title: Pathophysiologic observations and histopathologic recognition of the portal hyperperfusion or small-for-size syndrome.
abstract: In an attempt to more completely define the histopathologic features of the portal vein hyperperfusion or small-for-size syndrome (PHP/SFSS), we strictly identified 5 PHP/SFSS cases among 39 (5/39; 13%) adult living donor liver transplants (ALDLT) completed between 11/01 and 09/03. Living donor segments consisting of 3 right lobes, 1 left lobe, and 1 left lateral segment, with a mean allograft-to-recipient weight ratio (GRWR) of 1.0 +/- 0.3 (range 0.6 to 1.4), were transplanted without complications, initially, into 6 relatively healthy 25 to 63-year-old recipients. However, all recipients developed otherwise unexplained jaundice, coagulopathy, and ascites within 5 days after transplantation. Examination of sequential posttransplant biopsies and 3 failed allografts with clinicopathologic correlation was used in an attempt to reconstruct the sequence of events. Early findings included: (1) portal hyperperfusion resulting in portal vein and periportal sinusoidal endothelial denudation and focal hemorrhage into the portal tract connective tissue, which dissected into the periportal hepatic parenchyma when severe; and (2) poor hepatic arterial flow and vasospasm, which in severe cases, led to functional dearterialization, ischemic cholangitis, and parenchymal infarcts. Late sequelae in grafts surviving the initial events included small portal vein branch thrombosis with occasional luminal obliteration or recanalization, nodular regenerative hyperplasia, and biliary strictures. These findings suggest that portal hyperperfusion, venous pathology, and the arterial buffer response importantly contribute to early and late clinical and histopathologic manifestations of the small-for-size syndrome.
authors.affiliation: Department of Pathology, Thomas E. Starzl Transplant Institute, University of Pittsburgh Medical Center, University of Pittsburgh, Pittsburgh, PA 15213, USA. demetrisaj@upmc.edu
authors.forename: Anthony J
authors.initials: AJ
authors.lastname: Demetris
authors.forename: Dympna M
authors.initials: DM
authors.lastname: Kelly
authors.forename: Bijan
authors.initials: B
authors.lastname: Eghtesad
authors.forename: Paulo
authors.initials: P
authors.lastname: Fontes
authors.forename: J
authors.initials: J
authors.lastname: Wallis Marsh
authors.forename: Kusum
authors.initials: K
authors.lastname: Tom
authors.forename: Heinke P
authors.initials: HP
authors.lastname: Tan
authors.forename: Thomas
authors.initials: T
authors.lastname: Shaw-Stiffel
authors.forename: Linda
authors.initials: L
authors.lastname: Boig
authors.forename: Paula
authors.initials: P
authors.lastname: Novelli
authors.forename: Raymond
authors.initials: R
authors.lastname: Planinsic
authors.forename: John J
authors.initials: JJ
authors.lastname: Fung
authors.forename: Amadeo
authors.initials: A
authors.lastname: Marcos
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006499:Hepatic Artery / Q000503:physiopathology; D006801:Humans; D008099:Liver / Q000098:blood supply; D008102:Liver Circulation / Q000502:physiology; D008107:Liver Diseases / Q000209:etiology* / Q000503:physiopathology* / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects* / Q000473:pathology; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D011168:Portal System / Q000503:physiopathology*; D011169:Portal Vein / Q000503:physiopathology; D011183:Postoperative Complications / Q000503:physiopathology*; D020858:Tissue and Organ Harvesting