While most eyes that underwent LSV for Stage 4A or 4B ROP maintain useful vision with long-term follow-up, approximately one-fifth of eyes had no functional vision, and in a further fifth, vision could not be measured due to severe neurological impairment.

ABSTRACT.Purpose: To evaluate the effect of the vitreomacular interface (VMI) on treatment efficacy of intravitreal therapy in uveitic cystoid macular oedema (CME). Methods: Retrospective analysis of CME resolution, CME recurrence rate and monthly course of central retinal thickness (CRT), retinal volume (RV) and best corrected visual acuity (BCVA) after intravitreal injection with respect to the VMI configuration on spectral-domain OCT using chi-squared test and repeated measures ANOVA adjusted for confounding covariates epiretinal membrane, administered drug and subretinal fluid. Results: Fifty-nine eyes of 53 patients (mean age: 47.4 AE 16.9 years) were included. VMI status had no effect on complete CME resolution rate (p = 0.16, corrected p-value: 0.32), time until resolution (p = 0.09, corrected p-value: 0.27) or CME relapse rate (p = 0.29, corrected p-value: 0.29). Change over time did not differ among the VMI configuration groups for BVCA (p = 0.82) and RV (p = 0.18), but CRT decrease was greater and faster in the posterior vitreous detachment (PVD) group compared to the posterior vitreous attachment (PVA) and vitreous macular adhesion (VMA) groups (p = 0.04). Also, the percentage of patients experiencing a ≥ 20% CRT thickness decrease after intravitreal injection was greater in the PVD group (83%) compared to the VMA (64%) and the PVA (16%) group (p = 0.027), however, not after correction for multiple testing (corrected p-value: 0.11). Conclusion: The VMI configuration seems to be a factor contributing to treatment efficacy in uveitic CME in terms of CRT decrease, although BCVA outcome did not differ according to VMI status.

Subjects, Participants, and/or Controls: One hundred and three patients with an identified germline, pathogenic DICER1 variant (DICER1-carriers) and 69 family control subjects underwent clinical and ophthalmic examination at the National Institutes of Health between 2011 and 2016. Methods: All participants were evaluated with a comprehensive ophthalmic exam including best corrected visual acuity, slit-lamp biomicroscopy and a dilated fundus examination. A subset of patients returned for a more detailed evaluation including spectral-domain optical coherence tomography, color fundus photography, fundus autofluorescence imaging, visual field testing, full field electroretinogram and genetic testing for inherited retinal degenerative diseases. Main Outcome Measures: Visual acuity and examination findings Results: Most DICERl-carriers (97%) maintained a visual acuity of 20/40 or better in both eyes. Twenty three DICERl-carriers (22%) had ocular abnormalities compared with four (6%) family controls (P=0.005). These abnormalities included retinal pigment abnormalities (N=6, 5.8%), increased cup-to-disc ratio (N=5, 4.9%), optic nerve abnormalities (N=2, 1.9%), epiretinal membrane (N=2, 1.9%) and drusen (N=2, 1.9%). Overall, we observed a significant difference (p= 0.03) in the rate of retinal abnormalities in DICERl-carriers (N=11, 11%) vs. controls (N=1;1.5%). One patient had an unexpected diagnosis of retinitis pigmentosa with a novel variant of unknown significance in PRPF31, and one had optic nerve elevation in the setting of increased intracranial pressure of unclear etiology. Three patients (3%) had DICERl-related ciliary body medulloepithelioma (CBME), two of which were identified during routine examination, a significantly higher rate than that previously reported. Conclusions: Ophthalmologists should be aware of the ophthalmic manifestations of the DICER1 syndrome and individuals and families should be counseled on the potential signs and symptoms. We recommend that children with a germline pathogenic variant in DICER1, especially those under the age of 10 years, undergo annual dilated ophthalmic examination, looking for evidence of CBME, signs of increased intracranial pressure and perhaps changes in the retinal pigment epithelium. Précis Ciliary body medulloepitheliomas have previously been described in patients with DICER1 syndrome. In addition to these findings, DICER1-carriers should be monitored for changes in the optic nerve and retina.

PurposeTo report the first case of infectious surgically-induced necrotizing scleritis following strabismus surgery which was treated successfully with a tectonic corneal graft.ObservationsWe report a case of surgically-induced necrotizing scleritis after strabismus surgery in a 61-year-old gentleman with gout and a subconjunctival abscess. Surgical drainage of the subconjunctival abscess led to a diagnosis of scleral melt which was subsequently treated with a tectonic corneal graft along with aggressive medical management. Over the following eight months, the patient showed no signs of endophthalmitis, graft necrosis, nor graft dehiscence, and serial anterior segment optical coherence tomography imaging demonstrated anatomic stability.Conclusions and importanceThis case offers further insights into a rare but vision-threatening and potentially life-threatening diagnosis. In conjunction with aggressive local and systemic treatment, tectonic lamellar keratoplasty provides good therapeutic and tectonic results for scleral necrosis after strabismus surgery. This case also demonstrates the importance of screening for associated systemic risk factors in any patient with scleritis for appropriate, targeted therapy.

Purpose: To report a case of juvenile xanthogranuloma that simulated a chalazion and to discuss the association between juvenile xanthogranuloma and Langerhans cell histiocytosis. Method: Case report and review of literature. Results: A 13-year-old boy with a prior history of Langerhans cell histiocytosis was referred to our clinic for a possible chalazion. The patient had undergone treatment for Langerhans cell histiocytosis 10 years prior. The patient underwent an excisional biopsy. Histopathology revealed a proliferation of histiocytes and lymphocytes with Touton giant cells, consistent with a diagnosis of juvenile xanthogranuloma. Conclusions: Though the relationship between Langerhans cell histiocytosis and juvenile xanthogranuloma has yet to be fully elucidated, juvenile xanthogranuloma should be included in the differential diagnosis for any former Langerhans cell histiocytosis patient presenting with a new cutaneous lesion.