Primary amenorrhoea and clitoromegaly in a nulliparous woman: successful medical and surgical management

Clitoromegaly is an important sign of virilisation and poses difficulty in sex determination, when present since birth. The diagnosis and treatment in an adult is a major challenge to the treating gynaecologist. The primary reason for its development is androgen excess due to congenital adrenal hyperplasia, polycystic ovarian syndrome, ovarian virilising tumours, neurofibromas, adrenal neoplasm and prolonged intake of anabolic steroids. A case of young nulliparous married woman who presented with primary amenorrhoea and clitoromegaly and was managed successfully has been reported.

https://ift.tt/2Oksp5d

Giant left breast hamartoma in a 45-year-old woman

This is a case of a woman in her fifth decade of life who presented with a lump in her left breast. The patient underwent extensive breast investigations which did not confirm one particular diagnosis. The lesion was excised, and histological findings confirmed the diagnosis of a giant breast hamartoma. This case is interesting due to the largeness. As well as this, giant breast hamartomas are uncommon, and this case highlights the difficulty in their diagnosis and frequent misdiagnoses, potentially contributing to their reported low incidence rates. Diagnosis is difficult due to the complex appearance of the mass on imaging, raising the possibility of malignancy. Core biopsy is unremarkable, with the final diagnosis only possible on histological examination of the resected mass.

https://ift.tt/2Qi7Zaf

Hypoxic hepatitis and furosemide

Hypoxic hepatitis is a rather common complication of heart, circulatory or respiratory failure. We present the case of a patient with hypoxic hepatitis in the setting of heart failure and dehydration from furosemide as a reminder of an important clinical lesson. The pathogenesis of hypoxia (especially in the case of heart failure) is explained by a two-hit mechanism in which the liver at risk of hypoxic injury by passive hepatic congestion (right heart failure) is subsequently exposed to systemic hypoperfusion, which leads to a marked and transient elevation of aminotransferases. In the case presented, the use of furosemide (at least partially) promoted the second hit because it helped to generate hypotension and splanchnic hypovolaemia and favoured hepatic hypoxia.

https://ift.tt/2OvR5rD

Red colour venous flow in the suprasternal view: a red flag sign

Description 

A 3-day-old full-term neonate, born by normal vaginal delivery, was presented with respiratory distress and mild cyanosis. His room air saturation in all four limbs was 84%. The clinical examination was unremarkable. Chest X-ray showed cardiomegaly and increased pulmonary vascular markings. Transthoracic echocardiography revealed 8 mm ostium secundum atrial septal defect (ASD) with bidirectional shunt and dilated right atrium and right ventricle with absence of individual pulmonary veins (PV) draining into left atrium (LA). Suprasternal view showed vertical vein (VV) which was recognised as a vessel lateral to LA with red-coloured continuous flow on Doppler (figure 1). The PVs were draining into the venous confluence behind the LA which was draining into the VV. VV connects into the left innominate vein which finally forms the right superior vena cava (figure 1, video 1).

Figure 1

Suprasternal view demonstrating vertical vein....

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Use of long saphenous vein graft in acute on chronic mesenteric ischaemia

Acute mesenteric ischaemia is a relatively rare surgical emergency, but despite advances in diagnostic tests, the mortality of this condition remains stubbornly high (50%–80%). This is principally because of the non-specific nature of the presenting symptoms and subsequent delay in diagnosis. We report an unusual case of acute mesenteric ischaemia treated by emergency laparotomy, small bowel resection and revascularisation using reversed long saphenous vein graft.

https://ift.tt/2OuqZVW

Primary undifferentiated pleomorphic cardiac sarcoma with MDM2 amplification presenting as acute left-sided heart failure

Primary cardiac tumours are a rare clinical entity that can present with myriad of non-specific cardiopulmonary symptoms. We describe a case of a 61-year-old previously healthy woman who presented with progressive dyspnoea and lower extremity swelling, suggestive of acute left-sided heart failure. Transthoracic echocardiogram revealed a large, 3.7x3.2 cm intracardiac mass resulting in severe mitral valvular dysfunction. The patient underwent surgical resection of the mass, however, negative margins were not obtained, and the tumour quickly returned. Histological and molecular analysis was consistent with the diagnosis of undifferentiated pleomorphic sarcoma with murine double minute 2 (MDM2) amplification. Given the overall grim prognosis, the patient chose to pursue comfort-based care. She died at home 9 months after the initial diagnosis. Here, we provide an updated review of the literature for the classification of undifferentiated pleomorphic cardiac sarcoma and potential treatment modalities.

https://ift.tt/2QgTJ1o

A delayed diagnosis: recurrent fever and beta thalassaemia

Familial Mediterranean fever and beta-thalassaemia are two genetic disorders, with a largely common geographical distribution. However, they have not much else in common, as the first is an autoinflammatory disorder, while the other is a haemoglobinopathy. We describe a patient with known beta-thalassaemia intermedia who presented with recurrent fevers and he was diagnosed with familial Mediterranean fever 2 years later. We discuss whether there is an association between the two disorders and the cognitive biases that lead to the delay in the diagnosis of familial Mediterranean fever.

https://ift.tt/2OrAHIg

Imaging features of toxic megacolon

Description 

A 27-year-old woman presents with vomiting and abdominal pain for 2 days. She had been diagnosed with ulcerative colitis (UC) 2 weeks prior and was started on oral sulfasalazine and prednisolone. Currently, she is afebrile and not tachycardic. On examination, the abdomen was distended and tender centrally. Serial abdominal radiographs showed persistently dilated transverse colon with loss of haustra (figure 1). CT abdomen revealed diffuse bowel thickening involving the ascending colon, caecum and terminal ileum with multiple pseudopolyps at the ascending colon (figure 2). The transverse colon is dilated up to 6.8 cm (figure 3). There are also multiple enlarged right-sided mesenteric nodes. A diagnosis of toxic megacolon (TM) was made. She deteriorated during admission due to acute pulmonary embolism. Blood investigations showed marked leucocytosis (32x10⁹/L) and raised C-reactive proteins (98.7 mg/L). Subtotal colectomy involving the caecum until splenic flexure was performed followed by ileostomy and mucous...

https://ift.tt/2Qk8l04

Modified laparoscopic Janeway gastrostomy: a novel adjunct for the management of choledocholithiasis in Roux-en-Y patients

A 76-year-old woman with surgical history of Roux-en-Y gastric bypass presented with recurrent choledocholithiasis. Double balloon enteroscopy was unsuccessful in cannulating the biliary tree, thus, requiring surgically assisted endoscopic retrograde cholangiopancreaticogram (ERCP) access. Due to her stable clinical status, the non-urgent indication and multiple anticipated ERCPs for definitive biliary clearance, a more durable port of access to the ampulla was desired. A modified laparoscopic Janeway gastrostomy of the gastric remnant was performed and served as access for multiple subsequent endoscopic procedures with successful clearance of the biliary tree.

https://ift.tt/2OrAODG

DRESS syndrome due to vemurafenib treatment: switching BRAF inhibitor to solve a big problem

We present a case report of an early-onset drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) induced by vemurafenib (BRAF inhibitor) in a middle-age man affected by a metastatic, BRAF mutant melanoma who was started on first-line metastatic treatment with vemurafenib and cobimetinib.

After initiating the treatment, the patient presented an extensive cutaneous rash with eosinophilia and renal impairment. Due the constellation of signs and symptoms, a diagnosis of DRESS syndrome was made which strongly contraindicated the reintroduction of vemurafenib due to its hypersensibility reaction. Thus, vemurafenib was stopped immediately, and we started corticoid treatment with clinical improvement.

Due to the contraindication to start vemurafenib again, after multidisciplinary view of the case and having balanced the risks and benefits, we successfully performed a switch to another BRAF inhibitor in a progressively ascending pattern, without any skin toxicity and with a good response of the metastatic melanoma.

https://ift.tt/2QjVXgw

Duplicated middle cerebral artery associated with aneurysm at M1/M2 bifurcation: a case report

A duplicated middle cerebral artery arises from the internal carotid artery and supplies blood to the middle cerebral artery territory. A duplicated middle cerebral artery is sometimes associated with an intra...

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Hepatocellular carcinoma presenting as spinal cord compression in Native Americans with controlled hepatitis C: two case reports

Hepatocellular carcinoma is a common malignancy in Asia. It is associated with chronic hepatitis B virus or hepatitis C virus infection and alcoholic hepatitis. Commonly, the tumor metastasizes to the lungs, r...

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Diagnosing microscopic pancreatic neuroendocrine tumor using 68-Ga-DOTATATE PET/CT: case series

Abstract

Neuroendocrine tumors (NETs) are an uncommon diagnosis which often present asymptomatically or with vague symptoms. They can originate from many different organs such as the GI tract, lungs, pancreas and others. ⁶⁸Gallium DOTATATE positron emission topography/computed topography has been shown to be an effective imaging modality for diagnosing NET and evaluating management options for patients. Here, we present two cases of positive findings in the distal pancreas on the DOTATATE PET/CT scans without any morphological lesion found to be NET in a healthy 48-year-old male and 68-year-old male.

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Adult ileocecal intussusception induced by adenomatous ileal polyp: case report and literature review

Abstract

Intussusception is a rare cause of bowel obstruction in adults, and has generally an organic etiology. However, adenomatous polyp of the small bowel is an uncommon etiology. Moreover, there's a great difference with childhood intussusception in its presentation, etiology and management. We describe herein a case of adult ileocecal intussusception due to an adenomatous ileal polyp with a preoperative diagnosis made on computed tomography. We performed a right hemicolectomy, without attempting to reduce the intussusception, and an end-to-end ileotransverse anastomosis. The pathological examination of the surgical specimen revealed an adenomatous polyp with a high grade dysplasia on the terminal ileum, being the cause of the ileocecal intussusception.

https://ift.tt/2Ooe4oa

Biliary obstruction secondary to migrated intra-duodenal gastric band: a case report

Abstract

Adjustable gastric banding (AGB) was a previously popular bariatric procedure, but adverse events such as erosion have surfaced as common complications. We present an interesting case of an AGB causing biliary obstruction after eroding into the stomach.

https://ift.tt/2DFYsZa

Spontaneous rupture of the urinary bladder due to bacterial cystitis

Abstract

We report a case of spontaneous rupture of the urinary bladder (SRUB) due to bacterial cystitis in a 76-year-old woman with chief complaint of abdominal pain a day before presentation. She had fever (38.0°C), and her systolic blood pressure dropped to 70 mmHg; she was referred to our hospital, where she was admitted with a diagnosis of ileus. However, her abdominal pain worsened the following day, and abdominal CT showed free air. Emergency laparotomy was performed for suspicion of digestive tract perforation, which revealed a small hole at the dome of the urinary bladder and another at the peritoneum. Suture repair was performed. We reviewed the abdominal CT on admission and noted that the perforation of the urinary bladder was present during admission, whereas that of the peritoneum occurred the following day. SRUB is rare, and bacterial cystitis rarely causes it; thus, accurate diagnosis and proper treatment are essential.

