Treatment

Objective: definitive treatment to remove the affected segment of the colon and bring the normal ganglionic intestinal ends together

Procedures

• Total transanal endorectal pull-through: preferred method that can be done in one stage [6]

• Abdominoperineal pull-through (Soave procedure): traditionally performed in two stages:

  • First stage: diverting colostomy to relieve the dilated bowel .

  • Second stage

    • Resection of the aganglionic segment

    • Anastomoses of the normal ganglionic colon segment to either the distal modified rectum or normal (unmodified) distal rectum

    • Preservation of internal anal sphincter function is of the utmost importance.

Objectives

• Maintain normal fluid and electrolyte balance

• Reduction of bowel distention

• Prevent and manage complications

Initial medical management

• Intravenous fluid resuscitation

• Nasogastric decompression

• Intravenous antibiotics, if indicated

• Colonic irrigation may be required.

Postoperative medical management

• Routine colonic irrigation

• Prophylactic antibiotic therapy

Hirschsprung-associated enterocolitis [8]

• Symptoms of toxic megacolon (e.g., abdominal pain, fever, foul-smelling and/or bloody diarrhea)

• If not treated early, sepsis, transmural intestinal necrosis, and perforation are possible.

• Management: IV fluid resuscitation, broad-spectrum IV antibiotics (anaerobic and aerobic coverage), rectal irrigations, with possible colostomy

Fecal incontinence

Urinary dysfunction

Erectile dysfunction

Peritonitis due to bowel perforation

Good prognosis with early treatment

Higher mortality associated with younger age, length of aganglionosis, and preoperative enterocolitis