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Clinical features of congenital uveal coloboma Hironori Ozeki 1 , Shoichiro Shirai 1 , Miho Nozaki 1 , Kozo Ikeda 1 1Dept of Ophthalmol, Nagoya City Univ Med Sch pp.253-256
Published Date 1998/3/15
DOI https://doi.org/10.11477/mf.1410905736
  • Abstract
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We reviewed 76 cases of congenital uveal coloboma comprising 62 typical and 14 atypical ones. The 62 typical cases included 30 males and 32 females. It was bilateral in 28 cases, unilateral in 30 and unclassified in 4 due to contralateral severe microphthalmia. Associated anomalies of typical coloboma included microphthalmos in 26 eyes of 22 cases, persistent pupillary membrane in 26 eyes of 17 cases, and posterior embryotoxon in 19 eyes of 14 cases. Accompanying systemic anomalies included 18 cases each of ear anomalies, retarded growth and retarded mental development, 13 of heart anomalies, 12 of genital hypoplasia and 10 of facial palsy. The 14 atypical cases comprised 8 males and 6 females. It was bilateral in 3 and unilateral in 11. Accompanying ocular anomalies included microphthalmos in 8 eyes of 5 cases, persistent pupillary membrane in 6 eyes of 5 cases, and peripapillary staphyloma in 5 eyes of 3 cases. There were no systemic anomalies associated with atypical coloboma. The majority of associated anomalies of both typical and atypical coloboma were due to maldevelopment of neural crest cells. The findings suggest that congenital uveal coloboma arises from a developmental disorder of neural crest cells.


Copyright © 1998, 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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