Τετάρτη 29 Αυγούστου 2018

A left-sided cystic pancreatic incidentaloma with sigmoid colon adenocarcinoma: a case report

The synchronous colorectal malignancy is well described in the literature but combination of pancreatic incidentaloma with sigmoid cancer has not been well described and the association has not been described ...

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An unusual cause of a breast mass in a 13-year-old girl: a case report

Adolescents rarely present with breast lumps, and such lumps are usually due to benign causes. Foreign bodies in the breast are an uncommon finding and could be detected incidentally during imaging or be sympt...

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Tragedy of transition: hypertensive crisis in a young adult secondary to unilateral ureteropelvic junction obstruction following pyeloplasty as an adolescent

A 25-year-old man with a history of left ureteropelvic junction (UPJ) obstruction that was corrected surgically at the age of 16 presented with a chief complaint of syncope. He was found to have severe hypertension with evidence of end organ damage on laboratory evaluation. His blood pressure was controlled with intravenous and oral antihypertensives with improvement in end organ dysfunction. Workup for secondary causes of hypertension implicated failed left-sided pyeloplasty with resultant hydronephrosis as the aetiology. The patient was transitioned to an oral antihypertensive regimen and discharged with urological surgery follow-up. Blood pressure control was maintained with oral antihypertensives and a low-salt diet; however, evidence of chronic kidney disease persisted. This case highlights the importance of close follow-up and adequate transition of care in patients with UPJ obstruction who transitioned to adulthood.



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History conflict and non-formulary medicine: a threat for anaesthesiologist in low and middle-income countries

The practice of complementary and non-formulary medicine has gained tremendous popularity due to their claimed beneficial effects in cardiac, respiratory and chronic diseases, as also other disorders. The most threatening aspect related to these practices pertains to the non-disclosure of its use by patients at the time of their preoperative assessment in elective or emergency setting. We report a case of profound, long-lasting unexplained hypotension during and after anaesthesia in a patient presented for emergency vocal cord surgery (cordectomy). He was taking complementary medicine for last 2 years. Serum cortisol level was sent postoperatively in intensive care unit that was found extremely low.



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Acute horseshoe abscess of the hand after corticosteroid injection to treat trigger thumb

Local corticosteroid injections are frequently used in the management of trigger finger. We present a case of a 56-year-old woman who developed an acute horseshoe abscess of the hand after injection of corticosteroid and local anaesthetic into the left thumb. This was managed successfully with intravenous antibiotics, operative intervention and early mobilisation. This case highlights the possible complications that can occur with such a minimally invasive procedure. The pathophysiology behind this condition is explained by communication between the radial and ulnar bursae. Knowledge of the anatomy of the hand and its variants is therefore essential to assist in diagnosis. Prompt clinical diagnosis and surgical management is required to avoid disastrous complications.



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Acute spontaneous intracranial epidural haematoma and disseminated intravascular coagulation in a paediatric sickle cell patient

An African American teenage boy during an acute sickle cell crisis spontaneously developed acute bifrontal epidural haematomas (EDHs) in addition to disseminated intravascular coagulation (DIC). The successfully evacuated EDH reaccumulated postoperatively. After multiple transfusions, the patient underwent repeat surgery. Subsequent maximal medical therapy was unable to significantly improve the patient's neurological status, and due to family wishes, care was withdrawn. EDH are the most common emergent neurosurgical complication of sickle cell disease (SCD). Twenty-two such cases have been previously reported. We present one further complicated by DIC leading to reaccumulation of the patient's EDH. An understanding of the mechanisms of EDH formation in SCD and their associated radiological findings could help clinicians identify when a patient is at high risk of EDH formation and thus offer the potential for early intervention prior to the development of an emergency.



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Optical coherence tomography of iris mammillations

Description 

A 10-year-old girl presented with blurred vision of 2 months. Uncorrected visual acuity was 20/20 in both eyes (OU). Refraction revealed 0.75 diopters of astigmatism OU. Visual blur was related to a moderate convergence insufficiency. The superior 90% of the left iris was darkly pigmented and velvety in consistency with regularly spaced protuberances (mammillations) (figure 1).

Figure 1

Slit lamp photograph of the right normal iris (A) and left dark velvety iris with mammillations (B). The inferior sector of the left iris is normal. Anterior segment optical coherence tomography (OCT) of the normal right iris demonstrates smooth surface with random crypts (C). OCT of the left eye shows numerous micronodules on the iris surface with flattening of the entire iris (D).

