Congenital rubella retinopathy--a progressive disease

Congenital rubella retinopathy is characterized by disseminated dusty or mottled pigmentation with greatest density in the macula. Visual fields, dark adaptation, the electroretinogram and the electrooculogram are normal or close to normal. The intensity of the pigmentation increases over a number of years; as a rare complication it can lead to subretinal neovascularization. This complication is due to atrophy and necrosis of the pigment epithelium, both being induced by the rubella virus. After scarred healing the subretinal neovascularization leads to disciform maculopathy. This rare complication of rubella retinopathy is illustrated by two case reports of girls aged 14 and 16 years.     

Congenital rubella syndrome—major review

Congenital rubella syndrome is a rare disorder with devastating ocular and systemic consequences. Although efforts to eradicate the disease have been in place for some time, some areas of the world continue to be affected by this disease. The burden of the disease weighs heavily on patients and society; therefore, vaccination and other preventative strategies should continue to be strongly encouraged.     

Spontaneous reabsorption of a rubella cataract.

A case of spontaneous reabsorption of a rubella cataract is presented. The morphology of the capsular bag is recorded by Scheimpflug slit image and retroillumination photography.     

Congenital cataract: general review

Cataract is a loss of lens transparency because of a protein alteration. Etiopathogenesis is poorly understood but new mutations of different developmental genes involved are found in 25% of cases. Frequency of onset, particularly when different ocular development anomalies occur, is related to the lens induction phenomena on the eye's anterior segment structure during embryologic development. Genetic transmission is often found on the dominant autosomal mode. Diagnosis is based on a complete and detailed examination of the eye, often with general anaesthesia. This condition predisposes children to later, sometimes serious amblyopia. Different clinical aspects can be observed: from cataract with ocular and/or systemic anomalies to polymalformative syndrome, skeletal, dermatological, neurological, metabolic, and genetic or chromosomal diseases. A general systematic pediatric examination is necessary. Congenital cataract requires first and foremost early diagnosis and a search for all etiologies. Surgical treatment is adapted case by case but it has progressed with the quality of today's intraocular lenses even if systematic implantation continues to be debated. Life-long monitoring is absolutely necessary.     

Congenital cataract and intraocular lenses

We implanted nine intraocular lenses for the aphakic correction of congenital monocular cataracts in eight children (eight eyes). During follow-up periods ranging from 18 to 50 months in six children, there were no major complications connected to the surgery. Parental cooperation with treatment for amblyopia was satisfactory in all cases. The degree of strabismus, the fixation pattern, and the optokinetic nystagmus responses improved postoperatively in all six. Three children old enough to cooperate during visual testing had visual acuities better than 20/200 and two of these had visual acuities of 20/40. In all six children the sound eye is still patched for three to six hours a day. All six attend regular kindergartens and participate in their normal activities without difficulty.     

先天性白内障与缝隙连接蛋白Cx50

约1/3的先天性白内障与遗传有关。随着分子生物学技术的发展,近年来愈来愈多地发现Cx50缝隙连接蛋白与先天性白内障有关,其发病机制还不是很明确。本文通过对与缝隙连接蛋白Cx50相关的先天性白内障分子基础的研究做一综述,以期对缝隙连接蛋白分子结构、作用、致病机制有更深的了解。