Sphincter Reconstruction for Patients with Soiling after a Pull-Through for Hirschsprung Disease

We present a 10 year old boy with a prior pull-through for Hirschprung's disease. He suffered from fecal incontinence for many years, and when I first met him, after being referred from another institution, the exam showed a patulous anus and an absent dentate line. These anatomic problems result from an overstretching of the anal sphincters, and a dissection started too low during the initial procedure. To manage his incontinence, we gave him a Malone appendicostomy for anti-grade flushes. And he was able to be clean and socially continent, albeit mechanically. Now we present a novel technique for sphincter reconstruction, with the hope that he will improve his ability to squeeze the anus closed, and be able to have voluntary bowel movements. Here is a photo and an artistic diagram of what a normal anal canal looks like. Here you see him in prone position. Inspection of the anus shows a patulous anus and a missing dentate line. We performed a three-dimensional anorectal manometry, which confirmed objectively the absence of good sphincteric contraction, particularly on the anterior aspect. Here you see images of that 3D manometry. The operation begins in prone position. We place the lone starring and pins to provide circumferential exposure of the anal opening. These are placed just at the skin level, as there is no dentate line to preserve. The purple marks are placed at the mucocutaneous junction. Silk sutures are then placed in the bowel to provide for distributed tension for the dissection of the previous pull-through. We then start the dissection at the skin edge, trying to stay in the plane between the bowel wall and the surrounding sphincter muscle. The pins are replaced into this cut edge. The plane between the bowel and the muscle freeze up easily, as the muscle is not very adherent to the pull-through. You can see a nice areolar plane between the bowel and muscle, and see the sphincter muscle circumferentially. This is the external sphincter. Once the pull-through is dissected circumferentially, the surrounding external muscle is easily visualized. These are the muscles that need to be more firmly attached to the distal pull-through to provide adequate squeeze to close the anus. If the patient can detect the presence of stool in the anal canal, they will be able to close the anus in time to avoid an accident. We plan to have the patient practice this during their integrade flushes and anticipate they will get better and better at holding in their flush. Here we show the depth of dissection, which measures 3 to 3.5 centimeters. Deeper to this location is ischiectal fat. The muscle is now tacked to the bowel circumferentially. We are starting anteriorly, which was the most problematic area noted on the manometry. The prior suture is tagged to show the ideal location for the next suture. These are absorbable sutures for a viral from the serumuscular bowel layer to the muscle. These stitches are placed circumferentially. Once all the tacking is complete, the sutures are cut. We then suture the mucosal edge back to the skin circumferentially. You can now see the anus is more closed. Digital exam confirms that the anus is supple and easily distensible, but now compressed by the surrounding external sphincter. After a period of time with continued antigrade flushes, and with hopefully improvement in control, we anticipate trying to allow this patient to have their own bowel movements, and to demonstrate their own capacity for voluntary control. Repeat three-dimensional anorectal manometry confirmed objectively an improvement in the symmetry of the muscles around the pull-through, and in their increased tone.