Total colonic Hirschsprung disease: Ileostomy take down and ileoanal pull-through
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Patient presented with neonatal obstruction
Contrast enema was typical of total colonic Hirschsprung disease
All rectal and colonic biopsies showed no ganglion cells
Good ganglion cells were present at the ileum where the stoma was opened
Patient thrived and needed no enteral or parenteral nutrition supplementation
At age 1, stool was noted to be thick in the ileostomy, prompting pull-through
The distal ileostomy limb had about 25 centimeters of ganglionic ileum
All redo Hirschsprung cases are best handled prone
Transanal prone rectal dissection in untouched rectum helps understand anatomy of previously operated rectums
Dentate line should be preserved and hidden under retraction pins
Marking 0.5 centimeters from anal verge preserves the anal canal
There is a typical areolar plane in the full-thickness Swenson dissection
Tying off the distal rectum prevents spillage when entering the abdomen
Elliptical incision around stomas makes transverse incision easier to close
Downstream small bowel is very small in caliber after prolonged diversion
Best orientation for pull-through is with small bowel limb coming down the right pelvis
Marking 4 centimeters below superior aspect of pubic bone confirms pull-through segment will comfortably reach perineum
Intact vascular arcade supplies the distal ileal segment
Martini glass technique preserves V-shaped vessels and ligates the stem
Pull-through segment must be under no tension
Anastomosis uses seromuscular stitches from bowel to sphincters in 4 positions
Second anastomotic layer is mucosa of ileum to mucosa proximal to preserved anal canal