn’t differentiate if the lesion was a solid mass or a cystic one and its malignant potential, if any, so that the Sistrunk procedure was chosen for safety’s sake. A careful dissection of the lesion was performed, and the central portion of the hyoid bone was resected. In the postoperative course, the patient suffered from dyspnea and dysphonia, with relief of symptoms on the third post-operative day. At follow-up, 8 months after surgery, the patient was well, without evidence of disease.\nOn gross examination, the excised mass measured 20 mm in largest diameter; a central fibrous areas was detected, without evidence of cyst. The tissue was fixed in 10% buffered formalin, routinely processed and paraffin embedded. Histological examination revealed a preserved lymph node structure at the periphery, with reactive follicles and dilated sinuses, whereas, in the region of the hilum, the nodal parenchyma was completely replaced by a fibrous tissue (Figure\n) containing numerous irregular vessels and spindle cell bundles (Figure\n). On immunohistochemical analysis, the spindle cells appeared as smooth muscle fibers, as confirmed by their positivity for HHF35 and desmin. Consecutive sections did not document presence of a cystic lesion. All the aforementioned findings were consistent with a pathological diagnosis of AH of the lymph node. |
We report the case of a 37-year-old male with a past medical history of essential hypertension and hyperlipidemia who was transferred to our hospital for further ongoing care for colitis and hematochezia in settings of possible bleeding disorder. The patient was initially presented to another hospital with a one-week history of diffuse abdominal pain, bloating, and a three-day history of bright red blood per rectum. He also reported multiple episodes of vomiting and one episode of gross hematuria. The patient also reported a six-month history of fatigue, generalized body weakness, cold intolerance, swelling in his arms and legs, easy bruising, and unintentional weight loss of 20 pounds over six months duration. In October 2020, he had an episode of profuse gum bleeding after a dental procedure, and he has been currently being worked up by hematology as an outpatient for von Willebrand disease (VWD) versus hemophilia. He had a remote history of proteinuria and was following by nephrology outpatient, but a biopsy was not pursued in the setting of bleeding disorder workup. We were consulted as he was found to have nephrotic range proteinuria with a 24 hr urine protein of 4.61 gm.\nOn examination, he was afebrile with a blood pressure of 105/64, tachycardia with a heart rate of 112, and normal oxygen saturation on room air. He was in no acute distress. His cardiac and pulmonary exam was unremarkable. Abdominal examination revealed mild diffuse tenderness and dullness on percussion. He had +2 non-pitting edema in the lower extremities. The skin was dry, and no rash was noted. Ultrasound abdomen was done, which showed hepatomegaly with findings of portal hypertension with the slow flow in the main portal veins, recanalized umbilical vein, pericholecystic edema, reversal flow within the splenic vein at pancreas, splenic siderosis, splenic varices, and moderate ascites.\nLaboratory workup (Table ) revealed acute kidney injury (AKI) with serum creatinine of 1.88 mg/dl (baseline creatinine around 1 mg/dl), hyponatremia, hypoalbuminemia, and hyperlipidemia. Hepatitis and human immunodeficiency virus (HIV) serology were negative. Autoimmune workup showed negative antinuclear antibodies (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), and anti-glomerular basement membrane antibody (anti-GBM), normal complement C3, high complement C4, and elevated beta-2 microglobulin. Urine analysis and microscopy showed no white blood cells (WBC) or red blood cells (RBC), random urine protein above 500. 24-hour urine protein showed 4.61-gram proteins, urine protein to creatinine ration of 5.54, and normal serum immunofixation pattern. No monoclonal immunoglobulins were detected. Serum protein electrophoresis (SPEP) report showed increased alpha proteins. No monoclonal immunoglobulins were detected. A urine immunofixation report identifies a free lambda monoclonal immunoglobulin representing less than 10% of total urine protein. The free kappa level was high, and the free lambda level was high; however, the kappa to lambda ratio was low.\nA renal biopsy was performed. The tissue process for light microscopy is a single length of renal cortical tissue with a total of 11 glomeruli, four of which are globally sclerotic. The remaining glomeruli revealed a diffuse and global non-nodular increase in eosinophilic extra-cellular mesangial metrical material. The material was weakly PAS positive, silver negative, Congo red positive, and demonstrated apple-green birefringence when examined under polarized light (Figure ). There were occasional sheaf-like projections perpendicular to the glomerular basement membrane that were highlighted with the Jones silver stain. Tubule demonstrates PAS positive cytoplasmic protein droplets. There is no significant interstitial inflammation. There is mild interstitial fibrosis/tubular atrophy. The mesangial material demonstrated monotypic immunoreactivity for lambda light chains without dominant immunoglobulin expression. Ultrastructural examination confirmed the presence of fine randomly oriented fibrils consistent with amyloid of approximately 10 nm (Figure ). The kidney biopsy sample was sent to Mayo clinic for liquid chromatography-tandem mass spectrometry (LC-MS/MS): Liquid chromatography-tandem mass spectrometry (LC-MS/MS) detected a peptide profile consistent with AL (lambda)-type amyloid deposition. These findings support the diagnosis of amyloidosis and indicate AL (lambda)-type amyloid deposition.