[Focal nodular liver lesions associated with a congenital portocaval shunt in an adult woman]

The Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. Diagnosis is usually made in childhood following the finding of liver neoplasms combined with slightly increased liver enzymes. There is a female predominance. Further findings are malformations of the heart, skeletal system and kidneys. Short-term follow-up of the liver lesions is recommended. Progressive disease can be treated by partial liver resection or transplantation. Diagnosis of the Abernethy malformation is based on various imaging modalities. We report the MRI and MRA findings of an Abernethy malformation in a 35-year-old woman with multiple liver cell adenomas and review the 32 previously published cases.     «

The Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. Diagnosis is usually made in childhood following the finding of liver neoplasms combined with slightly increased liver enzymes. There is a female predominance. Further findings are malformations of the heart, skeletal system and kidneys. Short-term follow-up of the liver lesions is recommended. Progressive disease can be treated...     »