Late occurrence of additional ocular and intracranial pathologies in the linear naevus sebaceous (Feuerstein-Mims) syndrome |
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Authors: | J Brihaye M Brihaye-van Geertruyden J Retif A M Mercier |
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Institution: | (1) Department of Neurosurgery, Institut Bordet, Université Libre de Bruxelles, Belgium;(2) Department of Ophthalmology, Akademisch Ziekenhuis, Vrije Universiteit Brussel, Belgium |
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Abstract: | Summary The linear naevus sebaceous syndrome of Feuerstein-Mims belongs to the phacomatoses and classically is composed of a linear cutaneous lesion associated with mental retardation and epilepsy, sometimes also with ocular dystrophies.During an unusually long follow-up of 32 years of such a case, which is reported here, the late occurrence of additional ocular and intracranial pathology are demonstrated: conjunctival and bulbar tumours with progressive deterioration of visual acuity, bilateral intracranial arachnoidal cysts, dilatation and torstuosity of the middle cerebral artery.As a consequence of these observations the prognosis in cases with this syndrome has to be given with special caution. |
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Keywords: | Naevus sebaceous Feuerstein-Mims syndrom phacomatoses ocular tumours arachnoidal cyst cerebro-vascular pathology |
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