Meckel syndrome

Meckel syndrome (MKS) is a rare, lethal, genetic, multiple congenital anomaly disorder characterized by the triad of brain malformation (mainly occipital encephalocele), large polycystic kidneys, and polydactyly, as well as associated abnormalities that may include cleft lip/palate, cardiac and genital anomalies, central nervous system (CNS) malformations, liver fibrosis, and bone dysplasia.

Portal fibrosis

Fibroblast proliferation and fiber expansion from the portal areas to the lobule.

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PMID (PMCID)
3130875	MIXED_SAMPLE	Infant, Newborn
	Pathology of renal and hepatic anomalies in Meckel syndrome.
	Blankenberg TA, Ruebner BH, Ellis WG, Bernstein J, Dimmick JE. Am J Med Genet Suppl. 1987;3:395-410.
	The livers of the Meckel syndrome fetuses were compared to those of age-matched control fetuses, and it was concluded that the hepatic abnormality represents an arrested development of the intrahepatic biliary system at the stage of biliary cylinders with varying degrees of reactive bile duct proliferation, bile duct dilatation, portal fibrosis, and portal fibrous vascular obliteration superimposed on the arrested pattern.