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

The patient is a 10-year-old boy with a prior pull-through for Hirschsprung's disease who suffered from fecal incontinence for many years.

Physical exam showed a patulous anus and an absent dentate line.

These anatomic problems (patulous anus and absent dentate line) result from an overstretching of the anal sphincters and a dissection started too low during the initial procedure.

To manage his incontinence, a Malone appendicostomy was given for antegrade flushes.

The patient was able to be clean and socially continent, albeit mechanically, with the Malone appendicostomy.

The novel sphincter reconstruction technique aims to improve the patient's ability to squeeze the anus closed and enable voluntary bowel movements.

Three-dimensional anorectal manometry confirmed objectively the absence of good sphincteric contraction, particularly on the anterior aspect.

The operation is performed in prone position.

Lone Star retractor pins are placed just at the skin level, as there is no dentate line to preserve.

Dissection is started at the skin edge, staying in the plane between the bowel wall and the surrounding sphincter muscle.

The plane between the bowel and the muscle frees up easily, as the muscle is not very adherent to the pull-through.

A nice areolar plane is visible between the bowel and muscle, and the external sphincter muscle is seen circumferentially.

The surrounding external sphincter muscles 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.

The plan is for the patient to practice voluntary squeeze during their antegrade flushes, with the anticipation they will get better and better at holding in their flush.

The depth of dissection measures 3 to 3.5 centimeters; deeper to this location is ischiorectal fat.

The muscle is tacked to the bowel circumferentially, starting anteriorly, which was the most problematic area noted on the manometry.

Absorbable sutures are placed from the seromuscular bowel layer to the muscle.

After tacking is complete, the mucosal edge is sutured back to the skin circumferentially.

Post-operatively, the anus is more closed; digital exam confirms the anus is supple and easily distensible, but now compressed by the surrounding external sphincter.

After a period of time with continued antegrade flushes and hopefully improvement in control, the plan is to allow the patient to have their own bowel movements and demonstrate 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.