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.

A neonatal intestinal obstruction with unusual presentation.

A two days old male child was admitted with features of neonatal intestinal obstruction. Straight x-ray abdomen showed distended small intestinal loops with few air fluid levels. After resuscitation abdomen was explored. There was distal ileal atresia with duplication cyst. Resection of atretic segment along with duplication cyst was performed and end to side ileo-ileal anastomosis with proximal ileostomy was done. Patient recovered uneventfully. Till now patient is doing well.

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.