XXXX : diagnosis and management.

@entity1 @entity1136 @entity712 @entity417 @entity101 @entity548 @entity5 @entity5642 @entity1448 @entity3067 @entity382 @entity1320 @entity99 @entity288
Five cases of @entity99 are described, and a sixth (previously reported) is reviewed. Clinical features, investigation, and management are described to show the great variability of the complications and prognosis. Five @entity1 presented in the first 10 weeks of life with @entity382 ; 4 developed @entity712 ; 3 had cutaneous haemangiomata. One @entity1 presented at age 4 years with @entity382 and @entity417 , and on investigation had features of chronic @entity5642 . Focal decrease or @entity3067 in hepatic uptake of @entity1136 colloid, and abnormal intrahepatic circulation was shown in all cases. In 3 @entity1 liver biopsy was performed to exclude @entity5 . In one @entity1 there was spontaneous regression of the @entity5 by age 3 years. In 3 cases hepatic artery ligation was necessary to control @entity712 which had persisted despite treatment with digoxin, diuretics, and oral @entity288 , a procedure which was without complications after up to 8 years. One @entity1 with intractable portal @entity101 , @entity99 , and severe @entity1448 died with persisting alimentary @entity548 and @entity1320 . One @entity1 aged 4 years showed no immediate response to hepatic artery ligation but the size of her @entity5 got smaller and the clinical features diminished after irradiation. These @entity5 cause considerable morbidity and have a high reported mortality. If @entity712 is not rapidly controlled, hepatic artery ligation should be performed.