\nBone marrow biopsy revealed a plasma cell neoplasm, favor plasma cell myeloma (Figure ). Flow cytometry immunotyping showing CD (cluster differentiation) 19-, CD56-, CD117 +, lambda light chain restricted monotypic plasma cells consistent with plasma cell neoplasm. Echocardiography was done and showed an EF of 70%. The patient was started on CyBorD (Cyclophosphamide, bortezomib and dexamethasone) induction chemotherapy. Workup for bleeding disorders revealed that the patient has Type 2A von Willebrand Disease, likely secondary to the AL amyloidosis. |
A 54-year-old male needed the extraction of an upper wisdom tooth; the patient complained of pain, posteriorly, on the upper left side of the maxilla. After clinical and radiographic examinations, the upper left wisdom tooth was found with deeply a carious lesion with pulpal involvement, causing the pain.\nThe patient seemed to be cooperative, compliant and confident with the dental team and the surgery, even though the clinical history and medical record showed previous episodes of moderate anxiety related to dental procedures. Before the surgical intervention, topical analgesia (Lidocaine 15% spray) was used prior to LA injection. LA injection was administered on the vestibular and palatal aspects, for both pain control and vasoconstriction (Ecocain, 2x 20 mg/ml, 1,8 ml, 1:50:000 adrenaline). However, due to pulpal involvement and the presence of an infection, good pain control was not achievable\n. The patient showed distinct and comprehensible signs and symptoms of distress and an inability to withstand that surgical situation and the surgical steps. Thus, he rapidly became nervous and agitated. Not to lose the confidence and trust of the patient, we administered a benzodiazepine (bromazepam 15 drops\nper os) and nitrous oxide (Inhalation; Start: 10% nitrous oxide and 90% oxygen, progressively reaching 40% nitrous oxide and 60% oxygen). After 20 minutes of relaxation, we could perform the extraction with the perfect compliance of the patient and a better control of the intraoperative pain. At the end of the procedure, we administered 100% oxygen for 10 minutes for patient recovery. The patient reported satisfaction for the previous treatment at the suture removal visit.\nWhen facing a patient with special needs (anxiety and reduced compliance), especially before procedures such as a planned minor surgery, or an extraction or a mucogingival surgery or an osseous periodontal surgery or a simple case of implantology, the dental team should preserve patient confidence, reduce anxiety and obtain compliance. In addition to local analgesia, the dentist should be prepared to supplement LA infiltration with inhalation sedation and/or oral sedation, even in the absence of the specialist anaesthesiologist.\nThe combination of benzodiazepines and nitrous oxide positively affects both the patient and the dental professional: in fact, a good consciousness of time and space perception and deep conscious sedation are achievable by adjusting nitrous oxide titration; the dentist is more relaxed and concentrated on the procedure. Analgesia is then well controlled by means of local analgesia. |
An 8-year-old girl was admitted to our department with a mass in her left parotid area, which was present for one month and has increased in size over the last 7-day period. On examination a tender mass was palpated in the left parotid area. The lesion was elastic, moderately movable, and mildly painful, located in the left infra-auricular area, in close relation with the lobule and external auditory canal.\nThe patient underwent magnetic resonance imaging (MRI) of the head and neck, demonstrating the anomaly (Figures and ). The lesion appeared as a rather well-circumscribed mass in the left parotid area, within the parotid gland. Cystic origin was well established (high signal in T2-weighted sequence) and the presence of an internal septum was noted. In contrast, in enhanced T1 sequence (after intravenous gadolinium administration) both the wall of the lesion and the internal septum were enhanced. Moreover, the top side of the mass projects on the external auditory canal, duplicating the ear canal.\nA superficial parotidectomy was subsequently performed, with total excision of the cyst. During the operation it was confirmed that the cystic mass extended upwards, being in close contact with the cartilaginous part of the external auditory canal, but no communication with the canal or the middle ear was identified (). The lesion lied superficially to the facial nerve, as we expected considering its embryological origin (). The location of the facial nerve was identified anatomically with standard techniques and exposed with blunt dissection; electrophysiological monitoring throughout the procedure reduced the risk of injury. Once the facial nerve and its branches had been fully identified and carefully dissected, the anomaly was completely resected.\nPathological examination of the surgical specimen showed skin, squamous epithelium, and cartilage, confirming the diagnosis of first branchial cleft cyst (type II).\nNo sign of recurrence was noted after a follow-up period of 15 months. |
