Central Retinal Artery Occlusion in a Young Patient With a Hidden Unusual Sickle Cell Trait.

Sickle cell trait is considered a benign condition. Ophthalmic manifestations are infrequent but can result in significant visual deterioration. We present a case of a 33-year-old male, not known to have any medical illnesses, who presented to the ophthalmological emergency room complaining of a sudden onset of painless and profound left eye vision loss for 12 hours. The patient denied any medication use, past eye trauma, or surgery. On detailed ophthalmologic examination, the best-corrected visual acuity (BCVA) was 20/20 in the right eye and hand movement in the left eye. Dilated fundus examination of the left eye showed a central retinal artery occlusion (CRAO) with pale, white retinal swelling and a macular cherry-red spot. Fundus fluorescein angiography showed delayed arterial filling with persistently reduced macular perfusion. CRAO was diagnosed in an otherwise healthy young male. Systemic workup was negative except for protein electrophoresis, which showed sickle cell trait, and HbA1C was 7.8%. Later, atrophic macular changes with a pale optic disc were observed, and BCVA was reduced to light perception. CRAO in young patients amounts to diverse causes, which require extensive systemic workup. In addition, the concurrence of the sickle cell trait with diabetes mellitus might have a role in CRAO development.

Hydrogel (Miragel®) scleral explant, late orbital complication.

INTRODUCTION: The hydrogel Scleral Buckle is a soft and pliable subtype of buckles which has been used to repair retinal breaks and detachments externally. This case represents an unusual late orbital complication of the implant. CASE REPORT: A 70 years old male patient presented with drooping right upper lid and eye misalignment, associated with foreign body sensation and discomfort. The patient underwent scleral buckle surgery for a rhegmatogenous retinal detachment 32 years before his presentation using a hydrogel MIRAgel® explant. Orbital imaging showed a large cystic lesion in the superolateral quadrant of the orbit indicating a fluid-filled explant. DISCUSSION: In patients who were treated with hydrogel MIRAgel® explant for retinal breaks or detachments, detailed ophthalmic history, particular past eye surgery history, and examination, orbital imaging, and good clinical documentation. Serious orbital and neurological conditions need to be ruled out carefully. CONCLUSION: Late orbital complications of hydrogel MIRAgel® explant might develop late after surgery and might represent a diagnostic challenge for ophthalmologists.

Uveitic Granulomas Masquerading as Ocular Tumors.

Introduction: Solitary uveal lesions confer a diagnostic challenge to ophthalmologists. Uveitic lesions most abundantly appear amelanotic and commonly involve the choroid. Most amelanotic choroidal lesions are either neoplastic or inflammatory in origin. In our study, we aimed to describe six uveitic granuloma cases, which were referred to a tertiary ophthalmology center as intraocular tumors. Methods: Retrospective chart review of 6 patients (7 eyes) who had uveitic granulomas and were referred to a tertiary ophthalmology center as having intraocular tumors. Results: Mean age on presentation was 47 ± 12.5 years. One lesion was involving the ciliary body only, five lesions had pure choroidal involvement, and one had ciliochoroidal involvement. Mean visual acuity on presentation was 1.7 ± 0.75 (Snellen = 20/1,000) and ranged from 20/80 to light perception. Mean basal diameter of all lesions was 7.7 ± 1.8 mm. Three lesions had moderate echogenicity, two lesions were low to moderate echoic, and one lesion had moderate to high echogenicity on ultrasonography. Three lesions were associated with retinal detachments. Five eyes showed an early hypofluorescence with late hyperfluorescence. Leakage of fluorescein at borders was noticed in 3 lesions. Final diagnosis was presumed intraocular tuberculosis in 4 patients, probable ocular sarcoidosis in 1 patient, and idiopathic solitary uveitic granulomas in 1 patient. Upon treatment, the vision improved to 0.3 ± 0.27 (Snellen = 20/40) and ranged from 20/20 to 20/100 after 4.7 ± 2.9 years of follow-up. Conclusions: Uveitic granulomas can demonstrate features of ocular tumors. Proper uveitis management leads to a favorable visual outcome and ocular preservation.

