Nonperfusion of the Far Peripheral Retina in Highly Myopic Stickler Syndrome.

Purpose: To describe the clinical course of 3 patients with Stickler syndrome found on fluorescein angiography (FA) to have nonperfusion of the peripheral retina. Methods: Three patients with confirmed Stickler syndrome were examined under anesthesia. Genetic testing and FA were performed. Results: Each patient had characteristic ocular findings of Stickler syndrome, including high myopia with vitreoretinal degeneration. FA was performed on each patient and showed 360 degrees of nonperfusion of the retinal periphery in all eyes, with mild leakage in Case 3. Conclusions: The current series presents evidence of peripheral retinal nonperfusion in 3 consecutive patients with Stickler syndrome. Based on these findings, the authors recommend adopting FA as a standard imaging modality and using laser photocoagulation to treat the areas of retinal nonperfusion for all patients with Stickler syndrome.

Nd:YAG Laser Hyaloidotomy: A Therapeutic Approach for Valsalva Premacular Hemorrhage.

Subhyaloid hemorrhage, characterized by localized vitreoretinal detachment due to blood accumulation, often results in sudden vision loss, especially in the macular area. This case report highlights a 23-year-old female presenting with exercise-related Valsalva retinopathy leading to premacular subhyaloid hemorrhage. The patient underwent neodymium-doped yttrium aluminum garnet (Nd:YAG) laser hyaloidotomy, a non-invasive procedure, leading to rapid blood drainage and visual recovery. The patient's initial visual acuity was severely impaired, with a significant premacular hemorrhage obscuring the macula. A week later, due to the expanding hemorrhage, Nd:YAG laser hyaloidotomy was performed, demonstrating successful blood dispersion and restoration of vision. Follow-up revealed significant improvement with demarcation of the previous hemorrhage and no evidence of new findings. The case emphasizes the importance of prompt intervention and considers alternative treatments for premacular subhyaloid hemorrhage. While associated with ocular pathologies, such as macular holes and retinal detachment, Nd:YAG laser hyaloidotomy remains a safe and effective outpatient procedure for managing premacular subhyaloid hemorrhage, avoiding the risks of more invasive surgical interventions. The presented case highlights the significance of tailored interventions based on patient history, minimizing the need for invasive procedures and their associated risks.

External Compressive Ischemic Orbitopathy: A Rising Clinical Entity.

PURPOSE: Saturday night retinopathy, the term coined by Jayam et al . in 1974, is a rare condition in which external compression of the orbit during a drug and alcohol stupor causes a unilateral orbitopathy with ophthalmoplegia and ischemic retinopathy. This condition has been increasingly reported in the last decade, correlating with an increasing burden of substance use. This condition mirrors a similar entity typically reported in patients following spinal surgery, where a headrest supporting the patient's face compresses the orbit. The current authors combine these 2 entities, entitled external compressive ischemic orbitopathy, and present a comprehensive literature review describing this entity. METHODS: A systematic review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. All related publications of vision loss in the setting of orbital compression were reviewed. Data collected included patient demographics, precipitating circumstances of vision loss, presenting ocular symptoms, outcomes, and ancillary imaging. RESULTS: In total 31 articles were selected for inclusion, yielding 46 patients. A total of 10 patients suffered orbitopathy in the setting of a drug stupor, and 36 following prone-positioned surgery. However, 79% of patients presented with visual acuity of light perception or worse. Also, 86% of patients presented with ophthalmoplegia, 92% with proptosis and orbital edema, and 86% with varying degrees of retinal ischemia. When compared with iatrogenic cases, self-induced stuporous cases demonstrated worse presenting visual acuity, ophthalmoplegia, retinal and choroidal filling, and worse final outcomes. CONCLUSION: External compressive ischemic orbitopathy is a severe vision-threatening condition that has been increasingly reported in the last decade.

Can the Future be Bright with Advances in Diabetic Eye Care?

The prevalence of diabetic retinopathy is steadily increasing as the population of patients with diabetes grows. In the past decade, the development of anti-VEGF agents has dramatically changed the treatment landscape for diabetic retinopathy and diabetic macular edema (DME). Newer agents in development aim to reduce the treatment burden of diabetic retinopathy.

