Peripheral Avascular Retina Research Articles

Familial exudative vitreoretinopathy in a 4 generations family of South-East Asian Descendent with FZD4 mutation (c.1501_1502del)

BackgroundFamilial Exudative Vitreoretinopathy (FEVR) is a hereditary disorder characterized by peripheral avascular retina with neovascularization. Although FEVR has been thoroughly described in multiple literature publications from different countries, there are currently limited articles describing the phenotypes of FEVR among South-East Asian Descendent. This paper describes the clinical phenotype of the FZD4 gene with c.1501_1502 deletion in a 4-generation case series of a South East Asian family.MethodsWe reviewed a 4-generation case series of a South-East Asian descendent family consisting of 27 family members with 10 members diagnosed with FEVR. We observed the clinical phenotype of these series of patients, including some of the family members who underwent whole-exome sequencing, PCR amplification and DNA sequencing techniques to identify the mutated gene.ResultsFrameshift mutation (c.1501_1502del) were found in FZD4 gene in this series of patients with the age ranging from 1 month old to 69 years old. There was a 100% (4/4) of our paediatric patients being diagnosed within 21 days of life. It was also found that 75% of patients (6/8) less than 40 years old exhibited disease asymmetry of 2 stages or more and 80% (8/10) had a history of vitreoretinal surgery or diode laser photocoagulation, with a further 50% of the adult patients identified as legally blind; the mean age of blindness was 18-years-old.ConclusionsPhenotypic manifestation of FZD4 gene with c.1501_1502del mutation can be identified within the neonatal period. They have relatively greater clinical asymmetry of 2 stages or more compared to other mutations. Without treatment, most of them will have bilateral severe visual impairment around the adolescent age group.

Long-term visual outcomes of children screened for retinopathy of prematurity with telemedicine in New Zealand

ABSTRACT Clinical Relevance Children with a history of regressed retinopathy of prematurity (ROP) are at increased risk of peripheral avascular retina. Wide-field digital retinal imaging and telemedicine is an effective tool for ROP screening. Ophthalmologists and Optometrists should have a high level of clinical suspicion for peripheral retinal changes in children screened for ROP. Background Retinopathy of prematurity, a vaso-proliferative disorder of the pre-term retina, is a preventable cause of childhood visual impairment. The Auckland Regional Telemedicine ROP (ART-ROP) network, established in 2006, utilises wide-field digital imaging and telemedicine to screen at-risk infants for ROP. This prospective observational study reports the long-term ocular outcomes of ART-ROP network infants. Methods A comprehensive paediatric eye examination including cycloplegic autorefraction and wide-field retinal imaging was completed on all participants. Participants had been screened for ROP by the ART-ROP network between May 2008 and October 2011. Results A total of 69 children, with a mean age of 5 to 8 years old were assessed and divided into two groups: those with or without a history of ROP, 44 and 25 children, respectively. Infants with a history of ROP had significantly lower gestational age (26.6 ± 1.9 vs. 29.1 ± 1.6 weeks, p < 0.001) and birth weight (937 ± 237 vs. 1177 ± 311 grams, p = 0.001). No significant differences were detected between the two groups for visual acuity (p = 0.596), stereopsis (p = 0.219), refractive error (p = 0.472), or strabismus. Clinically significant refractive error was noted in 10 participants; none with moderate or high myopia. Retinal imaging exposed asymptomatic, persistent, peripheral avascular retina in four children, all of whom had a history of regressed ROP. Conclusion Visual and ocular outcomes did not vary based on history of ROP, with no participant having reduced vision as a result of undetected or untreated ROP. Further research is required into the long-term implication of persistent avascular retina in regressed ROP.

Хирургическое лечение ретинопатии недоношенных: исторический экскурс

Purpose. Тo study the change in approaches to surgical treatment of ROP in the historical aspect. Large-scale multicenter researches have begun in the world to study the mechanism of ROP development since 1951. For decades, knowledge about the pathogenesis of ROP has been expanded, confirmed in practice, and since the 80 s of the last century, the development of various methods for the treatment of active ROP, depending on the stage of the disease, has begun. It has been proven that cryo- and laser retinopexy of the avascular peripheral retina is an effective method of stabilizing the pathological process in the active stages. With the accumulation of experience, it became obvious that if after laser treatment it was not possible to achieve regression of the disease, and objective markers of its further progression are determined, then expectant tactics does not make sense. The search for a clear correspondence between a pathogenetically substantiated method of treatment and the timing of its implementation to the stage of the pathological process of ROP remains relevant nowadays. Conclusion. The history of the surgical treatment of ROP is the way of the development of knowledge about this disease. Currently, a promising direction in the management of patients with active PH is to shift the timing of treatment to earlier stages. Keywords: retinopathy of prematurity, surgical treatment, vitrectomy.

