Problemi speciali
Le dilatazioni cistiche delle vie biliari
Cystic malformations of the biliary tree
Marco Sciveres1, Francesco Cirillo1, Silvia Nastasio2, Giuseppe Maggiore2
1IsMeTT, Epatologia Pediatrica e Trapianto di Fegato, University of Pittsburgh Medical Center, Palermo
2Gastroenterologia Pediatrica, Dipartimento di Medicina Clinica e Sperimentale, Università di Pisa e Dipartimento Materno-Infantile, Azienda Ospedaliera Universitaria Pisana, Pisa
Maggio 2013 - pagg. 295 -301
Abstract
Cystic disease of the biliary tree is a rare and heterogeneous medical condition that may
be diagnosed during intrauterine life or can become symptomatic with cholestatic jaundice
or acute recurrent pancreatitis before adulthood. The etiology remains obscure although
abnormalities of the pancreaticobiliary junction may be the primary pathology in some,
with biliary dilatation following reflux of activated pancreatic secretions. Pancreaticobiliary
maljunction may however remain asymptomatic up to adulthood without or with minimal
choledochal dilatation, but carries a relevant risk of biliary carcinogenesis. The modified
Todani classification, which is based on anatomical characteristics, is the current standard
classification method of biliary cystic malformation. Most anomalies will present in
childhood with obstructive jaundice or abdominal pain; however, the classical triad of pain,
jaundice and palpable mass is not common. Complete cyst excision, where possible, and
biliary reconstruction are the treatment for most types. In case of biliary cystic disease associated
with ductal plate malformation, the therapeutic solution must be more radical and
these patients are candidate to liver transplant.
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Classificazione MeSH
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Corrispondenza: giuseppe.maggiore@unipi.it