P0356 THE MANAGEMENT OF HEPATIC HAEMANGIOENDOTHELIOMA IN CHILDREN

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Introduction: Hepatic haemangioendothelioma (HHE) in children is a rare, benign vascular tumour, characterized by rapid growth in the first 6 months of life followed by gradual resolution. A complication with high mortality is congestive cardiac failure and symptomatic children require aggressive medical and/or surgical management. Our aim was to review clinical features and management of HHE in a paediatric hepatology tertiary referral centre. Methods: Retrospective review of 31 children with HHE referred between 1979 and 2003. Results: 17/31 children were male (median age at presentation 2 months, range birth- 5.5 years). Three were diagnosed by antenatal ultrasound. Abdominal distension was present in 67%, pallor in 32%, poor feeding in 26%, jaundice in 12%. Two had Kasabach-Merritt syndrome; 8 were asymptomatic. Hepatomegaly was present in 90%, cutaneous haemangiomata in 32%, heart murmur in 29%, high output cardiac failure in 35%. Thyroid function tests were abnormal in 8/12 tested. Alpha-fetoprotein was elevated in 11/14 tested. Diagnostic imaging were ultrasound scan in all, CT in 20, angiography in 16 and MR angiography in 6. HHE involved both lobes in 64%, right lobe in 19% and left lobe in 15%. Liver histology in 10 children showed HHE in 7, with premalignant changes in 1, giant cell hepatitis in 2 and biliary cirrhosis in 1. Seven persistently asymptomatic children were treated conservatively and are alive and well (median follow up 2.2 years). Medical treatment in 19 children consisted of steroids (10), digoxin (16), frusemide (16), spironolactone (8), ace inhibitors (2), interferon (2) and chemotherapy (3, with radiotherapy in 1). Eighteen showing no improvement, as well as 5 who did not receive medical treatment, underwent surgical procedures: hepatic artery ligation (HAL) in 16, partial hepatectomy (PH) in 3, combined HAL and PH in 2 and primary liver transplantation (LT) in 2. Two children required LT after HAL because of rapid clinical deterioration. Twenty-seven children (87%) are alive and well (median follow up 2.7 years, range 1 month–17 years). Four children died: 1 after evolution to haemangiosarcoma, 1 soon after LT with CMV infection, 1 while waiting for LT of multi-organ failure and 1 one-year after LT of respiratory complications. The child with prema-lignant changes is listed for LT. Conclusion: In symptomatic HHE medical treatment is ineffective on its own, but if combined with HAL and/or PH, the prognosis is good. LT is indicated when medical and surgical management fail or in the presence of premalignant changes.