Hepatic vascular anomalies in infancy: A twenty-seven-year experience

• Published in: The Journal of pediatrics 1996-09, Vol.129 (3), p.346-354
• Main Authors: Boon, Laurence M., Burrows, Patricia E., Paltiel, Harriet J., Lund, Dennis P., Ezekowitz, R.Alan B., Folkman, Judah, Mulliken, John B.
• Format: Article
• Language: English
• Subjects: Arteriovenous Malformations - diagnosis; Arteriovenous Malformations - therapy; Biological and medical sciences; Diagnosis, Differential; Female; Gastroenterology. Liver. Pancreas. Abdomen; Hemangioma - congenital; Hemangioma - diagnosis; Hemangioma - therapy; Hepatic Artery - abnormalities; Hepatic Veins - abnormalities; Humans; Infant; Infant, Newborn; Liver Neoplasms - congenital; Liver Neoplasms - diagnosis; Liver Neoplasms - therapy; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Malformations; Medical sciences; Retrospective Studies
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/S0022-3476(96)70065-3

OBJECTIVE: Infantile hemangioma and arteriovenous malformation (AVM) of the liver have a similar presentation but a different natural history, and therefore require different treatment. This study was undertaken to clarify differential diagnosis and management of these two biologically distinct vascular disorders. STUDY DESIGN: We retrospectively analyzed the records of 43 children with hepatic vascular anomalies treated during the past 27 years. RESULTS: Ninety percent were hemangiomas (n = 39); 10% were AVM (n = 4). Infants with AVM or large solitary hemangioma had hepatomegaly, congestive heart failure, and anemia as presenting symptoms at birth. Multiple hepatic hemangiomas manifested at 1 to 16 weeks of age with the same clinical triad, plus multiple cutaneous lesions (19/23). The mortality rate after treatment of hepatic AVM was 50% (2/4). The mortality rates after treatment of liver hemangiomas were as follows: resection of solitary lesions, 20% (2/10); embolization, 43% (3/7); corticosteroids, 30% (3/10); and interferon alfa-2a, 15% (2/13). CONCLUSION: Solitary hepatic hemangioma cannot always be distinguished from hepatic AVM without radiologic studies. Multiple hepatic hemangiomas are differentiated from hepatic AVM by coexistence of multiple cutaneous hemangioma and by radiologic imaging. We recommend combined embolization and surgical resection for hepatic AVM and for solitary symptomatic hemangioma, if drug therapy fails. Pharmacologic treatment is used for symptomatic multiple liver hemangiomas. Embolization allows interim control of heart failure. A decreased mortality rate after interferon alfa-2a therapy is encouraging. (J P EDIATR 1996;129:346-54)