Referable Macular Hemorrhage-A Clinically Meaningful Screening Target in Newborn Infants. Position Statement of the Association of Pediatric Retina Surgeons.

Universal newborn eye screening facilitates early diagnosis of ocular abnormalities and mitigates vision loss. "Referral warranted" eye disease is present at birth in about 5.5% of term infants, with "macular hemorrhage impinging on the fovea" representing about 50% of referral warranted disease. The Association of Pediatric Retina Surgeons held a symposium on February 9, 2021 that culminated in a position statement on "referable macular hemorrhage" (RMH) in newborn infants. RMH is meaningful in that in can cause amblyopia through deprivation, can be readily captured with wide-angle photography in a safe and efficient manner, and may lead to early intervention with mitigation of vision loss. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:3-6.].

International Classification of Retinopathy of Prematurity, Third Edition.

PURPOSE: The International Classification of Retinopathy of Prematurity is a consensus statement that creates a standard nomenclature for classification of retinopathy of prematurity (ROP). It was initially published in 1984, expanded in 1987, and revisited in 2005. This article presents a third revision, the International Classification of Retinopathy of Prematurity, Third Edition (ICROP3), which is now required because of challenges such as: (1) concerns about subjectivity in critical elements of disease classification; (2) innovations in ophthalmic imaging; (3) novel pharmacologic therapies (e.g., anti-vascular endothelial growth factor agents) with unique regression and reactivation features after treatment compared with ablative therapies; and (4) recognition that patterns of ROP in some regions of the world do not fit neatly into the current classification system. DESIGN: Review of evidence-based literature, along with expert consensus opinion. PARTICIPANTS: International ROP expert committee assembled in March 2019 representing 17 countries and comprising 14 pediatric ophthalmologists and 20 retinal specialists, as well as 12 women and 22 men. METHODS: The committee was initially divided into 3 subcommittees-acute phase, regression or reactivation, and imaging-each of which used iterative videoconferences and an online message board to identify key challenges and approaches. Subsequently, the entire committee used iterative videoconferences, 2 in-person multiday meetings, and an online message board to develop consensus on classification. MAIN OUTCOME MEASURES: Consensus statement. RESULTS: The ICROP3 retains current definitions such as zone (location of disease), stage (appearance of disease at the avascular-vascular junction), and circumferential extent of disease. Major updates in the ICROP3 include refined classification metrics (e.g., posterior zone II, notch, subcategorization of stage 5, and recognition that a continuous spectrum of vascular abnormality exists from normal to plus disease). Updates also include the definition of aggressive ROP to replace aggressive-posterior ROP because of increasing recognition that aggressive disease may occur in larger preterm infants and beyond the posterior retina, particularly in regions of the world with limited resources. ROP regression and reactivation are described in detail, with additional description of long-term sequelae. CONCLUSIONS: These principles may improve the quality and standardization of ROP care worldwide and may provide a foundation to improve research and clinical care.

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

PURPOSE: 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. DESIGN: Retrospective, nonconsecutive, noncomparative, multicenter case series. PARTICIPANTS: Three hundred sixty-three eyes of 186 patients. METHODS: 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. MAIN OUTCOME MEASURES: Rate of RDs and factors conferring a higher risk of RDs. RESULTS: 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). CONCLUSIONS: 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.

Long-Term Outcomes of Total Exudative Retinal Detachments in Stage 3B Coats Disease.

