Multidisciplinary Motility Team Guides Care for Child with Hirschsprung Disease

Saul was born in 2013 at 32 weeks gestation and was diagnosed with trisomy 21 (Down syndrome). He began having explosive bowel movements during his six-week stay in the neonatal intensive care unit. Extensive testing led to a diagnosis of Hirschsprung disease, followed by a pull-through surgery at about 10 months of age. This was followed by multiple bouts of enterocolitis, significant difficulty stooling, abdominal pain and bloating. Concerned about Saul’s symptoms and unsure about the best course of action, Saul’s doctor recommended that his parents travel with him from Texas to see pediatric colorectal surgeon Jason Frischer, MD, director of Cincinnati Children’s Colorectal Center.

During the pre-visit planning process, Frischer considered redoing Saul’s pull-through procedure. But Saul’s colon was so inflamed, and his enterocolitis symptoms so serious, that Frischer instead performed an ileostomy. This was followed by a successful redo procedure three months later and a third surgery to close the ileostomy.

Saul improved, but by spring 2016 he was again experiencing explosive bowel movements. Frischer, unable to find anything anatomical to explain Saul’s symptoms, decided to share the case with the medical center’s multidisciplinary motility team, which had been established the previous year.

The multidisciplinary motility team—which includes Frischer, four other pediatric colorectal surgeons, four pediatric neurogastroenterologists and several nurses—meets weekly to discuss patients with complex conditions, such as Hirschsprung disease and anorectal malformation, who have ongoing issues with diarrhea and constipation. Together, the team develops a unified treatment plan for each patient that may include:

• Motility testing, such as colonic manometry
• Botox injections
• Additional surgical procedures
• Pelvic floor physical therapy
• Participation in the Colorectal Center’s Bowel Management Program
• Additional therapies from nutrition or behavioral medicine specialists
• Consults from other specialists, such as pediatric urologists and obstetrician/gynecologists

Cincinnati Children’s offers four motility clinics a month, where patients and families can see the entire care team in one place rather than make a separate appointment with each specialist. When a child needs to undergo a procedure, have testing or go through bowel management, appointments are scheduled in a coordinated way to streamline care.

“For some patients, surgical repair is beneficial but does not resolve certain symptoms, such as persistent constipation and diarrhea,” says Khalil El-Chammas, MD, a neurogastroenterologist who is part of the multidisciplinary motility team. “In Saul’s case, the team decided we needed more information about what his colon was doing. So he underwent high-resolution colonic manometry with an upper endoscopy and colonoscopy. Fortunately, the test showed normal colon motility through the entire colon, which meant we could essentially rule out total colonic aganglionosis. He also underwent anal Botox injection to help optimize his bowel movements. Then the team met to discuss next steps.”

Confident that Saul did not require a surgical repair, the team recommended that he go through the Bowel Management Program. Saul completed the program that week and returned home with a treatment plan that included laxatives and colonic irrigations. Since then, Saul has transitioned to daily enemas to further improve his bowel control. And in July 2019, he had a Malone procedure to simplify his colonic irrigations.

“We don’t know why some children who have Hirschsprung disease still experience symptoms after a successful pull-through procedure,” Frischer says. “Additional surgery may be needed, but doing more surgical repairs in an area where there’s already scar tissue and a lot of structures in a closed space, you run the risk of injuring nerves and other urinary tract structures. Taking a team approach with Saul meant that we could come up with non-operative ways to help him achieve continence and a much better quality of life.”