Related Experiment Videos
Strictures of the colon in cystic fibrosis
S J King1, D Van Velzen, R L Smyth
1Department of Diagnostic Radiology, Royal Liverpool Children's NHS Trust, Alder Hey.
Insights
Colonic strictures, a complication of cystic fibrosis (CF) distal intestinal obstruction syndrome (DIOS), require surgical consideration when medical management fails. Histopathology reveals post-ischaemic ulceration repair in these CF patients.
Area of Science:
- Pediatric Gastroenterology
- Colorectal Surgery
- Medical Imaging
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the gastrointestinal tract.
- Distal Intestinal Obstruction Syndrome (DIOS) is a common complication in CF patients, often managed medically.
- Refractory DIOS cases may necessitate surgical intervention, with colonic strictures being a potential underlying cause.
Purpose of the Study:
- To describe the radiological and histopathological findings in pediatric CF patients with DIOS.
- To highlight the significance of colonic strictures in CF patients unresponsive to medical DIOS management.
- To correlate imaging findings with surgical and histopathological results.
Main Methods:
- Retrospective review of five pediatric patients with CF and DIOS.
- Preoperative imaging including ultrasound and contrast enema in four patients.
- Surgical exploration and histopathological examination of resected colonic segments.
Main Results:
- Four patients presented with colonic strictures (ascending colon) confirmed by ultrasound, contrast enema, and surgery.
- All five patients exhibited histopathological evidence of post-ischaemic ulceration repair.
- One patient developed a subsequent descending colon stricture requiring resection.
Conclusions:
- Colonic strictures are a critical consideration in CF patients with DIOS refractory to medical treatment.
- Radiological and histopathological findings are key to diagnosing and managing these strictures.
- Early recognition and intervention for colonic strictures can improve outcomes in CF patients with DIOS.
Abstract:
We describe the radiological and histopathological findings in five children with cystic fibrosis who presented recently to our hospital. Each child underwent surgery after failing to respond to medical management for suspected distal intestinal obstruction syndrome. Four patients had preoperative ultrasound and contrast enema examinations. Wall thickening and dilatation of the ascending colon was seen on ultrasound and contrast enema revealed a stricture of the ascending colon in all four. At surgery these findings were confirmed. All five patients had histopathological changes of post-ischaemic ulceration repair. One child had symptoms of intestinal obstruction 5 months after right hemicolectomy. Radiological investigation revealed a stricture in the descending colon which was resected. The histopathological changes were the same as before. Colonic strictures should be considered in cystic fibrosis patients who do not respond to medical management of distal intestinal obstruction syndrome.