Authors : Jisha Yohannan

Volume/Issue : Volume 11 - 2026, Issue 4 - April

Google Scholar : https://tinyurl.com/bdezuvxz

Scribd : https://tinyurl.com/yy8n27pv

DOI : https://doi.org/10.38124/ijisrt/26apr1050

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Abstract : Hirschsprung disease (HD), also known as congenital aganglionic megacolon, is a developmental disorder of the enteric nervous system characterized by the congenital absence of parasympathetic ganglion cells in the myenteric (Auerbach) and submucosal (Meissner) plexuses of the distal colon and rectum. This absence results in functional intestinal obstruction and extreme proximal colonic dilatation. The recto-sigmoid colon is the most frequently affected segment. HD has a well-established predilection for the male sex. Clinical presentation varies significantly by age of onset: neonates typically exhibit delayed meconium passage, abdominal distention, and bilious vomiting, while older children present with chronic constipation, failure to thrive, and progressive abdominal distention. Diagnosis is confirmed through a combination of contrast enema studies, anorectal manometry, and definitive rectal biopsy demonstrating the absence of ganglion cells. Surgical management includes initial colostomy for intestinal decompression, followed by definitive resection of the aganglionic segment with primary anastomosis. Nursing assessment, perioperative care, and family education are central pillars of comprehensive management.

Keywords : Hirschsprung Disease, Congenital Aganglionic Megacolon, Paediatric Nursing, Enteric Nervous System, Colostomy, Rectal Biopsy, Neonatal Obstruction.

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