Cerebral Angiography in Encephalo-Trigeminal Angiomatosis

Abstract

Encephalo-trigeminal angiomatosis, as we understand it, consists essentially of a vascular anomaly of the skin of the face, in the area served by the trigeminal nerve, associated with a presumed intracranial vascular anomaly. Sturge originally described the syndrome as consisting of a facial nevus of the “port-wine” variety associated with an ocular abnormality, either buphthalmos or congenital glaucoma, and “cerebral involvement,” such as focal convulsive seizures. Since that time, other features have been added to the syndrome and endless subtypes and eponymic designations have appeared. These have served little purpose except to sustain the impression, shared by most modern authors, that encephalo-trigeminal angiomatosis is but one manifestation of a generalized dysgenesis which, like all such congenital abnormalities, can take a variety of forms and present itself in a whole gamut of “completeness.” We have used the term to include various vascular anomalies of the face, whether of the classical port-wine type or racemose angioma. Relatively few cases of this syndrome have been studied by means of cerebral angiography. Egas Moniz stated that no angiographic abnormalities could be found in these cases, and Bergstrand, Olivecrona and Tönnis, and later Riechert, agreed with that conclusion. Many investigators have been reluctant to subject children to cerebral angiography. Until Torkildsen and Koppang's publication appeared in 1950, there was no report specifically devoted to the procedure in this early age group. A recent study by the present writers demonstrated that it is at least as safe in children as in adults. Cerebral angiography has certain inherent drawbacks that may explain the disappointment of early investigators with this technic. Some vascular abnormalities are undoubtedly made up of vessels too small to opacify sufficiently to be seen on an x-ray film. A feeder vessel to a small lesion may be thrombosed and thus hide the latter during angiography. The introduction of serialograms obtained by rapid cassette changers and roll films has made possible a more complete visualization of the capillary and venous phases. Material We have collected 15 cases of encephalo-trigeminal angiomatosis which were subjected to cerebral angiography. All of these were studied at the Neurological Institute of the Presbyterian Hospital, New York. In addition, we have reviewed the available literature and found 37 cases—Sturge-Weber syndrome, encephalo-trigeminal angiomatosis, or some other designation—in which cerebral angiography had been carried out. Two of these cases (Cases 12 and 13 of DiChiro and Lindgren) are not included in our analysis, since there were no stigmata of the syndrome which would make them conform to our criteria for acceptance in this series.