Long-term efficacy and complications of intravitreal anti-vascular endothelial growth factor agents combined with ablative therapies in juvenile Coats disease: a five year follow-up study.

PURPOSE: To evaluate the long-term safety and efficacy of adjuvant intravitreal anti-VEGF therapy in juvenile Coats disease. METHODS: This retrospective, observational study included a total of 62 eyes in 62 pediatric patients with juvenile Coats disease treated with intravitreal anti-VEGF agents followed for a mean of 67.08 months (ranged from 60 to 93 months). All affected eyes were managed initially with one session of ablative treatment plus adjuvant intravitreal anti-VEGF agent (0.5 mg/0.05 ml ranibizumab or conbercept). Ablative treatment was repeated if telangiectatic retinal vessels were not completely regressed or recurred. Anti-VEGF therapy was repeated if subretinal fluid or macular edema still existed. Treatments above were repeated every 2 to 3 months. We reviewed clinical and photographic records of patients including the demographics, clinical characteristics and interventions. RESULTS: At final visit, all 62 affected eyes had partially or completely disease resolution; none progressed to advanced stage namely neovascular glaucoma or phthisis bulbi, respectively. No ocular or systemic side effects related to intravitreal injections were observed during follow-up. In terms of 42 affected eyes that could cooperate with visual examination, best corrected visual acuity improved in 14 (14/42, 33.3%) eyes, stabled in 25 (25/42, 59.5%) eyes, and worsened in 3 (3/42, 7.1%) eyes. In the field of complications, 22 (22/62, 35.5%) eyes developed cataracts; 33 (33/62, 53.2%) eyes developed vitreoretinal fibrosis, of whom 14 (14/33, 42.4%) eyes in the subgroup of stage 3B developed progressive TRD; 40 (40/62, 64.5%) eyes developed subretinal fibrosis. Multivariate regression analysis showed increased clinical stage may be associated with the development of vitreo- and subretinal fibrosis (adjusted odds ratio:16.77,17.59; 95% CI:4.50-62.53, 3.98-77.86, respectively, all P < 0.001). CONCLUSION: Adjuvant intravitreal ranibizumab or conbercept combined with ablative therapies may be a long-term safe and effective treatment for juvenile Coats disease.

Retinal honeycomb appearance and its role in patients with X-linked retinoschisis.

BACKGROUND: To investigate the clinical characteristics of retinal honeycomb appearance in a large cohort of patients with X-linked retinoschisis (XLRS) and to determine whether it is associated with complications like retinal detachment (RD) and vitreous hemorrhage (VH). METHODS: A retrospective observational case series. A chart review of medical records, wide-field fundus imaging, and optical coherence tomography (OCT) was performed on 78 patients (153 eyes) diagnosed with XLRS at Beijing Tongren eye center between Dec 2017 and Feb 2022. The chi-square test or Fisher exact test was performed on the 2 × 2 cross-tabulations of honeycomb appearance and other peripheral retinal findings and complications. RESULTS: Thirty-eight patients (48.7%), and 60 eyes (39.2%) had a honeycomb appearance of different areas on the fundus. The supratemporal quadrant was the most commonly affected (45 eyes, 75.0%), followed by the infratemporal (23 eyes, 38.3%), the infranasal (10 eyes,16.7%), and supranasal (9 eyes,15.0%). The appearance was significantly associated with peripheral retinoschisis, inner retinal layer break, outer retinal layer break, RD, and rhegmatogenous retinal detachment (RRD) (p < 0.01, p = 0.032, p < 0.01, p = 0.008, p < 0.01, respectively). All the eyes complicated with RRD had the appearance. None of the eyes without the appearance had RRD. CONCLUSIONS: The data suggest that the honeycomb appearance is not uncommon in patients with XLRS and is more likely to be accompanied by an RRD, and inner and outer layer breaks, thus should be treated with caution and close observation.

Abnormalities of the contralateral eye in unilateral congenital anophthalmic or blind microphthalmic patients.

