It is important for clinicians to consider exposure to toxic substances and nutritional deficiencies when diagnosing and managing cases of vision loss. In these cases, physiologic damage can alter the function of key components of the visual pathway before morphologic changes can be detected by traditional imaging methods. Electrophysiologic tests can aid in the early detection of such functional changes to visual pathway components, including the retina or optic nerve. This review provides an overview of various electrophysiologic techniques, including multifocal electroretinogram (mfERG), full-field ERG (ffERG), electrooculogram (EOG), pattern electroretinogram (PERG), and visual evoked potential (VEP) in monitoring the retinal and optic nerve toxicities of alcohol, amiodarone, cefuroxime, cisplatin, deferoxamine, digoxin, ethambutol, hydroxychloroquine, isotretinoin, ocular siderosis, pentosane, PDE5 inhibitors, phenothiazines (chlorpromazine and thioridazine), quinine, tamoxifen, topiramate, vigabatrin, and vitamin A deficiency.

Macular dystrophies are a heterogeneous group of genetic disorders that often severely threatens the bilateral central vision of the affected patient. While advances in molecular genetics have been instrumental in the understanding and diagnosis of these disorders, there remains significant phenotypical variation among patients within any particular subset of macular dystrophies. Electrophysiological testing remains a vital tool not only to characterize vision loss for differential diagnosis but also to understand the pathophysiology of these disorders and to monitor the treatment effect, potentially leading to therapeutic advances. This review summarizes the application of electrophysiological testing in macular dystrophies, including Stargardt disease, bestrophinopathies, X-linked retinoschisis, Sorsby fundus dystrophy, Doyne honeycomb retina dystrophy, autosomal dominant drusen, occult macular dystrophy, North Carolina macular dystrophy, pattern dystrophy, and central areolar choroidal dystrophy.

Purpose: To examine an underreported and underdiagnosed phenomenon with implications for the pathophysiological mechanisms of tattoo-induced uveitis. Methods: Two cases highlighting the clinical presentation of tattoo-related uveitis were evaluated. Results: A 28-year-old man with biopsy-proven sarcoidosis and ocular manifestations presented with worsening retinal vasculitis after acquiring a red-ink tattoo. Each subsequent flare followed acquisition of a new tattoo. A 31-year-old woman without systemic sarcoidosis presented with multiple episodes of bilateral intermediate uveitis and macular edema concurrent with inflammatory granulomas to recently acquired black-ink tattoos. A skin biopsy in both patients showed cutaneous noncaseating granulomas. Conclusions: These cases add to those reported in the literature and emphasize the importance of understanding the modifiable factors of inflammatory ocular disease. Future study is necessary to understand the mechanisms of tattoo-related uveitis.

Inherited retinal dystrophies encompass a diverse group of disorders affecting the structure and function of the retina, leading to progressive visual impairment and, in severe cases, blindness. Electrophysiology testing has emerged as a valuable tool in assessing and diagnosing those conditions, offering insights into the function of different parts of the visual pathway from retina to visual cortex and aiding in disease classification. This review provides an overview of the application of electrophysiology testing in the non-macular inherited retinal dystrophies focusing on both common and rare variants, including retinitis pigmentosa, progressive cone and cone-rod dystrophy, bradyopsia, Bietti crystalline dystrophy, late-onset retinal degeneration, and fundus albipunctatus. The different applications and limitations of electrophysiology techniques, including multifocal electroretinogram (mfERG), full-field ERG (ffERG), electrooculogram (EOG), pattern electroretinogram (PERG), and visual evoked potential (VEP), in the diagnosis and management of these distinctive phenotypes are discussed. The potential for electrophysiology testing to allow for further understanding of these diseases and the possibility of using these tests for early detection, prognosis prediction, and therapeutic monitoring in the future is reviewed.

Autologous retinal transplant (ART) has become an increasingly explored surgical option for managing large chronic holes refractory to standard surgical treatments. However, management strategies for patients who already failed a previous ART are less well-understood. Here, we report on a case of a successful repeat retinal transplant for a refractory macular hole after a previously dislocated ART graft. Subretinal injection of balanced salt solution was used to partially elevate the macular hole and secure the edge of the harvested retinal graft under the edge of the macular hole in the second operation. Postoperatively, the patient developed intraretinal fluid within the retinal graft with an appearance similar to cystoid macular edema, which was controlled with topical steroids. In addition, two separate choroidal neovascular membranes along the subretinal injection sites were seen and treated with vascular endothelial growth factor downregulation. This case illustrates successful repeat ART surgery, but further optimization of ART surgical techniques is necessary to minimize ART’s complication rate.

Purpose: To present a series of 4 patients from the Ohio Amish or Mennonite populations with isolated ectopia lentis. Methods: A case series was evaluated. Results: Four cases with bilateral lens subluxations were diagnosed with a homozygous c.767_786del pathogenic variant in ADAMTSL4. Their ages ranged from 2 to 22 years. Three cases were symptomatic and were managed surgically with lensectomy, vitrectomy, and endolaser photocoagulation with or without secondary intraocular lens (IOL) implantation. One asymptomatic patient was observed. The postoperative visual acuity ranged from 20/20 to 20/60 in nonamblyopic eyes. Conclusions: The pathogenic homozygous c.767_786del variant in ADAMTSL4 may be a cause of bilateral isolated ectopia lentis in the Ohio Amish and Mennonite populations, likely as a result of a founder effect. Vitrectomy and lens extraction with or without secondary IOL implantation may lead to good visual outcomes. There were no cases of retinal detachment.