digestive cystic fibrosis
Image Gallery
Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs.
Synopsis
neonatal digestive obstruction
- meconial ileus (meconium ileus)
- neonatal intestinal volvulus
- neonatal intestinal atresia
- neonatal intestinal perforation
- meconial peritonitis with abdominal calcifications
- colonic meconial plug syndrome
- colonic perforation
- right colon perforation (7175631)
- junction left colon-sigmoid colon perforation
- meconial peritonitis with abdominal calcifications
- colonic perforation
in older children with CF
- intestinal obstruction
- distal intestinal obstruction syndrome
- colonic stricture
- tenacious intestinal residue
- intestinal intussusception
- recurrent rectal prolapse
- distal intestinal obstruction syndrome
- thickened intestinal mucosal
- digestive pneumatosis (16702447)
- rare necrotizing enterocolitis
- peptic esophageal stricture due to gastroesophageal reflux
- duodenal ulcer
- enteritis (10799435)
- appendicitis due to inspissated secretions
- neonatal acute appendicitis
- Clostridium difficile-associated colitis (or antibiotic-associated colitis or pseudomembranous colitis (9930816, 9470027, 8014775)
- Crohn-like colitis (indeterminate colitis) (9587500)
- fibrosing colonopathy
pancreatic anomalies
- obstruction of ducts and ductules
- exocrine pancreatic insufficiency
- pancreatitis
- small pancreas
- largely replaced by fat
- calcifications
- macrocysts
hepatic anomalies
- cholestasis
- cholelithiasis
- biliary cirrhosis with portal hypertension
- steatosis
- gallstones
- shrunken nodular liver
- portal hypertension
- splenomegaly
- portosystemic collateral vessels
increased risk for developing gastrointestinal carcinomas, as early as the 3rd decade.
Localization
- cystic fibrosis colonopathy
Videos
Meconium obstruction with necrosis (by Washington Deceit)
References
Agrons GA, Corse WR, Markowitz RI, Suarez ES, Perry DR. Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics. 1996 Jul;16(4):871-93. PMID: 8835977