Chiari disease (or malformation) is in general a congenital condition characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal. The onset of Chiari syndrome symptoms usually occurs in the second or third decade (age 25 to 45 years). Symptoms may vary between periods of exacerbation and...

We studied the etiology, diagnosis and natural course of myeloproliferative disease (MPD) in 40 consecutive patients with Budd-Chiari syndrome (BCS). In 38% of the BCS patients with MPD another etiological factor was found. JAK2 mutation was present in 41% of the tested BCS patients. Survival was not significantly affected by the presence of MPD.

An 11 year old boy developed pancytopenia, haemolysis, and Budd-Chiari syndrome. The venous thrombosis extended to involve other intra-abdominal vessels before paroxysmal nocturnal haemoglobinuria was recognised as the underlying haematological abnormality. Earlier diagnosis would have made curative bone marrow transplantation a possibility.

We report the case of a 63 year old woman who developed the complications of cholangitis and Budd Chiari syndrome secondary to polycystic disease of the liver. The two complications were not present simultaneously, and both resolved after decompression of the liver cysts.

Hydatid cyst of the liver, causing compression of the inferior vena cava (IVC) and hepatic veins is a rare cause of secondary Budd-Chiari syndrome (BCS). As the hydatid disease is endemic in India, it is a rare but treatable cause of BCS. The early diagnosis and timely intervention can prevent hepatic complications leading to BCS in the affected patients.

Recently, we experienced a rare case of Budd-Chiari Syndrome. The case was a 57-year-old female patient. Venous return had been severely disturbed by the membranous occlusion of the IVC and a giant floating thrombus. After catheter directed thrombolysis combined with stepwise percutaneous angioplasty, the IVC was recanalized sufficiently and the thrombus was completely resolved.

Budd-Chiari syndrome (BCS) is an uncommon condition induced by thrombotic or non-thrombotic obstruction of hepatic venous outflow. BCS most often occurs in patients with underlying thrombotic diathesis, including such myeloproliferative disorders (MPDs) as polycythemia vera and paroxysmal nocturnal hemoglobinuria, and pregnancy, oral contraceptives, tumors, chronic inflammatory diseases, clotti...

Budd-Chiari syndrome was diagnosed in a 13 year old boy who presented with ascites. Angiographic studies showed occlusion at the ostia of the hepatic veins. This was treated surgically by the Senning operation of transcaval dorsocranial resection of the liver and hepatocaval anastomosis. The patient's ascites cleared and he remains well 10 months after surgery.

Management of the airway in Apert syndrome is complex and multidisciplinary. This rare syndrome, occurring in up to approximately 1 in 65,000 live births, results in airway compromise at various anatomic levels, in addition to abnormal central respiratory drive. Obstructive apneas arise because of decreased airway caliber, which may occur in the form of congenital bony nasal stenosis, choanal a...