Abdominal manifestations of cystic fibrosis

Abdominal manifestations in cystic fibrosis are common, varied and nearly all organ systems can be affected.

Only 39% of patients with cystic fibrosis (CF) have pulmonary symptoms as their sole complaint ¹.  7% of CF patients do not present until adulthood.

For a general discussion of cystic fibrosis, and a discussion of its other manifestations, please refer to :

• cystic fibrosis (parent article)
  • pulmonary manifestations of CF
  • head and neck manifestations of CF
  • musculoskeletal manifestations of CF

Hepatobiliary manifestations

Liver parenchymal disease

• 40% of CF patients will develop liver disease
• 1 - 8% progress to portal hypertension
• up to 40 % progress to focal cirrhosis and up to 12 % progress to multilobular cirrhosis ⁵, but development of HCC is unusual
• pathophysiology is multifactorial
• fatty infiltration seen in 30% of CF patients at biopsy, 30 - 50% at imaging and 60% at autopsy ²

Biliary tree

• gallstones : 12 - 24% of CF patients
• sclerosing cholangitis
• intrahepatic ductal strictures seen in 100% of patients with CF and liver disease and may correspond histologically to focal biliary fibrosis seen in 78% of CF patients >24 years old
• microgallbladder : seen in up to 30% of CF patients at autopsy ³

Pancreatic manifestations

Exocrine gland insuffciency affects 85 - 90% of all CF 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 CF patients

• fatty replacement : most common manifestation of the pancreas, which can progress to complete pancreatic lipomatosis
• acute pancreatitis : occurs in patients with residual pancreatic exocrine function
• pancreatic calcifications occur in 7% of patients
• pancreatic cysts and cystosis : typically microscopic 3mm diameter
• pancreatic duct strictures

Gastrointestinal manifestations

• gastro-oesophageal reflux and associated complications such as Barrett oesophagus : thought to be secondary to chronic cough, hyperinflation and diaphgramatic depression
• gastric and duodenal ulcerations : likely related to impaired bicarbonate secretion
• distal intestinal obstruction syndrome (DIOS)
• intussusception ⁴ : typically ileocolic and may result from adherent fecal residue or enlarged lymphoid follicles. 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 CF patients is lower than that in the general population
• colon : often abnormal in patients with CF, 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 CF diagnosis not yet made or young adults that are non compliant with treatment
• gastrointestinal tract malignancies : of which colorectal carcinoma is most common

Renal manifestations

• renal stones