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Three cases of Rieger's syndrome Hitomi Uchida 1 , Toshiko Yamana 1 , Masako Ichimiya 1 , Keiko Yoshimura 1 , Hajime Inomata 1 1Dept of Ophthalmol, Fac of Med, Kyushu Univ pp.1239-1242
Published Date 1988/11/15
DOI https://doi.org/10.11477/mf.1410210543
  • Abstract
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We observed Rieger's syndrome in 3 patients : a 5 -month-old male, 2-month-old female and 24-year -old female. The first case manifested posterior embryotoxon, hypoplasia of iris, and strands bridg-ing the iridocorneal angle, as well as polycoria, microcornea and congenital absence of tooth. Ocu-lar hypertension developed at the age of 4 years, without particular changes in visual acuity or visual field during the following 3 years. The sec-ond case manifested hypoplasia of iris, mi-crocornea, persistent pupillary membrane, cataract, strabismus and dysgnathia. Posterior embryotoxon was absent. The third case, her mother, manifested hypoplasia of iris, iris strands bridging iridocorneal angle, microcornea, cataract, glaucoma and absence of tooth. Posterior embryotoxon was absent.

All the three cases manifested, as common fea-tures of the disease, goniodysgenesis involving the cornea and iris, absence of tooth or dysgnathia. These abnormalities seemed to be clinical manifes-tations of dysplasia of mesenchymal tissue derived from the neural crest.

Rinsho Ganka (Jpn J Clio Ophthalmol) 42(11) : 1239-1242, 1988


Copyright © 1988, 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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