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Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; Update for 2022
Hira Ahmad1, Desale Yacob2, Devin R Halleran3
1Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Seattle, WA, United States.
Insights
Many children struggle after Hirschsprung disease (HD) surgery. This review outlines a systematic approach to evaluate and treat post-operative complications like incontinence or enterocolitis for better outcomes.
Area of Science:
- Pediatric Surgery
- Gastroenterology
Background:
- Hirschsprung disease (HD) requires surgical correction, typically a pull-through procedure.
- A significant number of patients experience persistent complications post-operatively, despite technically adequate surgery.
Purpose of the Study:
- To present a structured diagnostic and therapeutic strategy for managing patients with Hirschsprung disease who experience poor outcomes after pull-through surgery.
- To address common post-operative issues including fecal incontinence, obstructive symptoms, and recurrent enterocolitis.
Main Methods:
- Systematic review of diagnostic approaches for post-HD pull-through complications.
- Outline of treatment modalities including medical management, reoperation, and botulinum toxin injections.
Main Results:
- Post-operative problems in HD patients can manifest as fecal incontinence, obstructive symptoms, or enterocolitis.
- A systematic diagnostic evaluation is crucial for identifying the cause of these complications.
Conclusions:
- Effective management strategies, including medical interventions, surgical revision, or targeted treatments like botulinum toxin, can successfully address complications in most post-HD pull-through patients.
- A systematic approach ensures appropriate treatment for improved patient outcomes.
Abstract:
After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is unfortunately not the case for a significant number of patients who struggle following their pull-through procedure. Many clinicians are puzzled by these outcomes as they can occur in patients who they believe have had a technically satisfactory described operation. This review presents an organized approach to the evaluation and treatment of the post HD pull-through patient who is not doing well. Patients with HD who have problems after their initial operation can have: (1) fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis (a more severe subset of obstructive symptoms). After employing a systematic diagnostic approach, successful treatments can be implemented in almost every case. Patients may need medical management (behavioral interventions, dietary changes, laxatives, or mechanical emptying of the colon), a reoperation when a specific anatomic or pathologic cause is identified, or botulinum toxin when non-relaxing sphincters are the cause of the obstructive symptoms or recurrent enterocolitis.
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