We present a novel anterior optical coherence tomography (OCT) report of iris mammillations.1 2...



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SIADH and water intoxication related to ecstasy

Recreational drug use is a significant societal issue and remains a clinical challenge in emergency and critical care departments. We report on a 19-year-old woman admitted to hospital semiconscious and with severe hyponatraemia. Urinalysis was positive for methamphetamine and supported a diagnosis of hyponatraemia related to ecstasy use together with a syndrome of inappropriate antidiuretic hormone secretion (SIADH). The woman was transferred to an intensive care unit, where a hypertonic saline infusion was started. Three hours postadmission she developed polyuria. Follow-up urinalysis at this point was consistent with water intoxication. This case is a reminder that hyponatraemia is a potentially fatal complication after the ingestion of 3,4-methylenedioxymethamphetamine, illustrates the sequential nature of an SIADH and water intoxication and highlights the importance of considering the sequence of onset of hyponatraemia, as the patient may be admitted at any stage.



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Hypothermia as a forgotten sign of prolonged severe hypoglycaemia

Hypothermia is often a sign of serious illness. Commonly reported aetiologies include but are not limited to sepsis, exposure to cold and endocrine disorders. Hypoglycaemia, a common occurrence, is rarely associated with hypothermia. We present a case of prolonged, severe hypothermia due to hypoglycaemia. A 58-year-old man with diabetes who presented with chest pain and was diagnosed with Non-ST elevation myocardial infarction. He was given nothing per mouth in preparation for a left heart catheterisation but received his reported insulin glargine dosage at bedtime. A few hours later, he was noted to have diaphoresis and hypoglycaemia, and his temperature steadily started dropping which was unresponsive to local warming. Once his hypoglycaemia was successfully treated with dextrose infusion, his temperature improved. An extensive workup revealed no infective or endocrine disorder.



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Bronchobiliary fistula caused by diaphragmatic invasion of a hepatic tumour

A 71-year-old man presented with a productive cough consisting of yellow fluid. He had previously been treated for pneumonia without resolution in his symptoms. Sputum was tested for bilirubin using a urine dipstick given its similar appearance to bile, which was positive. Hepatobiliary scintigraphy scan revealed uptake of radiotracer in the right lower lobe of the lung. Endoscopic retrogade cholangiopancreatography confirmed diagnosis of a bronchobiliary fistula. The patient had a stent placed in the common bile duct promoting anterograde bile flow with complete resolution of symptoms.



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When your immune system falls out with your heart: an important lesson on antisynthetase myocarditis

A 57-year-old special Olympics athlete presented with subacute onset dyspnoea. Baseline investigations revealed troponin T rise and an abnormal ECG, which prompted coronary angiography. This was unremarkable, as was his transthoracic echocardiography (TTE). He re-presented 7 months later with progressive dyspnoea associated with significant weight loss, peripheral oedema and intermittent fevers. Examination revealed bilateral fine end-inspiratory crackles, peripheral oedema and fever. Investigations revealed elevated troponin T and raised inflammatory markers. ECG remained unchanged, whereas TTE revealed mild global impairment of left ventricular function. Chest radiography was suggestive of extensive interstitial lung disease, which was confirmed by high resolution CT. Presence of interstitial lung disease and myocarditis raised the suspicion of a systemic inflammatory condition. Subsequently, an autoimmune screen was positive for anti-Jo-1 antibody associated with antisynthetase syndrome. He was treated with high-dose steroids and rituximab with dramatic symptomatic improvement and immediate fall in troponin T level.



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Extrapulmonary tuberculosis: atypical presentation in otorhinolaryngology

Tuberculosis (TB) continues to be a major health burden globally more so in low/middle-income countries like India. There is an increase in the prevalence of extrapulmonary TB (EPTB) because of HIV epidemics and increased usage of immunomodulating drugs. EPTB constitutes 15%–20% of all patients with TB and >50% of HIV-TB coinfected patients. We present three such atypical presentations of EPTB in head and neck region. EPTB can mimic any disease, hence knowledge of the unusual presentations helps in making early diagnosis and thereby reduces the morbidity and mortality involved with the disease.



