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Related Experiment Videos

Appendicitis in cystic fibrosis.

M D Shields1, H Levison, J J Reisman

  • 1Department of Paediatrics, Hospital for Sick Children, Toronto.

Archives of Disease in Childhood
|March 1, 1991
PubMed
Summary

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.

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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.

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