Superficial and Deep Capillary Plexus Nonperfusion in Nonaccidental Injury on OCTA.

A 5-year-old Caucasian male with a history nonaccidental injury (NAI) at age 1 presented with new onset no light perception in the left eye due to a closed funnel retinal detachment. The right eye showed optic nerve pallor, peripheral vascular attenuation, and leakage. Optical coherence tomography angiography (OCTA) revealed significant parafoveal attenuation of the superficial vascular plexus, intermediate capillary plexus, and the deep capillary plexus. This correlated with inner and middle retinal layer thinning temporal to the fovea and preservation of the ellipsoid zone. The peripapillary vascular plexus was preserved. Laser photocoagulation was performed to the nonperfused peripheral retina and intravitreal bevacizumab was injected. OCTA may be used in patients with NAI to characterize macular ischemia changes. Attenuation of the superficial, intermediate, and deep capillary plexuses in our patient may represent chronic ischemic retinal changes that arise from traumatic injury to the vitreoretinal interface and inner retina in NAI.

Scleral Suture Fixation of Dislocated Posterior Chamber Intraocular Lens: Modification for Tapered Haptics.

PURPOSE: To present a surgical modification to a previously published technique that allows repositioning and scleral fixation of one-piece acrylic intraocular lenses with tapered haptics. METHODS: A retrospective review of three consecutive cases. RESULTS: Our technique modification enables repositioning using scleral suture fixation of one-piece intraocular lenses with tapered haptics by looping the haptic at its proximal, notched junction to the optic with the polypropylene suture. The suture knot is internalized which effectively suspends the intraocular lens and allows for better adjustment of the intraocular lens centration. Two cases were endocapsular dislocations; the third case was dislocated extracapsularly due through a posterior capsule rupture. All showed adequate lens centration at 3 months of follow-up. One case had suffered a vitreous hemorrhage a month postoperatively that is clearing. CONCLUSION: Repositioning using scleral fixation of one-piece intraocular lenses with tapered haptics is possible with a minor technique modification. This offers an alternative to intraocular lens exchange.

Penile Filler Injection Leading to Silicone Embolism Syndrome and Paracentral Acute Middle Maculopathy.

A 31-year-old male with acute blurred vision and respiratory distress occurring three days after a penile filler injection was found to have outer plexiform layer hyperreflectivity on optical coherence tomography (OCT), consistent with paracentral acute middle maculopathy (PAMM). He was diagnosed with diffuse bilateral pneumonitis and silicone embolism syndrome requiring inpatient management. Following systemic stabilization, his vision and OCT findings improved. PAMM may be associated with silicone embolism syndrome in the presence of acute central scotomas and minimal retinal findings. Penile filler injections could contribute to retinal vaso-occlusion via an embolic process. PAMM may be self-limited and result in functional vision. [Ophthalmic Surg Lasers Imaging Retina 2022;53:635-638.].

Intraocular inflammation secondary to intravitreal brolucizumab treated successfully with Sub-Tenon triamcinolone: A case report.

PURPOSE: To report on a case of successful treatment of intraocular inflammation (IOI) secondary to brolucizumab intravitreal injection that responded to a single sub-Tenon injection of triamcinolone. OBSERVATIONS: An 81-year-old female with a longstanding history of exudative age-related macular degeneration (AMD) was unresponsive to various regimens of anti-VEGF injections. Her AMD was treated with one intravitreal injection of brolucizumab (6mg/0.05ml) into the right eye. On a follow-up visit, she had a new-onset conjunctival injection, with anterior chamber and vitreous inflammation, in the right eye, which was diagnosed as non-granulomatous iridocyclitis. The patient was treated with one posterior sub-Tenon injection of triamcinolone (40mg/ml) into the right eye. Subsequently, there was a durable resolution of inflammation, and her vision improved along with the resolution of her exudation. CONCLUSIONS AND IMPORTANCE: This case suggests that some brolucizumab-related IOI episodes may be treated with posterior sub-Tenon triamcinolone. Further studies may serve to elucidate the role of sub-Tenon triamcinolone in brolucizumab-associated IOI.

