Author(s): Merza AP, Raiser MW
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Abstract 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.
This article was published in Am J Gastroenterol
and referenced in Surgery: Current Research