The authors refer to three cases of congenital dilatation of the biliary system in an adult, an extremely rare pathology in the West, with no specific symptomatology, and therefore often discovered accidentally and unexpectedly The first patient had suffered from unexplained periods of fever since childhood, the second had only complained of vague abdominal pains a years before diagnosis; for the third, a Romanian woman, it was the first time she complained of a biliary colic. All the patients had an Ultrasound, a CT scan and a MRCP (magnetic resonance cholangiopancretography). Based on the Todani classification, the first and the third case belongs to type IV A, while the second is a type I. In all three patients the pathology was resolved due to an operation entailing disconnection of the biliary-pancreatic tract, with complete surgical removal of the dilated bile ducts followed by a Roux-en-Y hepatico-jejunostomy. Nevertheless the procedures undertaken are not immune to criticism: in the first and second case the diagnostic imagery had not immediately produced a clear interpretation, leading to a delay in the diagnosis and the execution in the first of a not anodyne ERCP; in the third case a colecistectomy was performed as it were as an intermediary measure; finally there was probably omitted a thorough explanation to the patient with regard to a pathology deserving of a careful follow-up for the possible recidivist of the infectious phenomena and above all the onset of the dangerous degeneration carcinomatosa.
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