title: Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein.
abstract: Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and splenic venous drainage bypass the liver and drain directly into the systemic veins through various porto-systemic shunts. In this article, we illustrate a case of multiple focal nodular hyperplasia (FNH) with congenital absence of the portal vein in a male, which, to our knowledge, is the third reported case in the literature since its first description in 1793. Furthermore, we discuss the embryology of the portal vein and the Morgan and Superina classification of portosystemic anomalies, the association between portal vein agenesis and multiple FNHs, and, lastly, the use of a hepatocellular-specific MRI contrast agent as an important diagnostic tool in the confirmation of FNH.
authors.affiliation: Department of Radiology, Vancouver General Hospital, Vancouver, BC, Canada. chandler.tracy@gmail.com
authors.forename: Tracy M
authors.initials: TM
authors.lastname: Chandler
authors.forename: Manraj K
authors.initials: MK
authors.lastname: Heran
authors.forename: Silvia D
authors.initials: SD
authors.lastname: Chang
authors.forename: Aatif
authors.initials: A
authors.lastname: Parvez
authors.forename: Alison C
authors.initials: AC
authors.lastname: Harris
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000000981:diagnostic imaging; D003287:Contrast Media; D003937:Diagnosis, Differential; D020518:Focal Nodular Hyperplasia / Q000175:diagnosis* / Q000000981:diagnostic imaging; D006801:Humans; D008111:Liver Function Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000196:embryology; D014057:Tomography, X-Ray Computed; D018608:Ultrasonography, Doppler; D055815:Young Adult
chemical_list: D003287:Contrast Media