Congenital absence of the portal vein in a boy

Abstract

Congenital absence of the portal vein (CAPV) is a malformation that is generally thought to be limited to females. We encountered an 11-year-old boy with this malformation. In 17 previously reported cases of CAPV, 2 were male. Three male patients, including our case, were Abernethy type Ib malformation. They had no associated liver tumour and two had no additional congenital abnormality. Conversely, 13 of the 15 female patients had congenital abnormalities and 6 had liver mass lesions.

Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin–Johnson syndrome

“…This anomaly has been reported mostly as a congenital absence of the portal vein in association with liver tumors and other congenital anomalies [1][2][3][4][5][6][7][8][9][10]. In this report, we present the case of a patient with congenital extrahepatic portocaval shunt in association with Dubin-Johnson syndrome who developed hyperplastic nodules in the liver.…”

Congenital Absence of the Portal Vein with Splenomegaly and Hypersplenism in a Young Woman

“…To our knowledge, 27 cases have been described as summarized previously [1, 2, 3, 4, 5, 6, 7, 8]. There has been recent increased interest in this anomaly because, of the 27 cases, more than one third has been reported since 1999 – all of them in pediatric, radiology or pathology journals [2, 3, 4, 5, 6, 7, 8]. Almost all patients had liver or cardiac anomalies.…”

Insight into congenital absence of the portal vein: Is it rare?

“…Although most cases do not possess any liver abnormalities [1,3,9,27,[43][44][45][47][48][49]54,57] , CAPV patients suffer from different levels of LD [2][3][4]9,15,16,20,23,24,[26][27][28][29]34,37,38,44,47,55,56] possibly due to the lack of portal flow. Hepatic encephalopathy, hepatopulmonary syndrome (HPS), and hepatorenal syndrome are closely related to metabolic disorder because of liver lesions, including hyperammonemia [3,27,41,59] and g alactosemia [20,27] . Toxic compounds produced in the digestion process only bypass the liver into the systemic circulation in CAPV patients, and are prone to cause hepatic encephalopathy [23,26,27,34,40,49,56] .…”

“…The associated extrahepatic portosystemic shunts may occur due to the persistent subcardinohepatic anastomosis with the vitelline veins. The subcardinohepatic anastomosis connects the vitelline vein that develops into the portal system and the right subcardinal vein that develops into the renal segment of the IVC, as well as forms the hepatic segment of the IVC, thus accounting for the high incidence of draining points at the suprarenal IVC [3] . Cardiac malformations are frequently observed in patients with CAPV [11,72] , the close relationship between the development of vitelline veins and the heart in embryonic life may be responsible for the association between cardiovascular malformations and CAPV [12,40,45] .…”

“…Furthermore, type Ⅰ is sub-classified into types Ⅰa and Ⅰb depending on the anatomy of portal vein. In type Ⅰa, the splenic vein (SV) and superior mesenteric vein (SMV) drain separately in type Ⅰa, while both drain together in type Ⅰb after uniting to form a common trunk [3] . Howard and Davenport [4] suggested that the congenital diversion of portal blood away from the liver, by either an end-to-side or a side-to-side shunt, is known as the Abernethy malformation.…”