Congenital absence of the portal vein: Two cases and a proposed classification system for portasystemic vascular anomalies

Glyn Morgan, Riccardo Superina*

*Corresponding author for this work

Research output: Contribution to journalArticle

267 Scopus citations

Abstract

Congenital absence of the portal vein (CAPV) is an unusual condition that often is associated with other anomalies. This is the first report of reduced-size liver transplant in a patient with CAPV. Because the presence of this rare congenital portasystemic shunt, there was no portal-systemic pressure differential, and thus an absence of collateral vessels in the pretransplant state. As a result, surgery was complicated by severe mesenteric edema caused by an increase in portal pressure when the allograft was implanted. The morbidity associated with CAPV usually results from associated conditions, but if transplantation is necessary, careful management of mesenteric congestion is crucial to success. The authors' experience and a review of the literature indicate that the CAPV can be classified into one of two groups of portasystemic anomalies.

Original languageEnglish (US)
Pages (from-to)1239-1241
Number of pages3
JournalJournal of Pediatric Surgery
Volume29
Issue number9
DOIs
StatePublished - Jan 1 1994

Keywords

  • Portal vein, anomalies
  • liver transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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