Stickler syndrome associated with congenital glaucoma.

Department of Paediatric Ophthalmology, Strabismus, and Neuro-ophthalmology, Jasti V Ramanamma Children's Eye Care Centre, LV Prasad Eye Institute, KAR Campus, Hyderabad, India.
The Lancet (Impact Factor: 39.21). 02/2013; 381(9864):422. DOI: 10.1016/S0140-6736(12)61813-3
Source: PubMed
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    ABSTRACT: The occurrence of hypertonia during a surgically treated retinal disease is frequent because these disorders often involve the same population of patients. The main cause of postoperative hypertonia remains a preoperative unknown glaucoma. Hypertonia occurring before the treatment of a retinal detachment can result from angle recession glaucoma, ghost cell glaucoma, or Schwartz-Matzuo syndrome; all of which are frequently associated with trauma. Hypertonia occurring after the surgery of a retinal detachment can be caused by scleral buckling, a topical postoperative steroid treatment, or an internal tamponade with gas or silicone. The latter is responsible for severe hypertonia that is frequently resistant to treatment. Hypertonia occurring after the use of triamcinolone is usually controlled with medical treatment. Prior filtrating surgery can lead to technical problems during retinal surgery. The knowledge of pre-existing glaucoma may be reason for cautious management of retinal surgery.
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    ABSTRACT: Summary Background: We undertook this study to demonstrate the incidence of vitreoretinal dystrophies in a Mexican population. Methods: This was a retrospective, observational, descriptive and transversal study. We analyzed the files of patients treated at the Retina Department in a medical center for state employees (ISSSTE) from January 1991 to December 2006 to obtain the incidence of vitreoretinal dystrophies. Results: We studied 36,300 patient files. We found an incidence of 0.008 % for familial exudative vitreoretinal dystrophy, 0.008 % for X-linked juvenile retinoschisis, 0.005 % for Wagner disease and 0.005 % for Goldmann-Favre disease. We present here a representative case of each type of dystrophy. Conclusions: Vitreoretinal dystrophies are uncommon diseases and are difficult to diagnose. Even though they have a low incidence, the poor evolution to blindness requires identification of early signs in order to offer timely and opportune treatment.
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    ABSTRACT: Although the presence of 'cartilage-specific' collagens in the eye has been documented earlier, very little is known about their synthesis rates during ocular development, growth and aging. The purpose of the present study was to follow changes in the mRNA levels and distribution of key components of the extracellular matrix in the eyes of normal and transgenic Del1 mice, harboring a short deletion mutation in the type II collagen gene, during ocular growth and aging. Total RNAs extracted from mouse eyes were studied by Northern analysis for mRNA levels of type I, II, III, VI, IX and XI collagens, biglycan, fibromodulin and decorin. A predominant finding of the present study was the marked reduction in the mRNA levels of type I and II collagens in the eye upon aging. The changes in the mRNA levels of type III and VI collagen and proteoglycans were smaller. Localization of type II and IX collagen in the eye was performed by immunohistochemistry. Despite the reduction in the type II collagen mRNA levels, immunohistochemistry confirmed widespread distribution of the protein also in aging mouse eyes, suggesting its slow turnover. Although the Del1 mutation caused gradual degenerative lesions in the eyes, the distribution of the protein remained essentially unchanged. The widespread distribution and marked downregulation of type II collagen production in the mouse eye upon aging probably explain the gradual development of degenerative lesions, particularly in the eyes of transgenic Del1 mice, where production of mutant type II collagen chains also contributes to the process.
    Experimental Eye Research 05/2001; 72(4):423-31. DOI:10.1006/exer.2000.0972 · 3.02 Impact Factor