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

Appendix

• 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

DIOS

• See distal ileal obstructive syndrome section under Abdomen.

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.

Hernia

• 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

Intussusception

• 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.