Retinal findings similar to retinopathy of prematurity in a term-infant with congenital cytomegalovirus infection.

To present a term-born infant with congenital cytomegalovirus (C-CMV) infection with an atypical retinal finding mimicking retinopathy of prematurity (ROP). A term-born infant was referred to our clinic due to a C-CMV infection. Fundoscopic examination of both eyes revealed a demarcation line at zone II level and peripheral avascular retina along the temporal retina. There was no chorioretinitis, no vitritis, and no retinal vasculitis. There was no history of oxygen supplementation. The demarcation lines in both eyes regressed spontaneously within 3 weeks without any sequelae. It has been known for many years that C-CMV infection may affect the eye, and chorioretinitis is the most common finding. In this case report, we presented an atypical ROP-like retinal finding in an infant with C-CMV infection. We think that the development of retinal vascularization may be affected by C-CMV infection. We hope that this finding may provide a clue for future investigations to understand the relationship between the pathogenesis of ROP and infection-induced inflammatory processes in prenatal and perinatal life.

Prevalence of peripheral avascular retina in spontaneously regressed retinopathy of prematurity

Peripheral avascular retina (PAR) predisposes the development of lattice degeneration, atrophic holes, and retinal detachments in individuals with a history of retinopathy of prematurity (ROP). While PAR is commonly observed after vascular endothelial growth factor antagonist therapy for ROP, the prevalence of PAR in spontaneously regressed ROP is unknown. In this study, we evaluate the prevalence of PAR in a cohort of patients with a history of extreme prematurity.

Familial Exudative Vitreoretinopathy (FEVR); Pathophysiology, Findings, Diagnosis, and Treatment

Familial exudative vitreoretinopathy (FEVR) is an inherited disease occurring due to defective retinal angiogenesis. FEVR patients have an avascular peripheral retina which, depending on the degree of ischemia, causes the secondary complications of the disease (retinal neovascularization, exudation, hemorrhage, and retinal detachment) Mutations in the NDP, FZD4, LRP5, TSPAN12 and ZNF08 genes have been shown to contribute to FEVR. Cases can be inherited in an autosomal dominant, autosomal recessive, or X-linked manner, or can affect individuals with no family history. Examination with wide-field fluorescein angiography is essential and can identify the disease in its earlier stages, enabling the timely treatment, in addition to helping identify asymptomatic family members. The current treatment modalities involve laser photocoagulation of the avascular peripheral retina for neovascular complications and vitreoretinal surgery for retinal detachment. Studies are ongoing to better understand the pathogenesis of FEVR and to improve treatment

Imaging the pediatric retina: An overview.

Recent decade has seen a shift in the causes of childhood blinding diseases from anterior segment to retinal disease in both developed and developing countries. The common retinal disorders are retinopathy of prematurity and vitreoretinal infections in neonates, congenital anomalies in infants, and vascular retinopathies including type 1 diabetes, tumors, and inherited retinal diseases in children (up to 12 years). Retinal imaging helps in diagnosis, management, follow up and prognostication in all these disorders. These imaging modalities include fundus photography, fluorescein angiography, ultrasonography, retinal vascular and structural studies, and electrodiagnosis. Over the decades there has been tremendous advances both in design (compact, multifunctional, tele-consult capable) and technology (wide- and ultra-wide field and noninvasive retinal angiography). These new advances have application in most of the pediatric retinal diseases though at most times the designs of new devices have remained confined to use in adults. Poor patient cooperation and insufficient attention span in children demand careful crafting of the devices. The newer attempts of hand-held retinal diagnostic devices are welcome additions in this direction. While much has been done, there is still much to do in the coming years. One of the compelling and immediate needs is the pediatric version of optical coherence tomography angiography. These needs and demands would increase many folds in future. A sound policy could be the simultaneous development of adult and pediatric version of all ophthalmic diagnostic devices, coupled with capacity building of trained medical personnel.

