Hirschsprung’s Disease Causes And Diagnosis

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Hirschsprung's Disease
Hirschsprung’s Disease

Hirschsprung’s Disease( Congenital Aganglionic Megacolon )

Hirschsprung’s Disease: Is congenital anomaly that results in mechanical obstruction from inadequate motility of part of the intestine.

Etiology :

  • Arrest in embryological development affecting the migration of parasympathetic nerves of the intestine ( prior to the 12th week of gestation).
  • Unknown cause.
  • May be familial.

Clinical manifestations:

{ vary depending on a degree of involved bowel}.

  • Appearing at birth or within first weeks of life.
  • Failure to pass meconium within 24- 48 hours after birth.
  • Vomiting ( bile-stained or fecal).
  • Abdominal distention.
  • Reluctance to ingest fluids.
  • Overflow – type diarrhea.

Older children :

  • History may reveal obstipation at birth.
  • Abdominal distention.
  • Constipation ( relieved temporarily with enema).
  • Ribbon-like, foul – smelling stool.
  • Inadequate weight again.
  • Fecal masses may be palpable.

Complications :

  • Prior to surgery: Enterocolitis (the major cause of death), water intoxication ( enemas), perforation.
  • Post-op: Enterocolitis, a leak of an anastomosis, pelvic abscess.
  • Colostomy: hemorrhage, shock, and infection.

Diagnostic evaluations :

  • Rectal examination : no fecal material .
  • Barium enema: narrow intestine proximal to the anus, dilated intestine proximal to narrow segment.
  • Rectal biopsy: absence or reduced number of ganglion nerve cells.

Treatment :

  • Initially: colostomy or ileostomy to decompress the intestine rest normal bowel.
  • Definitive surgery: abdominal pull- through, at 9-12 months or 7-10 kg body wt.

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