Baby with Hirschsprung thrives after expert pediatric surgical care at Inova L.J. Murphy Children’s Hospital

After a healthy, uncomplicated pregnancy, Lauren welcomed her second child, a baby boy named Jack, in June 2023 at Inova Loudoun Hospital. All seemed fine, but after two days had elapsed without little Jack passing any stool, the neonatology team at Inova Loudoun Hospital transferred Jack to Inova L.J. Murphy Children’s Hospital, home to Northern Virginia’s only Level IV NICU, for evaluation.

Jack had Hirschsprung disease, a congenital condition affecting about one in every 5,000 live births. Babies with Hirschsprung are missing ganglion cells in some or all of their colon, small intestine and/or rectum. Without those nerve cells, stool cannot move through the affected portion of the intestinal tract. While about 80% of children have short-segment Hirschsprung, Jack was in the unfortunate minority. He had long-segment Hirschsprung affecting three-quarters of his colon.

Pediatric general and thoracic surgeon Stephen Kim, MD, Medical Director of Children’s Surgery at Inova Fairfax Medical Campus and Inova L.J. Murphy Children’s Hospital and Chief of Pediatric Surgical Specialties for Inova, handled Jack’s surgical care. “Based on early studies, Jack seemed to have a short-segment Hirschsprung disease. So, the initial surgery involved a diagnostic laparoscopy and getting biopsies along the different segments of the intestine to localize where the transition between abnormal and normal intestine was,” Dr. Kim said. “Unexpectedly, during the first surgery, the biopsies showed absence of ganglion cells in a much longer segment of intestine. I elected to perform a diverting ileostomy and wait for the final pathology.”

After recovering from the first surgery, Jack and his family went home, where he grew and developed with the addition of an ostomy bag. The initial ileostomy had relieved the obstruction and allowed Jack to feed and grow. The Isler family was grateful for Dr. Kim’s extensive experience with Hirschsprung disease and the pediatric colorectal surgeries needed to correct it.

“We love Dr. Kim, and we formed a great relationship with him over the course of Jack’s three surgeries,” Lauren said. “He literally knows Jack inside and out. Having a baby who needs surgery is next-level scary, but he really helped our family through it. He always remained professional, but you could tell he cared about our family, and we trusted him with our son.”

In December 2023, Jack was ready for his pull-through surgery to remove the nonfunctioning portion of his colon. Jack had a Swenson pull-through, which is a technique that attaches the normally functioning part of the colon to the anus, creating a new anal canal. Dr. Kim performed the procedure laparoscopically and transanally, avoiding a large abdominal incision.

“Jack’s case was a little bit more complicated because this was a long-segment Hirschsprung disease,” Dr. Kim said. “He had to have essentially all of his colon removed, in addition to his rectum and sigmoid. We then had to mobilize the more proximal intestine to bring it down into the pelvis and to provide a new channel down to the anus.”

The technical challenge of reconnecting Jack’s colon and intestine without impeding them was significant. During fluoroscopy between surgeries two and three, a radiologist remarked that Jack’s intestine “looked like a garlic knot,” Lauren remembered.

“At Inova L.J. Murphy Children’s Hospital, we have a full-service, multispecialty approach. Not only do we have pediatric surgery as well as pediatric anesthesia, radiology and pathology, all of which are crucial for Hirschsprung disease, but we also have family-centered care. Our nurses, case managers, child life and palliative care all provide support for our families when their children are at their most vulnerable,” Dr. Kim said.

“The child life team for our stays on the pediatric floor after Jack’s surgeries was amazing. They helped distract Jack when he was in pain, and they’d bring new toys every day to switch things up in his crib,” Lauren said.

Jack’s third and final surgery in March 2024 was to close the ileostomy. “After the third surgery, all we could do was to cross our fingers and hope that he could pass stool,” Lauren recalled. When she saw Jack’s first poopy diaper, she wept tears of joy.

Today, Jack’s life is indistinguishable from that of any other typical toddler. “No one who didn’t know us back then would know anything happened,” Lauren said. “He is just your typical, crazy 2-year-old who is running around and living his best life.”

Long-term, Jack will need to keep an eye on his fluid intake and take care if he gets a stomach bug, as he is particularly susceptible to dehydration. Other than that, Hirschsprung is “in the rearview mirror,” according to Lauren. Today, the Isler family is awaiting another happy addition, as Lauren is pregnant with baby number three.

“I am happy for Jack and his family, and his long-term outcome is very positive,” Dr. Kim noted. “I think he will be a thriving, happy young man who can do anything and everything he wants in life. This should not hold him back at all.”

Learn more about Inova Children’s pediatric surgery services.