A 57-year old Afro-Caribbean female presented with a T4N2M0 triple negative mucinous right breast carcinoma. She was 1-year post radical hysterectomy and adjuvant radiation for abnormal uterine bleeding, with the corresponding histology of endometrial carcinoma as well as multiple uterine leiomyomas. She had a 12-year history of left exophthalmos secondary to a sphenoid wing meningioma, for which she received external beam radiotherapy. She also had a history of rectal bleeding secondary to an anal polyp, which was resected and proved to be a tubular adenoma.\nSeveral dermatological lesions were noted on examination, which the patient reported to be present ‘since childhood’. These included flesh colored papules in the perinasal region, warty papules on the tongue, hands and feet as well as palmar pits. A visible right neck swelling was also noted and further questioning revealed symptoms of dysphagia as well as a history of prior left thyroidectomy for a multinodular goiter. She did not report any shortness of breath, palpitations or significant weight loss. A left cheek swelling was palpated, which was pulsatile in nature. Our patient also had a thoracolumbar scoliosis and complained of symptoms of sciatica. Unfortunately, as she was estranged from her family, her family medical history and genetic testing of family members could not be obtained.\nHematological and biochemical investigations as well as thyroid function tests were all within normal limits. Contrast enhanced CT of the brain, neck, chest, abdomen and pelvis was performed for staging purposes and to monitor known lesions. CT revealed a left sphenoid wing meningioma with extra-conal extension and resultant left optic nerve impingement and proptosis. Two other homogenously enhancing extra-axial lesions were noted, with one showing calcification, consistent with the presence of additional smaller meningiomas. These were stable on imaging for 5 years (Fig. ). The pulsatile left cheek swelling corresponded with a left parotid arteriovenous malformation, with the arterial feeder arising from the left external carotid artery (Fig. ). A left thyroidectomy was noted with multinodular enlargement of the right thyroid lobe and isthmus along with retrosternal extension and marked tracheal deviation to the left (Fig. ). The right breast lesion was partially imaged on CT preoperatively, along with level I and II axillary adenopathy (Fig. ). Of note was a thin walled lung cyst in the periphery of the left upper lobe (Fig. ).\nMultiple hypo enhancing splenic lesions were seen, likely representing hamartomas, as well as several subcentimeter fat density lesions within the pancreas (Fig. ). The pelvis showed post surgical changes with no evidence of local recurrence or lymphadenopathy. No metastatic disease was evident. Mammogram of the left breast showed no evidence of spiculated masses, clustered micro calcifications or architectural distortion. Our patient declined biopsy of her skin lesions however these were assessed by dermatologists and clinically confirmed to be facial trichilemmomas, acral keratosis, palmar pits and oral papillomas. MRI of the spine revealed a thoracolumbar scoliosis with spondylosis and multilevel disc disease however there was no evidence of metastatic disease, dural ectasia or extra medullary lesions. Genetic testing identified a pathogenic mutation in the PTEN gene: c.697C > T (pArg233*).\nFollowing neoadjuvant chemotherapy, our patient underwent a right modified radical mastectomy with axillary lymph node dissection. Although she declined debulking operative intervention for the sphenoid wing meningioma, she was treated with external beam radiotherapy; with stability of this lesion noted post radiation. Prophylactic mastectomy for the contralateral breast was discussed at the multidisciplinary team meeting, however our patient opted to instead have regular clinical and imaging examinations for screening of the left breast.\nThere has been no clinical or radiological evidence of local or nodal recurrence of her endometrial carcinoma. FNAC of the right thyroid showed no evidence of malignancy, however the patient is considering thyroidectomy due to worsening symptoms of dysphagia. She continues to be monitored in the outpatient clinic setting for her sphenoid wing meningioma and parotid AVM, however she is not keen on operative management. For follow up, she will be offered annual thorough clinical examination including dermatologic assessment as well as screening colonoscopies. She will also perform interval whole body contrast enhanced CT for continued surveillance for metastatic disease. On this examination, her kidneys will also be closely monitored for renal cell carcinoma. |
A 35-year-old, primiparous woman in Wuhan city with a singleton pregnancy of 37 weeks and 6 days was admitted to our hospital (Maternal and Child Health Hospital of Hubei Province) due to regular uterine contractions at 17:50 on February 8, 2020, after undergoing chest CT scans. The chest CT images revealed spotted and slightly high-density shadows scattered on the lateral side of the right lower lobe (Fig. a). Except for lower abdominal pain, the pregnant woman had no signs or symptoms related to COVID-19 infection, and she reported normal fetal movement. None of her family members were diagnosed with COVID-19 or developed any respiratory symptoms. Since the woman developed cervical dilation of 0.5 cm and her CT result was abnormal, she was given a surgical mask instead of her original one. She was then transferred to a designated isolation delivery room by designated medical staff with specialized infection control preparation and strict infection protection equipment, including disposable work caps, medical protective masks, protective goggles, work clothes, disposable protective suit, disposable latex gloves, and disposable shoe covers.