https://ift.tt/2OodVkC

Adenoma mimicking hyponatremia of SIAD

Hyponatremia is a common electrolyte disorder, with prevalence as high as 20% in inpatient settings. It is classified based on volume status, urine sodium and osmolality results. While this approach might help narrow down the differential diagnoses, it can leave other diagnoses unentertained. In this case, we report recurrent and refractory hyponatremia secondary to hypocortisolism due to non-functioning pituitary macroadenoma. Interestingly, urine studies mimicked syndrome of inappropriate antidiuresis, but exclusively responded to hydrocortisone replacement. Hospital course was also complicated by hyponatremia-induced rhabdomyolysis, which is a rare complication of severe hyponatremia. We also discuss the role of anchoring heuristics and how they influence the physician's decision leading to possible diagnostic errors. One way to minimise the effect of anchoring bias on physicians is their cognitive awareness of such bias. In addition, discussing complicated cases with all members of medical team can highlight the clinician's thought processes, share uncertainty and help broaden differential diagnoses.

https://ift.tt/2xKftfw

Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis

Pneumatosis cystoides intestinalis (PCI) refers to the presence of gas within the wall of the small or large intestine. The pathophysiology is incompletely understood and is probably multifactorial in nature. PCI is a known but rare complication of systemic scleroderma, and the aetiology of PCI in patients with scleroderma is not fully understood. We present the case of a patient who was referred to gastroenterology clinic by her general practitioner for investigation of 8 months of weight loss, urgency, diarrhoea, bloating and crampy abdominal pain. Extensive investigations were performed to exclude infective, inflammatory or malignant aetiologies for these symptoms. She was diagnosed with PCI on her colonoscopy and was subsequently screened for secondary causes. Our patient was diagnosed with the limited cutaneous (CREST) variant of systemic scleroderma. This case report illustrates that PCI could be an uncommon presentation of systemic sclerosis, therefore clinicians should be aware of the association between these conditions.

https://ift.tt/2DDZfKb

Correction: Non-operative management, supported by self-monitoring using web-based patient reported outcome measures (PROMs), in knee osteoarthritis

Webb E, Parkes RJ, Gough AT, et al. Non-operative management, supported by self-monitoring using web-based patient reported outcome measures (PROMs), in knee osteoarthritis. BMJ Case Rep 2018. doi:10.1136/bcr-2017-223560.

This article was published with an error in the funding statement. The correct funding statement should read: 'the authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors'.

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Perianeurysmal vasogenic oedema (PAVO) following aneurysm embolisation: a unique case of asymptomatic long-term progression and review of the literature

Perianeurysmal vasogenic oedema is a recognised although rare phenomenon following endovascular treatment of certain intracranial aneurysms. We present a unique case of asymptomatic perianeurysmal vasogenic oedema following bare platinum coil embolisation of an incidentally discovered right middle cerebral artery aneurysm that slowly increased over a period of 6 years before stabilising and regressing. During this time, the coiled aneurysm per se remained completely stable on serial magnetic resonance angiography.

https://ift.tt/2DGrjg1

Pneumopericardium due to bronchopericardial fistula in a patient with lung cancer

Description 

Documented cases of pneumopericardium in patients with lung cancer are extremely rare.

We report the case of a 66-year-old man with a 45 pack-year smoking history and an Eastern Cooperative Oncology Group performance status 4. He presented with dyspnoea and chest pain in the last 2 hours. There were no signs of cardiac tamponade present.

CT angiography of the chest revealed a 78 mm right pulmonary mass containing small areas with gas density and a pneumopericardium of 28 mm in maximum thickness (figure 1). Transthoracic echocardiography showed the air gap sign, identified as the loss of signal during the systolic phase presented in patients with pneumopericardium. Bronchofibroscopy showed extensive infiltration and destruction of the carina bronchial wall and of the main bronchi by malignancy and endobronchial disease (figure 2). Endobronchial biopsies revealed an invasive squamous cell carcinoma.

Figure 1

CT angiography of the chest...

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Unusual case of primary pulmonary Hodgkins lymphoma presenting with a continuous murmur

Systemic to pulmonary fistulas are an unusual entity, even more so in association with Hodgkin's lymphoma. We herein report a case of a 33-year-old woman that presented with an incidental lung lesion on a chest radiograph with an associated high-frequency continuous murmur over the lesion. The diagnosis of primary pulmonary Hodgkin's lymphoma, nodular sclerosis type, was obtained by a CT transthoracic biopsy. We achieved an excellent response after polychemotherapy with near-complete disappearance of the mass and a residual faint systolic murmur over the lesion.

https://ift.tt/2DHk4Er

Gardener-associated fibroma: an unusual cause of upper airway obstruction

We present the first case of upper airway obstruction secondary to a retropharyngeal Gardner-associated fibroma (GAF). A 16-month-old infant presented with a 3-month history of worsening dyspnoea and apnoeic episodes. Examination revealed stridor and left-sided retropharyngeal asymmetry. MRI demonstrated a mass in the retropharynx. Tracheostomy and pharyngeal biopsy under anaesthesia were performed, and histology confirmed a diagnosis of GAF. The mass was excised using a transcervical approach, and postoperative recovery was unremarkable. GAF is associated with Gardner's syndrome (GS) and familial adenomatous polyposis (FAP), both of which are associated with multiple colonic polyps and increased risk of colorectal malignancy. Subsequent testing for an APC mutation seen in GS and FAP was negative in our patient. The details of this unusual presentation of a rare disease are given in addition to a review of the literature.

https://ift.tt/2xKCQpd

Liver transplantation for inferior vena cava leiomyosarcoma: from a Maslows hammer to the Occams razor

Leiomyosarcoma (LMS) of primary vascular origin is a rare entity with only potentially curative option being complete surgical resection; despite which the prognosis remains dismal. Tumour recurrence is very common, and the benefits of adjuvant therapy are undefined. A 39-year-old woman presented with 6 months' history of abdominal pain, abdominal distension and pedal oedema. On evaluation, she was diagnosed to have chronic Budd-Chiari syndrome (BCS) secondary to a tumour arising from the inferior vena cava (IVC) on evaluation. Her liver decompensation included jaundice, gastrointestinal bleed and ascites. Following a detailed multidisciplinary team discussion, she underwent complete excision of the tumour along with a segment of the IVC with living donor liver transplantation. She remains disease-free 24 months following surgery. This is the first reported case of liver transplantation for IVC LMS causing chronic BCS.

https://ift.tt/2DCzrhC

Kiloh-Nevin syndrome associated with humeral shaft fracture

Fracture humerus may be associated with nerve injuries. However, among them median nerve is uncommonly involved and clinical affection of the anterior branch of median nerve is rare which can be easily missed and could be under-reported. A 42-year-old man presented with closed fracture of shaft of humerus right sided following fall. He had isolated weakness of flexor pollicis longus and flexor digitorum profundus of index finger without any other sensorimotor dysfunction. The findings were consistent with isolated palsy of anterior interosseous nerve (AIN). The patient was operated with fixation of humerus without exploring the AIN. At 6-month follow-up, the patient had complete recovery of both the muscles. This case highlights the classical clinical presentation of a rare injury associated with humerus fracture. Awareness and knowledge of this entity is of paramount importance for the practitioners.

https://ift.tt/2xKCKOn

Neuromyelitis optica with unilateral diaphragmatic paralysis

In this case report, we describe the course of a female patient who is known to have neuromyelitis optica (NMO) and developed left hemiparesis and unilateral diaphragmatic paralysis. She was initially treated with intravenous methylprednisolone 1 g daily for 5 days without improvement. Subsequently, she received five sessions of a plasmapheresis. Her hemiparesis had improved after few days. however, the phrenic nerve palsy remained.

https://ift.tt/2DNUuOm

Leptomeningeal dissemination of spinal pilocytic astrocytoma: a rare entity

Description 

A currently 12-year-old boy presented at the age of 8 months with sleepiness, irritability and a tense fontanelle with upgaze palsy on a background of a 2-month history of vomiting, difficulty feeding and weight loss. Acute communicating hydrocephalus was diagnosed using ultrasonography and CT and treated with Ventriculo-Peritoneal shunt insertion. Further investigation with MRI (2006) found a thoracic intramedullary mass with further intracranial and spinal leptomeningeal dissemination (LD) (figures 1 and 2). A biopsy of the thoracic intramedullary mass showed pilocytic astrocytoma (PA) (figure 3).

Figure 1

Initial MRI whole spine (2006). (A) T2 sagittal, (B) T1 sagittal and (C) postcontrast T1 sagittal. Black arrows in (A) and (B) show upper thoracic intramedullary mass. The white arrow in (C) shows no significant enhancement. Thick white arrows in (C) show leptomeningeal enhancement.

Figure 2

Initial MRI brain...

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Severe calcium pyrophosphate dihydrate deposition disease of the metacarpophalangeal joints

We report a case of calcium pyrophosphate deposition disease (CPPD) with an unusual presentation of severe chondrocalcinosis with atypical large burden deposited in the metacarpophalangeal joints as well as more typical deposition in wrists and knees as demonstrated on plain radiographs. A 77-year-old African-American woman 1-year status post parathyroidectomy for hyperparathyroidism initially presented to the rheumatology clinic to treat suspected rheumatoid arthritis given her pattern of joint involvement but was found to have CPPD. The patient's history is notable for end-stage renal disease which complicates medical management. This case illustrates radiographic findings of CPPD and explores the challenges of treating CPPD in the setting of comorbid conditions.

https://ift.tt/2DNUqhA

Effect of high-flow high-volume-intermittent hemodiafiltration on metformin-associated lactic acidosis with circulatory failure: a case report

Metformin-associated lactic acidosis is a well-known life-threatening complication of metformin. We here report the case of a patient who developed metformin-associated lactic acidosis without organ manifestat...

https://ift.tt/2xYyH00

Impact of non-invasive continuous blood pressure monitoring on maternal hypotension during cesarean delivery: a randomized-controlled study

Abstract

Purpose

This study aimed to investigate the efficacy of the ClearSight™ system (Edwards Lifesciences, Irvine, CA) for reducing the incidence of hypotension compared with the traditional oscillometric blood pressure monitoring in cesarean delivery under spinal anesthesia.

