Pediatric patients with cystic fibrosis present a special challenge to surgeons.  Here are some considerations gathered from literature and clinical experience.


  • Chronic antibiotic may mask the classic signs and symptoms of appendicitis.
  • The normal appendix may be larger in CF patients than other patient populations
  • Incidence of perforated appendix at time of diagnosis higher than for general population


Fibrosing Colonopathy

  • Result of colonic strictures and classically associated with high strength pancreatic enzyme replacement.
  • Gross appearance of the affected colon: The lumen is thickened and narrow. The mucosa has an erythematous cobblestone appearance.
  • 89% required operation.

Rectal Prolapse

  • 6% incidence in this series, but reported to occur in up to 23% of the CF population.
  • Occurs most commonly between the ages of 6 months to 3 years.
  • Most managed conservatively by manual reduction.
  • Surgical repair may be indicated for significant bleeding, incarcerated prolapse, severe pain, or failure of medical management.
  • Management consists of controlling the stool consistency and avoiding straining.


  • High incidence of inguinal hernia in the CF population.
  • Chronic cough and poor nutrition may contribute
  • Recurrence rate may be higher than general population

Thoracic Procedures

  • Tube thoracostomy or pleurodesis for pneumothorax, lobar or wedge resection for bronchiectasis, and lung transplantation.
  • Avoid talc pleurodesis if lung transplant anticipated
  • High mortality rate in CF patients with pneumothorax, and high prevalence of history of pneumothorax in those patients who died.
  • Lung transplant only therapy available for those with end stage lung disease.
  • The donor lung does not carry the CF gene.
  • Mortality rates changing rapidly.  Check with lung transplant website for current rate.

GERD with Aspiration

  • May aggravate poor weight gain and adversely affect pulmonary function, and may account for the high incidence of CF lung disease in RLL
  • Theophylline worsens GER
  • GER is present in about 81% in patients younger than 5 years old.
  • Children unresponsive to conservative therapy should consider fundoplication
  • Gastrostomy tube placement may improve nutrition


  • Occurs in about 1% of patients with CF.
  • Average age 9.5 years old
  • this is very different from the general pediatric population (age 6-18 months)
  • Abnormally thick stools adhere to the bowel wall and may act as a lead point.

Multi-disciplinary Approach to Care

  • Pediatric Surgeons
  • Pediatric Pulmonologists with interest in CF
  • Critical care specialists
  • Respiratory care specialists
  • Nutrition support specialists
  • Improved antibiotic and pancreatic enzyme therapy.