PURPOSE: To evaluate the long-term outcomes of treatment of total exudative retinal detachments (ERDs) secondary to Coats disease (stage 3B) and the role of vitrectomy. DESIGN: Retrospective, observational case series. PARTICIPANTS: A total of 16 eyes in 16 patients undergoing treatment for total ERDs secondary to Coats disease with at least 5 years of follow-up. METHODS: We reviewed the records of patients with stage 3B Coats disease. The interventions, including the timing of vitrectomy if used, and clinical course were recorded. MAIN OUTCOME MEASURES: The primary outcome measures were visual acuity at the most recent appointment, whether there was progression to neovascular glaucoma (NVG) or phthisis bulbi, and need for enucleation. RESULTS: All patients received ablative treatment (photocoagulation or cryotherapy), with 8 having scleral buckling (SB) and 6 having external drainage of subretinal fluid (XD). Of the 12 patients who had pars plana vitrectomy (PPV), 8 had early PPV (EV) in the first year after presenting, and 4 of 8 in the expectant management group had late PPV (late vitrectomy) at a mean of 4.3 years post-presentation for treatment of significant traction retinal detachment (TRD). The other 4 patients of 8 in the expectant management group did not require vitrectomy. Mean follow-up overall was 9 1/2 years. At the date of last follow-up, 50% had no light perception or light perception vision, which was consistent across the subgroups that underwent EV (4/8), late vitrectomy (2/4), or no PPV (2/4). A total of 4 of 16 patients had progression to NVG or phthisis, 1 of whom required enucleation. CONCLUSIONS: In this retrospective series of patients with Stage 3B Coats disease, ablative therapy with a combination of PPV, XD, or SB was effective in preventing progression to NVG or phthisis in the majority of patients, thus preserving the globe. Half of the patients (4/8) in this series who did not undergo PPV in the early vitrectomy group developed late-onset TRD, suggesting a possible role for early prophylactic vitrectomy with possible SB and XD; however, this is balanced by the other half (4/8) in the expectant management group who did not require any vitrectomy.

Spectral-domain optical coherence tomography of the macula in preterm infants treated with bevacizumab for retinopathy of prematurity.

BACKGROUND AND OBJECTIVE: To examine the macula in preterm infants treated with bevacizumab (Avastin; Genentech, South San Francisco, CA) for retinopathy of prematurity (ROP) using spectral domain optical coherence tomography (SD-OCT). PATIENTS AND METHODS: Twenty eyes (of 11 patients) treated with Avastin only or in addition to photocoagulation and vitreous surgery were included. Imaging was performed with a portable spectral-domain optical coherence tomography (OCT) device. Macular measurements, including the foveal and parafoveal index were calculated. RetCam (Clarity Medical Systems, Pleasanton, CA) photographs were obtained for all patients. RESULTS: Mean age of patients at OCT examination was 28.9 months (range: 4 to 50 months). Thirteen eyes (65%) developed foveal contour (FC), and seven eyes (35%) did not. Earlier gestational age and zone I disease were associated with absence of FC development. In patients without FC, two of the five eyes had vision better than 20/400, compared with 10 of 11 eyes with FC. CONCLUSION: Vision tends to be better in Avastin-treated eyes when an FC is present. Morphological characteristics of retinal anatomy may predict visual function.

Incidence of endophthalmitis after 20-gauge vs 23-gauge vs 25-gauge pars plana vitrectomy.

PURPOSE: To compare endophthalmitis rates after 20-gauge versus 23-gauge versus 25-gauge pars plana vitrectomy (PPV) in 2007-2008, and compare the rates with those of 2005-2006. METHODS: Multicenter study including all patients who developed endophthalmitis following PPV performed by any of the authors during 2005-2008, and all patients who developed endophthalmitis following PPV at Penn State College of Medicine and Bascom Palmer Eye Institute during 2005-2008. The endophthalmitis rates after 20-gauge, 23-gauge and 25-gauge PPV during 2007-2008 were compared to those from 2005-2006. RESULTS: The endophthalmitis incidence during 2007-2008 was 1/4,403 (0.02%) for 20-gauge PPV, 1/3,362 (0.03%) for 23-gauge PPV, and 1/789 (0.13%) for 25-gauge PPV. There is no significant difference among these rates between any two of the three groups. Compared with the endophthalmitis rates among the same group of surgeons during 2005-2006, the 2007-2008 endophthalmitis rates following 20-gauge and 23-gauge PPV were stable, and the rate following 25-gauge PPV was marginally lower (p = 0.056; odds ratio = 0.15; 95% CI: (0.003, 1.03)). CONCLUSIONS: There was no significant difference in the 2007-2008 rates of endophthalmitis following 20-gauge versus 23-gauge versus 25-gauge PPV; among the same group of surgeons, the 2007-2008 rate of endophthalmitis following 25-gauge PPV was marginally lower than the 2005-2006 rate.

Vitrectomy after intravitreal bevacizumab (Avastin) for retinal detachment in retinopathy of prematurity.

PURPOSE: The purpose of this study was to evaluate the feasibility of performing vitrectomies after using bevacizumab (Avastin, Genentech, South San Francisco, CA) for the management of retinal detachments in retinopathy of prematurity. METHODS: Eleven eyes of 8 children with retinopathy of prematurity retinal detachment that developed despite peripheral ablation with indirect diode laser were injected with 0.625 mg bevacizumab. Injections were given through the pars plicata, and the status of the central retinal artery was checked with indirect ophthalmoscopy. A vitreoretinal surgery was scheduled for 1 week after the bevacizumab injection. All eyes underwent surgery using 25- or 23-gauge instrumentation. Demographic data, neovascular activity, and the anatomic status of the retina were evaluated. Systemic and local perioperative complications were recorded. RESULTS: The average gestational birth weight was 950 g, and the average gestational age at birth was 25.7 weeks. Although 9 eyes had stage 4A, 2 had stage 4B retinal detachments. Six eyes had zone II and five eyes had zone I disease. Bevacizumab was injected at an average postgestational age of 38 weeks. A notable reduction in the vascular activity could be observed 1 week later when vitrectomy was performed. With a mean follow-up of 8.5 months (range, 2-13 months), a favorable outcome could be observed in all eyes. Only one eye with persistent vitreous hemorrhage required reoperation. No complications attributable to the bevacizumab injections were noted. CONCLUSION: These data show that vitrectomy after intravitreal bevacizumab can be effective in selected cases. The regression of the vascular activity induced by this drug might contribute to better results in some of these cases. There were no apparent short-term safety concerns for intravitreal bevacizumab injections in these babies. However, additional studies are needed to define the role of bevacizumab as a possible adjuvant in the management of retinopathy of prematurity retinal detachments.

Treatment of type 1 retinopathy of prematurity with intravitreal bevacizumab (Avastin).

PURPOSE: The purpose of this study was to report a noncomparative consecutive case series of eyes with type 1 retinopathy of prematurity treated only with intravitreal bevacizumab (Avastin). METHODS: Twelve consecutive eyes of 7 premature infants, with type 1 retinopathy of prematurity as stated in the Results of the Early Treatment for Retinopathy of Prematurity Randomized Trial, were treated with only one intravitreal injection of bevacizumab (0.625 mg). RetCam pictures were used to document the cases. RESULTS: Nine eyes had zone I and 3 eyes had zone II retinopathy of prematurity. The infants weighed between 600 and 1,100 grams (mean, 846.57 g). The gestational age ranged from 23 weeks to 28 weeks (mean, 25.57 weeks). All eyes showed regression of the disease with no additional treatment needed. CONCLUSION: Intravitreal bevacizumab is a useful therapy for type 1 retinopathy of prematurity. However, additional studies are needed to define the role of Avastin as the first-line therapy, in combination, or after the failure of laser photocoagulation.

Endophthalmitis after 25-gauge and 20-gauge pars plana vitrectomy: incidence and outcomes.

PURPOSE: To compare the rates of endophthalmitis after 20-gauge versus 25-gauge pars plana vitrectomy (PPV) and to investigate clinical features of, and visual acuity outcomes, for patients with endophthalmitis after PPV. METHODS: A computerized database search was performed at each author's institution to identify all patients who underwent PPV by any of the authors between January 1, 2005, and December 31, 2006, and were subsequently treated for endophthalmitis. In addition, all patients who underwent PPV and were subsequently treated for endophthalmitis at Pennsylvania State College of Medicine (Hershey, PA) and Bascom Palmer Eye Institute (Miami, FL) during the study period were included. The medical records of these patients were reviewed to confirm that the endophthalmitis was associated with PPV and to collect clinical data to meet the study objectives. RESULTS: The incidence of endophthalmitis during the study period was 2 cases per 6,375 patients (or 1 case per 3,188 patients; 0.03%) for 20-gauge PPV compared with 11 cases per 1,307 patients (or 1 case per 119 patients; 0.84%) for 25-gauge PPV (P < 0.0001). Of 11 eyes that developed endophthalmitis after 25-gauge PPV, 9 received endophthalmitis prophylaxis with subconjunctival cefazolin after surgery. Median intraocular pressure on postoperative day 1 was 13 mmHg (range, 5-27 mmHg). Median time between PPV and endophthalmitis presentation was 3 days (range, 1-15 days). Presenting vision was hand motions or better in all eyes. Initial treatment included vitreous tap and injection of antibiotics in nine eyes and PPV and injection of antibiotics in two. All patients received intraocular treatment with vancomycin, and 10 received ceftazidime treatment. Eight patients had final visual acuity of >/=20/400, and four had visual acuity of >/=20/63. Cultures were negative in three cases; no culture specimens were obtained in one case. Six of the seven isolates were coagulase-negative staphylococci, and one was enterococcus. Five of six isolates tested for sensitivity to vancomycin were sensitive, and both isolates tested for sensitivity to ceftazidime were sensitive. CONCLUSIONS: The rate of endophthalmitis after 25-gauge PPV was significantly higher than that after 20-gauge PPV. Endophthalmitis after 25-gauge PPV occurred within 15 days of PPV, was usually due to coagulase-negative staphylococci sensitive to vancomycin, and was associated with variable visual outcomes.

25-gauge lens-sparing vitrectomy for stage 4A retinopathy of prematurity.

PURPOSE: To evaluate the feasibility of performing lens-sparing vitrectomies using 25-gauge instruments for the management of stage 4A retinal detachments in Retinopathy of Prematurity (ROP). METHODS: 13 eyes of 10 patients with stage 4A retinal detachments underwent a lens-sparing pars plicata vitrectomy. All patients were operated using a three port transconjunctival 25-gauge sutureless technique. The sclerotomies were made 0.5 to 1.0 mm posterior to the limbus through the pars plicata. Core vitrectomy and membrane peeling were performed. A partial fluid-air exchange was used at the end of the procedure in the majority of the cases. The anatomic status of the retina was determined by ophthalmoscopy during routine follow up visits or under general anesthesia. Per-operative complications were recorded. RESULTS: Eight of the infants were female and two were male. The average postmenstrual age at birth was 26.4 weeks and the average postgestational age at time of surgery was 39.3 weeks (range, 33-44). Three out of the 13 eyes (23%) had zone I ROP. With a mean follow-up of 17 months (range, 4-36) 12 out of the 13 eyes (92%) achieved successful retinal reattachment. One eye developed post operative hypotony with a small choroidal detachment that resolved spontaneously. CONCLUSIONS: Surgical intervention with a three port 25-gauge transconjunctival sutureless vitrectomy is an effective technique to attach the retina in patients with stage 4A retinal detachment in ROP.

Zone I retinopathy of prematurity: clinical characteristics and treatment outcomes.

PURPOSE: To study the clinical characteristics and treatment outcomes after laser and surgical treatment in a series of infants with zone I retinopathy of prematurity (ROP). METHODS: Preterm infants less than 1,500 g at birth diagnosed with zone I ROP were studied. Fifty-seven consecutive eyes in 29 infants with zone I ROP were treated with indirect diode laser. Within zone I, two anatomic subgroups (anterior and posterior) were defined. Additionally, 27 eyes (15 patients) with retinal detachment secondary to zone I ROP were surgically treated. Structural outcomes were evaluated 3 months after surgery. RESULTS: Atypical morphologic characteristics were observed. Thirty-one out of 48 eyes (64.6%) with anterior zone I ROP and none of the 9 eyes with posterior zone I ROP had a favorable outcome (P < 0.001) after photocoagulation. In the surgery group, 14 out of 19 (74%) eyes with stage 4 and 1 out of 8 (13%) eyes with stage 5 had favorable outcomes. CONCLUSIONS: Atypical clinical features can be observed in zone I ROP. A posterior localization of the disease carries a worse prognosis after laser photocoagulation. Surgical intervention can effectively reattach the retina in a significant number of zone I ROP-related retinal detachments.

Retinal pigment epithelium translocation after choroidal neovascular membrane removal in age-related macular degeneration.

PURPOSE: To test the feasibility of a new surgical technique and to assess visual function over the translocated retinal pigment epithelium (RPE) cells in patients operated on for subfoveal choroidal neovascularization (CNV) secondary to age-related macular degeneration (ARMD). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Nine patients with previously untreated exudative ARMD underwent surgical excision of the subfoveal CNV with RPE translocation and were observed for 12 to 32 months. METHODS: The surgery consisted of a standard three-port pars plana vitrectomy, excision of the CNV, and RPE translocation. Pre- and postoperative ocular examination included best-corrected visual acuity measurement, fundus color stereo photography, and fundus fluorescein angiography. Optical coherence tomography and confocal laser scanning ophthalmoscopy (cLSO) were performed after surgery. A crossfixation target and a single-point flashing light were projected on different areas of the posterior pole using a cLSO. Photopic 10 to 2 perimetry, photopic fine matrix mapping, and cLSO microperimetry were also performed after surgery in six patients. MAIN OUTCOME MEASURES: Optical coherence tomography cross-sectional scans and cLSO RPE autofluorescence were recorded to detect the presence of viable translocated RPE. Visual acuity, fixation, photopic 10 to 2 perimetry, photopic fine matrix mapping, and cLSO microperimetry were used to test central visual function. RESULTS: Retinal pigment epithelium was translocated successfully at the time of CNV removal from the edge of the RPE defect to a subfoveal location in seven of nine patients. One patient experienced proliferative vitreoretinopathy, but significant hemorrhage was not a feature. Optical coherence tomography showed the translocated RPE as an area of increased optical reflectivity with optical shadowing external to it. Confocal laser scanning ophthalmoscopy showed autofluorescence of the translocated RPE. The crossfixation target was seen when projected on the translocated RPE. During eccentric fixation, the patients could see a flashing point-target projected on the translocated RPE. Photopic 10 to 2 perimetry, photopic fine-matrix mapping, and cLSO microperimetry showed the presence of central visual function. CONCLUSIONS: The authors propose that translocation of RPE at the time of CNV removal, from the edge of the RPE defect to a subfoveal location, may have a role in the surgical management of ARMD.

Vitreoretinal management of posteriorly dislocated plate haptic silicone intraocular lenses.

PURPOSE: To report the vitreoretinal management of posteriorly dislocated plate haptic silicone intraocular lenses (PHSIOLs) and to present a surgical algorithm for this problem. METHODS: A retrospective analysis of 12 cases of immediate and delayed posterior dislocation of a PHSIOL after phacoemulsification was performed. RESULTS: The ages of the patients ranged from 26 years to 82 years (mean, 68 years); six of the patients were male. Delayed posterior dislocation of the PHSIOL occurred in 7 eyes (after Nd:YAG laser capsulotomy in 4 cases), while intraoperative dislocation occurred in 5 eyes. Defects of the anterior capsulorhexis rim or posterior capsule were present in nine eyes. The mean time from PHSIOL dislocation to definitive treatment was 4.8 months (range, 0.25-13 months). All patients underwent pars plana vitrectomy and implant exchange or repositioning. The dislocated PHSIOL was removed through the transpupillary route in 8 cases, while it was segmented and delivered through the pars plana in 3. Two patients underwent implant repositioning in the ciliary sulcus, although subsequent redislocation in one case required transpupillary removal and exchange. The mean follow-up period was 6.5 months (range, 2-18 months), with a final best corrected visual acuity of 6/12 or better in 10 eyes. CONCLUSIONS: Delayed and immediate dislocation of PHSIOL can occur in eyes with a defect in capsular integrity. The implant may be repositioned or exchanged following vitrectomy with either transpupillary or pars plana removal, resulting in generally excellent postoperative visual acuity and minimal complications.