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Intussusception in cystic fibrosis.

M Holmes¹, V Murphy, M Taylor

¹National Children's Hospital, Dublin, Republic of Ireland.

Archives of Disease in Childhood

|June 1, 1991

Summary

Acute intussusception in children with cystic fibrosis can mimic meconium ileus equivalent, delaying diagnosis. Surgical intervention led to full recovery in both reported cases.

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Area of Science:

Pediatric Surgery
Gastroenterology
Cystic Fibrosis Research

Background:

Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs and digestive system.
Intussusception, a condition where one part of the intestine slides into another, is rare in older children but can occur in CF patients.
Meconium ileus equivalent (MIE) is a common complication in CF, presenting with abdominal obstruction symptoms.

Observation:

Two older children diagnosed with cystic fibrosis presented with acute abdominal symptoms.
The clinical presentation mimicked meconium ileus equivalent, leading to a diagnostic delay.
Both patients experienced intussusception requiring surgical management.

Findings:

The reported cases highlight a diagnostic challenge where intussusception in CF can be mistaken for MIE.
Prompt surgical intervention was necessary for both pediatric patients.
Despite the complexity, both children achieved complete and uncomplicated recoveries post-surgery.

Implications:

This case report underscores the importance of considering intussusception in the differential diagnosis of abdominal obstruction in older children with CF.
Early recognition and surgical treatment are crucial for favorable outcomes in CF-related intussusception.
Further research may elucidate the specific mechanisms linking CF to intussusception and MIE-like presentations.

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