Central Serous Chorioretinopathy Following Oral Use of Adulterated Honey Mixed with Tadalafil: A Case Report.

BACKGROUND: Central serous chorioretinopathy (CSCR) is an idiopathic condition characterized by serous retinal detachment and/or retinal pigment epithelial (RPE) detachment, the condition associated with a fluid leak through the RPE into subretinal space. This article reports on an unusual case of CSCR following taking adulterated honey mixed with tadalafil powder. CASE PRESENTATION: A 38-year-old male, not known to have any medical illnesses, came to our ophthalmology clinic complaining of sudden onset of blurred central vision in the left eye for five days after taking an adulterated honey which was claimed to improve sexual performance. On taking a history, the patient denied taking any medications or past eye trauma or surgery. On detailed ophthalmologic examination, the best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/80 in the left eye. Dilated fundus examination of the right eye showed a flat retina, normal macular reflex, healthy optic nerve head and the left eye showed blunt foveal reflex with neurosensory retinal detachment at the macula and subretinal fluid. Optical coherence tomography (OCT) of the left eye showed marked macular thickening, leading to the impression of central serous chorioretinopathy (CSCR). Four weeks after stopping the adulterated honey, BCVA improved to 20/20 in the left eye, with complete resolution of subretinal fluids. CONCLUSION: Our case adds to recently reported cases of a link between tadalafil and CSCR.

Poor Outcome in Camel-Related Eye Trauma with Ruptured Globe.

PURPOSE: To report the poor visual outcome of ruptured globe caused by camel bites. OBSERVATIONS: A 48-year-old camel caregiver presented to the emergency department after being bitten by a camel in the left side of his face. Ophthalmic examination revealed a superior scleral wound from 9 to 2 o'clock, about 6 mm from the limbus extending to the equator with prolapse of uveal and vitreous tissues, an opaque cornea, total hyphema, diffuse subconjunctival hemorrhage, and a lower lid laceration involving the lid margin and the nasolacrimal duct. The patient has undergone surgical repairs of ruptured globe and lid laceration, followed by retinal detachment surgery. Following these surgical interventions, the patient preserved a light perception vision with flat retina. CONCLUSION: Camel-related injuries might primarily involve the ophthalmic structures, especially in camel bites. Camel-related eye trauma might lead to poor visual and anatomical outcomes which might not improve following surgical interventions.

The Effect of Perioperative Uveitis Control on the Success of Glaucoma Surgery in Uveitic Glaucoma.

PURPOSE: To study the effects of perioperative uveitis control (PUC) on postoperative intraocular pressure (IOP) and uveitis activity in uveitic glaucoma (UG) patients who required glaucoma surgeries. PATIENTS AND METHODS: A retrospective chart review of 109 patients (120 eyes) which had glaucoma surgery for UG. A total of 66 eyes which had PUC were compared to 54 eyes which did not have. Measurements of IOP and uveitis activity were recorded preoperatively and over 2 years postoperatively. Average number of antiglaucoma medications and frequency of surgical failure were obtained in both groups. RESULTS: Over 2 years postoperatively, average IOP was lower in eyes which had PUC. Significant differences in IOP were found at 3 months (P = 0.004), 6 months (P = 0.001), 1 year (P < 0.001), and 2 years (P < 0.001). Lower grades of anterior chamber (AC) inflammation were found in eyes which had PUC. Significant differences were found at 1 month (P < 0.001), 3 months (P < 0.001) and 6 months (P = 0.001). Mean number of antiglaucoma medications at last visit was 0.7 ± 1.1 for eyes which had PUC and 2.6 ± 1.5 for eyes which did not have PUC (P < 0.001). Among eyes which had PUC, only two eyes required second glaucoma surgeries, while 16 eyes with no PUC required further glaucoma surgeries after 27.7 ± 12.5 months (P < 0.001). CONCLUSION: Proper PUC in patients going for UG surgeries results in lower IOP levels and less AC inflammation over 2 years postoperatively. A comprehensive PUC regimen is needed for uveitic glaucoma patients going for surgeries.