\nThe physical examination revealed a body temperature of 37 °C, pulse of 100 beats per minute, blood pressure of 135/86 mmHg, respiratory rate of 20 breaths per minute, and oxygen saturation of 99% while breathing ambient air. Considering that the pregnant woman and her family hoped that she would deliver vaginally and that there was no surgical indication for her, we decided to help her give birth naturally in the isolation delivery room. Fortunately, the labor went smoothly with no fever and no artificial intervention. The mother gave birth to a male infant with a birth weight of 3180 g at 04:58 on February 9, 2020. Apgar scores were 9 and 10 at 1 and 5 min, respectively. During her labor course, the laboratory tests (Table ) showed significantly decreased lymphocyte counts (0.65 × 109/L), which is a common finding in patients with COVID-19 []. Then maternal nasopharyngeal swab samples were collected and sent to the designed hospital for testing for SARS-CoV-2 with the recommended kit in accordance with the WHO guidelines for qRT-PCR [, ]. The results were reported 2–3 days later. Considering the abnormalities in her chest CT images and lymphopenia on hematological examination, the mother was suspected of being infected with COVID-19; we asked the new parents for their opinions on breastfeeding and neonatal isolation, and they decided to temporarily isolate with the newborn and suspend breastfeeding. Then, the infant was discharged home for further isolation, and the mother was transferred to the designated isolation ward after 2 h of observation. Low-flow nasal oxygen inhalation, antiviral therapy and antibiotics were implemented for her.\nThe woman received a positive result for SARS-CoV-2 on the 2nd day after sampling (on February 11). According to this test result, we transferred the mother to the designated hospital and followed up with her by telephone interviews. Luckily, by February 23, which was the 14th day after birth, neither the newborn nor his father and grandmother who took care of him developed any COVID-19-related symptoms. The mother received antiviral (arbidol hydrochloride tablets), antibiotic (azithromycin dispersible tablets) and traditional Chinese medicine (Lianhua-Qingwen capsule) treatments after she was transferred to the designated hospital. During her hospitalization, she did not show any COVID-19-related clinical symptoms. The woman was tested five times, all of which were negative for SARS-CoV-2, after referral (Fig. ). However, the immunoglobulin (Ig) G antibody for SARS-CoV-2 was detected in her serum sample on March 12, so she should be diagnosed as an asymptomatic carrier []. |
A 42-year-old female presented with a history of painless swelling on the left side of the neck from 6 months ago with hoarseness. On clinical examination, a firm, non-tender swelling was noted in the upper part of the anterior triangle of the neck, posterior to the angle of the mandible on the left side () with oropharyngeal bulge and deviation of the uvula and tongue to the right side. Indirect laryngoscopy revealed restricted left vocal cord mobility indicating weakness of the tenth cranial nerve. Clinical signs suggestive of ninth and eleventh cranial nerve involvement were also noted. Family history was non-contributory.\nPlain radiography of the neck () showed well-defined inhomogeneous soft tissue opacity with central amorphous calcifications in the supero-lateral aspect of the neck on the left side. Ultrasonography was done to confirm the mass lesion revealed well defined heterogeneous isoechoic mass lesion in relation to the left parapharyngeal region encasing the carotid vessels and causing displacement of adjacent soft tissue structures with maintained fat planes. Multiple central calcific foci giving post-acoustic shadowing were noted within the mass lesion (). Minimal vascularity was noted on color Doppler study.\nPredominant left parapharyngeal isodense mass lesion showing moderate heterogeneous contrast enhancement with central amorphous and dense calcifications was noted in the CT scan of the neck (). Superiorly, the lesion extended intracranially into the left cerebellopontine (CP) angle cistern through the left hypoglossal and jugular foramina. Encasement of the entire left internal carotid artery and a part of the left external carotid artery was noted. Left internal jugular vein was significantly compressed by the lesion in the upper neck with complete nonvisualization of its upper part. Bony hyperostosis was noted on the left side of the skull base with erosion and enlargement of the left jugular foramen and hypoglossal canal ().\nThe atypical finding on CT scan was presence of two to three continuous linear vertical hypodensities with surrounding calcifications giving the ‘tram track appearance’ () within the lesion possibly suggesting encasement of the cranial nerves (correlated with history, clinical signs and skull base foramen involvement). In correlation with the anatomical course [], it appears that the linear hypodensities with surrounding calcifications in relation to jugular foramen, extending along the posterolateral aspect of the left carotid artery indicates the left vagus nerve and the other with more dense surrounding calcification in relation to the hypoglossal canal extending inferiorly up to the level of oropharynx on the lateral aspect of the mass possibly indicates the left hypoglossal nerve. However, the glossopharyngeal nerve could not be appreciated clearly, which might be due to the lesser calcification around it.