Even after a successful keratoplasty with a clear graft, a high postoperative refractive error could occur too hard to correct with spectacles or contact lenses. Therefore, refractive surgery could be considered a good tool to correct these high postoperative defects. The authors showed the reasons involved in the refractive errors after successful penetrating (PKP) or lamellar transplantation (DALK), pre-, intra-, and post-operatively. Moreover, they presented different techniques to correct the refractive errors after transplantation for different corneal pathologies, in the plastic phase (managing of transplant sutures) as well as in the static phase (different refractive techniques: incisional (AK, FemtoAK), ablative (PRK, FemtoLASIK), or IOL implantation (Phakic IOL, PHACO + IOL)). Thus, it is necessary to study accurately every single clinical case to choose the best surgery for each patient. Due to the high risk of graft damage or graft rejection, the patient must be adequately informed about the risks and benefits of the surgery proposed and must specifically accept the possibility of a new corneal transplant in the event of surgery failure or graft damage. Certainly, the refractive surgeon must be able in managing all the different refractive surgery techniques to reach the best result in every single case.
Fabry disease (FD) is an X-linked lysosomal storage disorder, causing Gb-3 (globotriaosylceramide) buildup in cellular lysosomes throughout the body, in particular in blood vessel walls, neuronal cells, and smooth muscle. The gradual accumulation of this glycosphingolipid in numerous eye tissues causes conjunctival vascular abnormalities, corneal epithelial opacities (cornea verticillata), lens opacities, and retinal vascular abnormalities. Although a severe vision impairment is rare, these abnormalities are diagnostic indicators and prognostics for severity. Cornea verticillata is the most common ophthalmic feature in both hemizygous men and heterozygous females. Vessel tortuosity has been linked to a faster disease progression and may be useful in predicting systemic involvement. New technologies such as optical coherence tomography angiography (OCTA) are useful for monitoring retinal microvasculature alterations in FD patients. Along with OCTA, corneal topographic analysis, confocal microscopy, and electro-functional examinations, contributed to the recognition of ocular abnormalities and have been correlated with systemic involvement. We offer an update regarding FD ocular manifestations, focusing on findings derived from the most recent imaging modalities, to optimize the management of this pathology.
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