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Colorectal Channel
Total colonic Hirschsprung disease: Ileostomy take down and ileoanal pull-through
Chapter 1 of 4 · Case-Based Learning
Case presentation
Case presentation and initial management
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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
The patient presented with neonatal obstruction and contrast enema was typical of total colonic Hirschsprung's.
All rectal and colonic biopsies showed no ganglion cells.
There were good ganglion cells at the ileum where the stoma was opened.
The patient did well, thrived, and needed no enteral or parenteral nutrition supplementation.
At the age of 1, the stool was noted to be thick in the ileostomy, so a pull through was offered.
The distal side has about 25 centimeters of ganglionic ileum.
All redo Hirschprung's cases are best handled prone.
Doing dissection in prone position in an untouched rectum helps understand the anatomy of a previously operated on rectum dissected out in the same way.
The dentate line is hidden under the pins and preserved.
Marking 0.5 centimeters from the anal verge preserves the anal canal.
There is a typical areolar plane in the full thickness Swenson plane.
An elliptical incision around the stomas makes the transverse incision easier to close at the end of the operation.
The downstream small bowel is very small in caliber.
The best orientation is with the small bowel limb coming down the right pelvis.
Marking a spot 4 centimeters below the superior aspect of the pubic bone confirms that the pull-through segment will comfortably reach the perineum.
The intact arcade will supply the distal ileal segment.
In the martini glass technique, you want to save the V part of the glass and ligate the stem.
The planned ileal segment is straight and has excellent blood supply after the martini glass technique.
The pull-through segment is under no tension.
A seromuscular stitch is placed from the bowel to the sphincters in 4 positions.
The next layer is mucosa of the ileum to mucosa just proximal to the preserved anal canal.