\nAn MRI of the neck was performed to see the definitive status of vessels in relation to the lesion. Plain and contrast enhanced MRI with MRA (, , , & ) revealed a heterogeneously enhancing altered signal intensity mass lesion in the left parapharyngeal region with a small enhancing intracranial extradural extension through the jugular foramen and hypoglossal canal. Gradient images showed multiple signal void foci within the lesion suggesting the presence of calcifications. Splaying of carotid vessels with encasement of the internal carotid artery up to the skull base and encasement with significant compression of the internal jugular vein was noted.\nBiopsy of the mass lesion was carried out and histopathology revealed psammoma bodies suggestive of meningotheliomatous (transitional or psammomatous form) meningioma (). |
A 46-year-old man presented with painless swelling of his right buttock. There was a gradual increase in swelling for 2 months leading to difficulty in movement. He refused to have any history of fever, night sweats, and weight loss. The patient was a well-nourished middle-age man with no palpable cervical, axillary, and inguinal lymphadenopathy. Systemic examination was normal, except for a huge right gluteal mass which was not painful on palpation and seemed to be firm, fixed, and deep in the muscle, causing ipsilateral lower extremity weakness. CXR was normal and other laboratory findings including fasting blood sugar, complete blood count, serum electrolytes, liver function test, and coagulation tests were normal. Pelvic magnetic resonance imaging (MRI) detected an extended mass involving the right gluteal, iliopsoas, obturator, piriform muscle, associated with abnormal signals from the right iliac bone and sacrum ala whereas, pelvic organs were intact (Figure ).\nIncisional biopsy was carried out under local anesthesia. Histopathological findings reported a lipomatous tissue. Due to the undetermined diagnosis by incisional biopsy and an indication of soft tissue malignancy, particularly sarcoma, excisional surgery was performed. Findings from the surgery revealed a giant hard mass infiltrating the adjacent muscle extending to the sciatic groove and involvement of the sciatic nerve. Extensive resection but not en bloc resection was performed due to the involvement of the nerve and bone. Primary pathological analysis did not report a definitive diagnosis therefore immunohistochemical (IHC) staining was performed. Following surgery, the patient's right lower limb and genitalia had gradually swollen due to pitting edema. One month later, he was admitted to hospital again: Blood lactate dehydrogenase was 1281 IU/L, abdominopelvic CT scan revealed an abnormal infiltrative mass lesion at the right side of his pelvic cavity extending toward the ischioanal fossa, and an infiltrative mass was also seen at the surgical site (Figure ).\nThe IHC staining of a fixed specimen showed positive staining for leukocyte common antigen, CD3, CD20, Ki67 40% and negative for CD15, CD30, S100, anaplastic lymphoma kinase for the tumor cells. These features are in confirmation with diffuse large B-cell lymphoma; thus, the patient was referred to the department of hematology for further evaluation and treatment. |
The patient is an 82-year-old female who underwent laparoscopic cholecystectomy. She initially presented to the hospital with abdominal pain, nausea, and vomiting. Her abdominal pain was localized to her epigastrium and had progressively worsened. The most pronounced symptom was intractable nausea, despite receiving antiemetics. Additional history provided by a family member confirmed that the patient had only been able to tolerate small meals for the past year. The patient’s past medical history included hypertension, hyperlipidemia, and a large hiatal hernia. Physical exam demonstrated a soft, distended abdomen with right upper quadrant tenderness on palpation, without rebound tenderness or guarding. Her chemistry panel revealed mild hyponatremia, hypokalemia, and hypochloremia with normal liver enzymes, bilirubin, and alkaline phosphatase levels. In addition to her electrolyte derangements, she had a WBC count of 10.1 x109 cells/L with a left shift and neutrophil percentage of 81%. CT imaging demonstrated a large hiatal hernia and a dilated gallbladder with heterogeneous densities within the gallbladder, possibly related to sludge. There was a small amount of pericholecystic fluid, extending along the liver surface. The biliary tree was dilated but no calcified gallstones were appreciated (Figure ). Ultrasound findings demonstrated a distended gallbladder and thickened gallbladder wall measuring 6 mm without cholelithiasis (Figure ). A hepatobiliary iminodiacetic acid (HIDA) scan was subsequently obtained that showed delayed visualization of the gallbladder (Figure ). Given the provided history and labs, a presumptive diagnosis of cholecystitis was made.