Hirschsprung's Disease ( Congenital A ganglionic Megecolon )




Is congenital anomaly that results in mechanical obstruction from inadequate motility of part of the intestine .


Congenital A ganglionic Megecolon
Hirschsprung's Disease

Hirschsprung's Disease

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 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 temporary with enema ) .

- Ribbon like , foul - smelling stool .

- Inadequate weight again .

- Fecal masses may be palpable .


Complications :


- Prior to surgery : Enterocolitis ( major cause of death ) , water intoxication ( enemas ) , perforation .

- Post-op : Enterocolitis , leak of anastomosis , pelvic abscess .

- Colostomy : hemorrhage , shock , and infection .

- Diagnostic evaluations :

- Rectal examination : no fecal material .

- Barium enema : narrow intestine proximal to 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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