Choroidal calcifications in two cases of aplasia cutis congenita and oculoectodermal syndrome.

PURPOSE: To describe choroidal calcifications as an ophthalmic feature in aplasia cutis congenita (ACC) with oculoectodermal syndrome (OES). OBSERVATIONS: Two cases of ACC/OES with characteristic echographic evidence of choroidal calcifications are described. CONCLUSIONS AND IMPORTANCE: The ophthalmic manifestations of ACC/OES may be expanded to include choroidal calcifications. The presence of a choroidal calcification with B-scan ultrasound in a case suspicious for ACC/OES may facilitate a more timely diagnosis and inform future follow-up regimens to monitor ophthalmic and systemic manifestations of this disease.

Ophthalmic Manifestations of Hodgkin Lymphoma: A Review.

BACKGROUND: Hodgkin lymphoma (HL) is a hematopoietic neoplasm characterized by cancerous Reed-Sternberg cells. In contrast to ophthalmic manifestations by non-HL that are well recognized, there is paucity of the literature as it relates to ophthalmic manifestation by HL. We performed a comprehensive review of published studies (case reports and small case series) to characterize the ophthalmic manifestations of HL. SUMMARY: Thirty patients were identified with ophthalmic manifestation of HL. Thirteen (43%) were male, and 14 (46%) were female (in 3 cases, sex was not specified). The median age at ophthalmic presentation was 27 years. Diagnosis of HL was made after ophthalmic manifestation in 10 (33%) cases, whereas 11 (36%) cases had a prior diagnosis of HL. Ophthalmic manifestations can be classified into 3 main groups; direct infiltration, inflammatory reaction, and paraneoplastic process. Seven cases had infiltration of the optic nerve. Uveal inflammatory reaction was reported in 21 cases. The presence of intraocular Reed-Sternberg cells had been confirmed in 1 case with granulomatous uveitis. Conjunctival and corneal reaction was seen in 3 cases. HL was in stage 2 or higher, with only 1 case with stage 1A (12 cases HL stage not specified). Seven cases (22%) died of HD, all were diagnosed with advanced lymphoma, and none was treated with chemotherapy. KEY MESSAGE: Ocular involvement in HL is extremely rare. A few cases of histopathologically confirmed optic nerve/tract infiltration are within the spectrum of CNS involvement by HL. Inflammatory uveitis is the most common ophthalmic association of HL. In the presence of prior known diagnosis of HL, restaging should be considered to exclude recurrence. Toxicity or adverse reaction to drugs used to treat HL may also contribute to ophthalmic involvement.

Outcomes of Pars Plana Vitrectomy Alone versus Combined Scleral Buckling plus Pars Plana Vitrectomy for Primary Retinal Detachment.

PURPOSE: We compared the outcomes of primary uncomplicated rhegmatogenous retinal detachment (RRD) repair using pars plana vitrectomy (PPV) alone versus combined scleral buckling plus PPV (SB+PPV). DESIGN: Retrospective, observational study. PARTICIPANTS: Patients with primary RRD who underwent PPV or SB+PPV from June 1, 2014, through December 31, 2017. METHODS: We performed a single-institution, retrospective, observational study of 488 consecutive patients with primary RRD repaired via PPV alone or SB+PPV and gas tamponade. We excluded patients younger than 18 years and those with advanced proliferative vitreoretinopathy, giant retinal tear, trauma, or secondary forms of RRD. We performed logistic regression and Cox proportional hazard regression analyses to identify potential risk factors associated with a retinal redetachment. MAIN OUTCOME MEASURES: Single-operation anatomic success (SOAS), defined as adequate retinal reattachment achieved with only 1 procedure. RESULTS: The mean follow-up interval was 14.3 months. Single-operation anatomic success and final anatomic success were achieved in 425 eyes (87.1%) and 487 eyes (99.8%), respectively. Single-operation anatomic success was achieved in 90 of 111 eyes (81.1%) with PPV alone compared with 345 of 374 eyes (92.2%) with SB+PPV (P = 0.0010). Scleral buckling plus PPV showed greater SOAS than PPV alone in phakic eyes (P < 0.0001), but not in eyes with a posterior chamber intraocular lens (PCIOL). Retinal redetachments occurred on average at 1.5 and 9 months after the initial surgery. Significant best-corrected visual acuity improvement was associated with SOAS (P < 0.0001). CONCLUSIONS: Scleral buckling plus PPV resulted in greater SOAS outcomes than PPV alone for primary RRD repair. Phakic eyes achieved greater surgical success with SB+PPV, whereas eyes with a PCIOL achieved similar results with both methods. Most retinal redetachments occurred within the initial postoperative 3-month period. Single-operation anatomic success was associated with statistically significant visual improvement.

