From the Research
Hirschsprung disease is primarily treated with surgery, as medical management alone is not curative, and the most recent evidence from 2024 supports this approach 1. The definitive treatment involves surgical removal of the aganglionic bowel segment and reconnection of healthy intestine. Before surgery, medical management focuses on preventing complications and stabilizing the patient, including:
- Rectal irrigations with warm saline to decompress the bowel and prevent enterocolitis
- Nutritional support to maintain adequate hydration and nutrition
- Antibiotics if enterocolitis develops, typically a combination of metronidazole and a broad-spectrum antibiotic like piperacillin-tazobactam, as supported by studies on Hirschsprung-associated enterocolitis 2, 3. For severe cases, a temporary ostomy may be created to divert stool until definitive surgery can be performed. Postoperatively, patients may require laxatives, stool softeners, or enemas to manage constipation as the bowel adapts to its new configuration. Long-term follow-up is essential as some patients may experience persistent constipation, incontinence, or enterocolitis even after surgical correction, highlighting the need for a systematic evaluation and approach to address these complications, as discussed in the most recent study from 2024 1. The medical approach is supportive rather than curative because Hirschsprung disease results from the congenital absence of ganglion cells in the enteric nervous system, preventing normal peristalsis in affected bowel segments, which cannot be restored without surgical intervention, a concept further elaborated in a review from 2018 4. Current diagnosis and management strategies, including surgical techniques and prevention of complications, are continuously evolving, as noted in a 2017 article 5, but the fundamental approach of surgical correction with supportive medical management remains the cornerstone of treatment.