Total colonic Hirschsprung disease: Ileostomy take down and ileoanal pull-through

Total colonic Hirschrung's disease, ileostomy takedown, and ileoanal pull through. We are going to show you a case of a patient with total colonic Hirschprung's. The patient presented with neonatal obstruction, and a contrast enema was typical of total colonic Hirschprungs, and they underwent an ileostomy and colonic mapping. All biopsies, rectal and colonic, 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. Here you see the double barrel ileostomy. The functional side is the majority of the ileum, and the distal side has about 25 centimeters of a ganglionic ileum. After a total body preparation, we begin the operation in prone position. I like to do a transanal prone rectal dissection in such a case. Because I know that all redo Hirschprung's cases are best handled prone. So any opportunity that I have to do a dissection with this approach in prone position in an untouched rectum, I take as it helps understand the anatomy of a previously operated on rectum dissected out in the same way. I place the lone star pins carefully to show and to preserve the dentate line. You see how we have hidden the dentate line under the pins. I now mark 0.5 centimeters from the anal verge, which preserves the anal canal. And I cut this circle. And then place sutures full thickness in the rectal wall. We now look for the full thickness Swenson plane. There is a typical areolar plane one can see. It is helpful at this stage to move the pins again and fully retract back the anal canal. The mixit or clamp helps facilitate this dissection. And now I tie off the distal rectum so there won't be any spillage when we enter the abdomen and also tie off the silk sutures. And we turn the patient supine. I take down both limbs of the ileostomy. I like to make an elliptical incision around the stomas, which makes this transverse incision easier to close at the end of the operation. We're stapling off the ends of both limbs of small bowel. You can see the downstream small bowel, which is very small in caliber. And now I'm removing the a ganglionic colon. I run the small bowel to be sure it is properly oriented as we set up for the pull-through. The best orientation seems to be with the small bowel limb coming down the right pelvis. I now mark a spot 4 centimeters below the superior aspect of the pubic bone. This location confirms to me that the pull-through segment will comfortably reach the perineum. And now we are setting up for the illuanal pull through. You can see the intact arcade which will supply the distal ileal segment. And I now draw a martini glass. You want to save the V part of the glass and ligate the stem. And the planned ileal segment is now straight and has excellent blood supply. And here we have pulled through the ileum and reconfirmed its orientation to be sure I haven't twisted it. The pull-through segment is under no tension. I place a serum muscular stitch from the bowel to the sphincters in 4 positions. And then the next layer is mucosa of the ileum to mucosa just proximal to the preserved anal canal. I moved the pins back to show the anal canal. And complete the ileoanal anastomosis.