Please enable JavaScript.
Coggle requires JavaScript to display documents.
Hirschsprung's Disease (Congenital Megacolon): - Coggle Diagram
Hirschsprung's Disease (Congenital Megacolon):
Definition & Cause:
Hirschsprung's disease is a congenital disorder caused by the absence of ganglion cells in the intestinal wall (myenteric and submucosal plexuses), leading to functional obstruction. The aganglionic segment fails to relax, causing proximal bowel dilation.
ncidence & Affected Segments:
Male predominance (4:1 male-to-female ratio).
Most common site: rectosigmoid region.
Variants: ultra-short, short (most common), long, very long segments, or total colonic aganglionosis.
Pathology:
Three distinct zones:
Narrow distal segment (aganglionic, contracted).
Transitional zone (hypoganglionic).
Dilated proximal segment (normal, hypertrophied due to chronic obstruction).
Complications:
Enterocolitis (fever, distension, septicemia; life-threatening).
Perforation (rigid abdomen, air under diaphragm on X-ray).
Diagnostic Investigations:
Clinical Presentation:
Neonates:
Delayed passage of meconium (>48 hours).
Abdominal distension, bilious vomiting, constipation.
Infants/Children:
Chronic constipation, abdominal distension, foul-smelling stool.
Empty rectal vault on exam, with explosive stool expulsion after withdrawal.
Imaging:
Barium enema: Shows narrow distal segment, transitional cone, and dilated proximal colon.
Plain X-ray: Distension, air-fluid levels, or free air (perforation).
Functional Tests:
Rectal manometry: Absent rectoanal inhibitory reflex (RAIR).
Gold Standard: Rectal biopsy (histologic confirmation of aganglionosis).
Pre-Operative Management:
NG decompression, NPO, IV fluids/antibiotics, electrolyte correction.
Enemas to decompress bowel (avoided in enterocolitis).
Surgical Treatment:
Neonates:
Uncomplicated: Primary transanal endorectal pull-through (TEPT).
Complicated (e.g., enterocolitis): Colostomy (transverse/leveling) followed by definitive surgery.
Older Infants/Children:
TEPT, Soave’s pull-through, Duhamel procedure, or Swenson’s operation.
Laparoscopic options (minimally invasive).
Postoperative Considerations:
Monitor for anastomotic leaks, strictures, or recurrent obstruction.
Enterocolitis risk persists post-surgery; requires prompt treatment.
Early diagnosis (via rectal biopsy) prevents life-threatening enterocolitis.
Surgery is curative, with techniques tailored to age/complications.
Lifelong follow-up for bowel function and complications.