Conjunctival Melanoma Angiotropic Microsatellitosis: A Mechanism of Local Extravascular Migratory Metastasis.

PURPOSE: To report a case of local metastasis of conjunctival melanoma, which may occur via extravascular migratory metastasis (EVMM), and discuss its clinical relevance in conjunctival melanoma tumor staging and possible management implications. METHODS: Retrospective chart review of a single clinical case with clinicopathologic correlation. RESULTS: A 65-year-old male referred due to local recurrence of conjunctival melanoma at the caruncle was successfully treated after two excisional procedures with negative sentinel lymph node biopsies. Forty-eight months after initial presentation, the patient developed a nodular lesion representing local recurrence in the ipsilateral upper tarsal conjunctiva, distant from the primary tumor site. Histopathology showed nodules in the substantia propria in the absence of primary acquired melanosis. The tumor cells were found along the extravascular surface without intralymphatic or intravascular tumor cells consistent with local metastasis. One possible mechanism is angiotropic microsatellitosis leading to local EVMM. Additional neck CT imaging showed no lymphadenopathy. CONCLUSION: EVMM via angiotropic microsatellitosis is another possible mechanism of noncontiguous local recurrence of conjunctival melanoma. Angiotropic microsatellitosis may represent a high-risk finding possibly related to increased melanoma-related mortality.

Histopathologic Patterns of Recurrent Choroidal Melanoma Following I-125 Plaque Brachytherapy.

PURPOSE: Histologic correlation of clinical patterns of recurrent choroidal melanoma following I-125 plaque brachytherapy was performed to identify pathologic mechanisms of recurrence. METHODS: We reviewed 7 cases of recurrent choroidal melanoma following I-125 plaque brachytherapy managed with enucleation. Clinical characteristics included tumor dimensions, radiation dose, time to local recurrence, and clinical pattern of recurrence. Histopathology (hematoxylin and eosin and periodic acid - Schiff) and immunohistochemistry (Ki-67, CD-163, HMB45, and SOX10) were performed. RESULTS: Mean follow-up time and time to local recurrence were 42 and 21 months after brachytherapy, respectively. Tumor recurrences were described clinically as marginal in 43%, diffuse in 29%, and extraocular extension (EOE) in 29%. Eighty-six percent were classified as mixed cell type and 14% were epithelioid type. Tumor zonation (histologic demarcation between zones of recurrent and nonrecurrent tumor cells by immunohistochemistry) was present in marginal and EOE cases (n = 6) and absent in the diffuse cases (n = 2). Ki-67 proliferative index was higher in marginal and EOE recurrences, while diffuse cases showed uniform -Ki-67 staining. CD-163 staining was found to be greater in nonrecurrent tumor. HMB45 correlated with SOX10 with a greater staining in recurrent tumor. CONCLUSION: Our observations provide a correlation between histopathologic and clinical patterns of local recurrence of choroidal melanoma after brachytherapy.

Episcleral brachytherapy for retinoblastoma.