PURPOSE: To describe the prevalence, distribution, and features of anterior and posterior segment abnormalities in the contralateral eye in cases unilateral of anophthalmia and blind microphthalmia. METHODS: The medical records of patients with unilateral congenital anophthalmia and blind microphthalmia referred to Beijing Tongren Hospital between January 2017 and December 2021 were reviewed retrospectively to investigate the prevalence of abnormalities of the fellow eye. RESULTS: A total of 168 patients were included. Of these, 28 (16.7%) had fellow eye abnormalities, 4 (2.4%) with anterior segment involvement in the contralateral eye. All 28 had fundus abnormalities. The most common posterior segment finding was coloboma (7.7%), followed by optic nerve dysplasia (3.0%), familial exudative vitreoretinopathy (FEVR) or FEVR-like fundus (1.8%), morning glory disk anomaly (1.8%), and retinal nerve fiber layer defect (1.2%). High myopia fundus changes (0.6%), retinal folds (0.6%), maculopathy (0.6%), peripapillary staphyloma (0.6%), and Bergmeister optic disk (0.6%) were also noted. CONCLUSIONS: Patients with unilateral congenital anophthalmia or blind microphthalmia have a high probability of contralateral eye disease. The most common abnormality is coloboma.

Clinical Characteristics of Pediatric Coats' Disease With Retinal Cyst Using Wide-Angle Fluorescein Angiography.

Purpose: To assess the demographic and treatment features of pediatric patients of Coats' disease with retinal cyst using wide-angle FA. Design: A retrospective, hospital based, cross-sectional study. Participants: Pediatric patients of Coats' disease underwent wide-angle FA. Methods: A retrospective review of pediatric patients of Coats' disease who underwent wide-angle FA at a single center from January 2015 to July 2020. Demographic and treatment features were compared between patients with or without retinal cyst. Main Outcome Measures: Demographic and treatment outcomes. Results: There were 123 pediatric Coats' patients in our study, and 18.70% (23/123) of the patients developed complications with retinal cyst, 73.9% (17/23) of the retinal cysts were located in the inferior-temporal quadrant and 82.6% (19/23) of the retinal cysts were located in the peripheral retina anterior to the vortex veins. Compared with patients without retinal cyst, patients with retinal cyst had more clock-hours of telangiectasia on FA (7.32 vs. 5.41, p = 0.031), and may need more total treatments (7.47 vs. 3.53, p = 0.023) including laser photocoagulation (4.08 vs. 2.31, p = 0.019) or intravitreal anti-VEGF (3.13 vs. 2.23, p = 0.039), and also required a longer time for telangiectasia resolution (22.33 vs. 18.53 months, p = 0.043). Conclusion: Pediatric patients with Coats' disease complicated by retinal cyst presented with more clock-hours of telangiectasia on FA and needed more total treatments and longer time for telangiectasia resolution.

Novel transscleral sutureless subretinal fluid drainage using a 25-gauge trocar-cannula with a self-closing valve in patients with advanced Coats disease.

AIM: To assess surgical outcomes of a novel method of transscleral drainage of subretinal fluid using a 25-gauge trocar-cannula with a self-closing valve (DTV) in patients with severe exudative retinal detachment (ERD) in Coats disease. METHODS: Retrospective consecutive cases of 20 patients (20 eyes) of severe ERD due to Coats disease (stage 3B) in total 156 Coats patients between June 2015 and April 2019 were included in this study. The participants were aged 1 to 10y with a mean age of 3.50±1.79y. The mean follow-up time were 11.9mo. Subretinal fluid was drained transsclerally using a novel method of DTV. The height of the retinal detachment and the regression of abnormal vessels including telangiectasias and aneurysms were observed. Complications including vitreoretinal fibrosis, tractional retinal detachments (TRD), endophthalmitis, retinal holes, and hemorrhages were evaluated. RESULTS: Following surgeries, the patients showed the replacement of ERD and regression of telangiectatic retinal vessels observed with binocular indirect ophthalmoscopy. Six patients received retinal cryotherapies and 12 patients received laser photocoagulations following first external subretinal fluid drainage using DTV. All patients underwent intravitreal anti-vascular endothelial growth factor therapies to induce residual subretinal fluid absorption. During follow-ups, 8 patients underwent a second drainage operation, 17 patients received retinal laser photocoagulations and 7 patients received cryotherapies. Vitreoretinal fibrosis was found in 7 patients and 6 patients underwent micro-invasive vitrectomies during the follow-up period. Severe TRD, iatrogenic retinal holes, and hemorrhages were not found. CONCLUSION: The authors present a new therapeutic approach that successfully drains subretinal fluid in advanced stage 3B Coats disease with severe ERD. This is a simple, safe and less invasive approach when compared with traditional managements. However, it should be strictly selected for patients with high bullous ERD close to the central axis of the eye in order to avoid the complication of retinal holes.

