Abstract

Remnants of a persistent hyaloid artery can occasionally cause cataracts and traction on the retina at the posterior pole of the eye.It is the task of the ophthalmologist to weigh up the risk of amblyopia against the risks of vitrectomy and lensectomy. The retina is primarily intact. This is different from the group of hereditary vitreoretinal dystrophies where defects in the retina and vitreous body contribute equally to the overall clinical manifestations. Familial exudative vitreoretinopathy (FEVR) of childhood is sometimes misdiagnosed as uveitis. Retinal exudates leak from enormously permeable retinal vessels and it is necessary to completely remove such abnormal vessels to stop progression. The peculiar vitreous opacities consist of paper-like veils which are stacked like onion skins. The FEVR simulates retinopathy of prematurity and other exudative vitreoretinopathies. Stickler and Wagner syndromes are associated with a high risk of rhegmatogenous retinal detachment, similar to giant retinal tears. Occasionally, extraocular manifestations, such as hyperextensive joints are indicative of a systemic connective tissue disease.The peripheral retina is mechanically weak and susceptible to tears and giant or multiple tears can occur. The prognosis is further aggravated by an elevated risk of proliferative vitreoretinopathy (PVR). It is, therefore, essential to identify the risk profile in this constellation and bear the risk of PVR in mind when selecting a treatment regimen, similar to that for giant tear retinal detachment.

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