Methods

Forty patients undergoing cesarean delivery under spinal anesthesia were enrolled. The patients were randomly divided into two groups (Control and ClearSight groups). All patients received spinal anesthesia using 0.5% hyperbaric bupivacaine (11.5 mg) and fentanyl (10 µg). Blood pressure was managed with the same protocol using the ClearSight™ system (ClearSight group) and oscillometric blood pressure monitoring (Control group). Furthermore, we compared the accuracy of the ClearSight™ system with the traditional oscillometric monitoring for blood pressure measurement using Bland–Altman, four-quadrant plot, and polar plot analyses.

Results

The incidence of hypotension was significantly lower in the ClearSight group from induction to delivery (45% vs. 0%, p < 0.001) and to the end of surgery (50% vs. 20%, p = 0.049). Intraoperative nausea occurred more frequently in the Control group (45% vs. 10%, p = 0.012). The ClearSight™ system demonstrated acceptable accuracy with a bias of − 4.3 ± 11.7 mmHg throughout the procedure. Four-quadrant analysis revealed an excellent trending ability of the ClearSight™ system with a concordance rate of approximately 95%. In the polar plot analysis, the angular bias and concordance rate were − 13.5° ± 19.0° and 76.9%, respectively.

Conclusions

The accuracy and trending ability of the ClearSight™ system for blood pressure measurement was clinically acceptable in cesarean delivery under spinal anesthesia, leading to reductions in maternal hypotension and nausea.

https://ift.tt/2Qi5PYg

Total parenteral nutrition in the pleural space

Description  

A male infant was delivered spontaneously at 29+3 weeks and transferred to the local neonatal unit for uplift in care. This included 24 hours of ventilation, 12 hours of dobutamine and placement of a peripherally inserted central catheter (PICC) for total parenteral nutrition (TPN). By 24 hours he was extubated onto Optiflow at 6 L/min and Fraction of Inspired Oxygen (FiO₂) of 0.21. Expressed breastmilk was commenced whilst awaiting PICC placement.

A 22G 80 mm Leaderflex PICC was aseptically inserted on day 2 into the right antecubital fossa. The line did not bleed but flushed easily. A 0.9% sodium chloride infusion was commenced whilst awaiting confirmation of line position. Chest X-ray showed the PICC projected over the subclavian vein with mild residual airspace shadowing in both lungs (figure 1). The PICC position was felt to be acceptable when the X-ray was reviewed prospectively and retrospectively.

Figure 1

Chest X-ray showing peripherally inserted central catheter...

https://ift.tt/2OpFR81

Extracranial meningiomas concurrently found in the lung and vertebral bone: a case report

Primary pulmonary meningiomas are very rare, and primary intraosseous meningiomas outside the head and neck region have not yet been reported. We report an extremely unusual case of concurrent meningiomas aris...

https://ift.tt/2Qc7i2b

Suppressed descending pain modulatory and enhanced sensorimotor networks in patients with chronic low back pain

Abstract

Purpose

Although cerebral structural and functional changes were uncovered by neuroimaging in patients with chronic low back pain (CLBP), their associations remain to be clarified. We co-analyzed anatomical and functional magnetic resonance imaging data in those patients and tested whether cortical gray matter volume changes are associated with altered pain modulatory networks underlying chronification of pain.

Methods

In 16 patients with CLBP and 16 heathy controls, we performed functional magnetic resonance imaging during mechanical pain stimulation on the lower back followed by anatomical imaging. We performed voxel-based morphometry and functional connectivity analysis from the seeds with altered gray matter volume, and examined correlations between imaging and psychophysical parameters.

Results

Patients showed decreases in gray matter volume at the right dorsolateral prefrontal cortex, middle occipital gyrus, and cerebellum, and showed increases at the bilateral primary sensorimotor cortices, left fusiform gyrus, and right cerebellum compared with controls (P < 0.001). Dorsolateral prefrontal and fusiform volumes showed negative associations with affective comorbidity, whereas motor cortex volume with impaired daily activity (P < 0.05). Connectivity was decreased between the cerebellar and limbic, and increased between the bilateral sensorimotor regions (P_FDR < 0.05). Higher pain intensity and unpleasantness correlated with enhanced bilateral sensorimotor and dorsolateral–medial prefrontal networks, respectively (P < 0.05).

Conclusion

Patients showed a decreased volume of cortical center for descending pain modulation and an increased volume of sensorimotor network, in association with suppressed descending pain modulatory and cerebellum–limbic networks and enhanced sensorimotor network during pain. Such structural and functional alterations might be part of cerebral pathophysiology of CLBP.

https://ift.tt/2N6htDj

Paediatric ocular super glue injuries: assessment of two cases

In this report, we elaborate the clinical findings and the optimal management of two cases with ocular glue injuries in two paediatric patients. Both the patients were presented to the ocular emergency with completely closed eyelid and periocular erythema. The eyelids were completely closed due to matting of the eyelashes with glue retention up to lash roots. Thus, in both cases, after the application of local anaesthetic agent, immediate lash trimming was performed along with the removal of crystallised glue particles. However, the ocular surface showed only congestion in absence of any added corneal complications. Patients were followed up with standard treatment protocol as per chemical injuries. Until the end of 5 months, the clinical course was uneventful.

https://ift.tt/2QbJE64

Comorbid presentation of syringomyelia and Guillain-Barre syndrome, attributed to mycoplasma, in a 6-year-old female patient

Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord that can cause loss of sensation and muscle spasticity. Guillain-Barre syndrome (GBS) is a postinfection autoimmune disease, classified as an acute polyneuropathy. This report describes the emergency admission of a 6-year-old girl presenting with sudden pallor and pain in both lower limbs. The patient's reflexes were normal, as were the results of her sonography, radiography and biochemical tests; however, spinal MRI revealed extensive compartmentalised syringomyelia extending from C2 to T3. A sensory and motor nerve conduction study revealed a demyelinating type motor polyneuropathy which, along with positive Mycoplasma pneumoniae test, was suggestive of GBS. Intravenous immunoglobulin infusion showed excellent results. In conclusion, we report a rare paediatric case of syringomyelia coexisting with GBS. It is important to bear in mind the possibility of other coexisting diseases even if MRI reveals definitive characteristics of another condition.

https://ift.tt/2zA9FGq

Diagnosis of a tick-borne coinfection in a patient with persistent symptoms following treatment for Lyme disease

A 67-year-old woman presented with 5 days of myalgias and fevers on completion of a 21-day course of amoxicillin for Lyme disease (Borrelia burgdorferi infection). She was found to have profound thrombocytopenia, as well as new anaemia and leucopenia. Workup revealed Babesia microti as the causative agent of her symptoms. The patient quickly improved after appropriate antimicrobial therapy directed against babesiosis was started. This case illustrates the importance of basic microbiology, including epidemiology and common vectors, when creating a differential diagnosis. Because the Ixodes scapularis tick can harbour and transmit multiple parasites simultaneously, the possibility of coinfection should be considered in any patient not responding to appropriate initial medical therapy.

https://ift.tt/2Q8BUS6

Desire for lasting long in bed led to contact allergic dermatitis and subsequent superficial penile gangrene: a dreadful complication of benzocaine-containing extended-pleasure condom

Description 

A 30-year-old non-atopic, non-diabetic man presented with complaints of swelling, pain and patchy blackening of penile skin for the last 3 days. He stated that for improving the duration of sexual intercourse and for better sexual performance, he recently used a new brand of condom, which contained 5% benzocaine. He, however, denied about the use of any lubricants or topical substance at the time of sexual act. There was no history of fever, inguinal or scrotal swelling, genital trauma, insect bite, known drug allergy, substance abuse or similar complaints in the past. In the past, he was using non-medicated natural rubber latex condom for contraception without any adverse effects. Local examination revealed vesicle eruption, sloughing and patchy blackening of penile skin extending from prepuce to base of penis with a clear demarcation line with non-palpable inguinal lymph nodes suggestive of allergic contact dermatitis as shown in figure 1. The underlying...

https://ift.tt/2zzKPqe

Knotted electric wire in urinary bladder: Can such complex foreign body be retrieved endoscopically!

Various types of foreign bodies have been recovered from the urinary bladder and urethra including telephone cables, pocket battery, little fish, pen tips and so on. Management of such cases include either endoscopic removal or open surgery. We report the case of an 18-year-old boy who inserted an electric wire in the urethra for eroticism and was managed by endoscopic removal following fragmentation of wire under local anaesthesia using holmium laser.

https://ift.tt/2QdFESt

Recurrent aseptic meningitis with PIGT mutations: a novel pathogenesis of recurrent meningitis successfully treated by eculizumab

We report the case of a patient with PIGT mutations who experienced recurrent aseptic meningitis 121 times over 16 years before developing paroxysmal nocturnal haemoglobinuria (PNH). Each episode was preceded by urticaria and arthralgia. After developing PNH, haemolysis occurred prior to meningitis. Flow cytometry revealed deficiency of the glycophosphatidylinositol (GPI)-anchored complement regulatory proteins, CD59 and CD55, and he was diagnosed with PNH. All the symptoms disappeared on administering eculizumab, an anti-C5 antibody. We did not detect mutation in PIGA, which is regarded as the cause of PNH. However, we detected a germ-line mutation and a somatic microdeletion in chromosome 20q including PIGT; PIGT is essential for transferring GPI anchor to the precursors of CD59 and CD55, which play important roles in complement regulation. Loss of these proteins leads to complement overactivation, causing inflammatory symptoms, including recurrent meningitis. PIGT mutations should be considered a novel pathogenesis of recurrent meningitis of unknown aetiology.

https://ift.tt/2zAyO45

Obstructive urosepsis secondary to ureteric herniation into the sciatic foramen

A 65-year-old female patient presented with a 2-day history of worsening right-sided abdominal pain and clinical signs of sepsis, on the background of a recent admission for pancolitis. Imaging revealed right ureteric herniation through the sciatic foramen with subsequent hydroureteronephrosis and radiological signs of severe pyelonephritis. The patient underwent emergency decompression with a percutaneous nephrostomy, followed by insertion of an antegrade stent to correct the ureteric hernia once the sepsis was appropriately treated. We describe the presentation and management of this unusual case of herniation of the right ureter into the sciatic foramen.

https://ift.tt/2QfcMtc

Acromioclavicular joint cyst formation in a patient with rotator cuff-tear arthropathy: a rare cause of shoulder discomfort

We report a case of a 77-year-old man who presented to our shoulder department with a soft tissue mass on his right acromioclavicular (AC) joint. Previously attempted puncture aspiration had revealed serous fluid retention which recurred after each of several drainage attempts. Conventional radiography and MRI of the affected shoulder joint demonstrated a progressed cuff-tear arthropathy with an irreparable tear of the supraspinatus tendon, static superior migration of the humeral head, opening of the AC joint capsule and a superior joint-fluid 'eruption' and accumulation called 'Geyser sign'. Given that the patient's cuff-tear arthropathy was very well compensated, arthroscopic rotator cuff debridement and open cyst excision were performed. Closure of the superior aspect of the AC joint capsule was performed by the aid of a collagen matrix with additional closure of the deltotrapezial fascia. One year postoperatively, no cyst recurrence was noted.

