Portal venous deprivation in patients with portosystemic shunts and its effect on liver tumors

Abstract

AimsTo outline the use of radiological investigations in patients with congenital portosystemic shunts (CPS) and to assess the degree of portal venous deprivation in relation to the development of liver tumors. MethodsSingle center retrospective cohort study of all patients with CPS referred from 1990 to 2016. Radiological investigations were reviewed for the presence of intrahepatic portal veins. Two groups were defined: Group 1 — without evidence of preservation of an intrahepatic portal venous system and, Group 2 — with evidence of intrahepatic portal venous flow. These groups were compared for the development of liver tumors, serum ammonia, and nature of subsequent surgical intervention. The ratio of infraceliac to supraceliac aortic diameter and hepatic enhancement in biphasic CT scans were also used to infer hepatic arterial in-flow and compared to age matched controls. Nonparametric tests were used throughout. A P value of 0.05 was considered significant. Data are quoted as median (IQR). Results45 patients (Group 1, n = 12: Group 2, n = 33) were investigated for CPS at a median age of 8 months (1 month–14 years). Liver tumors were more common in Group 1 than Group 2 [11/12 (92%) versus 10/33 (29%); P < 0.001]. Aortic ratio was significantly lower in patients with CPS compared to control (0.82 versus 0.96; P < 0.001), but there was no difference between patients with or without tumors (0.82 versus 0.82; P = 0.52). Enhancement of the liver parenchyma was greater in portal venous rather than arterial phases by a median difference of 28 (15–50) Hounsfield units in controls, compared to 15 (8.5–23.5) in CPS patients (P = 0.04). A single stage closure was possible in 2/6 (33%) operated patients in Group 1 and 14/20 (70%) in Group 2 (P = 0.32). ConclusionsPatients without radiological evidence of intrahepatic portal venous flow were significantly more likely to have associated hepatic tumors with a relative risk 3.1. Levels of evidenceThis manuscript includes data that are III and IV Levels-of-Evidence.