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Flashes of light and floaters: an unusual presentation of urothelial carcinoma

Description 

A 59-year-old Caucasian woman with multiple sclerosis presented with blurred vision in the right eye, floaters and flashes of light for 2 weeks. She had right-sided optic neuritis which was treated 8 years prior. There was a 20 pack-year smoking history. Examination was remarkable for right-sided temporal field loss and a palpable breast lump. Ophthalmological examination revealed right exudative retinal detachment, with ultrasonography demonstrating a choroidal mass with medium to high internal reflectivity. MRI was significant for a 14 mm intraocular metastasis (figure 1). On further questioning, the patient described haematuria. Urine cytology demonstrated large cells with high nuclear to cytoplasmic ratio, irregular nuclear borders and necrosis, all suggesting high-grade malignancy. Abdominal imaging revealed an ill-defined heterogeneously hypoenhancing infiltrative lesion of the right kidney within the mid and superior portions measuring 7.5x6.6x8.5 cm (figure 2). Biopsy of both the renal and breast masses confirmed urothelial...



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Spontaneous bacterial empyema: an elusive diagnosis in a patient with cirrhosis

Hepatic hydrothorax refers to the presence of a pleural effusion (usually >500 mL) in a patient with cirrhosis in whom other causes of pleural effusion, such as cardiopulmonary causes, pleural disease or malignancy have been excluded. It is seen in 5%–10% of patients with end-stage liver disease. A subset of these patients can develop infection of the hepatic hydrothorax, called spontaneous bacterial empyema. They may present with fever, chills and dyspnoea. We present the case of an 83-year-old man with a history of cirrhosis who developed a large right-sided pleural effusion, confirmed to be empyema by pleural fluid analysis. We aim to highlight the occurrence of spontaneous bacterial empyema. While less common that spontaneous bacterial peritonitis as a complication of cirrhosis, it is equally serious with potential for adverse outcomes.



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Unusual discovery of a vestibular schwannoma following eradication therapy for Mycobacterium abscessus

A young man with cystic fibrosis in his early 30s presented to accident and emergency with acute onset unilateral lower motor neuron facial palsy, hearing loss and impaired balance following Mycobacterium abscessus eradication induction therapy. The hearing loss and impaired balance developed over a 3-day period prior to the onset of facial palsy. Further investigation with a CT scan and MRI scan led to a diagnosis of vestibular schwannoma. The facial palsy resolved with steroid treatment; however, the hearing loss is irreversible, which has had a profound impact on his life and career. This case is intriguing as the cause and association of events are unclear. A working diagnosis of incidental Bell's palsy and unilateral hearing loss caused by the vestibular schwannoma was applied. However, the onset of these symptoms in relation to M. abscessus eradication induction therapy promotes discussion.



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Use of cinacalcet in lithium-induced hyperparathyroidism

The case of a 61-year-old female patient with a long-standing history of bipolar affective disorder treated medically with lithium therapy for the past two decades. In late 2012, the patient was diagnosed with hyperparathyroidism secondary to lithium therapy. The patient underwent parathyroidectomy in August 2013. During surgery, only two glands were conclusively located and removed. This resulted in a reduction in the patient's plasma total calcium levels and parathyroid hormone. The psychiatric management of the bipolar affective disorder was reviewed, and lithium discontinued as a result of the findings. Over the following year, a variety of different mood stabilisers were trialled, however none were found to successfully maintain the patient's mental health. In August 2014, the patient was admitted with a severe depressive relapse of her bipolar affective disorder. Her admission tests showed hypercalcaemia, which may also have contributed to her mood symptoms and mental state deterioration. The patient was reviewed by the endocrinology team and subsequently commenced on cinacalcet treatment (30 mg twice a day). Over the following months, the patient's plasma total calcium levels returned to within normal range. The patient's depressive symptomatology gradually improved with a combination of physical and pharmacological treatments.



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Breast cancer and organising pneumonia: the importance of correlating the pathological findings with the clinical and radiological picture

This case describes a female patient with a history of breast cancer who presented with a persistent cough and weight loss after a 17-year disease-free period. Radiologically there were new bilateral intrapulmonary nodules and areas of consolidation with a broad differential diagnosis. Brushings gained via bronchoscopy were suggestive of malignancy, but subsequent video-assisted thoracoscopic surgery wedge resection demonstrated organising pneumonia (OP) with no evidence of malignancy. Diagnostic uncertainty remained after this, and after a period of observation, there was evidence of disease progression. Further tissue was obtained for cytological and histological assessment which provided conclusive evidence of metastatic breast cancer. In this case, OP was secondary to proximal bronchial obstruction due to metastatic infiltration, of which there are no reported similar cases in the current literature. We discuss the importance of differentiating secondary OP from cryptogenic OP.



