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Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease
J C Langer1,2, M D Rollins3, M Levitt4
1Division of General and Thoracic Surgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada. jacob.langer@sickkids.ca.
Insights
Postoperative obstructive symptoms are common in children with Hirschsprung disease after pull-through surgery. This guideline offers a structured approach to diagnose and manage these challenging issues.
Area of Science:
- Pediatric Surgery
- Gastroenterology
Background:
- Hirschsprung disease often requires surgical intervention (pull-through surgery).
- Postoperative complications, including obstructive symptoms, are frequent challenges in pediatric patients.
- Existing management strategies for these symptoms lack a standardized approach.
Purpose of the Study:
- To provide a rational and systematic approach for managing obstructive symptoms in children post-pull-through surgery for Hirschsprung disease.
- To consolidate current knowledge on the causes, diagnosis, and treatment of these postoperative issues.
Main Methods:
- Literature review and expert consensus were utilized.
- A Hirschsprung Disease Interest Group was established by the American Pediatric Surgical Association.
- An algorithm for diagnosis and management was developed.
Main Results:
- Identified causes of obstruction: mechanical, aganglionosis/hypoganglionosis, transition zone pull-through, internal sphincter achalasia, proximal intestinal dysmotility, and functional megacolon.
- Presented a stepwise diagnostic and management algorithm.
- Highlighted the importance of a logical approach to patient care.
Conclusions:
- A structured, stepwise approach can effectively facilitate the diagnosis and management of obstructive symptoms following Hirschsprung disease pull-through surgery.
- Addressing these common postoperative issues is crucial for improving patient outcomes.
- Further research may refine management strategies.
Abstract:
Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment. Level of evidence V.
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