Related Experiment Video
Updated: Jul 31, 2026
07:07
Evaluation of Planar-Cell-Polarity Phenotypes in Ciliopathy Mouse Mutant Cochlea
Published on: February 21, 2016
Distinctive cataract in the Stickler syndrome
C M Seery1, R C Pruett, R M Liberfarb
1Retina Associates, Eye Research Institute, Boston, MA 02114.
American Journal of Ophthalmology
|August 15, 1990
Summary
Stickler syndrome patients frequently develop unique wedge and fleck cataracts. These distinctive ocular findings can aid in the early clinical diagnosis of Stickler syndrome.
Area of Science:
- Ophthalmology
- Genetics
- Clinical Medicine
Background:
- Stickler syndrome is a hereditary connective tissue disorder.
- Ocular manifestations are common in Stickler syndrome.
- Cataract is a significant visual impairment associated with the condition.
Purpose of the Study:
- To describe the clinical characteristics of cataracts in patients with Stickler syndrome.
- To identify distinctive cataract types that may serve as clinical markers.
- To evaluate the prevalence of cataracts in this patient cohort.
Main Methods:
- Retrospective analysis of clinical data from 133 patients diagnosed with Stickler syndrome.
- Ophthalmic examination of 231 eyes to assess for cataracts and other ocular abnormalities.
- Classification and quantification of different cataract types observed.
Main Results:
- Cataracts or aphakia were present in 115 of 231 eyes (49.8%).
- Distinctive wedge and fleck cataracts were the most frequent lesions, accounting for 40 of 93 observed cataracts (43.0%).
- These specific opacities were identified as potential clinical markers.
Conclusions:
- Cataracts are highly prevalent in Stickler syndrome.
- Wedge and fleck cataracts are characteristic findings in Stickler syndrome.
- Early identification of these distinctive cataracts can facilitate timely diagnosis of Stickler syndrome.
Related Concept Videos
01:25Glaucoma: Overview
Glaucoma is an eye condition characterized by increased intraocular pressure that damages the retina and optic nerve, leading to irreversible blindness if left untreated. The human eye has various components, including the cornea, iris, pupil, lens, and optic nerve. Aqueous humor is secreted by the epithelium of the ciliary body in the posterior chamber and flows through the trabecular meshwork and canal of Schlemm, maintaining normal intraocular pressure. The trabecular meshwork and the canal...
01:22Photoreceptors and Visual Pathways
At the molecular level, visual signals trigger transformations in photopigment molecules, resulting in changes in the photoreceptor cell's membrane potential. The photon's energy level is denoted by its wavelength, with each specific wavelength of visible light associated with a distinct color. The spectral range of visible light, classified as electromagnetic radiation, spans from 380 to 720 nm. Electromagnetic radiation wavelengths exceeding 720 nm fall under the infrared category, whereas...