Congenital Cystic Lesions of the Biliary Tree

Diagnostic challenge of large congenital liver cyst in the newborn

“…8 Caroli syndrome is more common than Caroli disease alone. 8 Caroli disease is usually sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner. Caroli disease occasionally presents with familial clustering, and chromosomal banding studies have found structural rearrangement of genes or unbalanced translocation between chromosomes 3 and 8.…”

“…9 Caroli disease, if complicated by biliary ductal stricture, stones, cholangitis or liver atrophy, is managed with partial hepatectomy and Roux-en-Y hepaticojejunostomy. 8 Liver transplantation may be indicated in severe cases of refractory or chronic cholangitis, liver failure, or malignant transformation. 10 In the present patient, the presentation was not suggestive of Caroli disease in the beginning but the progressive dilatation of the right intrahepatic bile ducts observed over time with normal caliber extrahepatic bile ducts made us reconsider the diagnosis.…”

“…8,9 We describe a case of CPA on arrival that was due to secondary PHA associated with bilateral infected hydronephrosis. In this case, successful resuscitation and intensive care resulted in a …”

Choledochal cysts: sequel of the malformed embryological ductal plate?

“…The embryological ductal plate is a double-layered tubular column of precursor hepatoblasts which surround the portal vein radicle and progressively remodel to form the future bile ducts ( Fig. 1) [2][3][4][5][6]. A partial or complete arrest of normal remodeling of the embryological ductal plate results in persistence of embryonic cystic structures ( Fig.…”

“…During embryogenesis, it is the level of insult (intrahepatic small, medium, or large-sized bile ducts and/or involvement of the extrahepatic biliary tree) that determines the type of ductal plate disorder (Fig. 3) [2][3][4][5][6]. Choledochal cysts are considered to be a ductal plate malformation of the extrahepatic bile duct with or without intrahepatic bile duct involvement [2][3][4][5].…”