Angioma Racemosum Venosum of Spinal Cord with Segmentally Related Angiomatous Lesions of Skin and Forearm
1961; American Association of Neurological Surgeons; Volume: 18; Issue: 4 Linguagem: Inglês
10.3171/jns.1961.18.4.0546
ISSN1933-0693
Autores Tópico(s)Spinal Dysraphism and Malformations
ResumoIt is well recognised that vascular hamartomata of the central nervous system may co-exist with other neural or extraneural lesions of a similar dysplastic nature. The most familiar of these associations is Sturge-Weber-Dim itri disease. The cardinal features of this syndrome are an extensive capillaryvenous malformation of the leptomeninges, atrophy and calcification of the affected cerebral hemisphere and a facial port-wine naevus in the distribution of the ipsitateral trigeminal nerve. Another is the von Hippel-Lindau syndrome, in which a capillary hemangioblastoma of the cerebellar hemispheres occurs with similar tumours affecting one or both retinae. Other features include congenital cysts of the pancreas and kidney and capillary naevi of the skin. The analogous combination, however, of a racemose angioma of the spinal cord with a cutaneous angioma in the corresponding segment of skin is surprisingly rare. Newman 1~ was able to collect 6 such cases from the literature published previously. He cited the cases of Cobb, 1 Wyburn-Mason ~2 (Case 6), Silverman, Gilbert, and Henson and Croft 5 (Case 4). The following case is unusual in that not only were the vascular anomalies of the spinal cord and skin related segmentally, but in addition an abnormal fistulous communication between the arteries and veins of the forearm, in the same neuromere, was demonstrated by brachial arteriography.
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