| Main clinical features
|
association of CNS malformations (typically occipital meningoencephalocele), postaxial polydactyly (PD), multicystic kidney dysplasia, and ductal proliferation in the portal area of the liver
. an occipital meningo-oncephalocele with multicystic enlarged kidneys liver fibrosis, typically manifesting as impeded development of the intrahepatic biliary system and ductal plate malformation and post-axial polydactyly, cleft lip/palate, laterality defects and congenital heart malformations including dextrocardia, shortening and bowing of the long tubular bones and abnormal development of the male genitalia |