2026 Volume 62 Issue 1 Pages 65-71
Teduglutide, a recombinant analogue of glucagon-like peptide-2, has recently gained attention as a therapeutic agent for pediatric short bowel syndrome. Although there have been an increasing number of reports on its efficacy in such cases, there are no published case reports in Japan on treatments involving the use of teduglutide from infancy in patients with extensive Hirschsprung disease, leading to a successful definitive surgery. A male infant was born at 37 weeks and 6 days of gestation, weighing 2,994 g. He was transferred to our hospital on day 3 because of abdominal distension and bilious vomiting. Laparotomy on day 4 led to a diagnosis of extensive-type Hirschsprung disease. An ileostomy was created 75 cm distal to the Treitz ligament. Postoperative management was complicated by enteritis and a persistent high-output stoma, resulting in fluid and electrolyte imbalance and poor weight gain. At seven months of age, teduglutide therapy was initiated. Gradual improvement in stool consistency and intestinal adaptation was observed. Despite an episode of mucous fistula prolapse and infectious endocarditis requiring prolonged antibiotic treatment, parenteral nutrition was discontinued by 17 months of age. At 19 months, a laparoscopic pull-through was performed. The postoperative course was uneventful, and the patient was sent home. This case highlights the potential utility of teduglutide in promoting intestinal adaptation and improving stoma output in infants with extensive Hirschsprung disease, ultimately facilitating weaning from parenteral nutrition and enabling a definitive surgery. Our experience suggests that teduglutide can be a valuable adjunctive therapy in selected patients.
