Blue rubber bleb naevus syndrome is an uncommon disorder whereby patients possess cutaneous vascular malformations. Cranial malformations have been rarely reported in the literature. We report the first ever case of a patient with blue rubber bleb naevus syndrome (BRBNS), who was found to have a symptomatic Chiari malformation and prominent subcutaneous vascular malformations in communication w...

Introduction: Budd-Chiari syndrome is a rare condition which can be defined as hepatic venous outflow obstruction occurring at any level between the small veins and junction of inferior vena cava right atrium. Symptoms may vary from mild or even asymptomatic for many years to fulminant failure. Imaging mainstay diagnosis BCS. Laboratory ematological tests are invaluable in recognizing hematolog...

Focal nodular hyperplasia and nodular regenerative hyperplasia are occasionally seen in patients with hepatic venous outflow obstruction as a consequence of circulatory stress in the liver. In addition, neoplastic processes such as hepatic adenoma, hepatocellular carcinoma, and metastatic disease may arise in these patients. Histologic evaluation is necessary when imaging modalities are unable ...

Chiari malformation type I and Poland's syndrome are two rare diseases and their simultaneous presentation had not been previously described in the literature. We report the case of a 27 year old male with history of Poland's syndrome, who referred headache and motor impairment of the intrinsic muscles of the left hand. In a cervical spine MR a Chiari I malformation with syringomyelia from C1 t...

Budd-Chiari syndrome (BCS) is a hepatic venous outflow block generally resulting from disorders affecting hepatic venous system. Elevated hepatic venous pressure results in portal hypertension. BCS may also cause hepatic encephalopathy. Echinococcus multilocularis is a tapeworm parasite and the natural course of the disease may affect liver parenchyma as well as hepatic venous tree. It is the m...

This report is of a 63-year-old man with polycythemia vera who developed the Budd-Chiari syndrome due to right hepatic vein stenosis. Diagnosis was made by laparoscopy and liver biopsy, and confirmed by hepatic venography. The patient was treated by percutaneous transluminal angioplasty, and recovered completely from ascites, leg oedema and venous stasis. No pulmonary embolism was observed. One...

Budd-Chiari syndrome (BCS) is a rare condition which occurs when there is obstruction of the hepatic veins. It includes any condition in which there is obstruction to venous flow from the small hepatic veins to the inferior vena cava. It should be differentiated from veno-occlusive disease in which the sinusoidal epithelial cells of the hepatic venules are damaged; this mainly occurs after stem...

BACKGROUND Endovascular management is important for the treatment of primary Budd-Chiari syndrome, which is caused by inferior vena cava (IVC) obstruction. The aims of this study were to compare long-term outcomes of endovascular management for primary Budd-Chiari syndrome patients with membranous obstruction of IVC (MOVC) and segmental obstruction of IVC (SOVC) and explore the optimal endovasc...