Hirschsprung’s disease and associated syndrome and malformation: A case series of four cases

Hirschsprung’s disease is a genetic disorder characterized by the absence of ganglion cells in different lengths of the intestine. It is one of the common causes of intestinal obstruction in neonates. We treated four cases of syndromic Hirschsprung’s disease from January 2017 to January 2020 in a Tertiary care hospital. The biopsy specimens from spastic segment and colostomy or ileostomy sites were sent for evaluation of ganglion cells. Two different syndromes and one associated malformation and neoplasm were detected in patients of Hirschsprung’s disease during this 3-year case study.

Shanghai Fever: A Fatal Form of Pseudomonas Aeruginosa Enteric Disease.

Background: Outcome of pseudomonas enteric fever is unpredictable as multiple systemic lethal complications occur abruptly. Case characteristics: A 9-month-old girl with multiple ileal perforations, leukocoria, ecthyma gangrenosum, hemiplegia and a perforated ulcer in the soft palate. Blood culture suggested Pseudomonas aeruginosa infection. Operative repair of multiple ileal perforations and multidisciplinary management was provided. Outcome: On 10th post-operative day, patient succumbed to multiple organ dysfunction syndrome. Message: Early detection and management of complications of P. aeruginosa enteric disease is important.

Congenital epulis-a case report.

A full term newborn baby presented at the age of 2 days with a pedunculated mass arising from the mandibular gingiva and having difficulty in feeding. The mass was excised under general anaesthesia. Histopathology proved it to be a case of congenital epulis. The baby is doing well during follow-up one year after surgery.