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© 2023 Retina. All right reserved.Purpose:To report outcomes of pediatric patients with combined hamartoma of the retina and the retina pigment epithelium followed up conservatively or after pars plana vitrectomy.Methods:This retrospective multicenter study included 62 eyes of 59 pediatric patients with combined hamartoma of the retina and the retina pigment epithelium from 13 different international centers with an average age of 7.7 ± 4.7 (0.3-17) years at the time of the diagnosis and having undergone pars plana vitrectomy or followed conservatively. At baseline and each visit, visual acuity values, optical coherence tomography for features and central foveal thickness, and tumor location were noted. Lesions were called as Zone 1, if it involves the macular and peripapillary areas, and the others were called as Zone 2 lesions.Results:Twenty-one eyes of 20 patients in the intervention group and 41 eyes of 39 patients in the conservative group were followed for a mean of 36.2 ± 40.4 (6-182) months. Best-corrected visual acuity improved in 11 (68.8%) of 16 eyes in the intervention group and 4 (12.9%) of 31 eyes in the conservative group (P < 0.001). The mean central foveal thickness decreased from 602.0 ± 164.9 µm to 451.2 ± 184.3 µm in the intervention group, while it increased from 709.5 ± 344.2 µm to 791.0 ± 452.1 µm in Zone 1 eyes of the conservative group. Posterior location of tumor, irregular configuration of the foveal contour and ellipsoid Zone defect in optical coherence tomography, subretinal exudate and prominent vascular tortuosity were associated with poor visual acuity.Conclusion:Vitreoretinal surgery is safe and effective in improving vision and reducing retinal distortion in Zone 1 combined hamartoma of the retina and the retina pigment epithelium in children.

Topic An international, expert-led consensus initiative organized by the Collaborative Ocular Tuberculosis Study (COTS), along with the International Ocular Inflammation Society and the International Uveitis Study Group, systematically developed evidence- and experience-based recommendations for the treatment of tubercular choroiditis. Clinical relevance The diagnosis and management of tubercular uveitis (TBU) pose a significant challenge. Current guidelines and literature are insufficient to guide physicians regarding the initiation of antitubercular therapy (ATT) in patients with TBU. Methods An international expert steering subcommittee of the COTS group identified clinical questions and conducted a systematic review of the published literature on the use of ATT for tubercular choroiditis. Using an interactive online questionnaire, guided by background knowledge from published literature, 81 global experts (including ophthalmologists, pulmonologists, and infectious disease physicians) generated preliminary consensus statements for initiating ATT in tubercular choroiditis, using Oxford levels of medical evidence. In total, 162 statements were identified regarding when to initiate ATT in patients with tubercular serpiginous-like choroiditis, tuberculoma, and tubercular focal or multifocal choroiditis. The COTS group members met in November 2018 to refine these statements by a 2-step modified Delphi process. Results Seventy consensus statements addressed the initiation of ATT in the 3 subtypes of tubercular choroiditis, and in addition, 10 consensus statements were developed regarding the use of adjunctive therapy in tubercular choroiditis. Experts agreed on initiating ATT in tubercular choroiditis in the presence of positive results for any 1 of the positive immunologic tests along with radiologic features suggestive of tuberculosis. For tubercular serpiginous-like choroiditis and tuberculoma, positive results from even 1 positive immunologic test were considered sufficient to recommend ATT, even if there were no radiologic features suggestive of tuberculosis. Discussion Consensus guidelines were developed to guide the initiation of ATT in patients with tubercular choroiditis, based on the published literature, expert opinion, and practical experience, to bridge the gap between clinical need and available medical evidence.

Topic The Collaborative Ocular Tuberculosis Study (COTS), supported by the International Ocular Inflammation Society, International Uveitis Study Group, and Foster Ocular Immunological Society, set up an international, expert-led consensus project to develop evidence- and experience-based guidelines for the management of tubercular uveitis (TBU). Clinical Relevance The absence of international agreement on the use of antitubercular therapy (ATT) in patients with TBU contributes to a significant heterogeneity in the approach to the management of this condition. Methods Consensus statements for the initiation of ATT in TBU were generated using a 2-step modified Delphi technique. In Delphi step 1, a smart web-based survey based on background evidence from published literature was prepared to collect the opinion of 81 international experts on the use of ATT in different clinical scenarios. The survey included 324 questions related to tubercular anterior uveitis (TAU), tubercular intermediate uveitis (TIU), tubercular panuveitis (TPU), and tubercular retinal vasculitis (TRV) administered by the experts, after which the COTS group met in November 2019 for a systematic and critical discussion of the statements in accordance with the second round of the modified Delphi process. Results Forty-four consensus statements on the initiation of ATT in TAU, TIU, TPU, and TRV were obtained, based on ocular phenotypes suggestive of TBU and corroborative evidence of tuberculosis, provided by several combinations of immunologic and radiologic test results. Experts agreed on initiating ATT in recurrent TAU, TIU, TPU, and active TRV depending on the TB endemicity. In the presence of positive results for any 1 of the immunologic tests along with radiologic features suggestive of past evidence of tuberculosis infection. In patients with a first episode of TAU, consensus to initiate ATT was reached only if both immunologic and radiologic test results were positive. Discussion The COTS consensus guidelines were generated based on the evidence from published literature, specialists’ opinions, and logic construction to address the initiation of ATT in TBU. The guidelines also should inform public policy by adding specific types of TBU to the list of conditions that should be treated as tuberculosis.

Purpose To study the practice patterns for the management of acute postoperative and postinjection endophthalmitis. Design Retrospective, interventional, nonrandomized, multicenter study. Participants Data on 237 eyes diagnosed with acute endophthalmitis occurring after intraocular surgery or procedures provided by 57 retina specialists from 28 countries. Main Outcome Measures Rates of pars plana vitrectomy (PPV), repeat intravitreal injection, and adjunctive therapeutic regimens (local and systemic antibiotics and steroids). Results Of 237 analyzed eyes, acute endophthalmitis secondary to cataract surgery or secondary lens implantation represented 64.6% of cases (153 eyes), whereas the remaining were secondary to intravitreal injections (35 eyes [14.8%]), PPV (29 eyes [12.2%]), and other intraocular surgeries (20 eyes [8.4%]). All eyes received intravitreal antibiotics on the same day of diagnosis. Overall, early PPV was used within the first week of presentation in 176 eyes (74.3%). There was no statistical difference in the proportion of eyes requiring a second intravitreal injection of antibiotics whether the eye was managed primarily with intravitreal antibiotics alone versus early PPV plus intravitreal antibiotics (29.5% [18 eyes] vs. 25.0% [44 eyes], respectively). Adjunctive therapies in the form of intravitreal steroids, systemic steroids, and systemic antibiotics were used in 25.3%, 21.9%, and 66.6% of eyes, respectively. The absence of disc or macular view and absence of endophthalmitis after cataract surgery were associated with an increased likelihood for early PPV (odds ratios 4.1 and 5.1, respectively). Conclusions Pars plana vitrectomy was frequently performed regardless of the presenting vision in eyes with endophthalmitis after cataract surgery and intravitreal injections. Increased vitreous opacification was associated with a higher probability for performing PPV.

This study sought to evaluate the agreement between smartphone-based device assisted remote screening and gold standard indirect ophthalmoscopic examination in cases of referable Retinopathy of Prematurity (ROP).

In recent decades, the treatment paradigm for noninfectious intermediate uveitis, posterior uveitis, and panuveitis, a group of intraocular inflammatory diseases, has included systemic and local (periocular or intraocular) corticosteroids, biologics, and other steroid-sparing immunomodulatory therapy agents. Recently, an intravitreal formulation of sirolimus, an immunosuppressant that inhibits the mammalian target of rapamycin, a key regulator of cell growth in the immune system, was developed. On the basis of this mechanism and the local method of delivery, it was hypothesized that intravitreal sirolimus can improve ocular inflammation in patients with noninfectious intermediate uveitis, posterior uveitis, and panuveitis, with minimal systemic exposure and systemic adverse events (AEs). This review summarizes the pharmacokinetics, efficacy, and safety results of intravitreal sirolimus from 3 preclinical studies and 4 phase 1-3 clinical studies. Preclinical studies in rabbits showed that 22 to 220 μg intravitreal sirolimus results in sustained release of sirolimus in the vitreous for 2 months or more, with systemic concentrations below the threshold for systemic immunosuppression (approximately 8 ng/ml). Subsequently, 2 phase 1 studies (n = 50 and n = 30) established that intravitreal sirolimus improves ocular inflammation in humans. Further investigation in phase 2 and 3 studies (n = 24 and n = 347, respectively) suggested that 440 μg has the best benefit-to-risk profile. In the phase 3 study, the proportion of patients who showed complete resolution of ocular inflammation at month 5 was significantly higher in the 440-μg group than in the 44-μg group (22.8% vs. 10.3%; P = 0.025, Fisher exact test). In addition, 47 of 69 patients (68.1%) who were treated with systemic corticosteroids at baseline discontinued corticosteroid use at month 5. No sirolimus-related systemic AEs were reported in phase 1-3 studies. Collectively, these preclinical and clinical study data of intravitreal sirolimus support the therapeutic rationale of treating noninfectious uveitis with a local mammalian target of rapamycin inhibitor and suggest that 440 μg intravitreal sirolimus has the potential to be an effective and well-tolerated anti-inflammatory and corticosteroid-sparing treatment for noninfectious intermediate uveitis, posterior uveitis, and panuveitis.

Ocular tuberculosis has been recognized from the nineteenth century onward due to the work of Gueneau de Mussy, Jaeger, Fraenkel, and Bouchut. Ocular tuberculosis had a high prevalence worldwide in the twentieth century, but this prevalence has reduced significantly in the western hemisphere, a decline that has paralleled the decline of tuberculosis as a common systemic infection. In recent times, the prevalence varies from 18% (Spain) to 1.39% (India). The advent of the human immunodeficiency virus (HIV) epidemic has led to an increase in the prevalence of ocular tuberculosis to up to 23.5%. Manifestations vary depending upon the ocular tissue involved. The manifestations can either be due to direct tissue infection or be hypersensitivity reactions. Rare manifestations include the eyelids (lupus vulgaris), the conjunctiva (conjunctivitis), cornea (ulcers and phlyctenulosis), and the sclera (scleritis). The most common manifestations include uveal involvement in the form of tubercles, tuberculomas, and serpiginous-like choroiditis. Other manifestations include orbital apex syndrome and lesions associated with neurotuberculosis (disc edema and sixth nerve palsies). The diagnostic workup of patients with suspected ocular tuberculosis has both systemic and ocular components. The systemic investigations include (1) radiography: (a) chest X-rays and computed tomography of the chest and (b) abdominal CT scan or ultrasonography; (2) Mantoux testing; and (3) interferon-γ(gamma) release assays (IGRA). Ocular investigations of ocular fluids or tissue biopsies include (1) microscopy, (2) culture, and (3) PCR (polymerase chain reaction) techniques. The treatment includes the use of topical, periocular, or systemic corticosteroids with a four-drug regimen of antitubercular therapy.