Clinical spectrum, investigations and treatment of Budd-Chiari syndrome

The clinical presentation, investigations, therapeutic modalities, prognosis and outcome of 44 patients with Budd-Chiari syndrome (BCS) were reviewed. There were 27 women and 17 men. Median age at presentation was 37 years (range 14-60). Possible aetiological factors were identified in 31 patients (70%). Myeloproliferative disorders were the commonest aetiology. Abdominal pain and swelling were the commonest presenting symptoms. Sixteen patients underwent a shunt operation (14 mesocaval, 2 mesoatrial). Ten patients had liver transplantation. Eleven patients had angioplasty/stent as their only treatment, and seven were treated medically or died before any treatment was instituted. Radiological intervention was comparable to mesocaval shunt in relieving patients' symptoms and in achieving good long-term results. Medical treatment and liver transplantation both yielded poor results. Radiological intervention in the form of balloon angioplasty or stent placement gives good results in a subgroup of BCS patients, and should be tried first to relieve the hepatic outflow obstruction. Mesocaval shunts provide good results in selected cases. Underlying haematological causes should be intensively investigated and promptly treated.