Interruption of the inferior vena cava with azygos termination associated with congenital absence of portal vein

Abstract

The authors report an exceptional and well-documented case of interruption of the retrohepatic segment of the inferior vena cava with an "azygos continuation", combined with absence of the portal vein. The only known combination of congenital anomalies of the inferior vena cava and the portal vein was that of an "azygos continuation" and a preduodenal portal vein. The double interruption, portal and inferior caval, may be associated with a disturbance of preferential flows induced by the left umbilical thrust. According to hemodynamic theory, the left umbilical flow is the determining factor in organogenesis of the portal vein and the retrohepatic segment of the inferior vena cava.

Congenital anomalies of the IVC—embryological perspective and clinical relevance

“…The suprahepatic IVC is derived from the cranial segment of the right vitelline vein. The retrohepatic segment is derived from an anastomosis between the cranial segment of the right subcardinal vein and the right vitelline vein (1). The infrahepatic/suprarenal IVC is derived from the right subcardinal vein (2).…”

Anatomical Variation and Its Management in Transplantation

“…via the left renal vein) into the IVC as well as interruption of the IVC (51,52). Acquired absence of the portal vein is most likely to be secondary to previous portal vein thrombosis (51,53), and may account for many of the supposed congenital absences.…”

Congenital Absence of the Portal Vein Complicating Hepatic Tumors

“…To our knowledge, only 35 cases of CAPV have been reported in the literature (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16). The age at diagnosis ranges from 1 day to 64 years.…”