Conserved regression patterns of retinopathy of prematurity after intravitreal ranibizumab: A class effect.

PURPOSE: To determine if fluorescein angiographic (FA) findings after intravitreal ranibizumab (IVR) for retinopathy of prematurity (ROP) conform to a class effect previously described with bevacizumab. METHODS: Single-center retrospective case series of all infants treated with 0.2 mg (0.02 mL) IVR for Type 1 ROP from July 2016 to November 2018. FA were obtained at 40, 52, 62, and 72 weeks of postmenstrual age (PMA) using wide-angle photography. FA images were analyzed and the peripheral avascular areas measured with ImageJ using a reference disc diameter (DD). Based on the extent of the avascular area and tortuosity of the retinal vessels all eyes were classified into four categories: complete vascular maturity (vascularization within 2 DD of the ora serrata), VAA (avascular area >2 DD of the ora serrata), VAT (avascular area >2 DD of the ora serrata and posterior tortuosity), and reactivation (recurrence of stage disease). RESULTS: About 13 infants were enrolled and 24 eyes were available in this study. None of the eyes reached complete vascular maturity at an average PMA of 60 weeks, 7 (29%) eyes presented with VAA, 8 (33%) with VAT, and 9 (37.5%) reactivated. The reactivated eyes presented with the largest area of peripheral ischemia, followed by the VAT and then the VAA groups (p = 0.02). CONCLUSION: IVR conforms to the previously described regression patterns following intravitreal bevacizumab for ROP indicative of a class effect. Follow-up using FA might help to optimize the management of these infants after injection of the drug.

Risdiplam treatment has not led to retinal toxicity in patients with spinal muscular atrophy.

OBJECTIVE: Evaluation of ophthalmologic safety with focus on retinal safety in patients with spinal muscular atrophy (SMA) treated with risdiplam (EVRYSDI®), a survival of motor neuron 2 splicing modifier associated with retinal toxicity in monkeys. Risdiplam was approved recently for the treatment of patients with SMA, aged ≥ 2 months in the United States, and is currently under Health Authority review in the EU. METHODS: Subjects included patients with SMA aged 2 months-60 years enrolled in the FIREFISH, SUNFISH, and JEWELFISH clinical trials for risdiplam. Ophthalmologic assessments, including functional assessments (age-appropriate visual acuity and visual field) and imaging (spectral domain optical coherence tomography [SD-OCT], fundus photography, and fundus autofluorescence [FAF]), were conducted at baseline and every 2-6 months depending on study and assessment. SD-OCT, FAF, fundus photography, and threshold perimetry were evaluated by an independent, masked reading center. Adverse events (AEs) were reported throughout the study. RESULTS: A total of 245 patients receiving risdiplam were assessed. Comprehensive, high-quality, ophthalmologic monitoring assessing retinal structure and visual function showed no retinal structural or functional changes. In the youngest patients, SD-OCT findings of normal retinal maturation were observed. AEs involving eye disorders were not suggestive of risdiplam-induced toxicity and resolved with ongoing treatment. INTERPRETATION: Extensive ophthalmologic monitoring conducted in studies in patients with SMA confirmed that risdiplam does not induce ophthalmologic toxicity in pediatric or adult patients with SMA at the therapeutic dose. These results suggest that safety ophthalmologic monitoring is not needed in patients receiving risdiplam, as also reflected in the United States Prescribing Information for risdiplam.

Familial Exudative Vitreoretinopathy With Neurodevelopmental Delay and Hypoplasia of the Corpus Callosum.

A 2-year-old child was referred to the authors' pediatric retina service for bilateral retinal folds, strabismus, and psychomotor retardation, as well as marked thinning of the corpus callosum. Family history was unremarkable and genetic testing revealed a previously undescribed mutation in the LRP5 gene. Widefield fundus photography, fluorescein angiography, and spectral-domain optical coherence tomography were used to image the retinal fundus. The authors' case suggests a correlation between LRP5 and neurological development, since its variants may lead to a syndromic condition characterized by FEVR-like abnormalities along with neurodevelopmental delay and hypoplasia of the corpus callosum. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:588-591.].

Functional and Morphologic Findings at Four Years After Intravitreal Bevacizumab or Laser for Type 1 ROP.

BACKGROUND AND OBJECTIVE: To compare morphologic and functional status at age 4 years for patients treated in one eye with laser photocoagulation and the other eye with intravitreal bevacizumab (IVB) injection for Type 1 retinopathy of prematurity (ROP). PATIENTS AND METHODS: In this single-center, randomized, controlled trial, best-corrected visual acuity (BCVA) in logMAR was obtained along with spherical equivalent refraction (SER), fluorescein angiography (FA), optical coherent tomography (OCT), and OCT angiography (OCTA). RESULTS: Eighteen babies (36 eyes) were selected for this study. BCVA and SER were similar in the two groups, but six patients had anisometropia of 4 diopters or more. IVB-treated eyes tended to have thinner foveal thickness than laser-treated eyes (mean difference: -5.33 pixels; 95% confidence interval, -9.62 to -1.05). CONCLUSION: Although the differences found here are minimal between the IVB-treated and laser-treated groups, further long-term evaluation of not only FA, but also OCT and OCTA, are needed in larger studies. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:180-186.].