Shirai et al. Journal of Medical Case Reports 2011, 5:124 http://www.jmedicalcasereports.com/content/5/1/124 Figure 2 Images showing various features of re-stenosis after the first PTA. One year after the first PTA, angiograms of the IVC showed severe stricture of the IVC at the level of the hepatic veins (A: black arrow). Doppler US showed that the RHV and MHV were completely occluded with regurgitation in MHV, and thick shunt vessels from MHV to RHV were noticed (B: white arrow). status compared to his status before PTA and surgery (Table 1). Moreover, multi-slice CT imaging showed that the congestion of the liver had completely disap- peared with normal flow inside the vessels (Figure 4). Discussion BCS is a clinical condition characterized by hepatic venous outflow obstruction due to various underlying causes [1]. BCS is uncommon, with an incidence rate of about one in two and a half million people per year, and current knowledge about its etiology is still insufficient [2]. Most western cases of BCS have a known etiology such as protein-C or -S deficiency, and myeloprolifera- tive diseases, whereas most Japanese cases are idiopathic including acute BCS [4]. The Japanese Ministry of Health and Welfare Research Committee on Aberrant Portal Blood Flow carried out an epidemiological survey and clinical study on BCS, and reported that the Page 3 of 5 After surgery 1.0 3.3 0.74 1.0 6 Table 1 Laboratory data of the patient Before PTA Bilirubin (mg/dL) 1.8 Albumin (g/dL) 2.5 PT-INR 0.99 Creatinine (mg/dL) 0.7 MELD Score 9 Child-Pugh Score 8 Before surgery 10.6 2.0 1.86 1.3 25 14 majority of BCS patients in Japan are idiopathic, with an obstructing lesion in the IVC [5]. This was also the case in our patient. It has been reported that stenosis of the IVC at the level of the liver is a common cause of non- cirrhotic ascites, which develop within a short period in developing countries [6]. These patients generally have a poor nutritional status, which is now recognized as one of the causes of an immune-compromised state. Patients with such status frequently have thrombophlebitis of the IVC at the level of the liver, which probably results in stenosis or thrombosis of the IVC. Before the onset of symptoms, our patient suffered from alcohol abuse and did not have sufficient nutrition. It may be possible that he had a similar situation of malnutrition, which led to temporary acute stenosis of the IVC. Clinically, in most cases of BCS, leg edema, abdominal fullness, and subcutaneous venous dilatation over the trunk gradually appear. In some cases, severe clinical manifestations such as progressive refractory ascites and rupture of esophageal varices occur within a short per- iod; a condition classified as acute BCS [7]. Acute BCS often results in fulminant liver failure due to severe liver congestion. In our case, the clinical manifestations developed within a short period accompanied with severe liver dysfunction, which could be classified as acute BCS. Although the general prognosis of acute BCS is poor, the outcome may be fair with accurate early diagnosis and effective treatment. We were able to make an early diagnosis of acute BCS and identify the stricture Figure 3 Photomicrographs of the recipient liver at the time of living donor liver transplantation. (A) Macroscopically, the recipient liver was swollen and reddish, indicating severe secondary congestion. (B) Microscopically, there was evidence of impaired blood outflow, including congestion, coagulative necrosis, and loss of hepatocytes without the severe inflammatory infiltrates associated with fibrosis development. Original magnification is ×40. Figure 4 Multi-slice CT imaging one year after the transplantation. The sagittal (A) and enhanced images (B) show that the congestion of the liver completely disappeared with normal flow inside the vessels.