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Paediatric duodenal injury complicated by common bile duct rupture due to blunt trauma: a multispecialist approach

We report a case of late presenting duodenal perforation with common bile duct rupture secondary to blunt handlebar trauma in an 11-year-old boy. The patient presented with upper abdominal wall ecchymosis, pain and vomiting. He was discharged after 24 hours with resolving symptoms. However, the boy presented 2 days later febrile with signs of peritonitis. CT indicated duodenal perforation, which was confirmed during laparotomy where common bile duct rupture was also demonstrated. Primary repair of the duodenum was undertaken. Here, decompression was achieved with a nasogastric tube proximal to the injury and T-tube duodenostomy distally. Common bile duct repair was achieved over a biliary stent. This case represents a rare subset of duodenal injury for which there is a paucity of evidence for optimal surgical management, particularly in the paediatric setting. This operative plan will guide surgeons and junior doctors in managing complicated cases like this in future.



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Retzius space haematoma as a rare cause of concealed retroperitoneal postpartum haemorrhage following spontaneous vaginal delivery

A 34-year-old woman presented to hospital in shock 8 hours after a spontaneous home vaginal delivery. She is a known carrier of haemophilia A. Examination revealed a tender but firm, central and contracted uterus. There was a small second-degree perineal tear which was not actively bleeding and no other trauma or bleeding visualised in the vault or cervix. Bedside ultrasound showed a large 1 L blood clot anterior to the empty uterus. Exploratory laparotomy was performed which revealed a haematoma in the Retzius space with extension beneath the anterior serosa of the bladder, through the left broad ligament and into the retroperitoneal space. Bleeding from the Santorini plexus was controlled with haemostatic sutures, reinforced with FLOSEAL and intra-abdominal packing. A CT angiogram and relook laparotomy the following day confirmed haemostasis and facilitated removal of intra-abdominal packs. This case demonstrates a very rare cause of postpartum retroperitoneal haemorrhage.



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Primary Epstein-Barr virus infection in immunocompetent patients with acute transverse myelitis and a combination of polyradiculitis and anterior horn syndrome as neurological manifestations

Neurological manifestations of a primary Epstein-Barr virus (EBV) infection are rare. We describe a case with acute transverse myelitis and another case with a combination of polyradiculitis and anterior horn syndrome as manifestations of a primary EBV infection.

The first case is a 50-year-old immunocompetent male diagnosed with acute transverse myelitis, 2 weeks after he was clinically diagnosed with infectious mononucleosis. The second case is an 18-year-old immunocompetent male diagnosed with a combination of polyradiculitis and anterior horn syndrome while he had infectious mononucleosis. The first patient was treated with methylprednisolone. After 1 year, he was able to stop performing clean intermittent self-catheterisation. The second patient completely recovered within 6 weeks without treatment.

Primary EBV infection should be considered in immunocompetent patients presenting with acute transverse myelitis and a combination of polyradiculitis and anterior horn syndrome. Antiviral treatment and steroids are controversial, and the prognosis of neurological sequelae is largely unknown.



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Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection

Systemic capillary leak syndrome (SCLS), also known as Clarkson syndrome, is a rare disease with potential fatal outcome. The clinical picture involves leakage of fluid and protein from the bloodstream into peripheral tissues, resulting in hypoalbuminaemia, elevated haematocrit, oedema and hypotension. The spectrum of the symptoms ranges from discrete swelling/oedema of extremities to fulminant cardiogenic shock. We present a case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome. The severe episode of capillary leak was potentially triggered by influenza virus infection. With the benefit of hindsight, he presented with symptoms of SCLS 2 years prior the major acute episode. Here we describe this case and review some aspects of the literature on SCLS, with particular focus on the pathogenesis, treatment/prophylaxis and long-term physical and psychological complications.