https://ift.tt/2zAvNAC

Atypical presentation of molar pregnancy

The classic features of molar pregnancy are irregular vaginal bleeding, hyperemesis, enlarged uterus for gestational age and early failed pregnancy. Less common presentations include hyperthyroidism, early onset pre-eclampsia or abdominal distension due to theca lutein cysts. Here, we present a case of molar pregnancy where a woman presented to the emergency department with symptoms of acute abdomen and was treated as ruptured ectopic pregnancy. The woman underwent laparoscopy and evacuation of retained products of conception. Histological examination of uterine curettage confirmed the diagnosis of a complete hydatidiform mole. The woman was discharged home in good general condition with a plan for serial beta-human chorionic gonadotropin (beta-hCG) follow-up. Complete follow-up includes use of contraception and follow-up after beta-hCG is negative for a year.

https://ift.tt/2QbFTx8

Lupus causing small bowel obstruction

A 20-year-old female patient was admitted to hospital in 2015 with 1 year history of recurrent abdominal pain, distension, borborygmi and nausea. The patient had a background of systemic lupus erythematous (SLE) diagnosed 4 years before, with skin, joint and renal involvement. The initial investigations have shown a long segment of ileal inflammation with upstream obstruction. Differential diagnoses were mainly SLE enteritis or concomitant Crohn's. Patient failed the initial conservative management and had a laparotomy with small bowel (SB) resection and ileostomy. The histology was suggestive of autoimmune enteritis. Although bowel involvement is a frequent feature of SLE, surgery for obstruction is extremely rare. Postoperatively, she had an emergency admission and was diagnosed with SB volvulus with perforation. She underwent further resection and stoma refashioning in 2016. As a consequence, she developed short gut syndrome. Eventually, the stoma was reversed and parenteral nutrition was stopped and weight became stable.

https://ift.tt/2zzfbcw

Idiopathic macrocheilia

A 13-year-boy presented with painless swelling of upper and lower lips accompanied with gingival enlargement. The aetiology for these symptoms included vast pathological varieties but none of them could fit in. Clinical features were similar to orofacial graulomatosis but histopathological examination revealed chronic non-specific infection. Therefore, the final diagnosis was made as idiopathic macrocheilia through exclusion criteria. Management with intralesional triamcinolone acetonide 40 mg, twice a week for 3 weeks, resulted in significant remission in lip swelling without recurrence after a 6-month follow-up.

https://ift.tt/2QbT3ug

Massive gastric dilatation leading to acute respiratory distress 1 year after a Nissen fundoplication

Acute massive gastric dilatation (AMGD) is a recognised complication after Nissen fundoplication.¹ A 63-year-old man recently presented to our emergency department in acute respiratory distress, acute abdominal pain and distension, having had an elective umbilical port incisional hernia repair a day prior. In the year preceding his presentation, the patient had undergone a laparoscopic paraoesophageal hiatus hernia repair and excision of sac, posterior cruropexy, dual mesh reinforcement of repair and 360° fundoplication, as a day case. In between these two events, the patient was asymptomatic, and had a free diet with no further medical or surgical intervention. We hereby present successful management and discuss implications of this exceptional yet potentially life-threatening complication.

https://ift.tt/2zA92gp

Diabetic ketoacidosis and thyroid storm: coexistence of a double trouble

Diabetic ketoacidosis (DKA) and thyrotoxic crisis are both endocrine emergencies. Here, we present a case of a patient who presented with DKA and on further evaluation was found to also have a thyrotoxic crisis. The coexistence of both of these endocrine emergencies simultaneously is extremely rare and heralds grave prognosis unless identified and treated promptly.

https://ift.tt/2QaNWdG

Bilateral submandibular gland nodular oncocytic hyperplasia with papillary cystadenoma-like areas

A 57-year-old man presented in 2016 with a 4-month history of a right submandibular mass, having undergone left submandibular gland (SMG) excision in 2003. Imaging suggested a benign tumour and subsequent core biopsy findings suggested a nodular oncocytic hyperplasia, similar to the tumour removed from the contralateral side. This was confirmed on histological analysis following right submandibular gland excision which showed characteristic features of nodular oncocytic hyperplasia along with an unusual diffuse papillary cystadenoma-like ductal proliferation, similar to that seen in the 2003 specimen. A diagnosis of multinodular adenomatous oncocytic hyperplasia (MAOH) was rendered in order to communicate the unique histological features that have otherwise not been described in the literature. We believe that this is the first reported case of non-synchronous multinodular oncocytic hyperplasia and the first case affecting the submandibular glands.

https://ift.tt/2zzPVTw

Gossypiboma masquerading as nephrocutaneous fistula

Nephrocutaneous fistula is a rare complication of surgical procedures involving the kidney. Fistula formation is also a complication seen in gossypiboma. We present the case of a patient who was initially suspected to have nephrocutaneous fistula after open pyelolithotomy. Later while undergoing open resection of fistula, he was found to have a retained surgical sponge (gossypiboma) near the lower pole of kidney. Gossypiboma is a term used for mass formed around a surgical sponge accidentally left in the body. It is most of the times not diagnosed by radiological imaging and thus results in unnecessary investigations to rule out other causes for the patient's symptoms. Having a high index of suspicion may lead to an earlier diagnosis. But the prevention of gossypiboma is of the utmost importance. A meticulous approach while operating by the surgeon and operating rooms staff is very important in doing so.

https://ift.tt/2Qde4Vl

Chronic urinary retention due to diabetic cystopathy masquedering as mesenteric cyst

Description 

A 55-year-old previously diabetic man presented with progressive abdominal distension for the last three months. He denied any bowel/bladder complaints. His medical/surgical history was unremarkable. On examination his vitals were stable and a huge abdominal mass (10x11 cm) was palpable. The mass was having side-to-side mobility. There was no hepatosplenomegaly or clinically significant lymphadenoapathy. On evaluation with ultrasonography of abdomen and CT scan, there was evidence of huge, cystic and homogenous mass (13x11 cm) with clear margins occupying almost entire abdominal cavity (figure 1). There was no evidence of calcification, septations or nodules in the mass, and it appeared to be separate from both the kidneys and bowel. A provisional diagnosis of mesenteric cyst was made, and the patient was taken for laparotomy. A per-urethral catheter was placed intraoperatively and surgery was started. During laparotomy it came as an element of surprise that the swelling was actually chronically distended bladder...

https://ift.tt/2zA9taa

Fetishism in ADHD: an impulsive behaviour or a paraphilic disorder?

A boy with attention deficit and hyperactivity disorder (ADHD) presented with a fetish for and the subsequent stealing of female undergarments. He was predominantly inattentive and had been a slow learner. Psychological tests showed that he had significant cognitive and inattention problems without significant hyperactivity, and was at risk of dyslexia as well as conduct disorder.

https://ift.tt/2QddTJF

Pneumonia and bacteraemia caused by Gemella morbillorum in a previously healthy infant: first reported case in literature

A 5-month-old baby presented with a low-grade fever and tachypnoea and was found to have right upper lobe consolidation on chest radiograph. He was admitted with the diagnosis of bronchopneumonia and the treatment protocol for pneumonia was initiated. Blood culture samples were collected, and he was started on a course of intravenous amoxicillin–clavulanate. Blood culture results displayed pansensitive Gemella morbillorum bacteraemia and he was continued on intravenous antibiotic to which he responded in a short period and was discharged in good condition on the fourth day.

https://ift.tt/2zzCnXW

Gastric emphysema secondary to severe vomiting: a comparative review of 14 cases

Gastric emphysema is characterised by the presence of air within the wall of the stomach. The radiographic finding of gastric emphysema with hepatic portal venous gas is classically an ominous sign, associated with a high mortality rate. We report one case from our clinical experience and undertake a review of the previously reported cases of vomiting-induced gastric emphysema retrieved from the PubMed. A total of 14 cases were found to date. The mean age at the time of diagnosis was 45.6 years (range, 9 months to 81 years). Computed tomography abdomen was the frequently used diagnostic modality. Interestingly, conservative treatment led to a clinical cure and resolution of gastric emphysema as well as the associated hepatic portal venous gas in most of the patients. This review illustrates that vomiting-related gastric emphysema entails a more benign course and surgical intervention can be avoided with a prompt aetiology establishment in these patients.

https://ift.tt/2Q9X2Yj

Aneurysmal dilation of the vein of Galen and straight sinus with resultant obstructive hydrocephalus

Description 

A 20-year-old woman was admitted to the emergency department on a previous occasion with tonic-clonic seizures. Her medical history was significant for headaches and hypertension. The headaches were associated with nausea and vomiting. She had no history of developmental delay or epilepsy. CT scan of the brain showed signs of obstructive hydrocephalus due to venous hypertension as a result of vein of Galen malformation, and hence a ventriculoperitoneal shunt was placed. Now on follow-up, her MRI brain demonstrates a round flow void in the suprapineal cisterns with engorged straight sinus, compatible with vein of Galen arteriovenous fistulous communication and shunting (figure 1). The resultant venous hypertension leads to mild to moderate compensated hydrocephalus.

Figure 1

(A) Axial T2, (B) axial T1 and (C) sagittal T2 MRI brain. MRI brain demonstrates a round flow void in the suprapineal cisterns with engorged straight sinus (green arrow),...