Development of neovascular glaucoma after intraocular surgery in Pierson syndrome.

Purpose: To report a patient with Pierson syndrome who presented with neovascular glaucoma (NVG) after cataract surgery.Methods: Retrospective case report.Results: A 17-year old monocular female presented with sudden onset of pain and decreased vision in the right eye. On examination, she had intraocular pressure (IOP) of 50 mmHg, aggressive iris neovascularization (NVI) and 3-piece IOL. Fundus examination revealed pale disc with tessellated fundus and parapapillary atrophy. Vascular arcades were vertically stretched with avascular ischemic retina starting from the near periphery. Macula appeared thin and atrophic. An intravitreal injection of 0.05 mg/0.1 ml bevacizumab was given to the right eye followed by Ahmed glaucoma valve (AGV) implantation. Assessment of her brother revealed similar posterior segment changes. A subsequent urine analysis showed proteinuria and high albumin to creatinine ratio. Next-generation sequencing for LAMB2 gene revealed a homozygous c.4573 + 1 G > A variant confirming the diagnosis of Pierson syndrome.Conclusion: This case expands our knowledge on retinal ischemia in the setting of Pierson syndrome. Close monitoring after intraocular surgery is recommended to look for the development of NVG.

Clinical spectrum, genetic associations and management outcomes of Coats-like exudative retinal vasculopathy in autosomal recessive retinitis pigmentosa.

Background: Coats-like retinal vasculopathy in retinitis pigmentosa (RP) is rare. This study describes its clinical spectrum, management outcomes and genetic associations in patients with autosomal recessive RP (arRP).Materials and methods: Retrospective review of ophthalmic, multimodal imaging, genetic findings and treatment outcomes of arRP patients who developed Coats-like features. Identification of patients included searching a retinal dystrophy registry of 798 patients.Results: Ten eyes of six patients with arRP (4 males, 2 females, mean age 33 years) demonstrated Coats-like features, namely inferotemporal peripheral retinal telangiectasis combined with unilateral inferotemporal vasoproliferative tumor (VPT) in 4 eyes. Exudative retinal detachment (ERD) developed in five eyes of which four had VPT. Ablation of the vasculopathy using retinal laser photocoagulation and/or cryotherapy in eight eyes, allowed ERD and/or lipid exudation to decrease in seven eyes despite incomplete vasculopathy regression. Additional intravitreal triamcinolone acetonide injection in one eye failed to regress the ERD and associated VPT. Observation in one eye caused increased exudation. Six mutations, including three novel mutations, were found in CRB1, CNGB1, RPGR, and TULP1.Conclusions: Coats-like features in arRP range from retinal telangiectasis to VPTs with extensive ERD and occur predominantly in the inferotemporal retinal periphery. In addition to their classic association with CRB1 mutations, other genes are implicated. To the best of our knowledge, this is the first report describing CNGB1 mutations in Coats-like RP. Awareness of the vasculopathy spectrum is important, and timely ablation of the vasculopathy with long-term monitoring is recommended to prevent additional visual loss in RP patients.

Incidence and Natural History of Retinochoroidal Neovascularization in Enhanced S-Cone Syndrome.

