Congenital absence of the portal vein—Case report and a review of literature

Abstract

Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and the splenic venous drainage bypass the liver and drain into systemic veins through various venous shunts. To our knowledge, we have reviewed all 83 cases of CAPV, since first described in 1793. This equates to a rate of almost 2.5 cases per year over the last 30 years. Morgan and Superina (1994, J. Pediatr. Surg. 29:1239-1241) proposed the following classification of portosystemic anomalies; either the liver is not perfused with portal blood because of a complete shunt (Type I) or the liver is perfused with portal blood due to the presence of a partial shunt (Type II). In our case, abdominal venous blood drained into the suprarenal inferior vena cava via the left renal vein and dilated left gastric veins. After analyzing all reported cases, we recognize that more than 65% of patients are females and more than 30% of all published cases had been diagnosed by the age of 5 years. Additional anomalies are common in CAPV. In the reported cases, more then 22% of patients had congenital heart disease. Other commonly found anomalies include abnormalities of the spleen, urinary and male genital tract, brain as well as skeletal anomalies. Hepatic changes such as focal nodular hyperplasia, hepatocellular carcinoma, and hepatoblastoma are diagnosed in more then 40% of patients. This article also illustrates the radiological findings of CAPV. Radiological evaluation by ultrasound, CT, and MRI is helpful to detect coexisting abnormalities.

Congenital absence of the portal vein complicated by hepatocellular carcinoma in the liver of an adult woman: review of imaging, literature and management

“…Hepatic changes such as FNH, HCC and hepatoblastoma were seen in 40% of cases [1] . In this case, the patient presented with HCC.…”

“…To date, 4 cases of patients with CAPV have been reported to have HCC [1] . One case was reported in a 14-year-old female, however nature of the review focused on intestinal flora compensating to result in normal ammonia levels rather than tumor description and presentation [9] .…”

“…In conventional anatomy, the splenic vein (SV) and the superior mesenteric vein (SMV) join to form the PV. The PV is then subdivided into right and left branches, which form small vessels throughout the liver that eventually drain into the sinus venosus [1] . During embryological development, the PV originates from the right and left vitelline veins between gestational weeks 4 to 10.…”

Ausencia Congénita de la Vena Porta Hepática Asociada a Malrotación Intestinal y a Vena Cava Inferior Izquierda: Reporte de Caso

“…Hasta 2010 se habían reportado sólo 84 casos (Mistinova et al, 2010), sus edades han fluctuado entre recién nacido y 64 años, afectando principalmente a mujeres (65 %) con un alto índice de morbimortalidad por falla hepática (Mistinova et al). Un reciente reporte de Hao et al (2015), eleva esta cifra a 101 ca-sos en el mundo desde la primera notificación en 1793.…”

“…La ausencia congénita de la vena porta hepática es una malformación en extremo rara, situación que afecta el desarrollo de la porción infra hepática de la vena vitelina derecha hacia el final del período embrionario. Hasta 2010 se habían reportado sólo 84 casos (Mistinova et al, 2010), sus edades han fluctuado entre recién nacido y 64 años, afectando principalmente a mujeres (65 %) con un alto índice de morbimortalidad por falla hepática (Mistinova et al). Un reciente reporte de Hao et al (2015), eleva esta cifra a 101 ca-…”