Treatment of Hirschsprung's Disease
The definitive treatment for Hirschsprung's disease is surgical resection of the aganglionic bowel segment with pull-through of normally innervated intestine to the anus. 1, 2
Surgical Management
Primary Surgical Approach
- Surgery is the only curative treatment and involves removing the aganglionic segment and creating a ganglionated neorectum using proximal normally innervated bowel 1, 2
- The procedure connects normally innervated bowel down to the anus while preserving sphincter function 2
Surgical Technique Options
Multiple established procedures are available, all with favorable outcomes 3, 2:
- Transanal endorectal pull-through (TERPT): Minimally invasive one-stage procedure consisting of rectal mucosectomy, resection of aganglionic bowel, and colo-anal anastomosis 4, 3
- Laparoscopic approaches: Associated with shorter hospital stays and favorable cosmetic results 3
- Traditional procedures (Swenson, Soave, Duhamel): Well-established techniques that may include laparoscopic assistance 2
Critical Intraoperative Consideration
Frozen section evaluation of the entire circumference of the pull-through bowel is essential to confirm both ganglion cells and normal-caliber nerves are present, as the transition zone between aganglionic and ganglionic bowel is irregular 5
Post-Surgical Management
Common Post-Operative Problems
Even after successful surgery, patients may experience 1, 3:
- Obstructive symptoms and constipation
- Enterocolitis (most serious complication)
- Fecal incontinence or soiling
- Anastomotic stricture
Systematic Evaluation Approach for Post-Operative Symptoms
When symptoms persist after surgery, evaluate systematically for 1:
- Anatomic factors: Examination under anesthesia, contrast studies, endoscopy
- Inflammatory factors: Assessment for enterocolitis
- Behavioral factors: Toileting patterns and habits
- Motility factors: Anal sphincter function testing, colonic motility studies
Treatment of Post-Operative Complications
Medical management should be attempted first for most post-operative symptoms 1:
- Sequential antibiotic therapy for bacterial overgrowth (similar principles as chronic intestinal failure management) 6
- Bowel management programs tailored to specific symptoms
- Botulinum toxin injection to anal sphincter for obstructive symptoms 1
Reoperation is rarely necessary but may be indicated when 1, 5:
- Residual aganglionic or transition zone bowel is identified (found in 63% of reoperations) 5
- Severe anatomic stricture is present
- Medical management fails completely
Critical Pitfall to Avoid
Do not assume all post-operative bowel dysfunction is due to residual aganglionosis - multiple pathophysiological mechanisms can contribute, including sphincter dysfunction, motility disorders, and behavioral factors, each requiring different management strategies 1
Long-Term Follow-Up
Continued long-term monitoring is essential to assess 3:
- Quality of life outcomes
- Bowel function (constipation vs. incontinence)
- Psychological well-being
- Development of late complications like enterocolitis