Giant choledochal cyst. An unusual cause of obstructive jaundice in an adult

Soto-Darias, Iván Carmelo; García-Romera, Ángel; Rodríguez-Castellano, Desiré; Bravo-Felipe, Alberto; Alarcó-Hernández, Antonio

doi:10.1016/j.gastre.2019.03.024

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“…Prenatal diagnosis of Caroli syndrome is very rarely reported. To the best of our knowledge, only four cases have been published and all of them showed polycystic kidney and some degree of dilatation of intrahepatic bile ducts . Therefore, we report this case to describe prenatal ultrasound (US) appearance of Caroli syndrome, with the rare presence of a giant choledochal cyst.…”

Section: Introductionmentioning

confidence: 89%

Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of Caroli syndrome

Traisrisilp

Tongprasert

Wannasai

et al. 2019

J of Clinical Ultrasound

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Caroli syndrome is a developmental disorder caused by complete or partial arrest of ductal plate remodeling, leading to dilated bile ducts along with fibrosis surrounding the portal tracts. It is most commonly associated with autosomal recessive polycystic kidney (ARPKD). We report a unique case of Caroli syndrome, diagnosed prenatally at 24 weeks of gestation in a 29-year-old Thai woman. Ultrasound findings revealed the association of a fetal giant choledochal cyst with ARPKD. Autopsy findings showed ductal plate malformation, typical of Caroli syndrome, associated with giant choledocal cyst and ARPKD.

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Section: Introductionmentioning

confidence: 89%