A twisted pull through.

B tight muscular cuff.

C anastomotic stricture.

D motility disorder.

E aganglionosis.

[/size]

A hypoplastic ganglion cells.

B absence of ganglion cells.

C small ganglia within the myenteric plexus.

D giant ganglia within the submucosal plexus.

E decreased acetylcholinesterase activity in the lamina propria.[/size]

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]

a Injection of botulinum toxin into the internal sphincter.

b Revision of the pull through.

c Biofeedback therapy.

d Twice daily dilations.

e Lateral internal anal sphincterotomy.[/size]

Edit: available without restrictions in next post

a Twice daily dilations.

b Injection of botulinum toxin into the internal sphincter.

c Revision of the pull through.

d Lateral internal anal sphincterotomy.

e Biofeedback therapy.[/size]

[size=20px]a Diverting ostomy

b Revision Duhamel

c Rectal irrigation/disimpaction

d Revision Swenson

e Revision Soave[/size]

Edit: available without restrictions

"The diagnosis of HD must be confirmed by an experienced, reliable pathologist with a rectal biopsy. This technique is best suited for short-segment disease, and ideally the patient would have a barium enema study demonstrating a distal transition zone. Longer segment disease is a relative contraindication for this surgical option, and these patients may be better served by a Duhamel procedure l. Prematurity is a relative contraindication, as these patients may not have fully mature ganglion cells and histologic evaluation of biopsies may therefore not be reliable. The patients are managed with rectal stimulation and irrigation until term. Strict contraindications include significant malnutrition, active enterocolitis, or massively dilated proximal bowel; these patients should undergo a staged procedure, with an initial colostomy and a definitive operation once the contraindication has resolved."

[center]by Dr Hussain kotawala

see in next post[/center]