The congenital cystic dilatation of the liver and bile ducts (CDB): our experience

The congenital cystic dilatation of the liver and bile ducts (CDB) is a very uncommon disease. It often appears in females and most frequently in paediatric patients. Its etiopathogenesis is not yet completely defined. Its evolution includes, together with several complications, the malignant transf...

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Veröffentlicht in:Annali italiani di chirurgia 2005-07, Vol.76 (4), p.367-75; discussion 375-6
Hauptverfasser: Zito, Enzo Saverio, Romagnuolo, Giuseppe, La Rocca, Francesco, Castriconi, Maurizio, De Sena, Guido, Chianese, Francesco, Bartone, Giovanni, Giuliano, Maria Elena
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container_end_page 75; discussion 375-6
container_issue 4
container_start_page 367
container_title Annali italiani di chirurgia
container_volume 76
creator Zito, Enzo Saverio
Romagnuolo, Giuseppe
La Rocca, Francesco
Castriconi, Maurizio
De Sena, Guido
Chianese, Francesco
Bartone, Giovanni
Giuliano, Maria Elena
description The congenital cystic dilatation of the liver and bile ducts (CDB) is a very uncommon disease. It often appears in females and most frequently in paediatric patients. Its etiopathogenesis is not yet completely defined. Its evolution includes, together with several complications, the malignant transformation. The Authors present the outcomes of their experience based on a series of 5 patients observed from 1991 till today. Apart from the rarity of this disease, their series is so interesting because 4 out of 5 are adult patients and the fifth one is over 15. In addition, differentiating from the literature trend, the group included only male patients. Are also discussed the most important aspects referring to nosology, epidemiology, etiopathogenesis, clinical pattern and its evolution of CDB, looking over a wide review too. The Authors extensively examine the diagnostic problems; owing to that the patients were observed in the period 1991-1999 it is necessary to clarify that some imaging methods, i.e. CSTscan and the bile duct MNR, were not yet introduced. The surgical treatment has been investigated as well, developed during its historical evolution until nowadays and our solutions for the 5 cases have been presented and discussed. In their series the Authors haven't ever observed during hospitalisation a malignant transformation.
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The surgical treatment has been investigated as well, developed during its historical evolution until nowadays and our solutions for the 5 cases have been presented and discussed. 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source MEDLINE; EZB-FREE-00999 freely available EZB journals
subjects Adolescent
Adult
Bile Duct Diseases - congenital
Bile Duct Diseases - diagnosis
Bile Duct Diseases - surgery
Caroli Disease - diagnosis
Choledochal Cyst - diagnosis
Choledochal Cyst - surgery
Cysts - congenital
Cysts - diagnosis
Cysts - surgery
Diagnosis, Differential
Dilatation, Pathologic
Female
Humans
Liver
Liver Diseases - congenital
Liver Diseases - diagnosis
Liver Diseases - surgery
Male
Middle Aged
title The congenital cystic dilatation of the liver and bile ducts (CDB): our experience
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