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Limited surgery for lower-segment Hirschsprung's disease
1Department of Surgery, Children's Hospital and Medical Center, University of Washington, Seattle.
Insights
Rectal myectomy effectively treated older children with Hirschsprung's disease, improving constipation and bowel function. This less-extensive surgery offers a viable option for managing this condition in pediatric patients.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Chronic constipation and abdominal distension are common symptoms in affected children.
- Diagnostic challenges exist, particularly in cases with nondiagnostic barium enema studies.
Purpose of the Study:
- To evaluate the efficacy of posterior rectal myectomy as a treatment for Hirschsprung's disease in children presenting with chronic constipation and inconclusive diagnostic findings.
- To assess the long-term outcomes and patient-reported improvements following rectal myectomy.
Main Methods:
- A retrospective case series involving 14 pediatric patients (aged 2-14 years) with Hirschsprung's disease.
- All patients underwent posterior rectal myectomy.
- Outcomes were assessed over a mean follow-up period of 37.1 months, evaluating factors like laxative use, stool frequency, soiling, incontinence, and overall patient satisfaction.
Main Results:
- All 14 patients demonstrated clinical improvement after rectal myectomy, with assessments categorized as excellent (6), good (6), or fair (2).
- Improvements were observed irrespective of the presence or absence of ganglion cells in the surgical specimen.
- No patients experienced soiling or incontinence post-operatively, and seven required only intermittent laxative use.
Conclusions:
- Posterior rectal myectomy is a safe and effective surgical intervention for selected pediatric patients with Hirschsprung's disease.
- This less-invasive procedure should be considered for older children experiencing chronic constipation due to Hirschsprung's disease.
- The procedure yields favorable functional outcomes, including improved bowel regularity and absence of incontinence.
Objective:
To determine if rectal myectomy is an effective treatment for a specific group of patients with Hirschsprung's disease.
Design:
Retrospective series with follow-up of 6 to 132 months (mean follow-up, 37.1 months).
Setting:
Children's hospital.
Patients:
Fourteen children aged 2 to 14 years with chronic constipation and nondiagnostic barium enema contrast study findings.
Intervention:
Posterior rectal myectomy.
Main Outcome Measures:
Laxative use, frequency of stools, incidence of soiling or incontinence, and patient's assessment of improvement.
Results:
The conditions of all 14 patients improved regardless of the presence of ganglion cells in the myectomy specimen; there were six excellent, six good, and two fair assessments. laxatives were used intermittently by seven of 14 patients. No patients complained of incontinence or soiling.
Conclusions:
Rectal myectomy is an effective, less-extensive operation that should be considered for older children with Hirschsprung's disease.