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Appendicitis in cystic fibrosis
M D Shields1, H Levison, J J Reisman
1Department of Paediatrics, Hospital for Sick Children, Toronto.
Insights
Appendicitis is rare in cystic fibrosis patients, often presenting as an abscess. Delayed diagnosis is common, potentially due to milder symptoms or confusion with intestinal obstruction syndrome.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Pulmonology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the gastrointestinal tract.
- Appendicitis is considered uncommon and challenging to diagnose in CF patients.
- Understanding appendicitis presentation in CF is crucial for timely intervention.
Observation:
- A study reviewed nine pediatric patients with cystic fibrosis who developed appendicitis.
- Most patients (8/9) presented with appendiceal abscess at the time of surgery.
- A significant delay in diagnosis (over three days) occurred in four patients.
Findings:
- Delayed diagnosis in CF appendicitis may stem from indolent presentation or misdiagnosis as distal intestinal obstruction syndrome (DIOS).
- Contrast enema showing extrinsic caecal compression is a key diagnostic indicator.
- Diagnostic imaging modalities like ultrasound, CT, and gallium scans showed limited utility in this cohort.
Implications:
- Appendicitis should be included in the differential diagnosis for CF patients presenting with abdominal pain.
- Radiological findings, particularly extrinsic caecal compression on contrast enema, are vital for diagnosis.
- Further research is needed to improve diagnostic strategies for appendicitis in the cystic fibrosis population.
Abstract:
Appendicitis is said to be uncommon and difficult to diagnose in cystic fibrosis. The clinical and radiological features in nine patients with cystic fibrosis who had appendicitis were studied. All but one of the patients had an appendiceal abscess at surgery. Four patients had a delay in diagnosis of greater than three days before the correct diagnosis was made. This delay may have been due to a more indolent presentation or because these patients were initially considered to have distal intestinal obstruction syndrome. Appendicitis should be considered in the differential diagnosis if a contrast enema demonstrates extrinsic compression of the caecum. Ultrasound, computed tomography, and gallium scans were found to be of limited help in our series.