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. 1994 Oct;78(10):781–785. doi: 10.1136/bjo.78.10.781

Biometry in X linked megalocornea: pathognomonic findings.

F M Meire 1, J W Delleman 1
PMCID: PMC504935  PMID: 7803356

Abstract

Biometric study in a series of 11 affected males provides characteristic findings. The patients present with a large cornea with short radius, very deep anterior chamber depth (AC depth) exceeding the normal mean value of plus 2 SD, and a short vitreous length. Calculation of the postlimbal depth, a method applied in this study to obtain information about positioning of the iris and the lens, reveals a posterior positioning of the iris and lens. The posterior positioning of the iris and lens was proved to occur at the expense of the vitreous. The importance of biometric data for diagnosis and for differential diagnosis in primary infantile glaucoma and other diseases with megalocornea is discussed.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Awaya S., Tsunekawa F., Koizumi E., Miyake Y., Yokoyama K. [Studies of X-linked recessive ocular albinism of the Nettleship-Falls type--with special reference to the association of megalocornea]. Nippon Ganka Gakkai Zasshi. 1988 Jan;92(1):146–150. [PubMed] [Google Scholar]
  2. Chen J. D., Mackey D., Fuller H., Serravalle S., Olsson J., Denton M. J. X-linked megalocornea: close linkage to DXS87 and DXS94. Hum Genet. 1989 Oct;83(3):292–294. doi: 10.1007/BF00285176. [DOI] [PubMed] [Google Scholar]
  3. Fontana S. T., Brubaker R. F. Volume and depth of the anterior chamber in the normal aging human eye. Arch Ophthalmol. 1980 Oct;98(10):1803–1808. doi: 10.1001/archopht.1980.01020040655013. [DOI] [PubMed] [Google Scholar]
  4. Goldberg M. F. Clinical manifestations of ectopia lentis et pupillae in 16 patients. Ophthalmology. 1988 Aug;95(8):1080–1087. doi: 10.1016/s0161-6420(88)33043-5. [DOI] [PubMed] [Google Scholar]
  5. Huet F., Castier P., Langlois M., Woillez J. P., Degrave B. La mégalocornée hérédo-familiale. Bull Soc Ophtalmol Fr. 1987 Jun-Jul;87(6-7):835–838. [PubMed] [Google Scholar]
  6. JOANNIDES T. [Contribution to the study of megalocornea (with genealogical tree)]. Ann Ocul (Paris) 1961 Jan;194:45–53. [PubMed] [Google Scholar]
  7. MALBRAN E., D ALESSANDRO C., VALENZUELA J. MEGALOCORNEA AND MOSAIC DYSTROPHY OF THE CORNEA. Ophthalmologica. 1965;149:161–176. doi: 10.1159/000304759. [DOI] [PubMed] [Google Scholar]
  8. Mackey D. A., Buttery R. G., Wise G. M., Denton M. J. Description of X-linked megalocornea with identification of the gene locus. Arch Ophthalmol. 1991 Jun;109(6):829–833. doi: 10.1001/archopht.1991.01080060093033. [DOI] [PubMed] [Google Scholar]
  9. Meire F. M., Bleeker-Wagemakers E. M., Oehler M., Gal A., Delleman J. W. X-linked megalocornea. Ocular findings and linkage analysis. Ophthalmic Paediatr Genet. 1991 Sep;12(3):153–157. doi: 10.3109/13816819109029398. [DOI] [PubMed] [Google Scholar]
  10. Meire F. M., Delleman J. W. Autosomal dominant congenital miosis with megalocornea. Ophthalmic Paediatr Genet. 1992 Jun;13(2):123–129. doi: 10.3109/13816819209087612. [DOI] [PubMed] [Google Scholar]
  11. Meire F. M., Delleman W. J., Bleeker-Wagemakers E. M. Ocular manifestations of congenital Marfan syndrome with contractures (CMC syndrome). Ophthalmic Paediatr Genet. 1991 Mar;12(1):1–9. doi: 10.3109/13816819109023078. [DOI] [PubMed] [Google Scholar]
  12. Meire F. M. Hereditary ectopia lentis. A series of 10 cases of ectopia lentis et pupillae. Bull Soc Belge Ophtalmol. 1991;241:25–36. [PubMed] [Google Scholar]
  13. Missotten L., Evens L. Phacolytic glaucoma in an eye with megalocornea 18 years after the spontaneous luxation of the lens into the vitreous cavity. Bull Soc Belge Ophtalmol. 1979;183:113–117. [PubMed] [Google Scholar]
  14. Neuhäuser G., Kaveggia E. G., France T. D., Opitz J. M. Syndrome of mental retardation, seizures, hypotonic cerebral palsy and megalocorneae, recessively inherited. Z Kinderheilkd. 1975 Jul 1;120(1):1–18. doi: 10.1007/BF00443795. [DOI] [PubMed] [Google Scholar]
  15. OPPEL O. Zur Klinik der grossen Hornhaut. Klin Monbl Augenheilkd Augenarztl Fortbild. 1956;129(6):737–756. [PubMed] [Google Scholar]
  16. RUD E. Megalocornea in a Danish gipsy family. Acta Ophthalmol (Copenh) 1960;38:606–617. doi: 10.1111/j.1755-3768.1960.tb00227.x. [DOI] [PubMed] [Google Scholar]
  17. Sampaolesi R., Caruso R. Ocular echometry in the diagnosis of congenital glaucoma. Arch Ophthalmol. 1982 Apr;100(4):574–577. doi: 10.1001/archopht.1982.01030030576003. [DOI] [PubMed] [Google Scholar]
  18. Skuta G. L., Sugar J., Ericson E. S. Corneal endothelial cell measurements in megalocornea. Arch Ophthalmol. 1983 Jan;101(1):51–53. doi: 10.1001/archopht.1983.01040010053007. [DOI] [PubMed] [Google Scholar]
  19. Young A. I. Megalocornea and mosaic dystrophy in identical twins. Am J Ophthalmol. 1968 Oct;66(4):734–735. doi: 10.1016/0002-9394(68)91298-1. [DOI] [PubMed] [Google Scholar]
  20. deLuise V. P., Anderson D. R. Primary infantile glaucoma (congenital glaucoma). Surv Ophthalmol. 1983 Jul-Aug;28(1):1–19. doi: 10.1016/0039-6257(83)90174-1. [DOI] [PubMed] [Google Scholar]

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