Hirschsprung Disease

Posted by e-Medical PPT Tuesday, March 8, 2011
Hirschsprung's disease (HD), or congenital aganglionic megacolon, involves an aganglionic section of bowel(the normal enteric nerves are absent) that starts at the anus and progresses upwards. The length of bowel that is affected varies but seldom stretches for more than about 30 cm. It arises when the ganglion cells fail to develop and mature correctly. The result is a section of bowel that is essentially paralyzed.
With an incidence of 1/5000 births, the most cited feature is absence of ganglion cells: notably in males, 75% have none in the recto-sigmoid, and 8% with none in the entire colon. The enlarged section of the bowel is found proximally, while the narrowed, aganglionic section is found distally; the absence of ganglion cells results in a persistent over-stimulation of nerves within the affected region, resulting in contraction.
1) Delayed passage of meconium.
2) Abdominal distension.
3) Constipation.
Definitive diagnosis is made by suction biopsy of the distally narrowed segment.Diagnostic techniques involve anorectal manometry,barium enema, and rectal biopsy.Radiologic findings may also assist with diagnosis.

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