Surgery in cystic fibrosis--a 20-year review

A T Westwood1, J D Ireland, M D Bowie

  • 1Cystic Fibrosis Clinic, Red Cross War Memorial Children's Hospital.

Insights

Cystic fibrosis (CF) patients frequently require surgery, often for meconium ileus. When indicated, surgical intervention for CF demonstrates low mortality and morbidity rates, making it a viable treatment option.

Area of Science:

  • Pediatric Surgery
  • Pulmonary Medicine
  • Gastroenterology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Surgical intervention is sometimes necessary for CF complications.
  • Historical data on surgical outcomes in CF patients is valuable.

Purpose of the Study:

  • To analyze the surgical histories of pediatric patients with Cystic Fibrosis (CF).
  • To determine the types and frequency of surgeries performed for CF-related conditions.
  • To evaluate the morbidity and mortality associated with surgery in CF patients.

Main Methods:

  • Retrospective review of surgical histories for 111 pediatric patients diagnosed with Cystic Fibrosis (CF).
  • Data collected between 1972 and 1991.
  • Categorization of surgeries based on relation to CF, including abdominal and thoracic procedures.

Main Results:

  • 154 operations were performed on 57 children.
  • 84 operations were directly related to CF, with meconium ileus being the most common indication (26 major abdominal procedures).
  • Low rates of mortality (1 death within one month) and complications (11.9% anesthetic, 9.2% post-operative) were observed.

Conclusions:

  • Patients with Cystic Fibrosis (CF) have a high likelihood of requiring surgical intervention during their lifetime.
  • When surgery is indicated for CF, it can be performed with acceptable low morbidity and mortality.
  • Surgical management is a critical component in the comprehensive care of pediatric patients with Cystic Fibrosis (CF).