MicroRNA-126 inhibits pathological retinal neovascularization via suppressing vascular endothelial growth factor expression in a rat model of retinopathy of prematurity

Vascular endothelial growth factor (VEGF) is the principal growth factor responsible for the retinal neovascularization in the pathogenesis of retinopathy of prematurity (ROP). Current therapies for ROP include laser ablation and intravitreal anti-VEGF injection. However, these treatments either destroy the peripheral retina or associate with problems of persistent peripheral avascular retina or later recurrence of ROP. In the present study we investigated a new therapeutic approach by exploring the potential role of a specific microRNA, miR-126, in regulating VEGFA expression and retinal neovascularization in a rat oxygen-induced retinopathy (OIR) model. We demonstrated that miR-126 mimic and plasmid effectively suppresses VEGFA mRNA expression in both human and rat retinal pigment epithelium cell lines, quantified with qRT-PCR. Animal experiments on rat OIR model revealed that intravitreal injection of miR-126 plasmid efficiently downregulated VEGFA expression in the intraocular fluid and retinal tissues measured by ELISA, and significantly suppressed retinal neovascularization, which was confirmed by calculating sizes of neovascularization areas on fluorescence microscopic images of flat mounted retina stained with Alexa Fluor 594-conjugated isolectin B4 to visualize blood vessels. Together, these results showed that intravitreal injection of miR-126 plasmid could inhibit retinal neovascularization by down-regulating VEGFA expression, suggesting a potential therapeutic effect for ROP.

Ocular complications following intravitreal bevacizumab injection for retinopathy of prematurity and assessment of risk factors

PurposeLaser ablation of the avascular peripheral retina has been the standard method of ROP treatment. Intravitreal anti-VEGF is useful in the management of ROP patients, especially for aggressive posterior ROP. However, ocular and systemic complication after intravitreal bevacizumab was the main concern. This study aimed to investigate the treatment-related ocular and systemic complications of intravitreal bevacizumab (IVB) in patients with retinopathy of prematurity (ROP).MethodThis retrospective study included neonates receiving intravitreal injections of bevacizumab (IVB) (0.625 mg) to treat ROP. Medical records of the patients were evaluated about the ocular complications after receiving IVB from 2012 to 2019. Treatment-related complications (vitreous hemorrhage, glaucoma, cataract, hyphema, corneal abrasion/opacity, and endophthalmitis), and disease-progression signs including retinal fold or stage 4 or 5 detachment were documented. Any reports of systemic events after injections were also recorded.ResultMean gestational age and birth weight of 441 patients receiving IVB for type-1 ROP were 28 ± 2 (22–34 weeks) and 1121 ± 312 (550–2700 g), respectively. The median follow-up after treatment in all patients and patients with complications was 289.43 ± 257 days (5–1899 days) and 385.89 ± 311.59 (196–1192) days, respectively. Out of 865 eyes, 20 eyes (2.31%, 95% Clopper-Pearson Confidence Interval: 1.14–3.54%) have been affected by ocular complications. The rates of different complications included progression of retinopathy in 17 eyes (1.96%), cataracts in 2 eyes (0.23%), and vitreous hemorrhage in one eye (0.11%). No cases of endophthalmitis, thromboembolic events, or death occurred in this study. We evaluated the prevalence ratio (PR) on the multiple risk factors to determine the prediction of the complications. The existence of neovascularization of iris has the highest susceptibility to predict the complication (PR = 5.091, P-value 0.014) following by the presence of retinopathy in zone 1 of the infant’s retina (PR = 4.386, P-value = 0.010).ConclusionThe incidence rate of complications related to Intravitreal bevacizumab injection was low, which was compatible with previous studies. Bevacizumab injection seems well tolerated in most cases of ROP. Iris neovascularization and the presence of retinopathy in zone 1 were associated with a higher occurrence of complications than the absence of these risk factors.

Posterior Segment Characterization in Children With Pierson Syndrome.

