Physiological Aspects of Gastrointestinal Disorders

Hirschsprung's Disease: Pathophysiology and Enteric Nervous System Involvement

Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the distal colon, leading to functional obstruction.

Pathophysiology

The absence of ganglion cells in the enteric nervous system results in a lack of coordinated motility, causing chronic constipation and megacolon.

Clinical Presentation

• Failure to pass meconium in neonates
• Abdominal distension
• Chronic constipation
• Enterocolitis risk

Diagnostic Approach

Definitive diagnosis requires rectal biopsy confirming the absence of ganglion cells. Additional tests include anorectal manometry and contrast enema.

Management

Surgical intervention via pull-through procedures is the primary treatment. Postoperative care focuses on preventing complications like enterocolitis.