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Ocular involvement associated with linear nevus sebaceous syndrome Kazuko Kiribuchi 1 , Yukio Uchida 1 , Hiroshi Maruyama 2 1Dep. of Ophthalmol., Tokyo Women's Med. Coll. 2Matsudo Clin. pp.199-203
Published Date 1985/2/15
DOI https://doi.org/10.11477/mf.1410209345
  • Abstract
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We observed 2 cases of linear nevus sebaceous syndrome with the triad of midfacial nevus sebaceus, seizures, and mental retardation.

The first case, a 11-year-old male, presented with nevus sebaceus of Jadassohn in the left facial hemi-sphere, focal EEG abnormality in the left occipital cortex and marked dilatation of the left temporal subarachnoideal space. The left fundus was affected with marked disc cupping, sectorial hypopigmen-tation of the choroid extending upper-nasal from the disc, and scattered patches of hypoplasia of the choroid and the sclera.

The second case was a 10-year-old male with the above triad associated with hemangioma in theforearm and normal ocular findings.

The observed wide spectrum of disorders involv-ing the brain, skin and the eye seemed to represent the multiple ectodermal and mesodermal malfor-mation in a rare congenital neurocutaneous syndrome.


Copyright © 1985, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1308 印刷版ISSN 0370-5579 医学書院

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