Dual intestinal anomalies in dizygotic twins

We report on the case of two digestive malformations in dizygotic/dichorionic/diamniotic twins born at 31 weeks of gestation. The mother (gravida 1 para 0) was treated by hydroxychloroquine for systemic lupus erythematosus during pregnancy. Twin A presented an arch-like dilatation on antenatal ultrasounds, characteristic of segmental volvulus. After birth, twin B presented repeated vomiting on feeding, leading us to diagnose ileal atresia despite normal antenatal ultrasounds. Both twins underwent surgery and the postoperative period was uneventful. After 1 year of follow-up, the twins are in excellent health without digestive sequelae. Genetic testing for cystic fibrosis was negative. The placenta showed diffuse signs of hypoxia and ischaemia, indicating that the root cause was vascular. The pathophysiology of intestinal atresia is hypothesised to derive from a vascular incident during fetal development. We are therefore led to believe that an intrauterine vascular event is the most likely cause of the dual malformation.

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Embolic stroke, left atrial myxoma and gigantism in a patient with Carney complex with additional features suggestive of Marfan syndrome

A 16-year-old boy presented to the emergency department with a sudden weakness on the right side of the body and was diagnosed as having embolic stroke. Later on, the patient was diagnosed as having Carney complex (CNC). The neurological complication might be caused by left atrial myxoma as a feature of CNC. Surprisingly, the patient showed some additional features such as positive wrist and thumb signs, pectus carinatum deformity and plain flat feet, suggestive of Marfan syndrome. This case demonstrated that both of these syndromes might coexist in the same patient, suggesting that proper diagnostic and management were key factors that affected prognosis. He showed an improved condition after he had received medical treatments, undergone tumour excision and physiotherapy. Further evaluation was needed to improve patient outcomes.

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Unexpected manifestation of cardiac amyloidosis

This report discusses an unusual case of cardiac amyloidosis. We report a patient who presented with unexplained ascites on a background of stable hypertension and mild left ventricular systolic dysfunction, cardiovascular complaints commonly associated with age. Due to the unspecific nature of his cardiovascular symptoms, it took 2 years of recurrent, unresolved ascites, numerous investigations, shifting differential diagnoses and significant cardiovascular deterioration before cardiac amyloidosis was recognised, by which the disease was at end stage. This case emphasises the need for more discriminating clinical features in the diagnosis of cardiac amyloidosis and advocates unexplained, recurrent ascites as a possible candidate.

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Uterine didelphys with one cervix obscured by blind hemivagina: a lesson in rarity

A 14-year-old girl presented with increasing cyclical pain, scanty menses, pelvic mass and absence of the left kidney. With both radiological and clinical examinations (examination under anaesthesia), diagnosis of bicornuate uterus with single cervix could be made while on laparotomy, and it turned out to be uterine didelphys, with one cervix obscured by blind hemivagina with haematometra and haematocolpos in the left horn, for which hemihysterectomy was done. Post procedure the patient was relieved of cyclical pain and is menstruating properly.

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Treatment of nasal myiasis with ivermectin irrigation

We describe a case of nasal myiasis due to Musca domestica in a 97-year-old Peruvian farmer with a previously undiagnosed mucocutaneous leishmaniasis. Initial attempts to remove the fly larvae using manual extraction with a toothed forceps and normal saline irrigation were unsuccessful. On subsequent nasal irrigation with ivermectin solution, the patient self-expulsed approximately 50 larvae within 15 min. He also received a course of oral ivermectin. A post-treatment CT scan revealed clear sinuses. Here, we propose that ivermectin irrigation is a simple and effective treatment for nasal myiasis.

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Acute intestinal ischaemia from a portal vein thrombosis in a young female smoker on an oral contraceptive

We report the case of a 23-year-old woman who presented with bloody diarrhoea and multiple syncopal events. While the initial diagnosis clinically appeared to be inflammatory bowel disease, she was found to have a portal vein thrombosis (PVT) on MR cholangiopancreatography and acute intestinal ischaemia on colonic biopsy. The aetiology of this patient's PVT is attributed to her acquired prothrombotic state from an estrogen-containing contraceptive pill in conjunction with regular tobacco use. Extensive mesenteric venous thrombosis from an acute PVT has been shown to cause intestinal ischaemia, likely from venous obstruction and reflexive arterial constriction; however, the diagnosis is often delayed until surgery or autopsy. Our case report highlights this patient's clinical presentation, workup and treatment, as part of a review for the risk factors and guidelines recommendations for management of an acute PVT.

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Appendiceal schwannoma: a rare cause of perforated appendicitis

Description 

An 82-year-old woman presented to the emergency department with an acute abdomen. CT imaging revealed a proximal appendiceal mass with distal appendicitis. She underwent a laparoscopic appendicectomy with partial caecectomy and subsequent histopathology confirmed an appendiceal schwannoma.

The woman presented with a 1-day history of acute abdominal pain localised to the lower quadrants, worse on mobilisation, loss of appetite and fever. She denied any previous abdominal pain, nausea or vomiting, bowel or bladder symptoms. There was no history of weight loss, generalised fatigue or other symptoms of chronic anaemia. She had been well in the preceding weeks. Her medical history was significant for a cerebrovascular accident, hypertension and hypercholesterolaemia. She had never had previous abdominal surgery.

