Surgical Techniques for Long-Segment Hirschsprung Disease

Hirschsprung disease is a congenital condition in which children are born without ganglion cells in all or part of their colon. As a result, the smooth muscle within those areas retains a spastic status, creating a functional bowel obstruction, among other symptoms. There are three variations of Hirschsprung disease, two of which are treatable with a surgical technique called a pull-through procedure. The third, called long-segment Hirschsprung disease, is rarer and for some patients, it requires specialized techniques.

Children’s Hospital Colorado colorectal surgeon Luis De la Torre, MD, has mastered both the Deloyers and Turnbull procedures to treat long-segment Hirschsprung disease effectively. In a recently published retrospective study, he explains these surgical maneuvers and shares their outcomes to disseminate this life-saving knowledge to surgeons worldwide.

Developing expertise in Hirschsprung disease surgery

Most cases of Hirschsprung disease affect the rectum or proximal sigmoid, and another group is born without ganglion cells in the entire colon. Long-segment Hirschsprung disease falls somewhere in between, involving a large part of the colon and the rectum. As a result, the typical pull-through can cause severe and persistent issues with bowel obstruction after surgery and for the remainder of the patient’s life. Because of this, this condition necessitates a different surgical approach. These patients are often misdiagnosed because of the rarity of their condition and frequently experience an increased risk of death due to complications from misapplied surgical techniques.

While most surgeons are lucky to treat one child with Hirschsprung disease in five years, Dr. De la Torre has spent 35 years developing expertise in the condition. In fact, between April 2017 and April 2024 alone, his team operated on 13 patients with long-segment Hirschsprung disease.

“At the beginning of my career, we didn't know about these issues,” he explains. “But we realized that we have this group of patients where we can preserve at least 35% to 45% of the colon to preserve some large bowel. We found that in adults with colonic cancer, colorectal surgeons developed these techniques for this problem. So, we went back and read many things about colorectal surgery in adults and took these little pieces from adult surgery to help our kids.”

When faced with a case of long-segment Hirschsprung, the team first evaluates how much of the colon is involved. In patients with a significant portion of the colon affected, Dr. De la Torre uses the Turnbull procedure, in which he passes the healthy section of the colon through a window created in the mesentery, the connective membrane that holds the intestines in place. He then connects it to the anus.

When there isn’t enough healthy colon to complete the Turnbull, Dr. De la Torre turns to the more complex Deloyers procedure. Here, the surgeon frees the right colon and rotates it 180 degrees before connecting the colon to the anus.

Improving global understanding of Hirschsprung disease treatment

By adapting these procedures and adapting them to fit children’s bodies, Dr. De la Torre has helped establish Children’s Colorado as a leader in Hirschsprung disease treatment. However, not all kids can make the trip to Colorado, and not all surgeons know about this referral option. This study conveys the impacts of performing these procedures in this patient population and explains how to complete them, allowing doctors to appropriately treat kids in need.

Still, because so few patients are diagnosed with long-segment Hirschsprung disease, experience in performing these two procedures is rare. Without surgeons with this highly specialized expertise, kids often are faced with surgical complications that require further procedures and result in a host of challenges. This recent study aims to lessen the problem. Still, Dr. De la Torre also hopes it will raise awareness about the importance of establishing centralized programs dedicated to building expertise in the condition. That way, doctors would know exactly where to send these complex cases to ensure patients are in expert hands.

Citation

1. Zornoza, M et al. “Surgical maneuvers for long-segment Hirschsprung pull-through in unique patients.” Pediatric surgery international vol. 40,1 180. 8 Jul. 2024, doi:10.1007/s00383-024-05767-0.