Hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis

Abstract

A 22-year-old man was referred to our hospital because of thrombocytopenia. Abdominal computed tomography (CT) revealed hypoplasia of the right hepatic lobe, the development of porto-systemic collateral vessels, splenomegaly and a periaortic soft-tissue mass. Laboratory tests and needle liver biopsy indicated no evidence of liver cirrhosis. Consequently, a diagnosis of hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis was established. Portal hypertension and hypersplenism was thought to be the cause of the thrombocytopenia. CT arterioportography revealed that anomalies of the portal venous system could have resulted in the hypoplasia of the right hepatic lobe. This is the first report describing hypoplasia of the right hepatic lobe accompanied by supervening idiopathic retroperitoneal fibrosis.

Bifid liver accompanied by hepatolithiasis

“…We speculate that certain undefined embryological factors may promote the connective tissue to encapsulate the liver cell cord before the fusion of the two developing liver lobes. Clinically, while most liver variations are insignificant, the following complications have been reported in major liver abnormalities: ectopic gallbladder, portal hypertension, splenomegaly, cholecystolithiasis, cholangiolithiasis, and bile duct carcinoma (Hsu et al, 1997;Inoue et al, 1997;Maeda et al, 1998;Sato et al, 1998;Saigusa et al, 2001;Gathwala and Sen, 2003;Hisatomi et al, 2004;Noritomi et al, 2004;Yamaguchi et al, 2004). Accessory hepatic lobe and mobile liver might manifest diaphragmatic hernia, torsion, or clinical misdiagnosis (Robertson et al, 2006;Othman et al, 2008;Han and Soylu, 2009).…”

Imaging and intervention of retroperitoneal fibrosis

“…The condition has also been described to be associated with a wide variety of systemic and autoimmune diseases (Table 1). 7–17 Retroperitoneal disease is also associated with malignant disease 18 . Lymphoproliferative disorders, particularly Hodgkin’s disease, sarcomas, carcinoid tumours and carcinomas of the breast, lung, stomach, colon, bladder and prostate have all been associated with retroperitoneal fibrosis 7 .…”