Pierson syndrome is a rare genetic disease defined by congenital nephrotic syndrome in association with microcoria. The authors aim to describe the posterior segment and retinal features in Pierson syndrome. A retrospective chart review of nine patients diagnosed with Pierson syndrome was ascertained. Details of ophthalmic history, ocular examination, retinal imaging, and surgical interventions were obtained during a median duration of 17 months of follow-up (range: 6 to 60 months). Retinal interventions included scatter laser photocoagulation and surgical retinal repair. Sixteen eyes of nine patients were included. The axial length of five eyes with flat retina was 26.59 mm ± 0.99 mm. Highly myopic features including tessellated fundus with accompanying optic disc pallor, unidentifiable cup, and abnormal retinal vascular emanation from the disc were observed in all eyes (100%), whereas 12 eyes (75%) had parapapillary chorioretinal atrophy. Features of abnormal retinal vascularization included avascular peripheral retina on fluorescein angiography, aberrant course of the temporal arcades in 13 eyes (81.3%), and straightened nasal retinal blood vessels in 12 eyes (75%). Tortuous retinal blood vessels were observed in three eyes (18.75%). Surgical repair was performed in five out of seven eyes with rhegmatogenous retinal detachment (RRD). Recurrence was observed in all eyes, which required two to three procedures to achieve final reattachment. Combined features of high axial myopia with incomplete peripheral vascular maturation characterize the posterior segment in Pierson syndrome. Careful posterior segment examination is essential to detect RRD or retinal neovascularization. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:618-627.].

The Fovea in Retinopathy of Prematurity.

PurposeBecause preterm birth and retinopathy of prematurity (ROP) are associated with poor visual acuity (VA) and altered foveal development, we evaluated relationships among the central retinal photoreceptors, postreceptor retinal neurons, overlying fovea, and VA in ROP.MethodsWe obtained optical coherence tomograms (OCTs) in preterm born subjects with no history of ROP (none; n = 61), ROP that resolved spontaneously without treatment (mild; n = 51), and ROP that required treatment by laser ablation of the avascular peripheral retina (severe; n = 22), as well as in term born control subjects (term; n = 111). We obtained foveal shape descriptors, measured central retinal layer thicknesses, and demarcated the anatomic parafovea using automated routines. In subsets of these subjects, we obtained OCTs eccentrically through the pupil (n = 46) to reveal the fiber layer of Henle (FLH) and obtained adaptive optics scanning light ophthalmograms (AO-SLOs) of the parafoveal cones (n = 34) and measured their spacing and distribution.ResultsBoth VA and foveal depth decreased with increasing ROP severity (term, none, mild, severe). In severe subjects, foveae were broader than normal and the parafovea was significantly enlarged compared to every other group. The FLH was thinner than normal in mild (but not severe) subjects. VA was associated with foveal depth more than group. Density of parafoveal cones did not differ significantly among groups.ConclusionsFoveal structure is associated with loss of VA in ROP. The preserved FLH in severe (relative to mild) eyes suggests treatment may help cone axon development. The significantly larger parafovea and increased outer nuclear layer (ONL) thickness in ROP hint that some developmental process affecting the photoreceptors is not arrested in ROP but rather is supranormal.

The process of revascularization in the neonatal mouse retina following short-term blockade of vascular endothelial growth factor receptors.

Misdirected vascular growth frequently occurs in the neovascular diseases in the retina. However, the mechanisms are still not fully understood. In the present study, we created capillary-free zones in the central and peripheral retinas in neonatal mice by pharmacological blockade of vascular endothelial growth factor (VEGF) signaling. Using this model, we investigated the process and mechanisms of revascularization in the central and peripheral avascular areas. After the completion of a 2-day treatment with the VEGF receptor tyrosine kinase inhibitor KRN633 on postnatal day (P) 4 and P5, revascularization started on P8 in the central avascular area where capillaries had been dropped out. The expression levels of VEGF were higher in the peripheral than in the central avascular area. However, the expansion of the vasculature in the peripheral avascular retina remained suppressed until revascularization had been completed in the central avascular area. Additionally, we found disorganized endothelial cell division, misdirected blood vessels with irregular diameters, and abnormal fibronectin networks at the border of the vascular front and the avascular retina. In the central avascular area, a slight amount of fibronectin as non-vascular component re-formed to provide a scaffold for revascularization. Mechanistic analysis revealed that higher levels of VEGF attenuated the migratory response of endothelial cells without decreasing the proliferative activity. These results suggest that the presence of concentration range of VEGF, which enhances both migration and proliferation of the endothelial cells, and the structurally normal fibronectin network contribute to determine the proper direction of angiogenesis.

