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Published on: August 29, 2025
Abdominal manifestations of cystic fibrosis in children
Gulraiz Chaudry1, Oscar M Navarro, Daniel S Levine
1Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Ave., Toronto, Ontario M5G 1X8, Canada. gulraiz.chaudry@sickkids.ca
Insights
Cystic fibrosis (CF) primarily causes lung issues but often presents with gastrointestinal and pancreaticobiliary symptoms in children. This review details the varied abdominal imaging findings in CF patients from infancy to adolescence.
Area of Science:
- Pediatric Gastroenterology
- Radiology
- Cystic Fibrosis Research
Background:
- Pulmonary complications are the primary cause of mortality in cystic fibrosis (CF).
- Abdominal manifestations are prevalent in children with CF, occurring across all age groups.
- Gastrointestinal and pancreaticobiliary issues often present as the initial symptoms in pediatric CF cases.
Purpose of the Study:
- To provide a pictorial review of the diverse radiological appearances of abdominal manifestations in children with cystic fibrosis.
- To illustrate the spectrum of gastrointestinal and pancreaticobiliary conditions associated with CF.
- To correlate imaging findings with specific CF-related abdominal pathologies.
Main Methods:
- Review of radiological imaging (CT and MR) in pediatric patients diagnosed with cystic fibrosis.
- Compilation of images showcasing common and less-common gastrointestinal and pancreaticobiliary manifestations.
- Individualized discussion of conditions and their typical appearances across various imaging modalities.
Main Results:
- Neonatal presentation can include meconium ileus.
- Older children may develop distal intestinal obstruction syndrome or colonic strictures.
- Radiological findings include pancreatic cysts, calcification, fatty infiltration, biliary ductal obstruction, cirrhosis, portal hypertension, and gallstones.
- Exocrine pancreatic deficiency is a common finding.
Conclusions:
- Abdominal manifestations are a significant and varied feature of cystic fibrosis in children.
- Radiological imaging plays a crucial role in diagnosing and managing these gastrointestinal and pancreaticobiliary complications.
- Understanding these imaging patterns is essential for comprehensive CF patient care.
Abstract:
Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities.
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