Abstract
Purpose When an X-linked pedigree of posterior lenticonus with cataract was identified further evidence for X-linked inheritance of this condition was sought.
Methods Forty-three cases of posterior lenticonus were identified from a database of 354 children with cataract. Two children with the X-linked syndromes of Lowe and Nance–Horan and 3 children with Fanconi syndrome have been excluded from further analysis. None of the children was deaf. None of the non-syndromic cases had microcornea.
Results There were 38 cases of non-syndromic posterior lenticonus (∼11%). There were 15 children from 13 pedigrees and 23 apparently sporadic cases. Of the 106 cases on the database with unilateral cataract 15 had posterior lenticonus (∼14%). Eleven of 13 pedigrees were compatible with X-linked inheritance or autosomal dominant inheritance with variable expression. However, in 2 pedigrees there was father to son transmission.
Conclusions Posterior lenticonus is a common cause of unilateral infantile cataract, but is thought to be a rare cause of bilateral cataracts. This study suggests that posterior lenticonus is responsible for a significant proportion of childhood cataracts (∼14% of unilateral and ∼9% of bilateral cases). Posterior lenticonus is generally thought to occur as a sporadic condition. This study demonstrates that there is a family history of early-onset cataract in a significant number of bilateral cases (∼58%).
Similar content being viewed by others
Article PDF
References
Meyer F . Ein Fall von Lenticonus posterior. Zentralbl Prakt Augenheilkd 1888;12:41.
Butler TH . Lenticonus posterior. Arch Ophthalmol 1930;3:425–36.
Howitt D, Homblass A . Posterior lenticonus. Am J Ophthalmol 1968;66:1133–6.
Pollard ZF . Familial bilateral posterior lenticonus. Arch Ophthalmol 1983;101:1238–40.
Vivian AJ, Lloyd C, Russell-Eggitt I, Taylor D . Familial posterior lenticonus. Eye 1995;9:119–23.
Hiles DA . Intraocular lens implantation in children with monocular cataracts. Ophthalmology 1984;91:1231–7.
Author information
Authors and Affiliations
Corresponding author
Additional information
Part of this work was presented at ARVO 2000 as a poster
Rights and permissions
About this article
Cite this article
Russell-Eggitt, I. Non-syndromic posterior lenticonus a cause of childhood cataract: Evidence for X-linked inheritance. Eye 14, 861–863 (2000). https://doi.org/10.1038/eye.2000.237
Received:
Revised:
Issue Date:
DOI: https://doi.org/10.1038/eye.2000.237
Keywords
This article is cited by
-
Contemplating the necessity of surgical treatment for posterior lenticonus: a case report
BMC Ophthalmology (2023)
-
Newer insights into the clinical profile of posterior lenticonus in children and its surgical, visual, refractive outcomes
Eye (2022)
-
Linse mit zentralem Reflexmuster, wie weiter?
Der Ophthalmologe (2020)
-
Jellyfish sign for intraoperative identification of posterior lenticonus
International Ophthalmology (2017)
-
Persistent central posterior capsule bulging after cataract extraction for posterior lenticonus
Eye (2012)