Abstract

Hirschsprung’s disease (HD) in adults is rare, often underdiagnosed, and presents as long-standing, refractory constipation, distension, and recurrent obstruction. Delayed diagnosis increases risks like toxic megacolon, volvulus, and perforation. This review aimed to cover the adult-specific features, diagnosis, and surgical outcomes of HD. The PubMed, Web of Science, Google Scholar, and Cochrane Library were searched using terms related to adult HD, presentation, diagnosis, treatment, and outcomes, the findings were then analyzed qualitatively. Adults typically have lifelong constipation needing cathartics or enemas. Imaging shows a narrowed distal rectum with proximal dilation; histopathology confirms aganglionosis. Surgery, mainly pull-through procedures (Duhamel most common), is standard, with low rates of dehiscence (~3.9%), poor long-term outcomes (~1.3%), and mortality (~0.4%). The Jinling approach improves Wexner constipation scale (WCS)/bowel function score (BFS), and gastrointestinal quality of life index (GIQLI) scores, with ~97–98% satisfaction at 6 months. Diversion is often chosen for older, sicker adults with emergencies, leading to longer stays and higher early morbidity than immediate pull-through, more common in younger patients. Despite reconstruction, bowel dysfunction and HAEC remain frequent and impactful socially. In adults with constipation and obstruction, high suspicion for HD is critical, with targeted imaging and histopathology essential. Duhamel pull-through remains effective, but structured bowel management and long-term follow-up are vital to reduce dysfunction and HAEC. More high-quality studies are needed to improve procedure selection and outcomes.

Key words: Adult Hirschsprung’s disease, diagnosis, treatment, bowel function, enterocolitis, narrative review.