OBJECTIVE: We examined the incidence and natural history of macular retinochoroidal neovascularization (RCN) in enhanced S-cone syndrome (ESCS). DESIGN: Retrospective case series. METHODS: This single-center study included 14 of 93 patients with ESCS who had signs of active or inactive RCN in ≥1 eye. We conducted multimodal retinal imaging, full-field electroretinography, and molecular genetic analysis of NR2E3 gene. Our main outcome measures included the cumulative incidence of RCN in ESCS, type of RCN, and mode of evolution of RCN. RESULTS: Fourteen (15.1%) of 93 patients with ESCS had RCN in ≥1 eye at 2 to 27 years of age. All 22 RCNs (21 eyes of 14 patients) were macular. Twelve of the RCNs were active with exudates/hemorrhages. Of these, 5 appeared de novo in a subretinal location, with photographic evidence of no pre-existing lesions. The latter were compatible with type 3 neovascularization or retinal angiomatous proliferation and subsequently evolved into unifocal fibrotic nodules. The remaining active lesions all had some degree of pre-existing fibrosis and remained stable. Ten inactive fibrotic nodules, identical to end-stage de novo lesions, were found and were presumed to represent healed RCNs. CONCLUSIONS: RCN, a treatable condition, may occur as early as 2 years of age and may be much more common in patients with ESCS than previously estimated. It may be the primary cause of the unifocal submacular fibrosis that is commonly observed in this condition. Additional research is needed to establish the pathogenesis of RCN in patients with ESCS and its optimal management.

Manifestation of Panuveitis after Intraocular Surgery in a Child with Blau Syndrome.

Blau syndrome (BS) is a rare granulomatous disease with autosomal dominant inheritance. It is characterized by a triad of dermatitis, arthritis, and recurrent uveitis. This case presents the onset of panuveitis in BS after intraocular surgery. A 10-year-old boy presented to the outpatient clinic with retinal detachment in the left eye after 6 years following early-onset cataract surgery. Bilateral panuveitis occurred 3 weeks after surgical repair and resulted in a total visual loss in the left eye and was persistent to conventional treatment in the right eye. Genetic testing revealed a mutation in NOD2 gene. The addition of adalimumab to the treatment regimen resulted in long-term uveitis control and maintenance of 20/70 vision in the right eye. We propose that NOD2-mediated inflammatory cascade can be activated by intraocular surgery and results in the manifestation of BS.

Posterior Segment Characterization in Children With Pierson Syndrome.

BACKGROUND AND OBJECTIVE: Pierson syndrome is a rare genetic disease defined by congenital nephrotic syndrome in association with microcoria. The authors aim to describe the posterior segment and retinal features in Pierson syndrome. PATIENTS AND METHODS: A retrospective chart review of nine patients diagnosed with Pierson syndrome was ascertained. Details of ophthalmic history, ocular examination, retinal imaging, and surgical interventions were obtained during a median duration of 17 months of follow-up (range: 6 to 60 months). Retinal interventions included scatter laser photocoagulation and surgical retinal repair. RESULTS: Sixteen eyes of nine patients were included. The axial length of five eyes with flat retina was 26.59 mm ± 0.99 mm. Highly myopic features including tessellated fundus with accompanying optic disc pallor, unidentifiable cup, and abnormal retinal vascular emanation from the disc were observed in all eyes (100%), whereas 12 eyes (75%) had parapapillary chorioretinal atrophy. Features of abnormal retinal vascularization included avascular peripheral retina on fluorescein angiography, aberrant course of the temporal arcades in 13 eyes (81.3%), and straightened nasal retinal blood vessels in 12 eyes (75%). Tortuous retinal blood vessels were observed in three eyes (18.75%). Surgical repair was performed in five out of seven eyes with rhegmatogenous retinal detachment (RRD). Recurrence was observed in all eyes, which required two to three procedures to achieve final reattachment. CONCLUSIONS: Combined features of high axial myopia with incomplete peripheral vascular maturation characterize the posterior segment in Pierson syndrome. Careful posterior segment examination is essential to detect RRD or retinal neovascularization. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:618-627.].

Foveal Congenital Simple Hamartoma of Retinal Pigment Epithelium: A Report of Two Cases.

We report two cases with foveal congenital simple hamartoma of the retinal pigment epithelium (CSHRPE), as both patients presented to our retina services complaining of a unilateral decreased vision. Full ophthalmic examination and multimodal imaging were performed including fundus photography, fundus autofluorescence, optical coherence tomography, fluorescein angiography, and electrophysiological testing. Both patients presented with 20/80 vision in the affected eyes. Foveal CSHRPE was found in both eyes, along with parapapillary hyperpigmented rim, multiple pinpoint macular lesions, and few posterior pole hyperpigmented lesions. Multifocal electroretinogram showed diminished central amplitude in both eyes, with three-dimensional topography map showing blunted foveal peaks in one eye and the absence of a central peak in the other patient. Both patients had a stable vision and clinical examination of the CSHRPE during 5 and 6 years follow up, respectively. Foveal CSHRPE is usually symptomatic and results in a decline in visual acuity. Follow-up of these patients showed stable vision and clinical examination.