\nThe patient was taken for laparoscopic cholecystectomy on hospital day five, after resolution of electrolyte abnormalities and failure of conservative measures to resolve her symptoms. After Hasson entry and insufflation of the abdomen, laparoscopic visualization of the patient’s right upper quadrant demonstrated a significant amount of inflammation, with the omentum adherent to the abdominal wall and liver. Careful blunt dissection of the omentum away from these structures eventually revealed a large black, mass-like structure. The mass was acutely edematous. Further blunt dissection with a suction irrigator device allowed for the separation of the mass from the liver bed. The mass was found to be a necrotic gallbladder (Figure ). Once the gallbladder was removed from the surrounding adhesions, it was found to be torsed. Application of a blunt grasper and suction irrigator device successfully detorsed the gallbladder 360 degrees counterclockwise. A very long cystic duct was identified. Clips were applied to the cystic artery and this was divided. The cystic duct was stapled as it was too large to clip. No complications occurred during the procedure. The final surgical pathology revealed chronic cholecystitis with transmural necrosis. The gallbladder was devoid of bile, without calculi, and had wall measurements ranging from 0.1 to 0.6 in thickness with multiple hemorrhagic regions. |
We present the case of a 7-year-old male patient, diagnosed at the age of 3 years with PJS due to a surgical intervention for acute abdominal pain that revealed a rectal polyp associated with hyperpigmented maculae on the lips and oral mucosa. His family history revealed the same condition in his mother, who was diagnosed much later, at the age of 25 years. We mention that since the diagnosis, the patient was not appropriately monitored due to several factors related to the patient's compliance and available resources. We must also mention that during this entire period he presented chronic rectal bleeding.\nThe clinical exam at the time of admission revealed influenced general status, severe pallor of the skin and mucosa, multiple pigmented maculae on the lips and oral mucosa (Fig. ), abdominal pain, abdominal scar post-laparotomy, rectal bleeding, and anal extrusion of polyp during defecation.\nThe laboratory tests performed at the time of admission revealed severe anemia (Hb 6.3 g/dL, MCV 53.8 fL, MEH 13.8 pg, Htc 24.5%). Based on all history and his general status, we decided to administer blood transfusion. The abdominal ultrasound did not reveal any pathological findings. We performed an upper digestive endoscopy, which showed multiple polyps of different sizes within the stomach, the biggest one of approximately 10 mm (Figs. and ). We also performed a colonoscopy and we identified 2 polyps of different sizes at approximately 5 cm from the anal orifice (Fig. ), and we recommended their excision due to the troublesome anal extrusion of the bigger polyp. The small bowel was assessed using barium enteropgraphy, which revealed 3 polyps within the ileum, the biggest one of approximately 10 mm.\nBased on the previous findings, the patient was referred to the surgeon, and both recto-anal polyps were excised, with a favorable evolution. The histopathological exam of the gastric poly and both recto-anal polyps did not reveal any signs of dysplasia, showing specific hamartomatous polyps.\nWe decided to discharge the patient with the recommendations for clinical assessment at least every 6 months, annual laboratory tests, but also follow-up of the detected polyps and screening by upper digestive endoscopy, barium enterography and colonoscopy every 2 years. |
A 48-year-old gentleman presented as an emergency to our institute with left-sided hemiparesis and aphasia. He had no prior co-morbidities, and the family history was insignificant. On examination, he was found to have a power of 0/5 in all muscle groups, according to the medical research council (MRC) muscle grading system, on the left side along with complete aphasia. However, the function of cranial nerve XII was intact. However, the examination was the neck was unremarkable; there was no palpable mass or skin changes in the neck region. The carotid pulses were palpable bilaterally.\nOn initial workup, Magnetic resonance imaging with angiogram was done, which showed a right middle cerebral artery (MCA) territory infarct and a heterogenous lesion within the neck just above the level of the carotid bifurcation. Subsequently, post-contrast images from the head and neck region were also acquired (). The lesion's appearance and characteristics suggested a glomus tumor of the neck. Further evaluation with an ECG and cardiac echo was performed to rule out the cause of an infarct. ECG and cardiac echo were found to be normal. Computed tomography angiogram of the neck was done (), which showed a highly vascular mass, 2.8 x 3.4 x 6.0 cm in size, arising above the carotid bifurcation with its feeding vessel arising from the right external carotid artery. It was splaying the right internal carotid artery anteriorly and medially and extending up to the hypoglossal canal.\nThe radiologist suggested it as an atypical glomus tumor or a rare peripheral nerve sheath tumor. With consideration of the location, clinical presentation, and imaging features, a working diagnosis of glomus tumor of the neck was made.\nBased on our working diagnosis of Glomus tumor, surgical excision was planned. In order to control hemorrhage intra-operatively, angioembolisation of the tumor was done before surgery, and approximately 90% embolization was achieved ().\nUnder general anesthesia, surgical excision was started using the vertical right anterior cervical approach (). Upon exposure to the carotids, the tumor was found to be away and not compressing the internal carotid artery.