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An interesting case of vanishing shoulder

Description 

A 37-year-old woman with progressive difficulty in using her left arm and with discomfort in the left shoulder was evaluated. Her complaints were of insidious onset and progressed over a period of 3 months. She had a soft shoulder and passive movements in the left shoulder were increased in all ranges, with internal and external rotation of 180° each (figure 1A,B). Active abduction was up to  90°. Radiographs of the shoulder demonstrated the absence of humeral head and a small sliver of bone, reminiscent of the greater tubercle (figure 1C). She was evaluated for neuropathic arthropathy, infection, neoplastic, metabolic and endocrine causes of osteolysis using appropriate blood tests and imaging techniques. Her blood parameters were normal. MRI confirmed absence of the proximal humerus and resorption and expansion of the glenoid cavity (figure 2A,C). A syrinx extending from the C2–D9 spinal region (figure 2C,D) was incidentally...



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Type IV Laryngotracheoesophageal Cleft Associated with Type III Esophageal Atresia in 1p36 Deletions Containing the RERE Gene: Is There a Causal Role for the Genetic Alteration?

The causes of embryological developmental anomalies leading to laryngotracheoesophageal clefts (LTECs) are not known, but are proposed to be multifactorial, including genetic and environmental factors. Haploinsufficiency of the RERE gene might contribute to different phenotypes seen in individuals with 1p36 deletions. We describe a neonate of an obese mother, diagnosed with type IV LTEC and type III esophageal atresia (EA), in which a 1p36 deletion including the RERE gene was detected. On the second day of life, a right thoracotomy and extrapleural esophagus atresia repair were attempted. One week later, a right cervical approach was performed to separate the cervical esophagus from the trachea. Three months later, a thoracic termino-terminal anastomosis of the esophagus was performed. An anterior fundoplication was required at 8 months of age due to severe gastroesophageal reflux and failure to thrive. A causal role of 1p36 deletions including the RERE gene in the malformation is proposed. Moreover, additional parental factors must be considered. Future studies are mandatory to elucidate genomic and epigenomic susceptibility factors that underlie these congenital malformations. A multiteam approach is a crucial factor in the successful management of affected patients.

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Implication of Topical Steroids in the Onset of Osteoporosis

The systemic bioavailability of steroids has long been implicated as a cause for osteoporosis (OP); however, much less is known about the effect of topical steroids on bone homeostasis. This is a case of an 11-year-old male who is a known case of generalised pustular psoriasis for 8-year duration with frequent exacerbations controlled with topical betamethasone dipropionate. He presented with generalised progressive bone pain and positive history of bone fracture. The diagnosis of OP was established on the results of DEXA, which were −2.7 SD and −2.4 SD for the lumbar spine and whole body, respectively. Although the cutoff value is the same (

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Lamotrigine-Valproic Acid Interaction Leading to Stevens–Johnson Syndrome

Lamotrigine (LTG) is currently indicated as adjunctive therapy for focal and generalized tonic-clonic seizures and for treatment of bipolar disorder and neuropathic pain. A common concern with LTG in children is the frequency of appearance of skin rash. The intensity of this adverse effect can vary from transient mild rash to Stevens–Johnson syndrome (SJS), which can be fatal mainly when LTG is coadministered with valproic acid (VPA). Hereby, we present the case of an 8-year-old boy who suffered from SJS and other complications two weeks after LTG was added to his VPA treatment in order to control his seizures. VPA is known to decrease LTG clearance via reduced glucuronidation. In this case, the minor elimination pathway of LTG would play a more important role, and the formation of an arene oxide metabolite would be enhanced. As this reactive metabolite is detoxified mainly by enzymatic reactions, involving microsomal epoxide hydrolase and/or GSH-S-transferases and these enzymes are polymorphically expressed in humans, arene oxide toxicity is increased when epoxide hydrolase or GSH-S-transferases is either defective or inhibited or a depletion of intracellular glutathione levels is taking place. VPA can cause inhibition of epoxide hydrolase enzymes and/or depletion of glutathione levels leading to adverse cutaneous reactions.

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Ehlers–Danlos Syndrome: Not Just Joint Hypermobility

Ehlers–Danlos syndrome is an umbrella term for a group of heritable soft connective tissue disorders which is characterized by joint hypermobility, skin texture and elasticity abnormalities, and visceral and vascular fragility or dysfunctions. As the syndrome is rare, it is often underdiagnosed. Patients usually present late, with chronic moderate to severe pain which is attributed to the joint hypermobility and joint subluxations. If the clinician is aware of the syndrome, he/she can identify affected patients in order to prevent complications. We report a 60-year-old woman with arthralgia and back pain lasting for several months and recent metatarsophalangeal luxation of the left toe who was discovered to have Ehlers–Danlos syndrome.

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