A case of Budd-Chiari syndrome in a young woman, which started probably in the last trimester of pregnancy, is described. The diagnosis was made clinically and was confirmed by inferior venacavography and on exploratory laparotomy. The possible connection of the syndrome with the pregnancy is discussed.

SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decomp...

In a patient with the Wolff-Parkinson-White syndrome, Ebstein's anomaly of the tricuspid valve, a right atrial Chiari net and a patent foramen ovale two unusual complications developed after surgical epicardial dissection combined with cryoablation of the anomalous pathway. The first complication was that ablation of the right atrial wall led to changes in interatrial pressure gradients and the...

For two patients with partial Budd-Chiari syndrome secondary to hepatocellular carcinoma, dynamic CT was evaluated. The obstructed hepatic veins were both the middle and left hepatic veins in Case 1 and the right hepatic vein in Case 2. The area affected by obstructed hepatic vein(s) was seen as low density on both unenhanced and contrast enhanced CT in Case 1 and as high density on enhanced CT...

The authors present the case of a 17 year old girl admitted to hospital for poor general state, mild scleral jaundice, deficient nutritional state, oliguria and massive ascites. She was diagnosed with Budd-Chiari syndrome: thrombosis of the left suprahepatic vein and nonocclusive thrombosis of the inferior vena cava at the level of the 12th thoracal and the lumbar vertebrae. The specific featur...

Budd Chiari syndrome or hepatic venous outflow obstruction is a complex entity with multiple etiologies and various clinical manifestations. It is often difficult to establish the diagnosis. The most common cause is a hypercoagulable state due to either genetic disorders of blood coagulation or several acquired conditions such as hematological diseases, tumors, infections, chronic inflammatory ...

Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with p...

We have previously reported our experience in inferior vena cava resection and reconstruction techniques during liver transplantation for Budd-Chiari syndrome. Herein, we present on a case that demonstrates the importance of experience in complex vascular reconstruction techniques for living donor liver transplantation. A 15-year-old boy was scheduled for living donor liver transplantation for ...

We report a case of combined hepatocellular-cholangiocarcinoma with stem cell features, cholangiolocellular subtype arising about 15 years after placement of an inferior vena cava stent for primary Budd-Chiari syndrome. Pre-surgical differentiation of the tumor from hepatocellular carcinoma was difficult because of elevated levels of alpha-fetoprotein and hypervascularity in the arterial phase....