Purpose We describe a case of epivascular glia (EVG) masquerading as dry age-related macular degeneration (AMD). Observations A 64-year-old healthy man with 20/20 vision bilaterally was referred for evaluation of macular lesions thought to be drusen due to dry AMD. Fundus autofluorescence (FAF) was unremarkable, indicating preserved retinal pigment epithelium (RPE) and photoreceptor integrity. Spectral-domain optical coherence tomography (SD-OCT) demonstrated a normal ellipsoid zone and intact RPE without evidence of drusen but revealed hyperreflective epivascular material that co-localized with the findings on near infrared reflectance and on clinical examination. En face OCT segmented at the level of the internal limiting membrane showed hyperreflectivity corresponding to the macular lesions on near infrared reflectance and on clinical examination. Conclusions and Importance These lesions are consistent with benign epivascular glia, presumably a Muller cell derived entity, underscoring the value of recognizing this entity and use of multimodal imaging in avoiding misdiagnosis of AMD.
Purpose To present a case of squamous cell carcinoma of the right (OD) conjunctiva and eyelid in an eye with biopsy-confirmed, unilateral ocular cicatricial pemphigoid (OCP) in the ipsilateral eye. Observations A 68-year-old male patient presented with chronic irritation OD and a previous diagnosis of trichiasis. A diagnosis of OCP was confirmed by biopsy with direct immunofluorescence. Despite treatment with methotrexate and mycophenolate mofetil, he later began to experience accelerated and refractory inflammation and pain OD. Multiple cultures were negative, and inflammation was also refractory to anti-microbial therapy. A second and third repeat biopsy for histopathology showed infiltrating squamous cell carcinoma (SCC) of the right bulbar conjunctiva and eyelid, prompting exenteration of the eye given the extent of involvement. Retrospective review of the original, OCP-confirmatory biopsy showed no evidence of SCC. Conclusions and importance OCP that is unilaterally worse and refractory to treatment is an unusual presentation that warrants further investigation. This presentation can be concerning for development of SCC or similar cancers in the affected eyelid. Chronic inflammatory conditions such as OCP may increase the risk of developing SCC.
Purpose To report a case of partially reversible retinal dysfunction associated with pimitespib documented using multimodal imaging and full-field electroretinography (ERG). Observations A patient with advanced gastrointestinal stromal tumor developed nyctalopia and blurred vision after initiation of pimitespib therapy. Spectral-domain optical coherence tomography (SD-OCT) showed blurring of the ellipsoid zone and interdigitation zone. Widefield short-wavelength fundus autofluorescence demonstrated regional signal differences, with relatively increased autofluorescence in the peripheral retina. ERG revealed severe generalized photoreceptor dysfunction involving both rod and cone responses. Pimitespib was discontinued 3 months after initiation, and symptoms gradually improved. Partial structural recovery of the outer retina was observed on OCT, although some EZ abnormalities persisted in the temporal retina, and ERG responses showed partial recovery. The drug was subsequently reintroduced at a reduced dose without recurrence of symptoms or structural abnormalities during follow-up. Conclusions and importance Pimitespib-related retinal dysfunction in this patient manifested as outer retinal toxicity involving both rod and cone photoreceptors that showed only partial recovery after drug cessation, underscoring the importance of early recognition. Reduced-dose rechallenge was tolerated without further deterioration, suggesting that dose adjustment may permit continued therapy. Multimodal imaging and full-field ERG were useful for detecting and monitoring retinal toxicity in patients receiving pimitespib.
Purpose To describe the evolving near-infrared (NIR) imaging findings and cross-sectional optical coherence tomography (OCT) features in a patient with a resolving sub-internal limiting membrane (sub-ILM) hemorrhage. Observations An 85-year-old man presented with an ‘egg-shaped’ scotoma in the left eye with sudden onset 5 weeks ago. Examination revealed a large dome-shaped sub-ILM hemorrhage, as well as subhyaloid and subretinal hemorrhage due to a ruptured retinal macroaneurysm. The sub-ILM hemorrhage demonstrated hyporeflectivity on NIR and corresponding hyperreflectivity on OCT, which is brighter inferiorly, and results in less OCT signal transmission to the underlying retinal layers. By 11 weeks after onset, the sub-ILM hemorrhage was smaller with a superior serous and an inferior mixed hemorrhagic and dehemoglobinized component. The hemorrhage has become more hyperreflective on NIR in both superior and inferior components, compared to prior. OCT scans continued to show a hyperreflective signal from blood, which was brighter inferiorly, but there was greater OCT signal transmission to the underlying retinal layers. Conclusions and importance These changes on NIR and OCT appear to follow the Beer-Lambert law and may illustrate the evolution of hemoglobin from to its degradation products.
Purpose To report an unusually excellent visual acuity and binocular outcome after severe ocular trauma. Methods This is a retrospective case report of a 44-year-old male who presented with hand motion vision after a severe open globe injury caused by blunt trauma from a surfboard hitting the eye. Results After primary globe repair, vision declined to light perception only. Two months later, the patient underwent repair of closed incarcerated funnel retinal detachment with pars plana vitrectomy, membrane peel, and 360 retinectomy. He subsequently underwent two strabismus surgeries to address severe torsional diplopia and a later strabismus surgery to address subsequent convergence insufficiency type exotropia. He showed remarkable vision recovery to best corrected 20/20 visual acuity and ability to maintain single binocular vision. Discussion Despite the poor outcomes typically associated with globe ruptures and the several unfavorable prognostic factors with which he presented, this patient had an unusually good visual acuity recovery after repair of the closed funnel retinal detachment. The good vision was accompanied by intractable torsional diplopia that was addressed with complex strabismus surgery, resulting in excellent binocular fusion and vision functioning. Unusual and extreme strabismus surgery intervention can help restore binocular vision when trauma surgery and retinal detachment repair results in severe diplopia and underlying strabismus. This report highlights that ocular trauma management is complex, and an excellent visual acuity outcome can create its own rehabilitative challenges which may require an ophthalmic multidisciplinary team approach.
