Inferior vena caval and hepatic vein thrombosis: the Chiari syndrome in childhood.

The syndrome of hepatic vein thrombosis was first described by George Budd of King's College Hospital, London, in 1845. Chiari in 1899 reported three cases of his own and reviewed the literature. The first case in childhood (an infant of 17 months) was published by Gee in 1871, and since that time we have only been able to trace nine other examples of this syndrome under the age of 17 years in the world literature (Schuippel, 1880; Lazarus-Barlow, 1899; Fisher, 1902; Penkert, 1902; Fabris, 1905; Hess, 1905; Hutchison and Simpson, 1930; Thompson, 1947; Dodd, Johannsman and Rapoport, 1948; Bronte-Stewart and Goetz, 1952). We have recently investigated a boy aged 13 years with hepatic vein thrombosis. An associated panniculitis, together with other clinical and pathological findings which throw some light on the aetiology of the thrombosis, prompted this report.