Updated Systematic Review and Clinical Spectrum of Peripheral Exudative Hemorrhagic Chorioretinopathy.

Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a rare retinal vasculopathy that might cause subretinal and/or vitreous hemorrhages. Although the primary etiology is still unknown, choroidal neovascularization is mainly involved in the pathogenesis. The main risk factors are age and systemic hypertension. Ancillary testing such as fluorescein angiography, indocyanine green angiography and ultrasonography can be of great value for diagnosing this entity and distinguishing PEHCR from other lesions as choroidal melanoma and retinal vasoproliferative tumor. Various treatments have been reported including photocoagulation, cryotherapy, intravitreal injection of anti-vascular endothelial growth factor (Anti-VEGF) and surgical intervention as pars plana vitrectomy. This review handles an up-to-date perspective regarding PEHCR.

Long-term resolution of chronic macular edema after a single dose of intravitreal dexamethasone in familial retinal arterial macroaneurysm.

PURPOSE: To report a favorable effect of intravitreal dexamethasone implantation in Familial Retinal Arterial Macroaneurysms (FRAM). METHODS: Retrospective Case Report. RESULTS: A 32-year-old male who presented with bilateral retinal macroaneurysms. Whole Exome Sequencing (WES) revealed a homozygous c.830-1 G > A mutation in Insulin Growth Factor Binding Protein 7 (IGFBP7) gene, confirming the diagnosis FRAM. The left eye was lost in the course of the disease, whereas the right eye developed a persistent macular edema due to multiple leaking retinal arterial macroaneurysms and responded poorly to intravitreal ranibizumab and only partially to intravitreal aflibercept. Intravitreal dexamethasone implantation in the right eye, on the other hand, resulted in marked visual and structural improvement. CONCLUSION: Intravitreal dexamethasone injections have beneficial anatomical and visual outcomes in FRAM patients with persistent macular edema poorly responsive to intravitreal injections.

Successful surgical management of post-penetrating or deep lamellar keratoplasty Acquired Corneal Sub-Epithelial Hypertrophy (ACSH): A case series.

INTRODUCTION: Acquired Corneal Sub-Epithelial Hypertrophy (ACSH) has been described in patients with peripheral superficial corneal opacities following penetrating keratoplasty and might present similar to Salzmann's nodular degeneration (SND) or peripheral hypertrophic sub-epithelial corneal degeneration (PHSCD). We describe the clinical presentation, topographic findings and the surgical outcome of three cases, which fit the appearance and characteristics of ACSH. PRESENTATION OF CASES: Three patients (3 eyes) with paracentral or peripheral corneal opacification were reviewed to describe their clinical examination (SL), morphology of the opacity (depth, diameter and density) and document their topographic changes before and after surgical intervention by peeling of the epithelium with or without superficial keratectomy under the microscope in addition to brief description of their histopathological examination. DISCUSSION: All 3 cases were secondary to corneal procedures [Penetrating keratoplasty (PKP) in 1 for pseudophakic bullous keratopathy and deep anterior lamellar keratoplasty (DLK) in 2 for advanced keratoconus]. All cases presented with reduced vision, astigmatic changes in topography or manifest refraction. The visual acuity, symptoms, and topographical findings all improved after treatment. Histopathologically, all cases fit the newly described entity of ACSH. CONCLUSION: Careful clinical judgement guided by corneal topography are needed for proper the diagnosis of acquired corneal opacification that results in reduction of vision to identify ACSH from other similar conditions (PHSCD and SND). Peeling of the thickened epithelial and sub-epithelial tissue is curative avoiding the need for corneal re-grafting.

