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
- See distal ileal obstructive syndrome section under Abdomen.
- 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.
- 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
- 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.