BACKGROUND/AIMS: To report visual outcomes, survival outcomes and complications following episcleral brachytherapy (EB) for retinoblastoma. METHODS: Retrospective review of retinoblastoma cases treated with EB in a single institution. Survival outcomes were analysed using the Kaplan-Meier method. RESULTS: Eleven tumours of 11 eyes were treated with either iodine-125 or ruthenium-106 EB with a mean apical dose of 44 Gy. The tumours were classified as group B in 5 (46%), C in 3 (27%) or D in 3 (27%) eyes, respectively. Mean follow-up time was 75.4 months. EB served as primary treatment in 3 eyes (27%) and secondary treatment in 8 eyes (73%). Final visual acuity was better than 20/200 in 70% of cases. Globe preservation was achieved in 9 (82%) eyes. Local recurrence occurred in 18% of cases at a mean onset of 17.4 months after EB. Two group D tumours that recurred after secondary EB underwent enucleation. Mean onset of radiation retinopathy was 17.4 months following EB. No metastatic or fatal events were recorded. Kaplan-Meier analysis showed recurrence-free survival and ocular survival of 80% and overall survival of 100% at 5 years after EB. CONCLUSION: EB is an effective primary or secondary treatment modality for selected retinoblastoma eyes (groups B and C). Advanced group D tumours may represent a risk factor for local recurrence. Visually significant complications such as radiation retinopathy should be anticipated.

Primary hepatic carcinoid tumor metastasizing to eyelid: A case report.

PURPOSE: We report a rare case of a metastatic carcinoid tumor to the right lower lid masquerading as a chalazion. OBSERVATIONS: A 78-year-old Hispanic woman who presented with a 3-month history of a non-resolving chalazion on the right lower lid despite aggressive medical treatment. The patient had a history of noninfectious anterior uveitis and primary hepatic carcinoid tumor that was incidentally diagnosed during the initial uveitis work-up. The right lower eyelid lesion was biopsied and histological and immunopathological analysis revealed a well differentiated neuroendocrine tumor consistent with a carcinoid tumor. CONCLUSIONAND IMPORTANCE: Neuroendocrine tumors should be considered as part of the differential diagnosis of focal, vascularized eyelid masses. To the authors best knowledge this is the first reported case of primary hepatic carcinoid tumor with metastasis to the eyelids. We also highlight the importance of pursuing a histopathologic diagnosis, in the setting of a non-resolving or recurrent chalazion.

Differentiating Veins From Arteries on Optical Coherence Tomography Angiography by Identifying Deep Capillary Plexus Vortices.

PURPOSE: To introduce a simple method for differentiating retinal veins from arteries on optical coherence tomography angiography (OCTA). DESIGN: Cross-sectional pilot study. METHODS: Four default en face slabs including color depth encoded, grayscale full-thickness retina, superficial plexus, and deep capillary plexus (DCP) from nine 3×3-mm and nine 6×6-mm OCTA scans were exported and aligned. Nine ophthalmologists with minimum OCTA experience from 2 eye institutions were instructed to classify labeled vessels as arteries or veins in 3 stages. Classification was performed based on graders' own assessment at stage 1. Graders were taught that a capillary-free zone was an anatomic feature of arteries at stage 2 and were trained to identify veins originating from vortices within the DCP at stage 3. Grading accuracy was analyzed and correlated with grading time and graders' years in practice. RESULTS: Overall grading accuracy in stages 1, 2, and 3 was (50.4% ± 17.0%), (75.4% ± 6.0%), and (94.7% ± 2.6%), respectively. Grading accuracy for 3×3-mm scans in stages 1, 2, and 3 was (49.9% ± 16.3%), (79.2% ± 9.6%), and (96.9% ± 3.1%), respectively. Accuracy for 6×6-mm scans in stages 1, 2, and 3 was (51.4% ± 20.8%), (72.3% ± 7.9%), and (93.2% ± 3.3%), respectively. Grading performance improved significantly at each stage (all P < .001). No significant correlation was found between accuracy and time spent grading or between accuracy and years in practice (r = -0.164 to 0.617, all P ≥ .077). CONCLUSIONS: We describe a simple method for accurately distinguishing retinal arteries from veins on OCTA, which incorporates the use of vortices in the DCP to identify venous origin.

Variability of Bad Prognosis in Uveal Melanoma.

