Novel surgical procedure for sphincter reconstruction shows promising results in children with Hirschsprung disease

Surgeons in the Division of Colorectal and Pelvic Reconstruction at Children's National Hospital have pioneered a new surgical procedure that successfully addresses a common lingering challenge-incontinence-faced by some families with young children who have undergone surgery for Hirschsprung disease.

The details of the novel surgical procedure appear in the Journal of Pediatric Surgery.

"The goal of surgery for Hirschsprung disease is to give a child a chance to have normal bowel function, which plays a significant role in their quality of life," says Dr. Levitt, who served as senior author on the study. "But sometimes after a child receives their pull-through operation-the first surgical step to treat Hirschsprung-they continue to be incontinent. This novel follow-up procedure is offering these kids and their families new hope for a more normal life."

Hirschsprung disease is a congenital condition that affects one in 5,000 children each year. Children with this condition develop a host of health problems including intestinal blockage and severe constipation, caused by the inability of the colon, or large intestine, to work properly. In such children with Hirschsprung disease pull-through procedure is the best treatment. The procedure removes the portion of the intestine that does not function so that the child can regain control.

Unfortunately after the procedure, some children continue to experience incontinence that requires routine enemas for their entire lives. Before now there was no solution to this problem.

The new pilot study shows long-term outcomes for seven patients between the ages of 2 and 18 who underwent the novel technical approach called sphincter reconstruction. Each of the seven were diagnosed with an anatomic or physical issue contributing to their incontinence. It was during a repair procedure for the first patient that Dr. Levitt and his team had the idea of a technique for sphincter reconstruction. After that, six more patients with similar anatomy were offered the procedure.

Four of the six now have normal bowel function. They reported sleeping better, higher productivity, the ability to participate in sports and be away from home with confidence. The remaining two have behavioral conditions that have delayed their progress but are on their way to similar success.

"Parents and caregivers have told us repeatedly about how much enemas negatively impact their family quality of life," says Dr. Levitt. "That's what makes this exciting-this validated surgical approach offers new hope for families who previously thought their child would face a lifetime of issues that would limit their freedom. Now, we finally have something to offer."

Reference:

Elizaveta Bokova, Elise McKenna, Wilfried Krois, Carlos A. Reck, Tamador Al-Shamaileh, Shimon E. Jacobs, Laura Tiusaba, Teresa L. Russell, Anil Darbari, Christina Feng, Andrea T. Badillo, Marc A. Levitt, Reconstructing the Anal Sphincters to Reverse Iatrogenic Overstretching Following a Pull-through for Hirschsprung Disease. One-Year Outcomes, Journal of Pediatric Surgery, 2022, https://doi.org/10.1016/j.jpedsurg.2022.10.052