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A two-year old boy with trisomy 21 underwent a leveling colostomy for aganglionosis as a newborn. A Duhamel procedure was performed at seven months of age. He has been hospitalized on three subsequent occasions for increasingly severe enterocolitis. His abdomen is chronically distended and his parents have been diligent with rectal irrigations at home. A digital rectal exam is normal. Plain radiographs and a contrast enema are obtained.

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[size=20px]Anorectal manometry demonstrates poor relaxation of the internal anal sphincter.

 

The best next step in this postoperative Hirschsprung patient is

 

A addition of metronidazole to the rectal irrigation and oral probiotics.

 

B rectal biopsy and intrasphinteric botulinum toxin (Botox®) injection.

 

C internal anal sphincterotomy.

 

D anal myectomy.

 

E diverting colostomy.[/size]

B

B

correct

 

Botulinum toxin acts by blocking acetylcholine release at the neuromuscular junction. It produces a flaccid paralysis that lasts for three to six months. Although there is limited literature in children, its use in treating anal hypertonicity following surgical treatment of Hirschsprung disease, internal anal sphincter achalasia and chronic constipation shows considerable promise.

I Advice a Rectal Biopsy at the Site of Anastomosis after that Botox can be sed,Botox have a Tranzit effect neededto be injected again if needed.

B

B

The biopsy checks for residual aganglionosis or a transition-zone pull-through. Botox temporarily relaxes the internal anal sphincter to improve bowel emptying, addressing the outlet resistance suggested by manometry