Hirschsprung's Disease: a Clinical and Pathologic Study in Iranian Constipated Children

Maryam Monajemzadeh1, Mehdi Kalantari, Bahareh Yaghmai

  • 1Department of Pathology, Tehran University of Medical Sciences, Tehran, Iran ; Pediatrics Center of Excellence, Children's Medical Center, Tehran, Iran.

Insights

Hirschsprung

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Developmental Biology

Background:

  • Hirschsprung's disease (HD) is a congenital condition characterized by the absence of ganglion cells in the bowel wall, leading to functional obstruction.
  • Chronic constipation in children can be a symptom of HD, necessitating diagnostic evaluation.

Purpose of the Study:

  • To determine the optimal age for diagnosing Hirschsprung's disease (HD) in constipated children.
  • To reduce the incidence of unnecessary rectal biopsies by identifying age-specific likelihoods of HD.

Main Methods:

  • A retrospective review of 172 rectal biopsies performed on 168 children with chronic constipation over five years.
  • Analysis of patient age at biopsy, age of symptom onset, and presence of HD.

Main Results:

  • Of 168 children, 127 (75%) were diagnosed with HD.
  • The mean age of children with HD at biopsy was 18 months, significantly younger than the mean age of 39 months for all constipated children.
  • Constipation onset during the neonatal period was strongly associated with HD (91% of cases).

Conclusions:

  • Constipation presenting after the neonatal period is unlikely to be indicative of Hirschsprung's disease.
  • Delayed meconium passage in neonates is a critical early sign of HD, guiding diagnostic decisions.
Abstract

Related Concept Videos

Assessment of the Rectum and Anus01:25

Assessment of the Rectum and Anus

Evaluating the rectum and anus plays a crucial role in conducting a thorough physical examination of the gastrointestinal system. Although it may be uncomfortable and often embarrassing for the patient, it holds immense diagnostic value, particularly in detecting gastrointestinal diseases and abnormalities. This guide will explain how to perform this assessment using inspection and palpation methods.
Rectal Inspection
Begin by inspecting the perianal and anal areas for color, texture, rashes,...
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation01:30

Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation

Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation
Irritable Bowel Syndrome (IBS) is classified into subtypes based on the predominant bowel habits as determined by the Bristol Stool Form Scale (BSFS). The subtypes are:
Intestinal Obstruction II: Pathophysiology01:07

Intestinal Obstruction II: Pathophysiology

Intestinal obstruction triggers a series of physiological responses, starting with gas and fluid accumulation in the bowel segment proximal to the obstruction, leading to distension. This distended intestine compresses the diaphragm, hindering lung expansion and potentially leading to reduced respiratory effort, atelectasis, and pneumonia.To overcome the blockage, the gut intensifies contractions, causing colicky abdominal pain, nausea, and vomiting, which reduces fluid and food intake and...
Intestinal Obstruction I: Introduction01:29

Intestinal Obstruction I: Introduction

Intestinal obstruction is a partial or complete blockage of the small or large intestine that disrupts the normal flow of intestinal contents through the lumen. This interruption impairs digestion, absorption, and fluid balance, and may lead to serious complications if not treated promptly.Mechanical ObstructionMechanical obstruction occurs when a physical blockage prevents intestinal contents from passing, arising from within the lumen or the bowel wall, or from external compression.Adhesions,...
Inflammatory Bowel Disease V: Surgical Management01:21

Inflammatory Bowel Disease V: Surgical Management

Surgical interventions for inflammatory bowel disease (IBD), which includes ulcerative colitis and Crohn's disease, are essential in managing symptoms and addressing complications. The selection of surgical procedures is contingent upon the specific conditions and complications that stem from these illnesses.
Here are some common surgical interventions for IBD: