Imaging Pearls ❯ Pancreas ❯ Cystic Fibrosis
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- “In conclusion, pancreatic cystosis is a rare manifestation of CF and is thought to be caused by ductal dilatation resulting from ductal protein precipitation. The diagnosis can easily be made in the appropriate clinical setting by means of sonography, CT, or MRI; however, sonography should be the technique of choice for initial and follow-up evaluation of patients with these findings. Although CT and MRI allow an adequate assessment of the structure and content of the cysts and a more panoramic view of the pancreas than sonography, they do not provide any relevant additional information to the data provided by sonography.”
Pancreatic Cystosis in Children and Young Adults with Cystic Fibrosis: Sonographic, CT, and MRI Findings
Teresa Berrocal et al.
AJR 2005;184:1305–1309 - "Pancreatic involvement can result in exo- crine or endocrine insufficiency. The spectrum of pancreatic appearances has been described as follows: complete replacement of the pancreas by fibrofatty tissue, with enlargement of the pancreas corresponding to lipomatous pseudohypertrophy; partial replacement of the pancreas by fibrofatty tissue; complete atrophy of the pancreas without fatty replacement; diffuse pancreatic fibrosis; and cystic transformation of the pancreas. Calcifications may also be found.”
Pancreatic Cystosis in Children and Young Adults with Cystic Fibrosis: Sonographic, CT, and MRI Findings
Teresa Berrocal et al.
AJR 2005;184:1305–1309 - “Cyst are a relatively common feature in the CF-affected pancreas, but their size does not usually exceed a few millimeters. Macroscopic cysts measuring more than 1 cm are a rare condition. Macrocysts of different sizes distributed throughout the gland represent an extremely unusual form of pancreatic involvement in CF that has been described in only a few patients and is known as pancreatic cystosis.”
Pancreatic Cystosis in Children and Young Adults with Cystic Fibrosis: Sonographic, CT, and MRI Findings
Teresa Berrocal et al.
AJR 2005;184:1305–1309 - "The imaging features of pancreatic macro- cysts seen in our patients are similar to those of uncomplicated pancreatic cysts from any other cause. No parietal cyst calcifications were present in our series. A parietal calcification in one cyst and evidence of hemorrhage in another cyst during an observation period in one CF patient with pancreatic cystosis have been reported using sonography and CT.”
Pancreatic Cystosis in Children and Young Adults with Cystic Fibrosis: Sonographic, CT, and MRI Findings
Teresa Berrocal et al.
AJR 2005;184:1305–1309
- “Cystic fibrosis (CF) is an autosomal recessive disorder that is characterized by an abnormality of exocrine gland function. Adult patients represent a rapidly growing percentage of the CF population. Pulmonary changes are seen in nearly every case and are the most serious complication of CF. In advanced lung disease, bronchiectasis, emphysematous bullae, and subpleural blebs can frequently develop. Although pulmonary disease is the most common cause of death and morbidity among CF patients, there also can be involvement of other organs, particularly in adults, which show characteristic signs on CT and spiral CT. Pancreatic abnormalities are present in 85–90% of CF patients.”
Cystic fibrosis: Spectrum of thoracic and abdominal CT findings in the adult patient
Carlos H. Lugo-Olivieri,Philippe A. Soyer, Elliot K. Fishman
Clin Imaging Sep-Oct 1998;22(5):346-54. doi: 10.1016/s0899-7071(98)00031-x. - "The degree of pancreatic involvement varies, ranging from accumulations of mucus in the small ducts to totally plugged ducts, which can cause atrophy of the exocrine glands and progressive fibrosis. Pancreatic dysfunction on CT is demonstrated as fatty replacement and fibrosis of the pancreas. However, there may be scattered foci of pancreatic calcifications that can be detectable on plain radiographs.”
Cystic fibrosis: Spectrum of thoracic and abdominal CT findings in the adult patient
Carlos H. Lugo-Olivieri,Philippe A. Soyer, Elliot K. Fishman
Clin Imaging Sep-Oct 1998;22(5):346-54. doi: 10.1016/s0899-7071(98)00031-x. - “CT findings in the pancreas of adult CF patients parallel the degree of pancreatic involvement, which can range from accumulations of mucus in the small ducts to totally plugged ducts. Pancreatic dysfunction on CT is demonstrated as fatty replacement and fibrosis of the pancreas. In mild or early disease, the pancreas appears heterogeneous on CT with areas of fat attenuation as well as soft tissue attenuation. In more severe cases the gland is totally replaced by fat with only the pancreatic duct visualized as a higher attenuation structure.”
Cystic fibrosis: Spectrum of thoracic and abdominal CT findings in the adult patient
Carlos H. Lugo-Olivieri,Philippe A. Soyer, Elliot K. Fishman
Clin Imaging Sep-Oct 1998;22(5):346-54. doi: 10.1016/s0899-7071(98)00031-x. - "Hepatobiliary involvement follows the same pattern as pancreatic abnormalities. Bile canaliculi are plugged by mucinous material and when this plugging is of long duration, biliary cirrhosis with diffuse hepatic nodularity may develop. Such severe hepatic involvement is seen in only about 2–5% of patients, although minor hepatic alterations, such as diffuse fatty changes, are fairly common. Hepatobiliary involvement is characterized by hepatic nodularity, compatible with cirrhosis, splenomegaly, and ascites.”