TOPIC: Survival of patients with uveal melanoma classified to have a bad prognosis. CLINICAL RELEVANCE: To explore reasons for reported variability in survival of patients with uveal melanoma classified to have a bad prognosis. METHODS: We searched PUBMED, MEDLINE, and EMBASE for studies reporting survival data for uveal melanoma undergoing prognostic testing with chromosome 3 status by fluorescence in situ hybridization (FISH), comparative genomic hybridization (CGH), microsatellite analysis (MSA), multiplex ligation-dependent probe amplification (MLPA), single nucleotide polymorphism (SNP), gene expression profiling (GEP) class, and exon sequencing. Only studies reporting 1-year, 3-year, or 5-year survival were included in the study. RESULTS: The initial search resulted in 49 studies. Only 12 studies met inclusion criteria. Three studies reported survival data for FISH, 1 study reported survival data for CGH, 1 study reported survival data for MSA, 3 studies reported survival data for MLPA, 3 studies reported survival data for SNP, 3 studies reported survival data for GEP, and 2 studies reported survival data for a combination of tests. No studies reported survival data for exon sequencing. Six studies reported percent free of metastatic death, 2 studies reported metastasis-free survival (MFS), 2 studies reported overall survival (OS), and 2 studies reported probability of metastasis. Metastasis-free survival (5 years) for monosomy 3 by FISH was 40% to 60%, by MLPA was 30% to 40%, by SNP was 72%, and for GEP class 2 was not reported. Overall survival (5 years) for monosomy 3 and disomy 8 tumors by MLPA and GEP class 2 were not comparable (81% and 55%, respectively). CONCLUSIONS: Variability exists in reported survival for uveal melanoma with a bad prognosis. Several factors, including composition of study population (tumor size, exclusion of iris melanoma, duration of median follow-up), method of obtaining tumor sample, type of prognostic test, and use of variable outcome measures, can explain some of the observed differences in survival. Variations in determining the cause of death (metastatic or nonmetastatic) may be the major reason for the observed differences. Standardization of study methods and outcome measures will allow comparison of survival data derived from different prognostic tests.

Opacification of Akreos Hydrophilic Acrylic Lens After Retinal Detachment Repair with Silicone Oil Tamponade: A Case Report.

We report localized opacification of a sclera-fixated Akreos® hydrophilic acrylic intraocular lens after successful repair of rhegmatogenous retinal detachment with silicone oil tamponade in a nondiabetic patient. No intraoperative lens opacification during air-fluid exchange or lens dislocation was encountered. Granular opacities within the optic were noted at 5 months after surgery, and did not relent to scrubbing with a vitrector during oil removal. Akreos lens opacification under silicone oil is not well documented in the literature. Surgeons must be aware of this potential complication, which is known to occur with various types of hydrophilic acrylic lenses after exposure to air or gas.

Animal models of radiation retinopathy - From teletherapy to brachytherapy.

Radiation retinopathy is a serious vision-impairing complication of radiation therapy used to treat ocular tumors. Characterized by retinal vasculopathy and subsequent retinal damage, the first sign of radiation retinopathy is the preferential loss of vascular endothelial cells. Ensuing ischemia leads to retinal degradation and late stage neovascularization. Despite the established disease progression, the pathophysiology and cellular mechanisms contributing to radiation retinopathy remain unclear. Clinical experience and basic research for other retinal vasculopathies, such as diabetic retinopathy and retinopathy of prematurity, can inform our understanding of radiation retinopathy; however, the literature investigating the fundamental mechanisms in radiation retinopathy is limited. Treatment trials have shown modest success but, ultimately, fail to address the cellular events that initiate radiation retinopathy. Animal models of radiation retinopathy could provide means to identify effective therapies. Here, we review the literature for all animal models of radiation retinopathy, summarize anatomical highlights pertaining to animal models, identify additional physiological factors to consider when investigating radiation retinopathy, and explore the use of clinically relevant tests for studying in vivo models of radiation retinopathy. We encourage further investigation into the mechanistic characterization of radiation retinopathy in the hope of discovering novel treatments.