Imaging changes in the pancreas in cystic fibrosis: A retrospective evaluation of 55 cases seen over a period of 9 years

Background: Pathologic changes of the pancreas have been observed as early as the recognition of the disease termed initially "cystic fibrosis of the pancreas". Atrophy of the gland and its fatty infiltration were considered as usual features. The aim of this study was to follow-up the evolution of cystic fibrosis pancreas and to define its successive stages in correlation with the clinical, biochemical, and imaging findings. Methods: Fifty-five patients were followed up during 9 years. The patients' genetic backgrounds were systematically performed. Blood lipase levels wer e analyzed systematically at each consultation of the patients and in the e vent of bouts, of abdominal pains. Imaging using mainly echograms and tomod ensitometric scans were regularly performed: echograms every 6 months, and tomodensitometric scans every 1 to 2 years. Magnetic resonance imaging was performed in four patients. Results: Five groups of patients were identified on the basis of tomodensit ometric scan findings: normal pancreas (n = 4), incomplete lipomatosis of t he pancreas (n = 9), complete lipomatosis of the pancreas (n = 23), cystic pancreas (n = 5), macrocystic pancreas (n = 1), atrophic pancreas (n = 13). Pancreas exocrine function was not correlated with findings. Forty episode s of pancreatitis were observed in seven patients. They had bouts of abdomi nal pain and elevation of lipase levels. Five of these patients were compos ite heterozygotes (D508/other). Incomplete lipomatosis represents an interm ediate stage leading toward complete lipomatosis or toward atrophy after pa ncreatitis. Conclusions: Studies of pancreatic function should be performed routinely i n cystic fibrosis, especially in pancreatic sufficiency or in patients with normal pancreas images. Acute pancreatitis should be diagnosed and properl y identified to be differentiated from other acute abdominal syndromes occu rring in cystic fibrosis.