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Lateral Proboscis (Elephant Tusk) with Orofacial Clefts: A Report of a Rare Case

Lateral proboscis is a rare congenital anomaly. Lateral proboscis is a rare craniofacial malformation characterized by a rudimentary tubular, nose-like structure occurring in association with a wide spectrum of other anomalies. We presented a seven-month-old girl's lateral proboscis, cleft lip, and palate. Proboscis was excised by an elliptical incision, and the cleft was repaired at the same surgery.

https://ift.tt/2xS4nUH

Silicone breast implant associated fibromatosis

Abstract

Extra-abdominal desmoid tumors, also known as aggressive or deep fibromatosis, are uncommon soft tissue tumors that rarely involve the breast. Although the exact etiology is unknown, the development of these tumors has been correlated with sites of previous trauma, surgery or in association with familial adenomatous polyposis. Clinically, breast fibromatosis is often mistaken for carcinoma but lacks metastatic potential. It is locally aggressive with high rates of recurrence. The treatment is primarily wide local excision with negative margins. Adjuvant treatments have been suggested and include radiotherapy, chemotherapy and hormonal therapy, however, there are no evidence-based treatment protocols to support their use. Here, we describe a case of fibromatosis that developed within the capsule around a silicone breast implant treated with surgical excision alone. The patient remains recurrence free at 3 months post-operative magnetic resonance imaging.

https://ift.tt/2OY3qBS

Clinical improvement in a patient with monostotic melorheostosis after treatment with denosumab: a case report

A 20-year-old Danish woman with melorheostosis in her right femoral shaft and disabling pain in the affected area, whose symptoms did not in the long term respond to zoledronic acid, experienced continuous rem...

https://ift.tt/2IkrpIV

Spontaneous asymptomatic rupture of pseudocyst into stomach

Description 

A 27-year-old woman presented with complaints of pain in the epigastric region radiating to back for 20 days. She was treated initially at local hospital and was diagnosed as acute pancreatitis as her amylase and lipase levels were >1000 U/mL. She was managed with analgesics and intravenous fluids and was discharged in 4 days. She then presented to our emergency, with complaints of epigastric pain, awareness of lump in the epigastric region and non-passage of stool or flatus for past 3 days. She also gave history of fever and multiple episodes of bilious vomiting. Patient denied previous history of similar episodes in past and is non-alcoholic. On abdominal examination, a lump of size 20x15 cm was palpable in the epigastric region extending into the umbilical region. On ultrasonogram abdomen, a cystic swelling was seen along with multiple gall stones in the gall bladder. Contrast-enhanced CT (CECT) abdomen was suggestive of a large cystic...

https://ift.tt/2IntjIR

Primary retroperitoneal filariasis: a common disease of tropics with uncommon presentation and review of literature

Lymphatic filariasis is caused by nematode filariae Wuchereria bancrofti, Brugia malayi or Brugia timori. It is commonly seen in tropical and subtropical regions of the world and affects the lymphatic system of humans, who are the definitive host while mosquito is the intermediate host. The most common manifestation of the disease is hydrocele followed by lower limb lymphoedema and elephantiasis. Although filariasis is much more common entity in north India, its presentation as retroperitoneal cyst is very rare with reported incidence rate of 1/105 000. We present a case of primary retroperitoneal filariasis in a 52-year-old man, without any classic signsandsymptoms, diagnosed postoperatively after surgical resection following diagnostic uncertaintyandfailure of other medical therapies.

https://ift.tt/2OfbCjV

Gossypiboma: a ghastly find

A gossypiboma is a mass within a patient's body comprising a cotton matrix surrounded by a foreign body granuloma. We describe an unusual presentation of a gossypiboma presenting in a 32-year-old man with acute epigastric pain and haematemesis. His surgical history revealed an emergency laparotomy following a road traffic accident 16 years ago. Initial gastroscopy showed extrinsic stomach compression. An abdominal ultrasound scan followed by a CT scan evidenced a large, well-defined, predominantly cystic mass with some solid areas occupying the left hypochondrium. Conservative management with insertion of a percutaneous drain proved to be inefficient. A laparotomy was performed; intraoperatively, the cyst was found to be ruptured and within it, a large surgical gauze was found. This was removed but required a distal pancreatectomy and gastrectomy for complete excision. He was discharged on day 74 of admission with outpatient follow-up.

https://ift.tt/2Ih30Us

Primary renal diffuse large B-Cell lymphoma causing haemodialysis-dependent nephromegaly in a child

A 4-year-old boy presented with fatigue and was found to have severe kidney injury requiring haemodialysis. A renal ultrasound demonstrated bilateral nephromegaly with mild loss of corticomedullary differentiation but preserved echogenicity. He had a persistent isolated monocytosis. Renal biopsy revealed extensive infiltration by primary renal diffuse large B-cell lymphoma. He required haemodialysis for 18 days and received chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone, rituximab and intrathecal methotrexate. He achieved remission with an estimated glomerular filtration rate of 50 mL/min/1.73 m², and his kidneys returned to normal size. Nephromegaly due to renal-limited haematolymphoid disease is extremely rare, especially in children.

https://ift.tt/2ObiE9s

Congenital Granular Cell Tumor: Case Report and Review

Congenital granular cell tumors are infrequently occurring masses occurring on a neonate's gingiva/alveolus. These lesions are benign with no noted malignant transformation, and treatment of excision is based on its effect on the neonate's respiratory ability and/or nutritional intake. The purpose of this review is to discuss a case of a congenital granular cell tumor and its treatment and review of the literature including demographics, histopathology, and operative treatment.

https://ift.tt/2xUaZ5g

Use of a qualitative case study to learn lessons from severe preeclampsia causing a maternal near-miss: a case report

Maternal mortality is a critical indicator in assessing the quality of services provided by a health care system. Approximately 99% of all maternal deaths occur in developing countries; where a majority of the...

https://ift.tt/2xCK2DQ

Post-traumatic bilateral vesicocutaneous fistula of thighs treated with buccal mucosa graft urethroplasty and cyanoacrylate glue instillation: a novel treatment for management of a complicated fistula

Post-traumatic vesicocutaneous fistula (VCF) is a rare variant of urinary fistulas. These fistulas may externally communicate to abdomen, perineum, buttocks, scrotum or very rarely thigh. These fistulas usually develop at a variable time duration after trauma and are usually preceded with thigh swelling or abscess formation followed by spontaneous rupture. We, hereby, report a case of VCF of bilateral thighs with associated penobulbar urethral stricture after road traffic accident which was managed with dual modality of buccal mucosa graft urethroplasty surgery and cyanoacrylate glue injection in the fistulous tracts. To the best of our knowledge, this is first report of bilateral VCFs communicating externally to thighs. In literature, very few cases of VCFs of thigh are reported and are rarely managed with adhesive glue application.

https://ift.tt/2xFonL8

Cushings reflex secondary to neck haematoma following thyroidectomy

Neck haematoma following thyroid surgery can present with respiratory distress which is generally attributed to airway obstruction. We recently had a 63-year-old female patient who underwent total thyroidectomy for toxic nodular goitre. However, within 4 hours of surgery, she developed sudden respiratory distress which was managed by prompt evacuation of the neck haematoma. Just before the haematoma evacuation, the patient had hypertension and bradycardia along with the distress. The arterial blood gas analysis sampled at that time was normal. Intraoperatively, the tracheal framework was found rigid and non-pliable. Considering the various clinical–biochemical findings observed, we think that the cause of the respiratory distress in the index case was transiently elevated intracranial pressure, secondary to bilateral internal jugular veins' compression. We hypothesise that in many patients with immediate postoperative neck haematoma, the Cushing's reflex would at least contribute partly, if not solely to the respiratory distress.

https://ift.tt/2DwRF3T

Three differently timed presentations of dermatomyositis associated with advanced ovarian cancer

Each of the three patients reported in this article presented with dermatomyositis at various stages of their advanced ovarian cancer. Dermatomyositis was the presenting feature and preceded the diagnosis of ovarian cancer by several months in one patient. In another patient, dermatomyositis occurred just prior to the scheduled third cycle of palliative chemotherapy after surgical debulking for stage 4 disease. The third patient presented with pathognomonic diagnostic features of dermatomyositis after ovarian cancer recurrence. Diagnosis was delayed in at least two of these patients; however, once appropriately diagnosed, each patient responded well to immunomodulatory treatment. In one patient, initiation of oral prednisolone seemed to correlate with a steady improvement in her proximal myopathy. A pulsed methylprednisolone approach was used in another patient with conversion to a tapering dose of oral prednisolone to good effect. In the patient in whom the most severe myopathy affecting bulbar muscle groups was demonstrated, an infusion of 5 days of intravenous immunoglobulin produced an eventual improvement in her steroid-refractory myopathy.

https://ift.tt/2xBDS6M

Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent. Our case highlights the importance of multispecialty collaboration in the diagnosis and management of SLE with multisystem involvement.

https://ift.tt/2Q8i6OR

Bronchial Blocker Use in the Difficult Airway Patient Requiring Lung Isolation: Clarification as to What Blockers Are Actually Available

No abstract available

https://ift.tt/2DuByE4

Intraoperative Considerations for Transgender Patients

No abstract available

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In Response

No abstract available

https://ift.tt/2Dt9VeD

In Response

No abstract available

https://ift.tt/2QYBE9r

Programmed Intermittent Bolus Regimen for Erector Spinae Plane Blocks in Children: A Retrospective Review of a Single-Institution Experience

With few published reports on erector spinae plane block use in children, limited guidance on perioperative local anesthetic dosing exists. We present a series of 22 patients who received erector spinae plane catheters with programmed intermittent bolus for various surgeries. Median loading dose of 0.4 mL/kg (interquartile range [IQR], 0.1 mL/kg) ropivacaine 0.5%, intraoperative bolus of 0.3 mL/kg/h (IQR, 0.1 mL/kg) ropivacaine 0.2%, and a postoperative programmed intermittent bolus regimen of maximum 0.6 mg/kg/h resulted in highest pain scores on postoperative day 1 with a median score of 1.7 of 10 (IQR, 1.8) and highest morphine equivalents consumed on postoperative day 2 with a median score of 0.16 mg/kg up to 120 hours after surgery. Accepted for publication August 22, 2018. Funding: None. Conflicts of Interest: See Disclosures at the end of the article. Reprints will not be available from the authors. Address correspondence to Ban C. H. Tsui, MD, FRCPC, Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University School of Medicine, Lucile Packard Children's Hospital Stanford, 300 Pasteur Dr, 3rd Floor, Room H3584, MC 5640, Stanford, CA 94305. Address e-mail to bantsui@stanford.edu. © 2018 International Anesthesia Research Society

https://ift.tt/2Dw32Ji

Acute Stroke Management in the First 24 Hours: A Practical Guide for Clinicians

No abstract available

https://ift.tt/2QYBvmp

Obstetric Anesthesiology in the United States: Current and Future Demand for Fellowship-Trained Subspecialists

No abstract available

https://ift.tt/2Dwkcqq

Lack of Bias Evaluation and Inadequate Study Selection May Produce Misleading Results

No abstract available

https://ift.tt/2QYBgrv

Should We Always Continue β-Blocking Agents Preoperatively?

