Bilateral sequential ischemic retinopathy and optic neuropathy in IgG1 heavy chain deposition disease.

PURPOSE: To report a case of progressive ischemic retinopathy and optic neuropathy in a patient with heavy chain deposition disease (HCDD), a rare form of monoclonal immunoglobulin deposition disease (MIDD). OBSERVATIONS: Our case describes a 74-year-old woman diagnosed with IgG1 lambda HCDD. After treatment with daratumumab and intravenous IVIG therapy, the patient developed worsening ischemic retinopathy and optic neuropathy, neovascular glaucoma, and bilateral sequential vitreous hemorrhages, necessitating surgical intervention. We present multimodal imaging from the onset of ischemic retinopathy to end-stage maculopathy illustrated by optical coherence tomography (OCT) angiography. Despite discontinuing treatment with daratumumab and providing maximal ocular interventions to control the complications of neovascular disease, the patient's condition progressed, resulting in profound vision loss. CONCLUSIONS AND IMPORTANCE: Our case illustrates the potential for HCDD to cause end-organ disease, including ischemic retinopathy and optic neuropathy, possibly worsened by the patient's underlying cardiovascular risk factor status and medications. Daratumumab, a humanized IgG1 kappa monoclonal antibody that binds to CD38 used to treat specific blood cancers, has been reported to cause disturbances in retinal blood flow, including retinal artery and vein occlusions. It remains to be determined whether careful patient selection or dose adjustments and timing of HCDD treatments could protect vision by reducing the risk of these rare yet severe ocular complications.

Retinal Vasculitis in a Patient with Hansen's Disease.

PURPOSE: Hansen's disease is endemic in over 140 countries worldwide and a potentially blinding condition. We describe a case of retinal vasculitis in a patient with Hansen's disease with concomitant positive antiphospholipid antibody serology, a potentially under-reported complication in this setting. METHODS: A 37-year-old Brazilian man systemically stable on triple therapy (clofazimine, rifampin, dapsone) for Hansen's disease presented for a comprehensive ophthalmic evaluation. RESULTS: Dilated exam revealed diffuse peripheral intraretinal hemorrhages in his right eye. Fluorescein angiography showed peripheral non-perfusion, abnormal shunt vessels and leakage from the retinal veins in the right eye and peripheral non-perfusion and vascular leakage in the fellow eye, consistent with vasculitis. Laboratory workup was notable for positive antiphospholipid antibodies (lupus anticoagulant, anticardiolipin IgM, anti-beta-2 glycoprotein 1 IgM) and normocytic anemia. CONCLUSION: As antiphospholipid antibodies are present in a large proportion of patients with Hansen's disease, it is possible that retinal vasculitis may be more common than previously thought. The extent to which retinal vasculitis occurs in Hansen's disease remains uncertain and possibly underestimated due to the frequency of anterior segment scarring, which impedes retinal evaluation. Heightened surveillance for potential retinal vascular complications is warranted.

A Novel Video-Based Skills Curriculum for Incoming Ophthalmology Residents.

BACKGROUND: Given limited pre-residency ophthalmology exposure, skill training for PGY-2 ophthalmology residents is essential. However, orientation experiences vary, and skills acquisition is often not measured. OBJECTIVE: A novel video-based orientation curriculum was developed and implemented to standardize and effectively teach ophthalmic examination skills to incoming ophthalmology residents. METHODS: An instructional video library (VL) on ophthalmic exam skills was created in 2020. Prior to any instruction, PGY2s were recorded performing basic ophthalmic exams (BOE) using slit-lamp recording smartphone adapters. After a 2-week orientation involving live teaching, practice, and self-directed library review, ophthalmic exams were again recorded. A 36-point ophthalmic exam skills checklist expanding upon the Ophthalmic Clinical Evaluation Exercise (OCEX) was developed for scoring videos. Residents also completed pre- and post- surveys assessing their comfort with the ophthalmic exam. RESULT: 7 of 11 incoming PGY-2 residents (63.7%) ophthalmology residents participated. Average recorded OCEX score improved from 16.5 ± 5.8 to (p = .0002) to 30.9 ± 2.7. Surveyed resident comfort with the exam increased from 2.4 ± 0.6 to 4.2 ± 0.5 on a 5-point Likert scale (p < .0001). CONCLUSION: Our video library orientation curriculum was effective in rapidly increasing resident comfort and BOE skills. With the launch of the integrated internship model, the VL curriculum may be effective for training ophthalmology PGY1s, medical students and non-ophthalmology providers.

Water-drinking test in primary angle-closure suspect before and after laser peripheral iridotomy.

BACKGROUND: This study aimed to investigate whether performing a laser peripheral iridotomy in primary angle-closure suspects had an effect on the intraocular pressure response during the water-drinking test. DESIGN: Prospective study in a hospital setting. PARTICIPANTS: Primary angle-closure suspects scheduled for a laser peripheral iridotomy. METHODS: Primary angle-closure suspects were evaluated before the laser peripheral iridotomy and 2 weeks after. On each visit, subjects underwent the water-drinking test. Intraocular pressure was measured every 15 min within a 1-h period. MAIN OUTCOME MEASURES: Intraocular pressure fluctuations during the water drinking test. RESULTS: Twenty patients were enrolled. The mean age was 58.1 years (±10.2 SD), predominantly female (n = 14, 70%). Average intraocular pressure range (maximum-minimum) during the water-drinking test increased significantly from 2.6 ± 1.1 mmHg before the laser peripheral iridotomy to 3.5 ± 1.5 mmHg after (P = 0.04). The mean difference between peak and last intraocular pressure measurements increased after the laser peripheral iridotomy was performed, from 2.5 mmHg (±1.27 SD) to 3.3 mmHg (±1.66 SD), (P = 0.057). There was no change in mean peak intraocular pressure (maximum-baseline) before and after the laser peripheral iridotomy (P = 0.87). CONCLUSIONS: Primary angle-closure suspects who underwent the water-drinking test had a slightly more pronounced recovery of intraocular pressure after the laser peripheral iridotomy was performed, which may be attributed to increase in the area of exposed trabecular meshwork following treatment. Differences in intraocular pressure curves before and after laser peripheral iridotomy were minimal, and therefore, the water-drinking test had limited value as a provocative test in this patient population.