Cystic fibrosis is the most common lethal autosomal condition: a defect in chloride channels in exocrine glands resulting in abnormal secretions. While the primary manifestation of cystic fibrosis is usually related to respiratory pathology, the gastrointestinal tract is a common site of cystic fibrosis-related complications. Pancreatic insufficiency1
are well-known associations. Hepatobiliary complications range from fatty liver infiltrates, liver nodularity and biliary cirrhosis,1
and intrahepatic microlithiasis.5
Oesophageal reflux and Barrett's oesophagus are documented.6
Distal intestinal obstructive syndrome is not uncommon7
and has recently been reviewed.8
Ileocecal and colonic stenoses9
(with wall thickening) and fibrosing colonopathy10
may be related to replacement pancreatic enzyme treatment. Intussusception11 12
and rectal prolapse are other known associations.13
Acute appendicitis develops in up to 1.5% of cystic fibrosis patients—a rate that is significantly lower than the 7% seen in the general population.14
However, a high percentage of these patients present late with appendiceal abscesses as the disease process is frequently masked by antibiotic treatment prescribed for respiratory infections. For this reason, and since they generally have a poor respiratory status, their morbidity and mortality following appendicectomy is significantly higher.14
A detailed review of the literature revealed only five cases15–19
of fistulation between appendix and colon (in English language case reports) all in the presence of diverticular disease. A sixth case describes the first case of appendico-sigmoid fistulation in the absence of diverticular disease.20
We describe the second documented occurrence of isolated fistulation between the appendix and sigmoid colon—the first in the presence of cystic fibrosis and the increased risk of appendicular complications.
- Cystic fibrosis can present with a myriad of abdominal problems.
- While rates of appendicitis are low, they present late with appendiceal abscesses.
- Appendicocolic fistulation has been reported in the presence of diverticulitis; only one previous case has been documented in the absence of diverticulitis.