STURGE-WEBER-DIMITRIʼS DISEASE

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Abstract
The authors describe an abortive form of Sturge-Weber-Dimitri''s disease in which no superficial hemangiomatous lesions were demonstrable. They state that the anatomic basis for the disease is hemangiomatous mesenchymal dysplasia in the cephalic region with lesions predominantly in the mesenchymal layer bordering upon the ectoderm; namely, the subcutis, submucosa of the mouth, nose and throat, choroid coat of the eye, and the leptomeninx. The engorged veins produce venous and capillary stasis of the underlying cerebral cortex and this disturbance in circulation results in the deposition of concretions which appear in the roentgenogram as double contour lines outlining the cerebral convolutions. The concretions are generally rich in Ca or Ca mixed with Fe. Although the primary developmental defect is fixed, the secondary changes result in circulatory disturbances which are generally slowly progressive over a period of yrs.

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