Cystic fibrosis (abdominal manifestations)

Abdominal manifestations in cystic fibrosis are common, varied and nearly all organ systems can be affected, and it should be remembered that only 39% of patients with cystic fibrosis have pulmonary symptoms as their sole complaint 1. Not only that, but 7% of cystic fibrosis patients do not present until adulthood. 

This article focuses on abdominal manifestations of cystic fibrosis. For a general discussion, and a discussion of its other manifestations, please refer to the general article on cystic fibrosis

(Most commonly involved abdominal organ)

Exocrine gland insufficiency affects 85-90% of all cystic fibrosis patients and results from inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat.

Endocrine dysfunction occurs in 30-50% of cystic fibrosis patients:

  • fatty replacement
    • most common manifestation of the pancreas,
    • can progress to complete pancreatic lipomatosis
    • mean age is 17 years 6
  • acute pancreatitis: occurs in patients with residual pancreatic exocrine function
  • pancreatic calcifications occur in 7% of patients
  • pancreatic cysts and cystosis: typically microscopic 3 mm diameter
  • pancreatic duct strictures
  • up to 72% of adult cystic fibrosis patients will develop liver disease 6
  • focal biliary cirrhosis (pathognomonic for cystic fibrosis and present in 78%): focal portal fibrosis and cholestasis; thickened hyperechoic peri-portal tissue on ultrasound (>2 mm thickness)
  • up to 12% progress to multilobular cirrhosis 5, but the development of HCC is unusual
  • 1-8% progress to portal hypertension
  • hepatic steatosis (23-67% 6; seen in 30% of cystic fibrosis patients at biopsy, 30-50% at imaging and 60% at autopsy 2)
  • pathophysiology is multifactorial
  • gallstones: 12-24% of cystic fibrosis patients
  • sclerosing cholangitis
  • intrahepatic ductal strictures are seen in 100% of patients with cystic fibrosis and liver disease and may correspond histologically to focal biliary fibrosis seen in 78% of patients >24 years old
  • microgallbladder: seen in up to 30% of cystic fibrosis patients at autopsy 3
  • gastro-esophageal reflux and associated complications such as Barrett esophagus: thought to be secondary to chronic cough, hyperinflation, and diaphragmatic depression
  • gastric and duodenal ulcerations: likely related to impaired bicarbonate secretion
  • distal intestinal obstruction syndrome (DIOS)
  • intussusception: typically ileocolic and may result from adherent fecal residue or enlarged lymphoid follicles 4; a chronically distended appendix may be the lead point
  • despite the common distention of the appendix by inspissated secretions, the reported prevalence of acute appendicitis in cystic fibrosis patients is lower than that in the general population
  • colon: often abnormal, with proximal colonic wall thickening, pericolonic fat proliferation, and mesenteric fat infiltration
  • pneumatosis intestinalis: confined to the colon and typically coincides with the development of obstructive lung disease
  • rectal mucosal prolapse: typically in young children in whom a diagnosis of cystic fibrosis has not yet been made or young adults that are noncompliant with treatment
  • gastrointestinal tract malignancies: of which colorectal carcinoma is most common
  • renal stones: symptomatic stones are twice as common in cystic fibrosis patients (~4.5%) compared to the general population (2%) 6
  • nephrotic syndrome from secondary amyloidosis (poor prognostic sign) 6
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Article information

rID: 6959
Synonyms or Alternate Spellings:
  • Abdominal manifestations of cystic fibrosis
  • Abdominal manifestations of CF

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Cases and figures

  • DIOS
    Case 1: with cirrhosis and portal hypertension
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  • Mucus impaction
    Case 2: with pancreatic fatty atrophy
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  • Case 3: with DIOS and pancreatic calcification
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  • Case 4: with portal hypertension and cirrhosis
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