Peripheral Vascular Abnormalities Detected by Fluorescein Angiography in Contralateral Eyes of Patients With Persistent Fetal Vasculature

To describe peripheral retinal vascular abnormalities in the contralateral eyes of patients with unilateral persistent fetal vasculature (PFV). Retrospective medical record review of fluorescein angiography (FA) findings of patients with unilateral PFV. Width of the temporal peripheral avascular retina in contralateral eyes was measured in disc diameters (DD). Vascular abnormalities were described. In 45 included patients, mean width of temporal peripheral avascular areas in contralateral eyes was 1.87 ± 0.71 DD. Forty-three patients (95.6%) had temporal peripheral avascular areas of 1 DD or greater in contralateral eyes, with 16 having temporal peripheral avascular areas of 2 DD or greater. Vessel shunts, vascular tortuosity and dilatation, circumferential vessels, and abnormal capillary beds in the peripheral retina were observed in 23 (51.1%) contralateral eyes of patients with unilateral PFV. PFV patients should undergo careful evaluations of both eyes, preferably with FA, to identify vascular changes that are not visible clinically. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:506-511.].

Study protocol for prognosis and treatment strategy of peripheral persistent avascular retina after intravitreal anti-VEGF therapy in retinopathy of prematurity

BackgroundProphylactic peripheral photocoagulation has been proposed to be applied to persistent, peripheral, avascular retina for retinopathy of prematurity (ROP) patients who have received an intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) agents. However, there are doubts regarding the necessity of this prophylactic action regardless of fundus fluorescein angiography (FFA) results. The adverse prognosis for persistent avascular retina after anti-VEGF therapy in ROP patients is not well understood. The relationship between vascular leakage and an adverse prognosis is also unknown. Therefore, it would be of value to study the above issues to shape the treatment strategy of persistent avascular retina after intravitreal anti-VEGF therapy in ROP patients.Methods/designThis is a prospective study of ROP patients with persistent avascular retina who have received anti-VEGF intravitreal therapy and have never received laser therapy. All the eyes being studied will be followed up and examined by FFA after anti-VEGF treatment and categorized into two groups, a leakage group and a non-leakage group according to the extent of vascular leakage from FFA results. The eyes being studied in the leakage group will be further randomized into two groups, a laser group and a non-laser group. A cohort study (observational) will be conducted on the non-leakage group and the non-laser group (with leakage) to investigate the incidence of an adverse prognosis for reactivation, retinal tear and retinal detachment; as well as to investigate the relationship between vascular leakage from FFA results and the abovementioned pathological changes. A randomized controlled study (experimental) will be conducted on the leakage group to compare the prognosis between the laser group and the non-laser group.DiscussionThe present study aims to investigate the occurrence rates of an adverse prognosis including reactivation, retinal tear and retinal detachment after anti-VEGF therapy in ROP patients with persistent avascular retina; to assess the relationship between vascular leakage from FFA results and the abovementioned pathological changes; to compare the prognosis of persistent avascular retina with or without prophylactic peripheral photocoagulation in these patients; to shape the treatment strategy and provide evidence for the indications of prophylactic photocoagulation.Trial registrationChinese Clinical Trial Registry (ChiCTR), ID: ChiCTR-ROC-17013253. Registered on 5 November 2017. http://www.chictr.org.cn/showproj.aspx?proj=22703

Retinopathy of Prematurity: Evolving Treatment With Anti–Vascular Endothelial Growth Factor

To discuss the evolution in retinopathy of prematurity since its first description as retrolental fibroplasia in the United States, including the changes in the understanding of pathophysiology; methods of diagnosis; destructive, anti-vascular endothelial growth factor (anti-VEGF), and supportive treatments; and differences in retinopathy of prematurity manifestations worldwide. The overall goal is to clarify retinopathy of prematurity currently and formulate questions to optimize future care. Literature review and synthesis. Critical review and consideration of the literature with inclusion of historical articles and those regarding pathophysiologic risk factors, retinopathy of prematurity worldwide, basic and clinical science particularly regarding anti-VEGF mechanisms and agents tested in clinical trials. Retinopathy of prematurity has evolved from affecting infants approximately 2months premature to affecting extremely premature infants. Worldwide, retinopathy of prematurity differs and, in emerging countries, has features similar to that experienced in the United States when retinopathy of prematurity first manifested. Treatments have evolved from destruction of the peripheral avascular retina to inhibit angiogenic stimuli to anti-VEGF agents, which inhibit pathologic angiogenesis but also extend normal intraretinal angiogenesis by ordering the development of intraretinal vessels. Clinical trial evidence is accruing with the goal to develop less destructive treatments to optimize vision and that are protective to the retina and infant. Goals for retinopathy of prematurity are to optimize prenatal and perinatal care, improve diagnostic acumen worldwide and refine treatment strategies, including with anti-VEGF agents, to inhibit intravitreal angiogenesis and facilitate vascularization of the previously avascular retina, which include supporting neural and vascular development of the premature infant and retina.

FLUORESCEIN ANGIOGRAPHY FINDINGS IN UNILATERAL PERSISTENT FETAL VASCULATURE.

This study aims to examine retinal vascular findings for affected eyes and contralateral eyes as well in typical cases of unilateral persistent fetal vasculature. We retrospectively reviewed all patients evaluated at Chang Gung Memorial Hospital, Linkou, for unilateral persistent fetal vasculature between January 2008 and July 2017. All patients underwent fluorescein angiography (FA) examination under general anesthesia. FA was performed using RetCam 3 (Clarity Medical Systems, Inc, Pleasanton, CA). Ten patients (eight male and two female) were identified as having adequate clinical data for the final analysis. The mean age at diagnosis was 13.7 ± 17.2 months (range 1-58). The mean axial length was shorter in the affected eyes as compared to the fellow eyes (17.27 ± 2.8 vs. 20.2 ± 1.7 mm; P = 0.024). In the affected eyes, nine cases (90.0%) showed a concomitant retrolental stalk, avascular peripheral retina, regional capillary dropout, and absence of foveal avascular zone. Hyperfluorescent stalk was seen in seven cases (70.0%). Four eyes (40.0%) showed leaking vessels. Terminal supernumerary branching was seen in two cases (20.0%). Popcorn hyperfluorescence was noted in one case (10.0%). In the fellow eyes, peripheral avascular zone was noted in nine eyes (90.0%), of which six (60.0%) had peripheral zones greater than two-disk diameters. Seven eyes (70.0%) presented with regional capillary dropout and abnormal choroidal filling. Three eyes (30.0%) had abnormal vessel straightening. Aberrant circumferential vessels and leaking spots were seen in two eyes (20.0%). Regional dilation of disk vessels, peripheral vessel dilation, and terminal bulbing were noted in one eye (10.0%). The mean best-corrected visual acuity of the fellow eyes was 20/39 (0.29 in logarithm of the minimum angle of resolution). Retinal vascular abnormalities in the affected eyes and fundoscopically normal fellow eyes of unilateral persistent fetal vasculature patients were found in 100% and 90.0% of patients, respectively. Fellow eyes had some subtle abnormalities that were only revealed through FA. These unilateral persistent fetal vasculature cases were still bilaterally affected.

Late-Onset Retinal Findings and Complications in Untreated Retinopathy of Prematurity

To investigate late retinal findings and complications of eyes with a history of retinopathy of prematurity (ROP) that did not meet treatment criteria and did not receive treatment during infancy. Retrospective, nonconsecutive, noncomparative, multicenter case series. Three hundred sixty-three eyes of 186 patients. Data were requested from multiple providers on premature patients with a history of ROP and no treatment during infancy who demonstrated late retinal findings or complications and included age, gender, gestational age and weight, zone and stage at infancy, visual acuity, current retina vascularization status, vitreous character, presence of peripheral retinal findings such as lattice retinal tears and detachments (RDs), retinoschisis, and fluorescein findings. Rate of RDs and factors conferring a higher risk of RDs. The average age was 34.5 years (range, 7-76 years), average gestational age was 26.6 weeks (range, 23-34 weeks), and average birth weight was 875 g (range, 425-1590 g). Findings included lattice in 196 eyes (54.0%), atrophic holes in 126 eyes (34.7%), retinal tears in 111 eyes (30.6%), RDs in 140 eyes (38.6 %), tractional retinoschisis in 44 eyes (11.9%), and visible vitreous condensation ridge-like interface in 112 eyes (30.5%). Fluorescein angiography (FA) was performed in 113 eyes, of which 59 eyes (52.2%) showed leakage and 16 eyes (14.2%) showed neovascularization. Incomplete vascularization posterior to zone 3 was common (71.6% of eyes). Retinal detachments were more likely in patients with a gestational age of 29 weeks or less (P < 0.05) and in eyes with furthest vascularization to posterior zone 2 eyes compared with zone 3 eyes (P= 0.009). Eyes with ROP not meeting the treatment threshold during infancy showed various late retinal findings and complications, of which RDs were the most concerning. Complications were seen in all age groups, including patients born after the Early Treatment for Retinopathy of Prematurity Study. Contributing factors to RDs included atrophic holes within peripheral avascular retina, visible vitreous condensation ridge-like interface with residual traction, and premature vitreous syneresis. We recommend regular examinations and consideration of ultra-widefield FA examinations. Prospective studies are needed to explore the frequency of complications and benefit of prophylactic treatment and if eyes treated with anti-vascular endothelial growth factor therapy are at risk of similar findings and complications.