Outcomes of secondary sulcus intraocular lens implantation in unilateral anterior persistent fetal vasculature.

AIM: To evaluate the surgical results of sulcus intraocular lens (IOL) implantation in children with unilateral anterior persistent fetal vasculature (PFV) underwent primary vitrectomy combined with lensectomy and preservation of the peripheral anterior capsule. METHODS: Twenty-two eyes of 22 children with unilateral anterior PFV who underwent sulcus secondary IOL implantation were analyzed. Main outcome measures were preoperative and postoperative visual acuity, and complications both intraoperatively and postoperatively. RESULTS: Review of 22 consecutive patients identified best-corrected visual acuity (BCVA) improvement from 1.37±0.84 to 0.73±0.57 logarithm of the minimal angle of resolution (logMAR) after IOL implantation (P<0.001) with a mean follow-up was 16.55±5.86mo. Average age at secondary IOL implantation was 41.05±15.41mo. Three eyes (13.64%) achieved BCVA of 0.3 logMAR at the final visit. Transient intraocular pressure rise (4 eyes; 18.18%), postoperative increased inflammation (3 eyes; 13.64%) and postoperative hypotony (2 eyes; 9.09%) were common complications. CONCLUSION: Properly preservation of the anterior lens capsule during the primary surgery facilitated secondary sulcus IOL implantation in pediatric patients with anterior PFV, with favorable postoperative visual outcomes and compatible percentage of complications.

Universal ocular screening of 481 infants using wide-field digital imaging system.

BACKGROUND: Universal ocular screening of infants is not a standard procedure in children's health care system in China. This pilot study investigated prevalence of ocular abnormalities of 6 weeks-age infants using wide-field digital imaging system. METHODS: Infants aged 6 weeks around were consecutively enrolled in a public hospital between April 2015 and August 2016. All the infants who were enrolled in the study underwent vision assessment, eye position examination, external eye check, pupillary light reflex, red reflex examination, anterior and posterior ocular segments were examined using flashlight, ophthalmoscope, and wide-field digital imaging system. RESULTS: A total of 481 infants at 45.1 ± 6.1 days after birth were enrolled in the study. 198 infants had abnormal findings (41.2%). Retinal white spots and retinal white areas were the most common findings (42.9% of abnormalities and 17.7% of all infants screened). The second major finding was retinal hemorrhage (16.2% of abnormalities and 6.7% of all infants screened). Other abnormal findings include retinal pigmentation, concomitant exotropia, neonatal dacryocystitis, retinopathy of prematurity, 'albinism-like fundus', congenital nasolacrimal duct obstruction, familial exudative vitreoretinopathy, immature retina, corneal dermoid tumor, large physiologic cupping of optic disc, congenital persistent pupillary membrane, entropion trichiasis, subconjunctival hemorrhage, congenital cataract, vitreous hemorrhage, ptosis and choroidal nevus. Intervention of any form was required in 22 infants, which accounted for 11.1% of abnormalities detected and 4.6% of all infants screened. CONCLUSION: Universal ocular screening is not only necessary for preterm infants but also for full-term infants. Addition of red reflex examination with wide-field digital imaging system can enhance the sensitivity of screening for ocular fundus abnormities. Further study with a long-term follow-up is needed in the future.

The role of intravitreal ranubizumab in the treatment of familial exudative vitreoretinopathy of stage 2 or greater.

AIM: To evaluate the role of intravitreal ranubizumab (IVR) in the treatment of familial exudative vitreoretinopathy (FEVR) of stage 2 or greater either as primary or an ajunct to conventional treatments. METHODS: Retrospective, non-controlled clinical study. Thirty patients (37 eyes) diagnosed with FEVR were enrolled. Twenty patients (66.67%) were male and 10 patients (33.33%) were female. Age ranged from 0.4 to 35 years old (median 3y). IVR was used either as primary or as a combined therapy according to the retinal neovasuclar activities. The follow up ranged from 1 to 57mo with mean 16.73±15.73 (median 11)mo. The treatment effect of retinal neovasuclar activites were recorded as well as the ocular and systemic side effects. RESULTS: Among 30 patients (37 eyes), 10 eyes received single IVR, 1 eye received 2 injections. Three eyes were treated with IVR and simutanous laser photocoagulation. Laser indirect ophthalmoscopy (LIO) was applied in 5 eyes 1mo after the primary IVR. Seven eyes were treated surgically following the primary IVR due to persistent retinal neovasuclar activities and retinal traction. IVR was used as combined treatment with vitrectomy in 11 eyes. Retinal neovascular regression was notified 1mo following the primary IVR in all eyes. Neither systemic nor ocular complications were recorded. CONCLUSION: IVR may be an effective modality in the treatment of FEVR either as primary or as an ajunct to the conventional therapies. The long term effect and safty of IVR still need further research.