\nIn further dissection, it was revealed that the tumor was originating from the right hypoglossal nerve, completely encasing it, with some of its portion lying intracranially, and only the distal end was visible just below the angle of the mandible. Unsure about the further step, a literature search was done, and it was found that there were only a few cases reports regarding such tumors with at least two reports suggesting radiosurgery as an alternative to surgical management ().\nHence, with no possibility of tumor resection without sacrificing the hypoglossal nerve, the procedure was abandoned, and the patient was planned for cyberknife therapy. No immediate post-operative complications were noted, and the patient underwent five cycles of radiosurgery (cyberknife). Three months' post-treatment MRI was done, which showed a 45% reduction in the size of the tumor () with significant improvement in symptoms and complete resolution of aphasia. |
A 34-year-old pregnant female (gravida 4, para 2, abortion 1, intrauterine death 1) at 32 weeks of gestation presented with preterm premature rupture of membranes. The patient had a background history of dermatomyositis with interstitial lung disease in remission for the last 4 years. She was initially optimized on steroids and mycophenolate mofetil and subsequently switched to tacrolimus after detection of pregnancy. She underwent an uneventful emergency cesarean section under regional anesthesia. During the next day, she developed a moderately severe headache, which was holocranial and continuous. She had no features of raised intracranial tension. Her headache was not relieved with analgesics, and on the second postoperative day, she developed a generalized seizure during sleep. She continued to be in a comatose state following the seizure with a Glasgow Coma Scale score of eye opening (E)1, verbal response (V)2, best motor response (M)5. Serial blood pressure readings were normal in the post-partum period. Examination revealed bilateral pupils 1.5 mm in size and a sluggish reaction to light, along with a paucity of movements in the right extremities. Magnetic resonance imaging of the brain showed a large intra-parenchymal hematoma involving the left ganglio-capsular and frontotemporal regions causing a midline shift of 16 mm with intra-ventricular extension and uncal herniation (). She underwent emergency craniotomy and hematoma evacuation. A postoperative computed tomography (CT) scan of the brain showed good evacuation of the hematoma and correction of midline shift (). Computed tomography angiography (CTA) showed mild focal narrowing and irregularities in distal branches of bilateral middle cerebral arteries, anterior cerebral arteries, and posterior cerebral arteries (). In the background of dermatomyositis and radiological findings, the possibility of vasculitis was considered. Erythrocyte sedimentation rate was 25 mm/h and C-reactive protein was 6 mg/L. Autoimmune workup for vasculitis including antinuclear antibody, antineutrophil cytoplasmic antibodies, rheumatoid factor, and antiphospholipid antibodies were negative. Viral serology for hepatitis B, hepatitis C, and human immunodeficiency virus was negative. Intravenous pulse methylprednisolone was given for 3 days; however, she continued to be in a deep coma. Serial follow-up CT scans of the brain did not show any hydrocephalus or bleed. Electroencephalogram did not show any epileptiform abnormalities. On the twelfth postoperative day, an examination revealed the patient to be in a quadriparetic state because of a newly developed left-sided weakness. A consequent CT brain showed multiple infarcts in the territories of the right anterior and middle cerebral arteries (). Digital subtraction angiography (DSA) done on the same day showed severe narrowing and irregularity of bilateral anterior, middle, and posterior cerebral arteries, which showed excellent reversibility to intra-arterial milrinone infusion over 15 minutes (, ). She was continued on intravenous milrinone infusion for 7 days. The diagnosis of RCVS was made secondary to her post-partum status, along with a possible etiological implication of tacrolimus therapy. Despite an RCVS2 score of only 4, which implies a low diagnostic sensitivity for the syndrome, the diagnosis of RCVS as opposed to vasculitis, was supported by excellent reversibility of angiographic abnormalities to milrinone []. Subsequently, the patient gradually recovered from her comatose state and started moving her right upper and lower limbs. CTA done before the discharge from the hospital in the sixth postoperative week showed no significant abnormalities. She was conscious and alert at the time of discharge with a power of grade 3/5 and 2/5 in her right and left extremities, respectively. Her neurological recovery is expected to be delayed due to bihemispheric dysfunction and multiple infarcts in the right cerebral hemisphere before milrinone therapy. |
