Biliary tract manifestations of the Marfan syndrome.
The diagnosis of the Marfan syndrome rests on the four criteria of characteristic musculoskeletal, cardiovascular, ocular, and familial features. Biliary tract manifestations have not been described. A case of Marfan syndrome with recurrent biliary obstruction is presented. Endoscopic retrograde cholangiograms presented a grossly ectatic biliary system which is postulated to be an unrecognized manifestation of this syndrome. In addition, the patient developed choledocholithiasis, which may have resulted from biliary stasis secondary to bile duct ectasia. Biliary obstruction developed in the presence and absence of choledocholithiasis, the latter apparently through kinking of the ectatic common bile duct. Obstruction was clinically relieved by biliary endoprosthesis. The presence of a Zenker's diverticulum in this patient, also unreported in the Marfan syndrome, is of incidental interest.
D000368:Aged; D002779:Cholestasis / Q000503:physiopathology*; D003137:Common Bile Duct Diseases / Q000503:physiopathology*; D005260:Female; D042882:Gallstones / Q000503:physiopathology; D006801:Humans; D008382:Marfan Syndrome / Q000175:diagnosis / Q000503:physiopathology*