Cardiac Perivascular Epithelioid Cell Tumor: Report of a Child and Review of the Literature.

Cardiac tumors presenting as mediastinal masses in childhood are rare. This report describes the case of a 6-year-old girl who presented to us after a failed attempt at resection of a mass arising from the left atrial appendage. Computed tomography-guided biopsy suggested a perivascular epithelioid cell tumor (PEComa).

The distal level of normally innervated bowel in long segment colonic Hirschsprung's disease.

Purpose: The definitive surgical management of Hirschsprung's disease (HD) depends on an unambiguous histological diagnosis of bowel aganglionosis, as well as an accurate assessment of its proximal extent from the rectum or 'leveling'. This study aimed to evaluate and compare the transition zone in rectosigmoid and long segment colonic forms of HD.

Methods: One hundred and fifteen cases of rectosigmoid Hirschsprung's disease (RSHD) and 20 cases of long segment colonic Hirschsprung's disease (LScHD) managed from 1998 to 2008 with a consensus prospective protocol were included in this clinicopathological analysis.

Bilateral spontaneous asymptomatic urinoma: report of an unusual case.

Urinoma, defined as a collection of chronically extravasated urine, is uncommon in children. The condition is usually secondary to obstructive uropathy, commonly posterior urethral valves. Non-obstructive causes include trauma to the kidney or collecting system.

Undescended testes.

Undescended testis or cryptorchidism is a common problem encountered in children. Despite the abundant literature on the pathology and management of undescended testis, it is the subject of numerous misgivings and controversies. The article reviews available literature on the entity and their relevance in clinical practice.

Neonatal colonic mucormycosis--a tropical perspective.

Neonatal gastrointestinal (GI) mucormycosis is a rare, usually fatal, opportunistic fungal infection, which is difficult to diagnose early or preoperatively. We report three babies, only one of whom survived, with a review of the literature. All three had similar findings of necrosis of colon with multiple perforations.

Traumatic posterior urethral disruptions in boys: experience with the perineal/perineal-transpubic approach in ten cases.

The management of traumatic posterior urethral disruption in children has ranged from primary realignment/repair to delayed urethroplasty. The operative approach may be perineal or transpubic; the advocates of either cite comparable outcome. The anatomic considerations in a child differ from the adult and the management is individualized.

A modified technique for the diagnosis of Hirschsprung disease from rectal biopsies.

Background: A diagnosis of Hirschsprung disease requires the demonstration of acetylcholinesterase fibres on frozen sections obtained from snap frozen biopsies of the rectum. This histochemical technique is generally not available in laboratories in developing countries. We improvised on the methodology of tissue preservation to make the staining technique more user-friendly, economical and reliable in demonstrating acetylcholinesterase activity in fresh rectal mucosal biopsies for the diagnosis of Hirschsprung disease.

Pyeloplasty in infancy.

Objective: The advent of routine maternal ultrasound has increased the number of neonates diagnosed to have ureteropelvic junction obstruction. Though the debate concerning its ideal management continues, recent years have witnessed reports on pyeloplasty in infancy. This study presents the feasibility and outcome of pyeloplasty in neonates and infants from a single centre.