CLINICAL SPECTRUM, INVESTIGATIONS AND TREATMENT OF BUDD-CHIARI SYNDROME

The clinical presentation, investigations, therapeutic modalities, pro gnosis 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 identif ied in 31 patients (70%). Myeloproliferative disorders were the common est aetiology. Abdominal pain and swelling were the commonest presenti ng symptoms. Sixteen patients underwent a shunt operation (14 mesocava l, 2 mesoatrial). Ten patients had liver transplantation. Eleven patie nts had angioplasty/stent as their only treatment, and seven were trea ted medically or died before any treatment was instituted. Radiologica l 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 inte rvention in the form of balloon angioplasty or stent placement gives g ood results in a subgroup of BCS patients, and should be tried first t o relieve the hepatic outflow obstruction. Mesocaval shunts provide go od results in selected cases. Underlying haematological causes should be intensively investigated and promptly treated.