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BMJ Case Rep. 2010; 2010: bcr0220102714.
Published online 2010 September 7. doi:  10.1136/bcr.02.2010.2714
PMCID: PMC3029547
Unusual association of diseases/symptoms

Appendico-colic fistula complicating appendicitis in cystic fibrosis


The case of a boy with cystic fibrosis who presented with an unusual complication of appendicitis is reported. Delayed presentation, with complications of appendicitis such as perforation and abscess formation, is not uncommon in cystic fibrosis; however, this case represents the first report of an isolated appendico-colic fistula following appendicitis in association with cystic fibrosis.


Cystic fibrosis is the most common autosomal recessive condition and can present with a myriad of potential causes for abdominal pain.

Case presentation

A 13-year-old boy with cystic fibrosis presented with an 18-month history of intermittent, mild central abdominal and right-lower quadrant pain. There were no other associated gastrointestinal symptoms. Medications included prophylactic antibiotics and pancreatic enzyme supplementation. On examination the patient was afebrile with a heart rate of 80 bpm. Abdominal examination revealed a mass in the right iliac fossa, which was mildly tender but with no evidence of rebound tenderness or guarding.


Haematological and biochemical investigations were normal. Abdominal ultrasound demonstrated a small ill-defined fluid collection in the right iliac fossa. Gastrograffin enema identified a soft tissue mass, which was displacing the ileum, together with a fistula between the sigmoid colon and the caecum (figure 1).

Figure 1
Gastrograffin enema demonstrating a right iliac fossa soft tissue mass displacing the terminal ileum and an appendico-colic fistula (white arrow) between the base of the appendix (dashed arrow) and the sigmoid colon (dotted arrow).


Following optimisation of respiratory function with antibiotic treatment and intensive physiotherapy the patient underwent a laparotomy. Dense adhesions were identified involving the ileum, caecum and sigmoid colon. A fistula was evident running from the stump of a retrocaecal appendix to the sigmoid colon. The sigmoid colon adjacent to the fistula was oedematous and inflamed as was the terminal ileum and caecum. The fistula was excised and a sigmoid colectomy performed together with a right hemicolectomy.

Outcome and follow-up

The patient made a good but slow recovery and was discharged on the 11th postoperative day. Histological examination of the resection specimen indicated that the fistula was lined by chronically inflamed colonic mucosa suggesting that the primary pathology had been acute appendicitis.


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 and pancreatitis2 are well-known associations. Hepatobiliary complications range from fatty liver infiltrates, liver nodularity and biliary cirrhosis,1 cholelithiasis3 4 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 cases1519 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.

Learning points

  • 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.


We would like to thank the patient and his parents for consenting to publication of this case report.


Competing interests None.

Patient consent Obtained.


1. Lugo-Olivieri CH, Soyer PA, Fishman EK. Cystic fibrosis: spectrum of thoracic and abdominal CT findings in the adult patient. Clin Imaging 1998;22:346–54 [PubMed]
2. De Boeck K, Weren M, Proesmans M, et al. Pancreatitis among patients with cystic fibrosis: correlation with pancreatic status and genotype. Pediatrics 2005;115:e463–9 [PubMed]
3. Doherty DE, Schonfeld SA. Cholelithiasis in adult cystic fibrosis. South Med J 1983;76:1580–1 [PubMed]
4. Scott WJ, Block GE. Biliary stone disease in adults with cystic fibrosis. Surgery 1989;105:671–3 [PubMed]
5. Magruder MJ, Munden MM. Intrahepatic microlithiasis: another gastrointestinal complication of cystic fibrosis. J Ultrasound Med 1997;16:763–5 [PubMed]
6. Hassall E, Israel DM, Davidson AG, et al. Barrett's esophagus in children with cystic fibrosis: not a coincidental association. Am J Gastroenterol 1993;88:1934–8 [PubMed]
7. Haanaes OC, Kongerud J, Storrøsten OT, et al. [Meconium ileus-equivalent in adult patients with cystic fibrosis]. Tidsskr Nor Laegeforen 1996;116:733–5 [PubMed]
8. Speck K, Charles A. Distal intestinal obstructive syndrome in adults with cystic fibrosis: a surgical perspective. Arch Surg 2008;143:601–3 [PubMed]
9. Pohl M, Krackhardt B, Posselt HG, et al. Ultrasound studies of the intestinal wall in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1997;25:317–20 [PubMed]
10. Schwarzenberg SJ, Wielinski CL, Shamieh I, et al. Cystic fibrosis-associated colitis and fibrosing colonopathy. J Pediatr 1995;127:565–70 [PubMed]
11. Fishman DS, Sailhamer EA, Cohen DT, et al. Intussusception of the appendix causing small bowel obstruction in a patient with cystic fibrosis. J Pediatr Gastroenterol Nutr 2010;50:1. [PubMed]
12. Syrimi M, Courtney ED, Mills AD, et al. Large caecal intussusception associated with three jejunal intussusceptions in a child with cystic fibrosis. Int J Colorectal Dis 2008;23:1141–2 [PubMed]
13. Siafakas C, Vottler TP, Andersen JM. Rectal prolapse in pediatrics. Clin Pediatr (Phila) 1999;38:63–72 [PubMed]
14. Shields MD, Levison H, Reisman JJ, et al. Appendicitis in cystic fibrosis. Arch Dis Child 1991;66:307–10 [PMC free article] [PubMed]
15. Yiangou C, Holme TC. Sigmoido-appendiceal fistula due to diverticulitis. J R Soc Med 1998;91:544. [PMC free article] [PubMed]
16. van Hillo M, Fazio VW, Lavery IC. Sigmoidoappendiceal fistula – an unusual complication of diverticulitis. Report of a case. Dis Colon Rectum 1984;27:618–20 [PubMed]
17. Libson E, Bloom RA, Verstandig A, et al. Sigmoid-appendiceal fistula in diverticular disease. Diagn Imaging Clin Med 1984;53:262–4 [PubMed]
18. Marshak RH, Maklansky D, Lindner AE. The radiology corner. Sigmoid-appendiceal fistula in diverticulitis. Am J Gastroenterol 1976;66:292–6 [PubMed]
19. Smith HJ, Berk RN, Janes JO, et al. Unusual fistulae due to colonic diverticulitis. Gastrointest Radiol 1978;2:387–92 [PubMed]
20. Ganesh JS, Ali MA. A case of appendiceal-sigmoid fistula of non-diverticular origin. Trop Gastroenterol 2001;22:50–1 [PubMed]

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