title: Meckel syndrome with Caroli disease and choledochal cysts.
abstract: Meckel syndrome is a lethal autosomal recessive disorder characterized by the triad of cystic renal dysplasia, occipital encephalocele, or other anomaly of the central nervous system and post-axial polydactyly. Malformation of the ductal plate is an integral component of Meckel syndrome. Ductal plate malformations include congenital hepatic fibrosis, biliary hamartoma, autosomal dominant polycystic liver disease, Caroli disease, and choledochal cyst. The occurrence of cystic hepatic disease, Caroli disease, and choledochal cyst have not been highlighted. This is a report of a 26-week fetus with features of Meckel syndrome, Caroli disease, and choledochal cyst.
authors.affiliation: Department of Pathology, PSG Institute of Medical Sciences & Research, PSG Hospitals, Peelamedu, Coimbatore, India. sandhyavenkatachala@yahoo.co.in
authors.forename: Sandhya
authors.initials: S
authors.lastname: Venkatachala
authors.forename: Ammu
authors.initials: A
authors.lastname: Sivaraman
mesh_terms: D000328:Adult; D001649:Bile Duct Diseases / Q000151:congenital; D016767:Caroli Disease / Q000473:pathology*; D015529:Choledochal Cyst / Q000473:pathology*; D002925:Ciliary Motility Disorders / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D004677:Encephalocele / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005333:Fetus / Q000473:pathology*; D006801:Humans; D008107:Liver Diseases / Q000151:congenital; D007690:Polycystic Kidney Diseases / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D011247:Pregnancy; D012174:Retinitis Pigmentosa