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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
05:45

Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5

Published on: April 26, 2019

Mortality due to constipation and short-segment Hirschsprung's disease

S J Singh1, S Arbuckle, D Little

  • 1Department of Paediatric Surgery, University Hospital, Queen's Medical Centre, Nottingham NG7 2UH, UK. shailinder.singh@mail.qmcuh-tr.trent.nhs.uk

Pediatric Surgery International
|October 19, 2004
PubMed

Insights

Severe constipation caused a fatal intestinal obstruction in a 12-year-old. Postmortem revealed no enteric nervous system abnormalities. Short-segment Hirschsprung

Area of Science:

  • Pediatric Gastroenterology
  • Clinical Pathology
  • Medical Case Reports

Background:

  • Constipation is a common pediatric issue.
  • Intestinal obstruction is a rare but serious complication.
  • Hirschsprung's disease is a congenital disorder affecting the large intestine.

Observation:

  • A 12-year-old boy with chronic constipation presented with intestinal obstruction.
  • The patient died post-laparotomy.
  • A neonate died from Hirschsprung's disease, specifically short-segment aganglionosis.

Findings:

  • Postmortem examination of the 12-year-old showed no enteric nervous system abnormalities, suggesting idiopathic constipation as the cause of obstruction.
  • Short-segment Hirschsprung's disease was identified postmortem in the neonate.
  • Enterocolitis is a rapid and potentially fatal complication of short-segment Hirschsprung's disease.

Implications:

  • This case highlights the potential for severe outcomes from idiopathic constipation in children.
  • Postmortem diagnosis of Hirschsprung's disease is feasible.
  • Prompt recognition and management of enterocolitis in Hirschsprung's disease are critical for survival.

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