On examination the patient had a temperature of 37.7°C. There were no signs of haemodynamic instability. Her abdomen was rigid with severe tenderness on palpation, worse in the right lower quadrant....

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Iliac bone tuberculosis with bicompartmental abscess

Description 

A 39-year-old man presented with insidious onset back pain and groin pain for 2 weeks, and limp for 7 days to orthopaedic outpatient department. He had anorexia for the past fortnight and had noticed weight loss of about 7 kg, though he remained afebrile. On examination, he had a pseudoflexion deformity of the right hip and tenderness in the right iliac fossa. An X-ray of the pelvis was done which showed an ill-defined lytic lesion in the anterior half of the right iliac blade with minimal periosteal reactions (figure 1). His laboratory investigation showed a high haemoglobin of 12 g/dL, erythrocyte sedimentation rate of 64 mm fall at the end of first hour, a raised total lymphocyte count of 13x10⁹/L with lymphocytosis 40%. His renal, liver and nutritional parameters were within normal range (albumin >3500 mg/dL, absolute lymphocyte count >1500/mm³). X-ray of the dorsolumbosacral region and chest was unremarkable. An MRI was done which...

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Sensory ganglionopathy associated with drug-induced hypersensitivity syndrome caused by mexiletine

Although various causes are reported for sensory ganglionopathy, drug-induced hypersensitivity syndrome (DIHS) has not been considered a possibility. We describe a 70-year-old woman, previously administered mexiletine hydrochloride for 4 weeks, who presented with systemic oedematous erythema and subacute progressive gait disturbance. Evaluation revealed lymphadenopathy with atypical lymphocytosis and eosinophilia, and human herpesvirus 6 (HHV-6) reactivation. Neurological examination indicated the almost complete loss of joint positional sense in her extremities; her tendon reflex was lost and there was marked pseudoathetosis and Romberg's sign. Skin biopsy revealed spongiosis with lymphocyte infiltration. Based on these findings, we diagnosed acute sensory ganglionopathy secondary to DIHS. Although her DIHS-induced symptoms subsided after methylprednisolone treatment, partial remission of sensory ganglionopathy occurred, even after subsequent intravenous immunoglobulin therapy. This case suggests the possibility that reactivation of HHV-6 may be involved in the pathomechanism of sensory ganglionopathy.

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Inflammatory myofibroblastic tumour mimicking a vocal cord polyp

Description 

A 9-year-old child presented with the complaint of voice change of 8 months in duration, which was gradually progressive, and a recent-onset noisy breathing at night noticed by the parents. There were no signs of airway distress or feeding issues on presentation, and the growth of the child was appropriate for her age.

On indirect laryngoscopic examination, a smooth, large polypoidal mass was seen arising from the anterior commissure, partially obscuring the laryngeal airway. Bilateral vocal cords were mobile. The initial impression was that of a benign vocal cord polyp (figure 1).

Figure 1

Laryngoscopic image showing smooth polypoidal mass arising from the anterior commissure.

The rest of the clinical examination was normal.

The patient was then planned for microlaryngoscopic laser-assisted excision of the polyp under general anaesthesia. Intraoperatively, the mass was engaged using a suspension laryngoscope and was seen  to arise from the anterior commissure and found to be firm on palpation....

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Analgesic effects of methadone and magnesium following posterior spinal fusion for idiopathic scoliosis in adolescents: a randomized controlled trial

Abstract

Purpose

To provide optimal conditions for neurophysiological monitoring and rapid awakening, remifentanil is commonly used during pediatric spinal surgery. However, remifentanil may induce hyperalgesia and increase postoperative opioid requirements. We evaluated the potential of methadone or magnesium to prevent remifentanil-induced hyperalgesia.

Methods

Using a prospective, randomized, blinded design, adolescents presenting for posterior spinal fusion to treat idiopathic scoliosis were assigned to receive desflurane with remifentanil alone (REMI), remifentanil + methadone (MET) (0.1 mg/kg IV over 15 min), or remifentanil + magnesium (MAG) (50 mg/kg bolus over 30 min followed by 10 mg/kg/h). Primary outcomes were opioid requirements and postoperative pain scores. Secondary outcomes included intraoperative anesthetic requirements, neurophysiological monitoring conditions, and emergence times.

Results

Data analysis included 60 patients. Total opioid requirement (hydromorphone) in the REMI group (received perioperatively and on the inpatient ward) was 0.34 ± 0.11 mg/kg compared to 0.26 ± 0.10 mg/kg in the MET group (95% confidence interval (CI) of difference: − 0.14, − 0.01; p = 0.035). The difference in opioid requirements between the REMI and MET group was related to intraoperative dosing (0.04 ± 0.02 mg/kg vs. 0.02 ± 0.01 mg/kg; 95% CI of difference: − 0.01, − 0.02; p = 0.003). No difference was noted in pain scores, and no differences were noted when comparing the REMI and MAG groups.

Conclusion

With the dosing regimens in the current study, the only benefit noted with methadone was a decrease in perioperative opioid requirements. However, given the potential for hyperalgesia with the intraoperative use of remifentanil, adjunctive use of methadone appears warranted.

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