Persistent Avascular Retina in Infants With a History of Type 2 Retinopathy of Prematurity: To Treat or Not to Treat?

To investigate persistent avascular retina in infants with type 2 retinopathy of permaturity (ROP) that persisted after 45 weeks' post-menstrual age when regular ROP screening ceased. A prospective observational study where fundus fluorescein angiography (FFA) was completed on consecutive infants who had a history of type 2 ROP and avascular retina during ROP screening that persisted after 45 weeks' post-menstrual age. FFA was completed on 72 eyes of 36 infants (53% male), with a mean gestational age of 26.0 ± 2.2 weeks and a mean birth weight of 834.6 ± 216.3 grams. The mean age at discharge from ROP screening was 47.6 weeks' post-menstrual age. All patients had type 2 ROP at the worst stage of their disease, with predominantly stage 2 disease. FFA was performed at a mean age of 18.8 ± 10.3 months post-menstrual age. All patients had detectable avascular retina in peripheral zone II or III on FFA. Peripheral vessel leakage was present in 3 eyes of 2 infants (5.5%), who both subsequently received peripheral laser treatment. Premature infants with type 2 ROP may have persistent peripheral avascular retina with unknown long-term ocular complications, which can present management dilemmas. Retinal FFA is recommended to determine retinal ischemia and aid decision making for treatment in these cases. [J Pediatr Ophthalmol Strabismus. 2019;56(4):222-228.].

Peripheral retinal avascularity and capillary leakage in central serous chorioretinopathy

PurposeTo report a novel finding of peripheral retinal avascularity (PRA) and peripheral capillary leakage (PCL) on wide-field fluorescein angiography (WFA) in non-dependent quadrants, in eyes with bilateral chronic central serous chorioretinopathy (CSCR). MethodsForty six patients with bilateral CSCR were studied. Four patients had PRA and PCL, and 42 patients did not. The demographic profile, clinical findings, and imaging characteristics of the two groups were compared. ResultsThere was no significant difference between those patients with and without PRA and PCL with respect to the demographic profile, clinical findings, and imaging characteristics. Laser photocoagulation to extrafoveal points of leakage seen on fluorescein angiography (FA) was sufficient to cause complete resolution of CSCR in these 4 patients. PRA areas were not treated. ConclusionThe PRA and PCL in CSCR are novel findings, which have not been previously described.

Management of retinopathy of prematurity in a neonatal unit: Current approach

Retinopathy of prematurity (ROP) is a blinding morbidity affecting preterm infants. It currently represents the leading preventable cause of childhood blindness worldwide. Most data indicate an increasing incidence of ROP disease in both industrialized countries and in the developing world. There are neither symptoms of ROP nor can a specific visual behavior in a preterm infant herald a concern for ROP. Hence, an effective screening is essential for prompt diagnosis of ROP. The available evidence suggests that the majority of premature infants who go blind from ROP do so due to screening failure. Timely screening of premature infants at risk is as important as early treatment in the management of ROP. The screening protocol at each neonatal intensive care unit (NICU) should be evidence-based, should be based on preferences of neonatologists, ophthalmologists, and NICU nurses. All at-risk infants should be identified and receive adequate dilated retinal examinations at appropriate times. Appropriate screening and follow-up guidelines and timely treatment protocols need to be implemented in every NICU by pediatricians and ophthalmologists to reduce the ROP-related blindness in the community. The ultimate goals of treatment of ROP are prevention of retinal detachment or scarring and optimization of visual outcome. The standard treatment involves ablation of peripheral avascular retina preferably by indirect retinal laser photocoagulation when the ROP progresses to a stage which needs intervention since vascular endothelial growth factors (VEGF) are known to play a major role in ROP pathogenesis and its progression, injection of anti-VEGF drugs intravitreally has been found to be effective in arresting the ROP disease process. This newer emerging pharmacotherapeutic option has the potential to improve treatment outcomes.