Hypogenesis of right hepatic lobe accompanied by portal hypertension: Case report and review of 31 Japanese cases

Abstract

We report a rare case of hypogenesis of the right lobe of the liver with portal hypertension and a review of 31 cases of agenesis or hypogenesis of the right hepatic lobe reported in Japan. A 74-year-old man consulted our hospital for further examination after a mass screening for gastric cancer. On physical examination liver enlargement was palpable, but liver function tests were normal. Abdominal ultrasonography, computed tomography, technetium-99m liver scintigraphy, and endoscopic retrograde cholangiopancreatography revealed a small right hepatic lobe and moderate splenomegaly, in contrast to a hypertrophic lateral segment of the left hepatic lobe, as well as ectopic dislocation of the gallbladder. Endoscopic examination revealed esophageal varices, indicating portal hypertension. Abdominal angiography demonstrated mild shunt flow between the hepatic artery feeding from the gastroduodenal artery and the portal vein. A biopsy specimen taken from both lobes of the liver showed normal liver tissue histologically. Based on these findings, we made a definite diagnosis of hypogenesis of the right lobe of the liver with portal hypertension. The present case appears to be the first such case accompanied by portal hypertension reported in Japan.

Agenesis of Right Lobe of Liver; A Rare Anomaly- A Case Report

“…Agenesis of right lobe of liver may predispose the patient to the development of portal hypertension and oesophageal varices especially when the left lobe is enlarged. 20 In our case the patient had developed the portal hypertension along with ascites and varices were present.…”

“…20 In case of such congenital liver anomaly the gall bladder is often placed on the right side of liver against the diaphragm in a vertical position. 16 In our case the position of gall bladder is also on the right side of liver in a vertical position between the right costal arch and right dome of diaphragm.…”

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).…”

Achado incidental de agenesia do lobo hepático esquerdo em paciente com colecistite aguda

“…Nesses casos, os pacientes podem apresentar episódios de sangramento das varizes esofágicas antes dos 30 anos de idade. A provável etiologia da hipertensão portal parece ser devido à diminuição dos ramos intra-hepáticos da veia porta que não foi compensada pelo aumento do lobo esquerdo 7 . Entretanto, existem relatos de pacientes com lobo hepático esquerdo não aumentado e sem evidências de hipertensão portal.…”

“…Alguns autores sugerem que essas anomalias decorrem de fluxo portal inadequado durante a embriogênese 2,5,7,9 . Entretanto também é possível que o fator desencadeante das anomalias ocorra antes da organização do sistema hepático venoso portal definitivo 2 .…”