Shirai et al. Journal of Medical Case Reports 2011, 5:124 http://www.jmedicalcasereports.com/content/5/1/124 frequently leads to severe life-threatening acute liver failure. We report the case of a patient with recurrent BCS in whom the first PTA was successful, but the sec- ond PTA failed to re-open the stenosis of the IVC. Since our patient showed manifestations of acute liver failure, we decided to perform salvage living donor liver transplantation (LDLT) from his brother. The transplan- tation was successful, and all clinical manifestations were remarkably alleviated. Case presentation A Japanese 26-year-old man without any relevant medi- cal history had progressive refractory massive ascites of unknown origin. He shortly developed hematemesis. An endoscopy revealed spurting bleeding from esophageal varices, and he was referred to and then admitted into our hospital. He underwent emergency endoscopic injection sclerotherapy (EIS). The laboratory data on admission showed severe liver dysfunction without any suggestion of chronic liver diseases, such as cirrhosis. The hepatitis viral markers, including hepatitis B and C, were all negative. Color Doppler ultrasonography (US) revealed a heterogeneous enlarged liver, shunt vessels between his middle and right hepatic veins (MHV and RHV, respectively), and retrograde flow in MHV. Com- puted tomography (CT) is a more sensitive modality for showing parenchymal abnormalities. In our case, enhanced CT revealed much clearer heterogeneity of the liver with decreased attenuation in the periphery. It also showed shunt vessels between MHV and RHV. The latter was markedly dilated, and it seemed to have some flow into the IVC, whereas his left hepatic vein was obstructed. Furthermore, sagittal CT images suggested severe stricture of his IVC at the level of the liver. Angiograms confirmed marked narrowing of his IVC at the level of the liver (Figure 1A). Magnetic resonance imaging (MRI) revealed findings similar to those of the CT. From these findings, and the rapid onset of the clin- ical manifestations, we diagnosed this patient as having acute BCS. Within one week, our patient developed massive hematemesis again. Subsequently, he showed manifestations of acute liver failure, possibly due to “shock liver”. Since we could not find a donor for deceased liver donor transplantation (DLDT), we decided to perform PTA first. A balloon catheter was inserted into his right femoral vein, and the stricture of the IVC at the level of his liver was dilated using a 12 × 40 mm balloon catheter. After PTA, the pressure differ- ence between his IVC and his right atrium decreased from 250 mmH2O to 50 mmH2O, and color Doppler US showed that the blood flow in his MHV had recov- ered to the normal antegrade flow. His clinical manifes- tations, such as ascites, dramatically improved, and his liver function also gradually recovered with time. About Page 2 of 5 one month after the PTA, an enhanced 3 D CT exami- nation revealed that the heterogeneity of the liver, the IVC stricture at the level of the liver, and the liver dys- function had markedly improved. The endoscopic find- ings of the varices were also alleviated. Our patient was discharged, and regular follow-up was performed. After one year, color Doppler US showed that the hepatic venous outflow had deteriorated again, despite our patient being given an anticoagulant after the initial PTA. Sagittal CT images again showed severe stricture of his IVC at the level of the hepatic veins. Our patient then developed massive hematemesis again due to a rupture of the varices. We performed emergency EIS, to control the bleeding. Although we performed PTA again, balloon dilatation was not effective this time. Doppler US showed that his RHV and MHV were com- pletely occluded with regurgitation in his MHV, and thick shunt vessels from his MHV to RHV were noticed (Figure 2). His clinical condition deteriorated over sev- eral weeks, including the development of severe hepatic failure. This time, our patient’s brother volunteered to become a donor for LDLT. After the physician and sur- geon obtained written informed consent from the donor, LDLT was performed using the left lobe of the brother’s liver. Macroscopically, the recipient liver was swollen and reddish, indicating severe secondary conges- tion (Figure 3A). Microscopic examination showed evi- dence of impaired blood outflow, including congestion, coagulative necrosis, and loss of hepatocytes without severe inflammatory infiltrates associated with fibrosis development (Figure 3B). After the operation, our patient’s general condition improved and his manifesta- tions were dramatically alleviated. One year after the LDLT, our patient was discharged with his liver func- tioning well. The laboratory data indicated a healthy Figure 1 Angiograms of the IVC before and after PTA. The IVC showed marked narrowing before PTA (A: black arrow). A balloon catheter was inserted into the right femoral vein, and the stricture of the IVC at the level of the liver was dilated using a 12 × 40 mm balloon catheter. After the first PTA, the stenosis was significantly improved (B: white arrow).