MR imaging of pancreas in cystic fibrosis

Murayama, Sadayuki; Robinson, Arvin E.; Mulvihill, Denise; Goyco, P. G.; Beckerman, Robert C.; Hines, M. R.; Stallworth, J. Manly

doi:10.1007/bf02011384

Cited by 22 publications

(8 citation statements)

References 10 publications

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“…This finding correlates well with findings at pathologic analysis of mature adipose tissue separated by fibrous septa and containing isolated clusters of islets of Langerhans and a few residual pancreatic ductules (14). The highsignal-intensity appearance of complete fatty replacement on T1-weighted images correlates relatively well with clinically advanced pancreatic disease (8,10), and a relationship has been demonstrated between reduction in T1 values and the degree of clinical compromise due to exocrine insufficiency (12). However, some cystic fibrosis patients with a normal pancreas at MR imaging have clinically apparent exocrine insufficiency (9,10); consequently, the clinical utility of this observation is limited.…”

Section: Pancreatic Manifestationssupporting

confidence: 76%

“…Complete fatty replacement is the most common MR imaging finding in pancreatic abnormalities (8)(9)(10)(11)(12). This finding correlates well with findings at pathologic analysis of mature adipose tissue separated by fibrous septa and containing isolated clusters of islets of Langerhans and a few residual pancreatic ductules (14).…”

Section: Pancreatic Manifestationssupporting

confidence: 73%

“…Apparent diffuse pancreatic fibrosis, which has very low signal intensity with all imaging sequences, was reported in three of 18 patients (17%) by Murayama et al (10). To our knowledge, however, this disease entity has not been described at MR imaging by any other authors.…”

Section: Pancreatic Manifestationsmentioning

confidence: 55%

“…However, some cystic fibrosis patients with a normal pancreas at MR imaging have clinically apparent exocrine insufficiency (9,10); consequently, the clinical utility of this observation is limited. At MR imaging, the reported prevalence of diffuse fatty change varies from 51% to 75% (8)(9)(10)(11)(12), with partial fatty replacement seen in 7%-29% of cases and pancreatic atrophy in 27%-35% (9,11,12). In an autopsy series of 27 cases of cystic fibrosis that spanned a period of 5 years, diffuse fatty replacement was reported in 15 cases (56%) (14).…”

Section: Pancreatic Manifestationsmentioning

confidence: 99%

“…The severity of the pancreatic and hepatobiliary changes has been shown to increase with age (3), and advances in the management of pulmonary complications of cystic fibrosis, along with better treatment of patients with pancreatic exocrine insufficiency, have led to increased survival rates. Several studies have described the magnetic resonance (MR) imaging appearances of pancreatic disease in patients with cystic fibrosis (8)(9)(10)(11)(12), although hepatobiliary manifestations at MR imaging are less well documented (9). Several studies have described the magnetic resonance (MR) imaging appearances of pancreatic disease in patients with cystic fibrosis (8)(9)(10)(11)(12), although hepatobiliary manifestations at MR imaging are less well documented (9).…”

Section: Introductionmentioning

confidence: 99%

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Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances

King

Scurr²,

Murugan³

et al. 2000

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Magnetic resonance (MR) imaging and MR cholangiopancreatography are useful, noninvasive techniques for the assessment of pancreatic and hepatobiliary complications in cystic fibrosis. Abnormalities of the pancreas in cystic fibrosis are typically characterized by fat deposition, which has increased signal intensity on T1-weighted MR images, and pancreatic fibrosis, which has low signal intensity on both T1-and T2weighted images. Pancreatic cysts are a relatively common finding; these cysts are typically quite small but are well demonstrated at MR imaging and MR cholangiopancreatography. Pancreatic duct abnormalities are also occasionally seen. Hepatic manifestations range from hepatomegaly and diffuse fatty infiltration to severe cirrhosis with fibrotic change, regenerative nodules, and portal hypertension. Splenomegaly is often characterized by siderotic nodules that manifest as multiple focal areas of abnormal low signal intensity within the spleen. Biliary manifestations include cholelithiasis, stricturization, and narrowing or dilatation of intra-and extrahepatic bile ducts. Gallbladder abnormalities including microgallbladder are also readily demonstrated. MR cholangiopancreatography can be used to help determine the presence and severity of biliary complications without resorting to more invasive procedures and, in conjunction with MR imaging, may prove useful in the assessment of patients with cystic fibrosis who present with abdominal symptoms that suggest hepatobiliary involvement.Abbreviations: FLASH = fast low-angle shot, RARE = rapid acquisition with relaxation enhancement

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Section: Pancreatic Manifestationssupporting

confidence: 76%

Section: Pancreatic Manifestationssupporting

confidence: 73%

Section: Pancreatic Manifestationsmentioning

confidence: 55%

Section: Pancreatic Manifestationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances

King

Scurr²,

Murugan³

et al. 2000

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Magnetic Resonance Imaging for Diagnosis of Shwachman's Syndrome

Lacaille,

Mani,

Brunelle

et al. 1996

J. pediatr. gastroenterol. nutr.

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SummaryShwachman's syndrome is a rare disease characterized by the association of chronic diarrhea due to exocrine pancreatic insufficiency, metaphyseal dysostosis, and neutropenia. The diagnosis requires demonstration of lipomatosis, or fatty replacement of the pancreas, which is the typical pathological feature of the disease. Magnetic resonance imaging (MRI) was performed in 13 patients with exocrine pancreatic insufficiency, 7 with Shwachman's syndrome, 2 with Pearson's syndrome, 1 with normal sweat test later diagnosed as cystic fibrosis, and 3 without identified syndrome, and in 7 control children. Ultrasonography in the patients did not differentiate between atrophy and lipomatosis and could not be performed in 3. MRI visulized the pancreas in all. The same image was noted in all patients with Shwachman's syndrome with a normal‐sized or enlarged pancreas, a hyperintense signal on T1‐ and T2‐weighted image, and a null signal on short time inversion recovery (STIR)‐weighted image, characteristic of fat. In all other patients, the findings were very different: The pancreas was a small structure surrounded with fat. In 1 patient without identified syndrome, the pancreas appeared to be partially replaced with fat. MRI is an excellent imaging technique to correlate the nature of a tissue and its radiological representation, especially fat, which gives a very typical signal. In our brief series of patients with Shwachman's syndrome, MRI had 100% positive predictive value in demonstrating lipomatosis. In atypical cases of pancreatic insufficiency in which some of the clinical features of Shwachman's syndrome are absent, MRI is an invaluable aid in the diagnostic procedure.

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Exocrine Pancreatic Insufficiency and Nutritional Complications

Mandalia

DiMagno

2020

Respiratory Medicine

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MR imaging of pancreas in cystic fibrosis

Cited by 22 publications

References 10 publications

Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances

Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances

Magnetic Resonance Imaging for Diagnosis of Shwachman's Syndrome

Exocrine Pancreatic Insufficiency and Nutritional Complications

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