Cystic fibrosis: Spectrum of thoracic and abdominal CT findings in the adult patient
Carlos H. Lugo-Olivieri,Philippe A. Soyer, Elliot K. Fishman
Clin Imaging Sep-Oct 1998;22(5):346-54. doi: 10.1016/s0899-7071(98)00031-x. - "Fibrosing colonopathy is commonly seen in patients receiving high strength pancreatic enzyme replacement to control intestinal malabsorption. CF patients with fibrosing colonopathy often develop marked colonic strictures, and longitudinal shortening with abnormal haustra, due to submucosal fibrosis and thickening of the muscularis mucosa. Nonobstructive bowel manifestations of CF include thickened, nodular mucosal folds as seen on barium enema images; mild, generalized small bowel dilatation with diffuse distortion and thickening of the mucosal folds; duodenal ulcer; pneumatosis intestinalis; and gastroesophageal reflux.’
Cystic fibrosis: Spectrum of thoracic and abdominal CT findings in the adult patient
Carlos H. Lugo-Olivieri,Philippe A. Soyer, Elliot K. Fishman
Clin Imaging Sep-Oct 1998;22(5):346-54. doi: 10.1016/s0899-7071(98)00031-x. - “Nevertheless, there are several other etiologies for fatty replacement of the pancreas, such as the metabolic syndrome, diabetes and more rarely hereditary stenosis of the pancreatic duct, Shwachmann-Bodian-Diamond syndrome or the Johanson Blizzard syndrome.”
Uniform and diffuse fatty replacement of the pancreas revealing cystic fibrosis in a 48-year-old man: CT findings
N. Alberti et al.
Diagnostic and Interventional Imaging,Volume 98, Issue 4,2017,Pages 365-366,
- “Cystic fibrosis (CF) is the most common inher- ited fatal disease in whites, affecting approximately 1 in every 3500 children born yearly . Inheritance is autosomal recessive, and the gene mutations have been identified at a single locus on the long arm of chromosome 7. The CF gene and its product, the CF transmembrane conductance regulator (CFTR), cause abnormal chloride ion transport on the apical surface of exocrine gland epithelial cells. This results in abnormally thickened, viscous secretions, which affect multiple organ systems. Mucous plugging, bronchiectasis, and recurrent infection are the well-known pulmonary manifestations, with respiratory failure the most common cause of mortality.”
Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient Michael B. Robertson et al. RadioGraphics2006;26:679–690 - “Exocrine gland insufficiency affects 85%–90%
of all CF patients and is a result of inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat . Patients with CF diagnosed in adulthood are far more likely to be pancreatic enzyme sufficient than those with CF diagnosed in childhood. Those patients with residual pancreatic exo- crine function are prone to recurrent episodes of acute pancreatitis . Endocrine gland dysfunction is reported in 30%– 50% and is thought to result from fibrosis and gland atrophy.”
Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient Michael B. Robertson et al. RadioGraphics2006;26:679–690 - “Complete fatty replacement is the most common pancreatic finding at imaging in adult CF patients, and the mean age of fatty replacement is 17 years.”
Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient Michael B. Robertson et al. RadioGraphics2006;26:679–690 - “Pancreatic cysts are relatively common in CF patients, are usually small, measuring 1–3 mm, and are best demonstrated with T2- weighted MR imaging or MR cholangiopancreatography. Occasionally, aggregates of true epithelium-lined cysts completely replace the pancreas, a condition referred to as pancreatic cystosis. It is thought to occur when the functional secretory capacity of the gland is maintained in the presence of ductal obstruction.”
Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient Michael B. Robertson et al. RadioGraphics2006;26:679–690 - “ Pancreatic carcinoma, although uncommon in CF patients, is one of the digestive tract malignancies for which CF patients are at increased risk.”
Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient Michael B. Robertson et al. RadioGraphics2006;26:679–690
- Cystic Fibrosis: CT Findings
- Complete replacement of the pancreatic gland by fibrofatty tissue
- Enlargement of the pancreas corresponding to lipomatous pseudohypertrophy
- Atrophy of the pancreas without fatty replacement
- Cystic transformation of the pancreatic gland
- Solitary pancreatic cysts of various sizes - “ The imaging findings of pancreatic macrocysts seen in our patients are similar to those of uncomplicated pancreatic cysts from any other cause. None of our patients suffered abdominal manifestations attributable to pancreatic cysts.”
Pancreatic Cytosis in Children and Young Adults with Cystic Fibrosis: Sonographic, CT, and MRI Findings
Berrocal T et al.
AJR 2005; 184:1305-1309