No abstract available

https://ift.tt/2DGm1RN

Is Tube Thermosoftening Helpful for Videolaryngoscope-Guided Nasotracheal Intubation?: A Randomized Controlled Trial

BACKGROUND: Thermosoftening of the endotracheal tube (ETT) and telescoping the ETT into a rubber catheter have been suggested as a method for reducing epistaxis during nasotracheal intubation (NTI). However, thermosoftening technique is known to make it difficult to navigate the ETT into trachea without the use of Magill forceps during NTI. The cuff inflation technique has been suggested as an effective alternative to the use of Magill forceps to improve the oropharyngeal navigation of the ETT, irrespective of their stiffness, during direct laryngoscope-guided NTI. We evaluated whether thermosoftening of the ETT telescoped into rubber catheters has an additional benefit in reducing nasal injury. Simultaneously, we also evaluated whether thermosoftening of the ETT worsened orotracheal navigability during cuff inflation-supplemented videolaryngoscope-guided NTI. METHODS: One hundred forty patients were randomly assigned to 1 of the 2 groups depending on whether the ETT was softened by warming or not. The primary outcome was the incidence of epistaxis during NTI. The secondary outcome was nasotracheal navigability of the ETT, assessed by navigation grade and time required for insertion of ETT in each phase (from nose to oropharynx, from oropharynx to glottic inlet aided by cuff inflation if needed, and from glottic inlet to trachea). RESULTS: The ETTs were successfully inserted through the selected nostril of all 140 patients. In the thermosoftening group, the incidence and severity of epistaxis was significantly lower (7% vs 51%; difference of 44.2%; 95% confidence interval, 29.9%–56.2%; P .99 and P = .054, respectively) and from the glottic inlet to the trachea (P > .99 and P = .750, respectively) between the 2 groups. In both groups, all ETTs could be navigated into the trachea without the use of Magill forceps. CONCLUSIONS: Supplemented with cuff inflation during videolaryngoscope-guided NTI, thermosoftening of the ETT telescoped into rubber catheters has a substantial benefit because it significantly reduces the incidence of epistaxis without worsening the oropharyngeal navigability of the ETT. Accepted for publication August 17, 2018. Funding: None. The authors declare no conflicts of interest. This study was approved by the Institutional Ethics Committee (institutional review board [IRB] approval number 2017-03-020, IRB contact information: Institutional Review Board, Hallym University Kangnam Sacred Heart Hospital, B1, 12, Siheung-daero 187-gil, Yeongdeungpo-gu, Seoul 07441, Republic of Korea. E-mail: dandelionc@hallym.or.kr. Reprints will not be available from the authors. Address correspondence to Joo Hyun Jun, MD, PhD, Department of Anesthesiology and Pain Medicine, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, 1, Singil-ro, Yeongdeungpo-gu, Seoul 07441, Republic of Korea. Address e-mail to ilpleut@naver.com. © 2018 International Anesthesia Research Society

https://ift.tt/2QRHvxv

Multimodal General Anesthesia: Theory and Practice

Balanced general anesthesia, the most common management strategy used in anesthesia care, entails the administration of different drugs together to create the anesthetic state. Anesthesiologists developed this approach to avoid sole reliance on ether for general anesthesia maintenance. Balanced general anesthesia uses less of each drug than if the drug were administered alone, thereby increasing the likelihood of its desired effects and reducing the likelihood of its side effects. To manage nociception intraoperatively and pain postoperatively, the current practice of balanced general anesthesia relies almost exclusively on opioids. While opioids are the most effective antinociceptive agents, they have undesirable side effects. Moreover, overreliance on opioids has contributed to the opioid epidemic in the United States. Spurred by concern of opioid overuse, balanced general anesthesia strategies are now using more agents to create the anesthetic state. Under these approaches, called "multimodal general anesthesia," the additional drugs may include agents with specific central nervous system targets such as dexmedetomidine and ones with less specific targets, such as magnesium. It is postulated that use of more agents at smaller doses further maximizes desired effects while minimizing side effects. Although this approach appears to maximize the benefit-to-side effect ratio, no rational strategy has been provided for choosing the drug combinations. Nociception induced by surgery is the primary reason for placing a patient in a state of general anesthesia. Hence, any rational strategy should focus on nociception control intraoperatively and pain control postoperatively. In this Special Article, we review the anatomy and physiology of the nociceptive and arousal circuits, and the mechanisms through which commonly used anesthetics and anesthetic adjuncts act in these systems. We propose a rational strategy for multimodal general anesthesia predicated on choosing a combination of agents that act at different targets in the nociceptive system to control nociception intraoperatively and pain postoperatively. Because these agents also decrease arousal, the doses of hypnotics and/or inhaled ethers needed to control unconsciousness are reduced. Effective use of this strategy requires simultaneous monitoring of antinociception and level of unconsciousness. We illustrate the application of this strategy by summarizing anesthetic management for 4 representative surgeries. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Accepted for publication June 11, 2018. Funding: This work was supported by the National Institutes of Health (Bethesda, MD): R01 GM104948 (to E.N.B.) and P01GM118269 (to E.N.B.); and by the Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Boston, MA. Conflicts of Interest: See Disclosures at the end of the article. A glossary of terms is available in the Appendix. Reprints will not be available from the authors. Address correspondence to Emery N. Brown, MD, PhD, Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, 55 Fruit St, Grey-Jackson 444, Boston, MA 02114. Address e-mail to enb@neurostat.mit.edu. © 2018 International Anesthesia Research Society

https://ift.tt/2DvSLgp

Performance of Air Seal of Flexible Reinforced Laryngeal Mask Airway in Thyroid Surgery Compared With Endotracheal Tube: A Randomized Controlled Trial

BACKGROUND: Flexible reinforced laryngeal mask airway (FLMA®) has gained popularity in thyroid surgery, but air leak and displacement are still concerns. METHODS: In this randomized, single-blinded, noninferiority, controlled trial, we randomized patients scheduled for elective radical thyroidectomy to an endotracheal tube (ETT) group or a FLMA group. The primary outcomes were ventilation leak volume, peak airway pressure, and partial pressure of end-tidal carbon dioxide (PetCO2). Data for primary outcomes were collected after insertion of ETT/FLMA, at incision, and at 10-minute intervals during surgery. Ten milliliters, 5 cm H2O, and 10 mm Hg were used as the noninferiority deltas for ventilation leak volume, peak airway pressure, and PetCO2, respectively. We assessed noninferiority of FLMA to ETT on the primary outcomes over time using the results of a linear mixed-effects model. The position of FLMA mask was evaluated before and after surgery, and the airway complications were recorded. RESULTS: A total of 132 patients were included: 65 in ETT group and 67 in FLMA group. Differences (FLMA group minus ETT group) of ventilation leak volume, peak airway pressure, and PetCO2 from the mixed-effects models were 2.09 mL (98.3% confidence interval [CI], –6.46 to 10.64), −0.60 cm H2O (98.3% CI, –2.15 to 0.96), and 1.02 mm Hg (98.3% CI, 0.04–1.99), respectively. Score of fiber-optic position of FLMA was significantly higher after surgery than before. There was no severe shift, loss of the mask seal, regurgitation, or aspiration in the FLMA group. One patient in the FLMA group experienced brief and easily controlled laryngospasm. CONCLUSIONS: In thyroid surgery, FLMA is noninferior to ETT in the peak airway pressure and PetCO2 although mild to moderate mask shift could occur during surgical manipulation. There is no evidence for a higher complication rate when FLMA is used. Accepted for publication July 27, 2018. Funding: None. The authors declare no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's website (https://ift.tt/KegmMq). Clinical Trial Number: ChiCTR-IOR-15006602. LMA Flexible and LMA Classic are registered trademarks of Teleflex Incorporated or its affiliates. Reprints will not be available from the authors. Address correspondence to Jie Yi, MD, Department of Anesthesiology, Peking Union Medical College Hospital, Beijing 100730, China. Address e-mail to easyue@163.com. © 2018 International Anesthesia Research Society

https://ift.tt/2R0ndlB

Novel in-frame deletion in MFSD8 gene revealed by trio whole exome sequencing in an Iranian affected with neuronal ceroid lipofuscinosis type 7: a case report

The neuronal ceroid lipofuscinoses are a group of neurodegenerative, lysosomal storage disorders. They are inherited as an autosomal recessive pattern with the exception of adult neuronal ceroid lipofuscinosis...

https://ift.tt/2xBuQ9Q

The effect of the volume of supra-inguinal injected solution on the spread of the injectate under the fascia iliaca: a preliminary study

Abstract

The fascia iliaca compartment is the compartment confined by the fascia iliaca (FI) and a muscular layer formed by the iliac- and psoas muscle. This compartment creates a virtual tunnel that contains the femoral nerve (FN), the obturator nerve (ON), and the lateral femoral cutaneous nerve (LFCN) of the lumbar plexus. In this pilot study, we aimed to determine the suggested volume needed to reach the three target nerves of the lumbar plexus (FN, ON, and LFCN) with a single-injection ultrasound-guided supra-inguinal fascia iliaca compartment (S-FICB). A computer tomography (CT scan)-guided step-up/step-down sequence was used to determine the suggested injection volume to target all three nerves. Subsequently, an anatomist blinded for the injected volume and CT findings, dissected the cadavers, and evaluated the spread of dye underneath the fascia iliaca. In total, seven pelvic areas of four cadavers were evaluated on CT scan and dissected. Distribution of dye underneath the FI in relation to the FN, ON, and the LFCN was recorded in all dissected cadavers. Combining CT and dissection findings, the suggested volume to reach the FN, ON, and LFCN with an S-FICB was 40 mL.

https://ift.tt/2NwGdtL

Anesthesiologists should bring in their expertise during the early postoperative period to improve surgical outcome

No abstract available

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Ambulatory anesthesia: what's new in 2018

No abstract available

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Goblet cell carcinoid of the appendix – diagnostic challenges and treatment updates: a case report and review of the literature

Goblet cell carcinoid is a rare but distinct entity of appendiceal tumors which is a hybrid or mixed tumor consisting of both epithelial (glandular) and neuroendocrine elements containing goblet cells. This en...

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Varicella causing remission of Cushings disease

An 18-year-old male with Cushing's disease presented with generalised skin eruptions and backache. He was diagnosed with varicella infection. During the course of the illness, he developed persistent vomiting. Hormonal evaluation suggested adrenal insufficiency. MRI of brain showed features of pituitary apoplexy. Initially, he required hydrocortisone replacement. Later on his hypothalamic–pituitary–adrenal axis recovered and he is currently asymptomatic without any treatment.

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Extreme scoliosis from CKD-MBD resulting in respiratory failure and death

Description 

A 64-year-old man presented to our hospital because of dyspnoea. Twenty years ago, he had started peritoneal dialysis (PD) for chronic kidney disease. He had been diagnosed with secondary hyperparathyroidism 7 years before his presentation to our hospital, with an intact parathyroid hormone (PTH) 1227 pg/mL (normal range 10–60 pg/mL), calcium level of 10.1 mg/dL (normal range 8.8–10.5 mg/dL), phosphorus level of 5.5 mg/dL (normal range 2.4–4.1 mg/dL) and alkaline phosphatase level of 377 IU/L (normal range 140–338 IU/L). His medications included cinacalcet. Ultrasonography showed a 5-milimetre nodule at the lower pole of the parathyroid, but  99mTc-hexakis2- methoxyisobutylisonitrile (MIBI)scintigraphy revealed no abnormal accumulation.

