Foveal hypoplasia is a retinal disorder in which there is a lack of full development of the morphology of the fovea. The optical coherence tomography (OCT) and functional findings are presented in relation to the underlying genetic and developmental conditions. Recent advancements of high-resolution OCT imaging have unveiled characteristics of foveal hypoplasia that were not detected by conventional imaging methods. An absence of a foveal pit does not necessarily imply poor visual acuity, and the maturation of the cone photoreceptors is important for the visual acuity. Regardless of the degree of the development of the inner retinal layers, the visual acuity can be preserved as in diseases such as Stickler syndrome that is a newly identified retinal disorder associated with foveal hypoplasia.

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Stickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR). The surgery for the combined GRT with PVR has limited structural results and the vision mainly remains impaired. In order to improve the visual outcomes, we propose an organized program oriented toward early diagnosis and surveillance. Adding an effective prophylaxis may maintain normal vision in a high percent of patients. The critical diagnostic moments for this program are prenatal and at birth. The tools include a directed history, general physical exam and advanced ophthalmologic exam looking for the particular features of SS. Some features may need advanced skills transfer, because they are not reliably taught in retina fellowships. Much of this program requires a partnership with obstetricians, pediatricians, neonatologists and geneticists. Finally, we review the evidence regarding prophylaxis and discuss our approach in the absence of guidance from a randomized clinical trial.

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Retinopathy of prematurity (ROP) is one of the most common causes of preventable blindness in children. In spite of the availability of various treatment options, and favorable results with timely intervention, many infants present to the ophthalmologists in the advanced end stage of the disease due to lack of awareness especially in the developing nations. This blinding or Stage 5 of ROP presents with total retinal detachment and has to be managed surgically. The surgical techniques for Stage 5 ROP are unique and demanding. The successful anatomical results after surgery are only seen in 20%–50% of cases. In spite of a successful anatomical result, the visual outcome may be slow and limited. The use of newer pharmacological adjuncts has shown promising results. Because of heterogeneity of presentation of the disease severity, a genetic predisposition has also been proposed. A concerted effort from the pediatricians, ophthalmologists, and healthcare workers is required to establish effective screening and treatment guidelines to prevent blindness due to ROP. Till then surgical management has to be done. Parents must be educated regarding the limited visual benefits of surgery and the need for prolonged follow-up. This review gives a comprehensive overview of the pathogenesis, clinical aspects, surgical interventions, and their outcomes and future prospects of Stage 5 ROP.

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PURPOSE: The purpose of this study was to report the rate of reactivation and structural outcome, after the laser or bevacizumab treatment for aggressive posterior retinopathy of prematurity (APROP). METHODS: Retrospective chart review was conducted on consecutive infants with APROP treated with (1) laser or (2) bevacizumab, followed by fluorescein angiography and prophylactic laser to the persistent avascular retina. RESULTS: Thirty-six eyes of 19 patients were included in this study. The mean gestational age was 24.5 weeks with a mean birth weight of 632 g in the bevacizumab group and 24.7 weeks and 777 g in the laser group. Unfavorable outcome occurred in 1 of 22 eyes treated with bevacizumab and in 5 of 14 eyes in the laser group (P = 0.002). Reactivation requiring treatment was common in both groups, 9/22 after bevacizumab and 6/14 after laser (ns). CONCLUSION: Regardless of the initial treatment reactivation requiring retreatment is common in eyes with APROP. The unfavorable structural outcome was significantly more common after initial laser treatment than after initial bevacizumab treatment.

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Retinoblastoma, the most common primary intraocular malignancy of the young, is a prototype hereditary cancer. Due to its fairly predictable Mendelian inheritance, easily examinable tumors, and early age of presentation, RB has served as the most extensively studied model for genetics in cancer. The genetic strides in RB have progressed at an exponential rate since the 1970s. The highly morbid, reasonably curable and distinctly predictable inheritance pattern of RB; not to mention its propensity to affect our young, forms the basis of aggressively encouraging genetic diagnosis in all patients of RB. In this article, we present the basic and clinically relevant concepts of the genetics retinoblastoma.

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In this review, the authors present special considerations a vitreoretinal surgeon should take into account before embarking on surgery in a pediatric eye. First, the anatomy of a pediatric eye is different from an adult and changes as the child grows. This is important especially in relation to the placement of transconjunctival ports. The structural characteristics of the sclera are also different, with lower scleral rigidity found in pediatric eyes. When considering vitrectomy, a posterior pars plicata lens-sparing technique should be considered. However, this may not be possible in complicated total detachments where anterior translimbal vitrectomy may be the method of choice. Scleral buckles are preferred for certain cases, and division of the encirclage is advocated in children below the age of 2 years, once the retina has stabilized. Enzymatic vitreolysis has been described as a preoperative adjunct to enhance complete detachment of the posterior hyaloid and reduce iatrogenic retinal breaks. However, its use in pediatric eyes has been limited, and larger studies are warranted. Finally, postoperative visual rehabilitation and treatment of amblyopia are key to maximizing functional outcomes in the pediatric patient. Co-management with a pediatric ophthalmologist and enlisting the co-operation of the parents are essential.

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In this review, we present a concise summary of the more commonly seen types of retinal detachments (RDs) that one can encounter in pediatric patients. A spectrum of diseases from rhegmatogenous RD in Stickler syndrome, Marfan syndrome, and choroidal coloboma to exudative RD in Coats disease, to tractional RD in persistent fetal vasculature, and combined RDs in familial exudative vitreoretinopathy are described with the management pearls for each.

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Retinopathy of prematurity (ROP) is a leading cause of childhood blindness worldwide. ROP screening and interventions (e.g., laser ablation and anti-vascular endothelial growth factor [VEGF] therapy) at the right time can reduce disease activity and prevent retinal detachment. However, sometimes, ROP is refractory to treatment, leading to tractional retinal detachment (TRD), requiring surgical intervention, such as vitrectomy. Vitrectomy for Stage 5 ROP (total retinal detachment) is beneficial in preventing total blindness in some patients. However, it has poor anatomical and functional results. Vitrectomy (lens-sparing vitrectomy, if possible) should be performed at Stage 4A ROP (partial TRD not involving the macula) because the anatomical and functional results are much better.

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We report a case with multiple recurrences of retinal detachment (RD) with severe proliferative vitreoretinopathy in a 12-year-old child who underwent vitrectomy using a heavy liquid perfluoro-n-octane (PFO) as a short-term postoperative tamponade. He had an encircling band and three vitrectomies with gas, followed by silicone oil tamponade for retinal redetachment prior to the use of PFO as a short-term tamponade. Short-term PFO tamponade was used in which inferior retinal reattachment was considered to be difficult with conventional gas or silicone oil tamponade, especially in regard to proper postoperative posturing. The PFO was left in the eye for 1 week, and the retina remained reattached after removal of the PFO which was replaced with silicone oil tamponade in this case. PFO appears feasible and tolerable as a short-term postoperative tamponade in the management of complex pediatric RD.

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