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A retrospective study was made of 106 cases of facial port-wine stains. It was concluded that only patients with lesions located in the ophthalmic (or V1 trigeminal) cutaneous area are at risk for associated neuro-ocular symptoms. It is proposed that Sturge-Weber syndrome results from a dysmorphogenesis of cephalic neuroectoderm.
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Enjolras et al. (1985) studied this question.
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