Sickle cell disease and the eye.

PURPOSE OF REVIEW: To review recent literature pertaining to sickle cell retinopathy (SCR) and, in particular, sickle cell maculopathy. RECENT FINDINGS: Several recent studies suggest that macular perfusion abnormalities seen in patients with sickle cell disease of various genotypes may affect both the superficial and deep capillary plexi, with a predilection for the deep capillary plexus. Further, these changes may be associated with areas of macular thinning, as well as with peripheral retinal ischemia, even in individuals without visual symptoms, contrary to what has previously been described in both diabetic retinopathy and retinal vein occlusion. Several cases also suggest that paracentral acute middle maculopathy may be the pathophysiologic mechanism by which microvascular occlusion leads to macular thinning. SUMMARY: Sickle cell disease can manifest in a number of ways within the orbit as well as intraocularly because of its nonspecific vasoocclusive episodes. However, SCR is the most common ophthalmic manifestation of this disease. Historically, SCR has been considered a peripheral retinopathy, but the development and use of spectral-domain optical coherence tomography and optical coherence tomography angiography suggest that significant macular vascular changes occur early in this disease, even in asymptomatic individuals.

Placement of ProKera in the management of ocular manifestations of acute Stevens-Johnson syndrome in an outpatient.

PURPOSE: To report the clinical use of ProKera (Bio-Tissue, Inc., Miami, FL) under topical anesthesia in an outpatient for the management of ocular manifestations of acute Stevens-Johnson syndrome (SJS). METHODS: Interventional case report. RESULTS: A 19-year-old woman developed acute SJS with ocular involvement after ingestion of oral antibiotics. Slit-lamp examination performed 2 weeks later showed severe inflammation and areas of ulceration along all 4 lids and complete, bilateral sloughing of bulbar and palpebral conjunctivae, including the limbus. ProKera was placed under topical anesthesia without sedation in both eyes instead of an amniotic membrane due to excess risk of general anesthesia. Three weeks after placement, slit-lamp examination showed complete re-epithelialization of both corneas and conjunctivae, with only trace conjunctival injection and minor limbal epithelial irregularities. Three months postprocedure, there were no signs of clinically significant scarring, and the visual acuity (VA) was 20/20 bilaterally. Fourteen months postprocedure, VA remained stable, and the patient did not have dry eye, photophobia, clinically significant scarring, or symblepharon. CONCLUSIONS: ProKera placement performed under topical anesthesia may be appropriate for the treatment of ocular surface manifestations of acute SJS particularly in those patients followed in an outpatient setting with milder forms of disease and/or with contraindications to general anesthesia.

Posterior placoid-like maculopathy and macular hole associated with vitamin A deficiency.

Purpose: To report a case of bilateral posterior placoid-like maculopathy and a macular hole associated with vitamin A deficiency. Observations: A 72-year-old male presented with nyctalopia and progressive vision loss in both eyes. Examination and multimodal imaging were consistent with posterior placoid-like maculopathy bilaterally and a macular hole in the right eye. A workup for infectious, inflammatory, and paraneoplastic etiologies revealed a severely low serum vitamin A level. Two months after initiation of vitamin A repletion, there was improvement in best-corrected Snellen visual acuity as well as macular hole closure. A diagnosis of posterior placoid-like maculopathy in the setting of vitamin A deficiency (VAD) was made. Conclusions and importance: VAD should be considered when symmetric posterior pole placoid-like lesions are observed and other, more common etiologies have been ruled out.

A case of ustekinumab-induced sclerouveitis.

PURPOSE: Biologics have proven to be essential therapeutic agents in immune-mediated systemic diseases. Ophthalmologic adverse effects have been reported in the use of more traditional agents, such as TNF-α inhibitors, but there are limited data on ocular side effects associated with the newer generation of biologics. OBSERVATIONS: In this report, we present a case of a suspected ustekinumab-associated recurrent sclerouveitis. CONCLUSIONS: To the best of our knowledge, this is the first reported case of this type seen with ustekinumab injections. Our goal is to add to the existing literature in order to better understand the mechanism and management of this condition.

Describing Adverse Events Associated with Bilateral Same-Day Intravitreal Dexamethasone Implants.

