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Published on: November 4, 2010
Treatment and prognosis of rectal prolapse in cystic fibrosis
Insights
Rectal prolapse is a common early sign in cystic fibrosis patients, often resolving spontaneously or with enzyme therapy. Early diagnosis is key, and a sweat test is recommended for any child with rectal prolapse.
Area of Science:
- Pediatric Gastroenterology
- Genetics
- Surgical Pediatrics
Background:
- Rectal prolapse is a notable symptom in pediatric cystic fibrosis (CF) cases.
- Physicians often overlook rectal prolapse as an early indicator of CF.
Purpose of the Study:
- To investigate the incidence and significance of rectal prolapse in cystic fibrosis patients.
- To evaluate the relationship between rectal prolapse and CF diagnosis and treatment.
Main Methods:
- Retrospective analysis of 18.5% (112 of 605) cystic fibrosis patients with rectal prolapse.
- Observation of prolapse onset relative to CF diagnosis and response to therapy.
Main Results:
- Rectal prolapse preceded CF diagnosis in 48 patients.
- Prolapse often resolved with pancreatic enzyme replacement therapy post-diagnosis.
- Many patients self-managed prolapse, with surgery rarely needed.
- Spontaneous resolution occurred in some undiagnosed cases by age 3-5.
Conclusions:
- Rectal prolapse warrants consideration as an early sign of cystic fibrosis.
- A sweat test is crucial for any child experiencing rectal prolapse.
- Management strategies vary, with conservative approaches often effective.
Abstract:
Rectal prolapse occurred in 112 (18.5%) of 605 cystic fibrosis patients. In 48 patients prolapse preceded diagnosis of cystic fibrosis, but physicians (pediatricians, pediatric and general surgeons, and proctologists) rarely appreciated its importance as a symptom of this disease. Prolapses frequently cease with institution of pancreatic enzyme replacement therapy following diagnosis of cystic fibrosis. However, even when the disease remains undiagnosed, the symptom is often transient and frequently resolves at 3-5 yr of age. Prolapse occurring initially after cystic fibrosis is diagnosed rarely responds to manipulation of diet or enzyme doses. Many patients develop a method of reduction which involves voluntary abdominal, perineal, and gluteal muscles and does not require manual pressure on the prolapsed segment. Most patients do not need specific treatment for the prolapse. Surgery is rarely necessary. A sweat test should be obtained on any child who has had even a single episode of rectal prolapse.
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