Werner syndrome is a rare autosomal recessive disorder characterized by clinical signs of premature aging, short stature, scleroderma-like skin changes, endocrine abnormalities, cataracts, and an increased incidence of malignancies. We report on a 48-year-old woman with Werner syndrome associated with intracranial meningiomas who had extensive musculoskeletal manifestations including osteoporosis of the extremities, extensive tendinopathy about the ankles, osteomyelitis of the phalanges of the first left toe, abundant soft-tissue calcification, and two dense ossified soft-tissue masses, with cortical bone and trabeculae arising from the posterosuperior aspect of the calcanei and extending into Kager fat pads. A review of previous descriptions of the radiological abnormalities of Werner syndrome indicates that the presence of soft-tissue calcifications has either not been noted or been mentioned only briefly. Moreover, there is no mention of bony masses associated with Werner syndrome in the world literature, and this would appear to be the first report of this kind.
Cystic dystrophy of the duodenal wall in heterotopic pancreas, recently described as paraduodenal pancreatitis, is a rare condition characterized by multiple cysts or pseudocysts located in the submucosa or muscularis propria of a thickened duodenal wall. They result from multiple episodes of obstruction of the small ducts in aberrant pancreatic islets. Cystic dystrophy of the duodenal wall in heterotopic pancreas usually affects alcoholic males, but here we present the case of a female without a history of alcohol abuse who was referred to our emergency department with abdominal pain and vomiting. She was treated by a pancreas-preserving surgical approach, thanks to a proper pre-operative differential diagnosis. Even though differentiating this benign condition from pancreatic cancer is a challenge, some characteristic findings on multidetector CT scan and MRI/MR cholangiopancreatography, such as a thickened duodenal wall containing cysts and sheet-like tissues in the pancreaticoduodenal groove, could lead to the correct diagnosis.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.