The patient's intact PTH increased to 2606 pg/mL 6 years prior to presentation. At that time, the patient deferred parathyroidectomy. A year later, he switched from PD to haemodialysis. Intact PTH remained elevated to more than 4000 pg/mL. His thorax became scoliotic curving to the left. He had experienced dyspnoea on...

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Barriers to healthcare for female patients in Papua New Guinea

A 25-year-old woman presented to hospital in the remote highlands of Papua New Guinea (PNG) with a 3-year history of increasing abdominal distension, amenorrhoea and syncope. Ultrasound showed a large unilocular ovarian cyst. During her work-up, she was found to be HIV positive. She was treated with antiretroviral therapy, and once her CD4 count improved, she underwent a laparotomy and removal of the ovarian cyst with immediate improvement in symptoms. PNG has high levels of HIV particularly in young women and children.¹ This is partly due to a lack of screening and treatment facilities and partly due to significant gender discrimination. PNG is considered one of the most dangerous places in the world for females; women are treated as second-class citizens with few human rights or access to services such as healthcare.² Rape, sexual assault and domestic violence are common, and their lives are dictated to them by their husbands or male relatives.^{2 3} The lack of healthcare resources and significant levels of gender discrimination meant that this patient had a delayed presentation resulting in potentially grave complications.

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Uncommon cause of pelvic inflammatory disease leading to toxic shock syndrome

A 44-year-old Caucasian female with a history of endometriosis is admitted to the intensive care unit due to severe left lower quadrant abdominal pain, nausea and vomiting. With patients' positive chandelier sign on pelvic examination, leucocytosis, elevated erythrocyte sedimentation rate and elevated C-reactive protein indicated that she had pelvic inflammatory disease (PID). PCR tests were negative for Neisseria gonorrhoeae and Chlamydia trachomatis; however, her blood and urine cultures grew Group A streptococci (GAS) with a negative rapid Streptococcus throat swab and no known exposure to Streptococcus. On further review, patient met criteria for GAS toxic shock syndrome based on diagnostic guidelines. The patient was promptly treated with intravenous antibiotics and supportive care, and she acutely recovered. This case demonstrates a rare cause of PID and an atypical aetiology of severe sepsis. It illuminates the importance of considering PID as a source of infection for undifferentiated bacteraemia.

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On a knife-edge: clinical uncertainty with an extensive knife blade in situ in the craniofacial region

A 25-year-old man presented to the trauma department following a penetrating stab wound to his left infraorbital margin with retained knife blade causing superoposterior displacement of the globe. Plain skull X-ray revealed an extensive retained blade with subsequent CT imaging revealing the tip of the blade had reached the right styloid process with no neurovascular compromise. Initial concern was primarily for the left eye leading to ophthalmology being the first specialty requested to review the patient. However, once the extent of the injury was established, ophthalmology requested further review from maxillofacial, ENT and neurosurgery. This resulted in an 84 hours wait between the initial injury and the removal of the knife blade. Incredibly, the patient had no initial sequelae from such an extensive injury and had an unremarkable recovery with no further complications aside from a laceration to the left inferior rectus muscle that was conservatively managed.

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Mast cell deposition and activation may be a new explanation for epiploic appendagitis

Epiploic appendagitis is as an acute painful condition of the fat on the outside of the intestine. Thus far, there have been no publications to our knowledge that appendagitis can be caused by mast cells or can be associated with chronic pain. A patient with multisystemic disorders suffered with both chronic and acute attacks of abdominal pain for a year. The worst attack led to surgical resection of an enlarged sigmoid colon epiploic appendage. Careful review of her complex medical history and mast cell stains of gastrointestinal biopsies led to the diagnosis of mast cell activation syndrome. Re-examination of the resected appendage using an immunohistochemical stain demonstrated a high mast cell density which is a new histopathological finding. Treatment of mast cell activation syndrome and other related syndromes led to marked improvement in her health, including all types of chronic abdominal pain.

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Spontaneous resolution of a 'ping-pong fracture at birth

'Ping-pong' fractures are depressed skull fractures in newborn infants that occur as inward buckling of the calvarial bones, forming a cup shape. These fractures are often associated with maintenance of bone continuity. These fractures may occur spontaneously during the intrauterine period or secondary to birth trauma. Currently, there is no standard protocol for the management of depressed skull fractures. Neurosurgical or non-surgical approaches may be administered depends on the severity of the fracture. Most untreated ping-pong fractures resolve spontaneously within 6 months. Therefore, it is recommended to reserve surgical elevation or non-surgical techniques for infants not demonstrating spontaneous resolution during this period. In addition, neurosurgical interventions are usually considered for cases with intracranial pathology or neurological deficits or for infants who do not respond to conservative treatment. Herein, we report a case of a newborn infant with a spontaneous intrauterine ping-pong fracture, which spontaneously resolved, without surgical or non-surgical intervention.

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External auditory canal lesion: colorectal metastatic adenocarcinoma

The patient presented to the ear, nose and throat clinic with failed conservative treatment of persistent right otitis externa. On examination, the roof of the right ear canal was polypoid and the tympanic membrane could not be visualised. There was a fragile mass noted in the external auditory canal (EAC) which on microsuction started to bleed. CT internal auditory meatus and MRI internal auditory meatus identified soft tissue mass in the EAC. The patient underwent urgent examination under anaesthetic of the ear and biopsies were taken. He had a background of ascending colon cancer; Duke's C1, pT4, N1 M0, R0 resection and had undergone laparoscopic right hemicolectomy with adjuvant chemotherapy, in the previous year. The biopsy results proved that the mass in the EAC was due to metastatic deposit of colorectal primary tumour. The patient also had a full body CT which revealed other new metastases. The patient is being treated with palliative chemotherapy.

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Delusional infestation in the treatment of ADHD with atomoxetine

The case describes an adult diagnosed with attention deficit hyperactivity disorder and treated with atomoxetine who quickly developed a florid case of delusional infestation. The patient described very distressing experiences that were significantly impacting her daily life. The symptoms improved with the withdrawal of atomoxetine and resolved completely with antipsychotic medications. Atomoxetine is proposed as the putative causative agent in this case.

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Novel mutation in the CHST14 gene causing musculocontractural type of Ehlers-Danlos syndrome

Musculocontractural type of Ehlers-Danlos syndrome (MC-EDS) is a recently recognised connective tissue disorder. MC-EDS is caused by homozygous or compound heterozygous mutation in the carbohydrate sulfotransferase 14 (CHST14) gene on chromosome 15q15. Herein, we report a case of a 3-year-old boy with MC-EDS in whom a novel mutation in the CHST14 gene was discovered. Besides being the second report of this rare disorder from India, the child till 3 years has not had any bleeding tendency as described in the earlier reports of this disorder.

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Expand the differential...think beyond rheumatoid arthritis

A 31-year-old male patient with severe, migratory arthralgias presented to our academic medical centre after being erroneously diagnosed and treated for rheumatoid arthritis for over 1 year. Multiple immunomodulatory therapies for rheumatoid arthritis were attempted with no relief of symptoms. Eventually, the pain was so bothersome that the patient became bedridden for 1 month prior to presenting to our facility. Our assessment revealed that the patient met the diagnostic criteria, known as the Yamaguchi criteria, needed to diagnose adult-onset Still's disease. Yamaguchi criteria include migratory inflammatory arthritis, quotidian fevers, leucocytosis and a salmon-coloured maculopapular rash. These signs and symptoms may go unnoticed or overlooked if adult-onset Still's disease is not considered. The patient was treated with anakinra (a recombinant human IL-1 receptor antagonist) and had rapid improvement in his symptoms, with the restoration of mobility.

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Posterior urethral valve associated with a foreign body in posterior urethra: a first of its kind report

Posterior urethral valve and foreign body are among the important causes of male urethral obstruction. Although one is congenital and the other is acquired, both entities are rare in children with only a few reported cases. Because of myriad of symptoms associated with both conditions, a conclusive diagnosis requires both physical examination and radiological imaging. We report a first of its kind association of posterior urethral valve with foreign body in the posterior urethra in a 6-year-old male child which was eventually managed by endoscopic intervention.

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Management of C-shaped root canal configuration in mandibular second molar

Description 

The C-shaped root canal configuration is an anatomical variation of root fusion and a type of taurodontism commonly seen in the mandibular second molar with prevalence ranging from 2.7% to 45.5% in different populations.1 In this configuration, the root canals are connected by slit or web with varying anatomy along the root length which makes thorough debridement obstacle for the clinician.2 Cone-beam CT (CBCT) is a three-dimensional imaging technique that facilitates and improves understanding the complex morphology. This article presents a case of C-shaped root canal configuration of mandibular second molar managed by using CBCT and modification in obturation techniques.

A 40-year-old female patient reported with pain in the lower left back teeth region. Medical history of the patient was unremarkable. Intraoral examination revealed deep class I carious lesion on tooth 37 with tenderness on percussion. Thermal test was performed to check the vitality...

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Over-the-counter drug-induced lung injuries with both diffuse alveolar haemorrhage and diffuse alveolar damage

Description 

A 41-year-old Japanese man with no underlying disease was admitted to our hospital with haemoptysis and dyspnoea. Two weeks prior to admission, he had caught a cold and took an over-the-counter drug (topikku GX), subsequently general fatigue and cough were worsening progressively. Hence, he presented to another hospital, owing to acute onset of dyspnoea on effort and haemoptysis, where chest X-ray was taken and revealed bilateral abnormal shadow in the middle lung fields, after that he was referred to our hospital.

On examination, his general appearance was moderately ill; blood pressure was 126/72 mm Hg, heart rate was 90 beats/min, oxygen saturation was 90% under ambient air, respiratory rate was 20 breath/min and body temperature was 38.2°. Chest auscultation revealed bilateral inspiratory mid to late crackles. Laboratory blood test showed mild elevation of liver function tests (Aspartate aminotransferase 42 IU/L, Alanine aminotransferase 34 IU/L), Lactate dehydrogenase 379 IU/L, C-reactive protein 28.2 mg/L, the rest of...

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Skin metastasis: a rare presentation in testicular germ cell tumour

A 35-year-old man presented with a history of cough, haemoptysis, weight loss for 2 months along with ulceroproliferative lesions on the chin and the scalp. On evaluation he was found to have non-seminomatous germ cell tumour, stage 3 c, poor risk with Eastern Cooperative Oncology Group Performance Status of 4. The skin lesions were proven to be metastasis by fine-needle aspiration cytology. He showed significant improvement with a 3-day protocol of abbreviated etoposide and cisplatin chemotherapy and is planned for 4 cycles of VIP. This case describes an uncommon presentation of germ cell tumour in the form of skin metastasis with excellent response to chemotherapy.

