Congenital Absence of the Portal Vein

Abstract

A 14-year-old girl presented at the hospital after discovering an abdominal tumor. CT scan and ultrasonography indicated a hepatic tumor and also revealed the absence of the portal vein. The patient was admitted to excise the hepatic tumor. It was found that the venous blood from the small intestines flowed into the left renal vein and then emptied directly into the inferior vena cava. A tumor extending from the right lobe through the middle portion of the liver was excised. The postoperative course was satisfactory and marked regeneration of the residual hepatic tissue was observed. Also the blood level of ammonia in the superior mesenteric vein was low, approximately 120 micrograms/dl, compared to the normal value of 350 micrograms/dl in the portal vein. This low blood level may indicate the presence of some homeostatic control mechanism.

Extrahepatic Portosystemic Venous Shunt without Portal Hypertension.

“…That is, hyperammonemia induced encephalopathy regardless of age. Nakasaki et al (16) reported that production of ammoniawas suppressed in the intestine as homeostasis in a case of congenital absence of the portal vein. They investigated the patient' s intestinal bacterial flora, and discovered distinct differences from the normal flora with respect to both varieties and numbers of the intestinal flora.…”

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

“…Several patients have been found to have combined hepatocellular adenoma [31,36,37] . These are generally considered benign parenchymatous lesions, and also have the potential to deteriorate into hepatoblastoma [26,28] and hepatocellular carcinoma (HCC) [12,20,38,39] .…”

“…Congenital cardiac diseases including atrial septal defect (ASD) [2,21,26,28,37,44,45] or patent foramen ovale [43] , ventricular septal defect (VSD) [16,17,21,26,31,40] , and patent ductus arterious (PDA) [12,23,43,44] are frequently observed along with CAPV. Bellah et al [45] have also detected a fatal congenital hypertrophic cardiomyopathy with ultrasound in an infant.…”

“…The urinogenital system including cystic dysplasia of the kidneys [22,24] , bilateral ureteropelvic obstruction of the kidneys [45] , vesicoureteral reflux [33] , crossed fuses renal ectopia [33,40] , and hypospadias [56] , is also involved. Nonfunctioning pancreatic tumor [25] , ulcerative colitis(UC) [33] , juvenile polyposis [21] , inguinal hernia [23,37] , and even situs inversus viscerum [2,4] , are sporadically observed in CAPV patients.…”

“…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] .…”