Definition: Hirschsprung’s disease is a congenital condition characterized by the absence of ganglion cells in the distal colon and rectum, leading to a functional obstruction.
Types:
Classic Hirschsprung’s disease: Most common type, characterized by the absence of ganglion cells in the distal colon and rectum.
Ultra-short-segment Hirschsprung’s disease: Characterized by the absence of ganglion cells in a very short segment of the rectum or anal canal.
Total colonic aganglionosis: A rare condition where the entire colon lacks ganglion cells.
Overlapping Hirschsprung’s disease: Characterized by a mixture of ganglionic and aganglionic segments in the colon and rectum.
Causes: The exact cause of Hirschsprung’s disease is unknown, but it is believed to result from a genetic mutation or a combination of genetic and environmental factors.
Clinical features:
Bowel obstruction: The most common symptom, characterized by abdominal distension, vomiting, and constipation.
Diarrhea: May occur in patients with a short-segment Hirschsprung’s disease.
Intestinal perforation: A life-threatening complication that can occur in severe cases.
Investigations:
Barium enema: A diagnostic imaging test that can help identify the absence of ganglion cells in the distal colon and rectum.
Rectal biopsy: A diagnostic test that involves removing a small sample of tissue from the rectum for examination under a microscope.
Complications:
Intestinal perforation: A life-threatening complication that can occur in severe cases.
Chronic constipation: May occur in patients with untreated Hirschsprung’s disease.
Intestinal obstruction: May occur in patients with untreated Hirschsprung’s disease.
Management:
Surgical resection: The primary treatment for Hirschsprung’s disease, involves removing the aganglionic segment of the colon and rectum.
Colostomy: May be performed in patients with a life-threatening intestinal obstruction or in cases where surgical resection is not possible.
Treatment:
Surgery: The primary treatment for Hirschsprung’s disease, involves removing the aganglionic segment of the colon and rectum.
Medications: May be prescribed to manage symptoms such as constipation or diarrhea.
Blood supply:
Superior rectal artery: Supplies blood to the rectum and sigmoid colon.
Middle rectal artery: Supplies blood to the rectum and sigmoid colon.
Inferior rectal artery: Supplies blood to the anal canal.
Nerve supply:
Pudendal nerve: Supplies nerve fibers to the anal sphincter and the external anal sphincter.
Hypogastric nerve: Supplies nerve fibers to the rectum and sigmoid colon.
Clinical anatomy:
Anal canal: The terminal portion of the large intestine.
Rectum: The final portion of the large intestine.
Sigmoid colon: The part of the large intestine that connects the descending colon to the rectum.
Homoeopathic therapeutic medicines:
Calcarea phosphorica: Indications include constipation, abdominal distension, and bowel obstruction.
Natrum muriaticum: Indications include diarrhea, abdominal cramps, and intestinal obstruction.
Lycopodium clavatum: Indications include bowel obstruction, abdominal distension, and constipation.
Sulphur: Indications include diarrhea, abdominal cramps, and intestinal obstruction.
Arsenicum album: Indications include diarrhea, abdominal cramps, and intestinal obstruction.