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Chronic cutaneous lupus erythematosus and topical clindamycin

Description 

A 64-year-old Caucasian woman was seen in our office for several years for management of her chronic cutaneous lupus erythematosus (CCLE). Her condition consisted of violaceous papules and plaques with some focal atrophy distributed over the upper arms, face and scalp. Histopathology from two 4 mm punch biopsies performed on separate occasions demonstrated features on H&E stain consistent with CCLE: epidermal atrophy and interface dermatitis with superficial perivascular, deep dermal and periadnexal lymphohistiocytic infiltrates containing some plasma cells. Her rash had frustrated her for over 5 years, worsening with increased sun exposure. Her collagen vascular serologies were negative. She had been managed with potent topical corticosteroids with improvement, however, the patient preferred to reserve this for flares of her condition. Interestingly, the patient had correlated dramatic clearing of her CCLE inadvertently after 3 months of applying topical clindamycin gel 1% twice daily (figures 1 and 2),...

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Primary tonsillar tuberculosis

Upper aerodigestive tract involvement with tuberculosis is relatively rare and may be seen in up to 2% of patients with pulmonary tuberculosis. Isolated tonsil involvement with tuberculosis is not commonly seen in clinical practice. We report a case of a 22-year-old postpartum mother who presented with odynophagia, fever, loss of weight and submandibular swelling of 3 months' duration. Clinical examination revealed a submandibular node, and oropharyngeal examination revealed necrotic slough overlying an enlarged left tonsil. Fine-needle aspiration cytology of the node and histopathological examination of the left tonsillectomy specimen revealed necrotising epithelioid cell granulomas, and stain for acid-fast bacilli was positive in the latter. She was diagnosed with tonsillar tuberculosis and was started on antituberculous treatment following which she improved clinically. This case serves to demonstrate an uncommon presentation of primary tuberculosis and reminds us to consider tuberculosis also as a microbiological aetiology for tonsillitis.

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Neuropsychiatric Disorder Associated with Group G Streptococcus Infection

Immune-mediated central nervous system manifestations of group A β-hemolytic Streptococcus (GABHS) infection include Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)—which includes tic and obsessive compulsive disorders—and a variety of neurobehavioral disorders. We report a case of Streptococcus dysgalactiae subspecies equisimilis (group G Streptococcus) (GGS) infection associated with involuntary movements, complex tics, and emotional lability in an 11-year-old Japanese girl. Serum IgM and IgG antibodies to lysoganglioside were positive, and she responded rapidly to intravenous immunoglobulin treatment. Neuropsychiatric disorder associated with GGS infection was ultimately diagnosed. The present findings suggest that neuropsychiatric disorders can result from GGS infection and that the pathogenic mechanism is similar to that of GABHS infection. Future large-scale studies should examine the relation between GGS infection and onset of neuropsychiatric disorder.

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Late onset hyperornithinemia-hyperammonemia-homocitrullinuria syndrome - how web searching by the family solved unexplained unconsciousness: a case report

Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a rare inherited urea cycle disorder, can remain undiagnosed for decades and suddenly turn into an acute life-threatening state. Adult presentation ...

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Sepsis and Pleural Empyema Caused by Streptococcus pyogenes after Influenza A Virus Infection

Streptococcus pyogenes (also referred to as group A streptococci, GAS) causes severe invasive diseases such as bacteremia, necrotizing fasciitis, pneumonia, osteomyelitis, septic arthritis, and toxic shock syndrome in children. However, there are only a few reports on pleural empyema caused by GAS in children. Here, we report the case of a 4-year-old boy who presented with pleural empyema due to GAS after influenza A virus infection. With intravenous antibiotic administration and continuous chest-tube drainage, followed by video-assisted thoracoscopic surgery, his condition improved. During the clinical course, cytokines induced in response to the influenza virus, especially IL-1β and IL-10, were elevated 1 week after influenza A infection, but these decreased as the symptoms improved. Reportedly, the IL-10 production increases during influenza virus-bacteria superinfection. These observations suggest that the immunological mechanisms induced by the influenza virus can play an important role in influencing the susceptibility to secondary bacterial infections, such as GAS, in children.

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Dermoscopy of skin metastases from breast cancer: two case reports

Cutaneous metastatic breast cancer is the most common cutaneous metastatic malignancy in women. The assessment of cutaneous metastatic disease can be perplexing because the clinical presentation appears simila...

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Rare case of Propionibacterium acnes-related splenic abscess

A 64-year-old woman with a medical history of morbid obesity, chronic hepatitis C, essential hypertension, multiple episodes of abdominal cellulitis, diabetes mellitus type 2 on insulin, intravenous and subcutaneous drug abuse presented to the emergency department complaining of left lower chest pain for 6 weeks along with multiple episodes of vomiting. Initial laboratory data revealed leucocytosis of 17 200x10³/μL with left shift. She reported multiple episodes of fever spikes. Abdominal and pelvic CT showed a splenic hypodense lesion. Specimens from interventional radiology aspiration and splenectomy grew Propionibacterium acnes. Following splenectomy, patient's symptoms resolved. To the best of our knowledge, this would represent the fifth reported case of P. acnes splenic abscess.

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Alpha-2 antiplasmin-associated aortic valve thrombus presenting as a STEMI in a patient with Graves disease

Description  

A 36-year-old Caucasian woman with a medical history of Graves disease presented to the emergency department with typical chest pain, and she was found to have a ST-elevation myocardial infarction (STEMI) via ECG and elevated troponins. Emergent cardiac catheterisation revealed distal blockages in the left anterior descending artery and second obtuse marginal artery (figure 1A,B). After distal balloon angioplasty, the decision was made to further treat the patient medically and discharge home after clinical stability was achieved. She was readmitted 2 days later for new onset of palpitations with up-trending troponins. Laboratory tests were significant for a low thyroid-stimulating hormone of <0.01 µLU/mL (reference range: 0.27–4.2 µLU/mL), and elevated free T4 of 4.02 µLU/mL (reference range: 0.9–1.7 ng/dL), free T3 of 4.8 pg/mL (reference range: 2.5–4.3 pg/mL) and a thyroid stimulating immunoglobulin of 386 (reference range: <140% baseline). She was immediately started on both methimazole 20 mg and potassium iodine oral solution (SSKI). Further workup...

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Haemorrhagic cholecystitis: a rare entity not to be forgotten

Haemorrhagic cholecystitis is a rare entity of acute cholecystitis that carries a high morbidity and mortality rate if management is delayed. Its clinical course can mirror that of acute cholecystitis. Characteristic findings on ultrasound or CT scan are useful clues to early diagnosis. Urgent cholecystectomy is required prior to progressing to perforation of gallbladder. Most of the literature are case reports with causes associated with anticoagulation. Herein, we described a morbidly obese patient with poorly controlled diabetes presenting with non-specific right upper quadrant pain and was subsequently diagnosed with haemorrhagic cholecystitis. A review of the literature was also performed to summarise the potential clinical presentations, distinctive imaging findings and management options available for this rare condition.

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Pneumomediastinum: a complication of reinserting a dislodged tracheostomy

Description 

A 54-year-old woman with a background of obesity and hypertension presented following a pulseless electrical activity (PEA) cardiac arrest. Cardiopulmonary resuscitation (CPR) was commenced on arrival of the paramedics and she was intubated and ventilated (figure 1). Return of spontaneous circulation was achieved in the emergency department following a total of 45 min of CPR. No clear cause of her arrest was identified. She was transferred to the intensive care unit (ICU) for post-arrest management.

Figure 1

Initial chest radiograph following return of spontaneous circulation, with endotracheal tube present (included as a baseline for comparison).

Seizure activity off of sedation necessitated further investigation. Her CT head was unremarkable, but her lumbar puncture showed features in keeping with meningitis. She was therefore treated with antibiotics and antiepileptics. Her cerebrospinal fluid viral PCR and blood HIV tests were negative.

Although appropriate seizure control was achieved,...

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Naclerios V sign and continuous diaphragm sign after endoscopy

Description 

An otherwise healthy 72-year-old woman presented with dyspnoea and systemic subcutaneous emphysema. She had undergone upper endoscopy, in which duodenal ulcer was found. The community hospital where the endoscopy was conducted transferred her to our tertiary hospital for further diagnosis and management. On arrival, she complained of severe difficulty in breathing; massive subcutaneous emphysema on her chest to the feet was observed on palpation. Chest X-ray image showed Naclerio's V sign (figure 1A, black arrow) and continuous diaphragm sign (figure 1A, white arrows). CT scan confirmed massive subcutaneous and mediastinal emphysema with pneumothorax, pneumopericardium and free air in retroperitoneal space (figure 1B,C). Emergent laparotomy revealed duodenal perforation, 9 mm in diameter at the descending part of duodenum (figure 1D, white arrow), and was surgically repaired. After the surgery, the emphysema gradually disappeared in weeks and had not recurred at 6-month follow-up.

...

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Hypertrophic pyloric stenosis following repair of oesophageal atresia and tracheo-oesophageal fistula in a neonate

Development of hypertrophic pyloric stenosis (HPS) after a few weeks of repair of an oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) is a rare condition in early infancy. Although vomiting or feeding intolerance in operated cases of OA+TOF are attributed to oesophageal stricture, gastro-oesophageal reflux and oesophageal dysmotility, it may also be caused by HPS. Herein, we report a newborn infant who had OA and TOF operation on day 2 of life and diagnosed to have HPS at 15th day of age. Even though it is a rare anomaly, HPS should be kept on mind in the presence of persistent vomiting following repair of OA.

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Primary renal angiosarcoma: a diagnostic and therapeutic challenge

Primary renal angiosarcoma is an exceedingly rare and aggressive neoplasm. Although it may occur in youth, this tumour is frequently reported in the sixth and seventh decades of life. The clinical presentation is frequently varied. Pathogenesis remains largely unknown and it has overlapping features with other tumours of the kidney. Current treatment options include variable combinations of surgery, chemotherapy and radiotherapy. Reports regarding the disease prognosis and natural history are limited. In this article, we chronicle the case of a patient with primary renal angiosarcoma presenting at an advanced stage as a widely metastasised tumour. Additionally, we undertake here a brief literature review highlighting the rarity and aggressiveness of this condition, its poor prognosis, and the lack of specific management guidelines.

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Intestinal obstruction with a twist: a rare case of congenital portal vein aneurysm causing intestinal obstruction

Bilious vomiting is often a presenting feature of upper intestinal obstruction in newborn. We present a case of intestinal obstruction in a newborn baby caused by abnormal vascular band arising from portal vein aneurysm in association with a midgut volvulus. Congenital anomalies of portovenous system are very rare, and it usually presents with portal hypertension in late infancy or childhood. In this particular child, the portal vein aneurysm contributed to intestinal obstruction due to both a failure of intestinal rotation and a mechanical band over the transverse colon.

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