A 35-year-old woman, gravida 4, para 2, was referred to our department with a history of 7-week amenorrhea and a positive pregnancy blood β-hCG assay performed for routine antenatal care. Her obstetric history included a laparoscopic salpingectomy for left tubal pregnancy two years ago.\nThe patient was completely asymptomatic, with stable hemodynamic parameters. Clinical examination was normal: no vaginal bleeding was found, and no lower abdominal or adnexal pain was perceived at combined vaginal examination along with abdominal palpation. Blood β-hCG assay levels were 29386 mUI/ml and then rose to 45057 mUI/ml three days later, while hemoglobin levels were within the normal limits.\nTransvaginal sonography at 7 weeks showed a mass of the right ovary measuring 18 mm. The uterine cavity was free of gestational sac, and no adnexal mass on the left side or fluid in the pouch of Douglas was found.\nThe clinical, biological, and imaging features thus far led to the suspicion of an ovarian pregnancy, for which a Pfannenstiel laparotomy was performed (lack of laparoscopic equipment). While exploring the pelvic cavity, we found a slightly enlarged and soft uterus with a missing left tube, removed from the isthmus. Both the right adnexa and left ovary were normal with no evidence of lesion or of pelvic adhesion. No fluid was found in the peritoneum. No evidence of ectopic intraperitoneal pregnancy could be found, for which we decided to resume the surgical exploration.\nWe concluded that the ovary mass was rather a corpus luteum, and there was no pelvic pregnancy.\nIn the following day, a repeated pelvic ultrasound confirmed the presence of the corpus luteum. Moreover, blood levels of β-hCG continued to rise, reaching 60000 mUI/ml in the third day after surgery.\nWe were facing the complex situation of an evolutive pregnancy given the β-hCG dynamics, in absence of pelvic pregnancy. We then performed an abdominal ultrasound that revealed a large mass in the left para-aortic region. The mass consisted of a gestational sac with an embryo with positive cardiac activity. We completed with an MRI that showed the gestational sac with the embryo and detailed its tight link with the great vessels alongside ().\nThe patient was then scheduled for an exploratory laparotomy by a multidisciplinary team composed of a gynecologist and an abdominal surgeon. The posterior peritoneum was intact. There was no fluid in the peritoneum and retroperitoneum. The retroperitoneal space was carefully dissected, revealing the oval mass of 6 cm attached to the left side of abdominal aorta () that corresponded to a gestational sac with the embryo. The sac was accidentally cracked but could be removed, along with the embryo of 20 mm and its trophoblastic tissue (). Minimal blood loss was reported, and hemostasis was ensured by bipolar diathermy.\nHistopathology examination revealed the presence of decidual tissue and a normal embryo with gestational sac and chorionic villi.\nWe decided not to administer systemic methotrexate postoperatively, as removal of the trophoblastic tissue appeared complete. Indeed, levels of blood β-hCG declined steeply postoperatively from 731 mIU/ml at day 1 to 55 mIU/ml at day 7 post surgery, indicating complete removal of trophoblastic tissue.\nPostoperative period was uneventful, and the patient left on the 7th day. Blood β-hCG levels were measured weekly and reached the undetectable threshold at the twenty-fifth postoperative day. |
A 3-year-old girl presented with darkened and swollen right lower limb and in ability to utilize the right side of the body following febrile illness with associated generalized body rash which started from the face and progressed to the whole body; this was preceded with complaint of coryza; she also had bilateral reddish eye discharge; this occurred during the 2013 period of measles outbreak in her community and she was not immunized for measles. A week after skin desquamation the mother noticed darkened and swollen right foot which was painful, progressive, and by 24 h it had involved up to the ankle; the toes then became blackened []. The patient had several episodes of generalized tonic-clonic seizures. At about same time after seizure control, mother noticed she could not use the right upper and lower limbs []. She was transferred to our hospital after initial management in a general hospital. On examination, she was not febrile; the cardiac and respiratory examinations were not remarkable. All peripheral pulses were palpable except for the right posterior tibial and dorsalis pedis arteries. At that point, she had regained consciousness, the right upper and lower limbs had reduced power (estimated power were 3/5 in both limbs); the left limbs were normal, there were increased tone in both right upper and lower limbs; and the right foot had areas of darkened and shrunken toes which were not tender with proximal areas of tender swollen purpuric skin. She had deranged clotting profile, that is, activated partial thromboplastin time (aPTT) of 90 s (aPTT = 60-85 s) and prothrombin time (PT) of 28 s (PT = 11-15 s) and thrombocytopenia of 90,000/mm3 (150,000-400,000/mm3) results from referring hospital; but the repeat in our hospital were normal, and there was no site of active bleeding. Magnetic resonance imaging (MRI) of the brain was normal [], similarly the echocardiograph was normal but the vascular Doppler study confirmed reduced flow in the right posterior tibial artery. Neither fresh frozen plasma infusion nor platelets transfusion was given at the referring hospital. The diagnosis was measles encephalitis with purpura fulminans. She was billed for transtibial amputation of the right lower limbs, but parents declined amputation despite adequate counseling that the grandparents refused amputation. She had honey dressing of the affect limb for 2-weeks with some good effect [] before they opted to leave against medical advice. |
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