The effects of a treatment combination of anti-VEGF injections, laser coagulation and cryotherapy on patients with type 3 Coat's disease.

BACKGROUND: To examined the curative effect of vitreous injection with ranibizumab,laser coagulation and cryotherapy in treating stage 3 Coats' disease with exudative retinal detachment. METHODS: Seventeen patients with stage 3 Coats' disease were enrolled in the study. All eyes were treated with vitreous injection of ranibizumab as initial treatment, and subsequent treatment depended on the absorption of subretinal fluid, Including cryotherapy and laser photocoagulation. Repeat treatment for the two treatment intervals occurred in ≥1 month. The mean follow-up time was 24.12 ± 5.99 months. The main data evaluation and outcome measurements included the patient's vision, intraocular pressure(IOP), optical coherence tomography (OCT), slit lamp examination, indirect ophthalmoscopy, color Doppler imaging (CDI) and color fundus image analysis. The following variables were compared between groups: abnormal vascular changes, subretinal fluid and exudate absorption, retinal reattachment and complications. The final follow-up results were used to determine the effectiveness of treatment. RESULTS: Of the 17 patients included, 88.24% were male and 11.76% were female. Visual acuity was less than 0.02 in 12 eyes before surgery and 8 eyes after surgery. Visual acuity improved in 7 eyes, accounting for 41.18% of cases, and remained unchanged in 7 eyes, accounting for 41.18% of cases. Three patients were too young to undergo the operation, accounting for 17.65% of cases. The best vision was 0.1. Patients were treated 1 to 5 times for an average of 2.82 ± 0.95 times each. There was no statistically significant difference (t = 1.580, p = 0.135) between the preoperative and postoperative intraocular pressures. However, there was a statistically significant difference between the preoperative and postoperative retinal detachment height (2- related samples Wilcoxon signed rank test with z = 3.517, p = 0.000). The results further showed that all patients had different degrees of subretinal fluid absorption, and some of the new blood vessels subsided. All patients were successfully treated with laser and cryosurgery. No ocular or systemic complications were observed during follow-up. CONCLUSIONS: Intravitreal ranibizumab (IVR), laser coagulation and cryotherapy were effective in the treatment of Coats' disease with exudative retinal detachment. TRIAL REGISTRATION NUMBER: We retrospectively registered our study, The trial registration number (TRN) is ChiCTR-ONC-17011161 and date of registration is April 16, 2017.

Ultrasound biomicroscopic imaging in paediatric ocular toxocariasis.

PURPOSE: To determine the potential and usefulness of ultrasound biomicroscopy (UBM) as a diagnostic procedure on a larger cohort of paediatric patients with ocular toxocariasis. METHODS: UBM was performed on 41 eyes of 41 patients with ocular toxocariasis in order to determine configuration of peripheral retina, pars plana and vitreous. Location and range of peripheral pathology using ophthalmoscopy with scleral indentation, UBM and intraoperative confirmation were recorded and compared. RESULTS: Pathological structures such as vitreous condensations or membranes of various configuration and extent were identified by UBM. UBM revealed peripheral granuloma and pseudocystic changes in the peripheral vitreous in 15 of 41 eyes. Compared with indirect ophthalmoscope, statistical analysis showed significant differences in the number of clock-hours of peripheral pathology detected by UBM (p=0.015), while statistical differences were not found between UBM and intraoperative peripheral examinations using scleral indentation (p=0.432). CONCLUSIONS: UBM seems to be a valuable and reliable diagnostic technique for the evaluation of patients with ocular toxocariasis. Longitudinal studies will have to determine the relevance of UBM findings for the individual clinical course and their influence on therapeutic decisions.