Intravitreal Triamcinolone Acetonide in Von Hippel-Lindau Patient with Novel Mutation.

von Hippel-Lindau (VHL) is a multisystemic inherited disease which most commonly affects the retina and central nervous system. The hallmark retinal manifestation of VHL in the eye is retinal capillary hemangioblastoma (RCH). Significant visual morbidity can result from exudative retinal detachments (ERDs) or tractional retinal detachments. Here, we present a 21-year-old male with long-standing poor vision in the right eye. On examination, he was found to have a massive ERD in the right eye with multiple RCH in both eyes. Genetic testing revealed a heterozygous (c.390dupT) mutation in the VHL gene. Intravitreal triamcinolone acetonide injection resulted in subretinal fluid absorption and near total resolution of ERD. Retinal flattening made RCH accessible for laser photocoagulation. Following multiple focal lasers to the RCH, the lesions were regressed with the flat retina and stable vision.

Ocular toxoplasmosis related macular traction: A case report and review of the literature.

This is a case of toxoplasmosis retinochoroiditis which has resulted in the formation of vitreomacular traction upon resolution which is rarely associated with ocular toxoplasmosis. A 39-year-old male came with an active toxoplasmosis retinochoroiditis. Best-corrected visual acuity, full ophthalmic slitlamp examination, colour fundus photography, spectral domain optical coherence tomography (SD-OCT), and fluorescein angiography were performed. Presumed ocular toxoplasmosis diagnosis was supported by serological tests. The patient was treated medically for 45 days and on his follow up he developed macular traction which was shown in SD-OCT with a good visual acuity. Vitreoretinal traction is a rare complication of ocular toxoplasmosis and ranges from mild to severe traction which might require surgery. We suggest a close follow up for patients with toxoplasmosis retinochoroiditis and early recognition could avoid exposing patients to surgery.

Perioperative Use of Rho-Kinase Inhibitors has Beneficial Effect on Corneal Endothelium after Phacoemulsification.

PURPOSE: Does perioperative use of Rho-Kinase (ROCK) inhibitors have beneficial effect on corneal endothelial cells after phacoemulsification? SETTING: This study was conducted at King Abdulaziz University Hospital in Riyadh. DESIGN: This was a prospective study assessing the effect of ROCK inhibitors on corneal endothelium after phacoemulsification. METHODOLOGY: Three patients have used ROCK inhibitor 1 day before and 1 week after phacoemulsification surgery, and specular microscopy and Pentacam were done preoperatively and 3 months postoperatively. RESULTS: Endothelial cell density decreased to 11.3%, 9.45%, and 4.09% in eyes with ROCK inhibitors and 23.9% in one eye without ROCK inhibitor. CONCLUSION: Perioperative ROCK inhibitor use has a possible protective effect on corneal endothelium.

Transition to Ophthalmology Residency Program and the Role of Basic Science Course.

BACKGROUND: Residency training is a difficult and a highly stressful task, especially at the beginning of the training. The aim of this study is to assess the roles of the Basic Science Course (BSC) and independent preparation on the transition to ophthalmology residency in Saudi Arabia. METHODOLOGY: A cross-sectional study was conducted among ophthalmology program residents. The questionnaire was administered to 100 residents. Responses were analyzed for association using univariate analysis. RESULTS: Nearly 88% of residents were satisfied with their knowledge exposure during the BSC, while 67% thought that the majority of the BSC was dedicated to theoretical lectures. Surgical exposure was limited to attending surgeries for 38% of residents before joining residency programs, and 91% thought that there was little time dedicated for hands-on surgical skills training during the BSC, while only 11% thought that their surgical exposure was beneficial. Almost half of the beginning residents were accompanied by senior colleagues during their initial calls. More than 50% of residents felt that their transition to ophthalmology residency was more stressful than the transition to internship. CONCLUSION: The knowledge exposure from the BSC was helpful to facilitate the transition to ophthalmology residency program, especially for the ones who prepared well before. Improvement in surgical skills training should be aimed to further facilitate transition to ophthalmology residency program.