BACKGROUND AND OBJECTIVES: To explore the incidence of adverse events after bilateral same-day intravitreal 0.7-mg dexamethasone implant (SDIDI) injections. MATERIALS AND METHODS: We performed an IRB approved, single-center, retrospective review of patients receiving bilateral SDIDI injections from January 1, 2016 to October 31, 2021 and reviewed adverse events that occurred within 3 months of injection. RESULTS: A total of 206 bilateral (412 eyes) SDIDI injections were performed in 59 patients. Ocular hypertension or the addition of intraocular pressure (IOP) lowering drops occurred in 121 (29.4%) eyes after IDI. Two (0.5%) eyes required glaucoma drainage surgeries. Of the 117 phakic eyes, 32 (27.4%) had progression of cataract or cataract extraction. There were two (0.5%) episodes of vitreous hemorrhage and one (0.2%) retinal tear with retinal detachment. There were no cases of endophthalmitis. CONCLUSION: Serious complication rates after bilateral same-day IDI injections appears low. Increased IOP that requires intervention can occur. [Ophthalmic Surg Lasers Imaging Retina 2022;53:612-618.].

Impact of Coronavirus Disease 2019 Restrictions on Retinal Detachment: A Multicenter Experience.

In this retrospective, multicenter study of 261 eyes (259 patients), patients who underwent rhegmatogenous retinal detachment repair during the coronavirus disease 2019 (COVID-19) post-lockdown period experienced an additional 22-day delay, leading to significantly more epiretinal membrane and proliferative vitreoretinopathy and lower single-surgery anatomic success rates. During lockdown, perfluoropropane gas was used more commonly, and pneumatic retinopexy was used more commonly in COVID-19-positive patients.

Relationship of Epiretinal Membrane Formation and Macular Edema Development in a Large Cohort of Uveitic Eyes.

Purpose: To identify the temporal relationship and clinical characteristics of epiretinal membrane (ERM) and macular edema (ME) formation in uveitic eyes.Methods: A total of 269 subjects (444 uveitic eyes) met study inclusion criteria. Comprehensive ophthalmic examination, spectral domain-optical coherence tomography (SD-OCT), and clinical testing were carried out.Results: Of the 444 uveitic eyes, 229 eyes (51.6%) developed an ERM, whereas 87 eyes (19.1%) developed ME. The odds ratios (ORs) of systemic disease causing uveitis and resulting in ERM and ME were significantly higher in posterior uveitis (OR 6.56, 95% CI 2.98-14.46; p < .0001) and panuveitis (OR 10.09, 95% CI 4.05-25.15; p < .0001). Temporal analysis revealed that an ERM was noted concurrently or prior to ME development in 93.8% of eyes.Conclusions: ERM and ME are primarily observed in posterior uveitis and panuveitis associated with systemic diseases. The temporal relationship highlights the importance of characterization of ERM as it relates to the development of uveitic ME.

Cytomegalovirus retinitis after treatment with topical difluprednate in an aphakic eye of an immunocompetent patient.

PURPOSE: To report a case of an immunocompetent 64-year-old man who developed cytomegalovirus (CMV) retinitis after using topical difluprednate. OBSERVATIONS: A 64-year-old man with type 2 diabetes developed hemorrhagic retinitis while using topical difluprednate after penetrating keratoplasty. Polymerase chain reaction of the vitreous was positive for CMV DNA. Complete blood count was within normal limits and 4th generation human immunodeficiency virus assay was negative. The retinitis resolved with oral valgancyclovir and intravitreal foscarnet injections. CONCLUSION AND IMPORTANCE: CMV retinitis may occur after topical difluprednate in an immunocompetent patient.

Cyclo-oxygenase inhibitors in the treatment of chronic non-infectious, non-necrotizing scleritis and episcleritis.

PURPOSE: To evaluate the use of selective vs. non-selective cyclo-oxygenase inhibitors (COXIs) for the treatment of chronic non-infectious, non-necrotizing scleritis and episcleritis. METHODS: Sixty-nine patients with scleritis and episcleritis treated for ≥2 months with COXIs were included. Outcome measures were rates of inflammation control, corticosteroid sparing, and COXI discontinuation, as well as side effects. RESULTS: Initial inflammation control was achieved in 78-81% of scleritis patients and 73-80% episcleritis patients on COXIs (p > .05). Rates of steroid sparing after 4-24 months of consecutive treatment were similar. Gastrointestinal side effects were observed in 1 (2.7%) patient on selective vs. 3 (9.4%) patients on non-selective COXIs (p = .33). Overall discontinuation rate was 6/37 (16%) for selective vs. 7/32 (22%) for non-selective COXIs (p = .76). CONCLUSIONS: Selective vs. non-selective COXIs were equally efficacious for the treatment of chronic non-infectious, non-necrotizing scleritis and episcleritis. Prospective studies to elucidate potential differences in side effect profiles may be warranted.