Surgical Management of Hemorrhagic Retinal Detachment Secondary to Peripheral Exudative Hemorrhagic Chorioretinopathy.

The purpose of the study is to report a case of peripheral exudative hemorrhagic chorioretinopathy (PEHCR), managed surgically with favorable visual outcome. A 66-year-old female presented with painless visual loss due to dense vitreous and subretinal hemorrhage extending from the far periphery to the macula. Pars plana vitrectomy (PPV) with subretinal tissue plasminogen activator (TPA) injection was performed resulting in good anatomical and visual outcome. PEHCR can present with severe visual loss. Surgical management with PPV and subretinal TPA injection might result in favorable anatomical and visual outcome.

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.

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.].

Comparison of outcomes of four different treatment modalities for diabetic vitreous haemorrhage.

We compared outcomes of four different management modalities for diabetic VH. Patients with diabetic VH were identified in this retrospective study undertaken at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. Eyes were grouped based on the treatment received: control (observation only), intravitreal bevacizumab (IVB) injection(s), pars plana vitrectomy (PPV), and preoperative single IVB injection before PPV. Best-corrected visual acuity (BCVA) and status of VH were noted at baseline and the last follow up (Minimum: 6 months, maximum: 29 months). The proportion of eyes with Snellen BCVA improvement by two lines or more and VH clearance at the last follow up were compared between groups. The four groups - Control, IVB, PPV, and IVB-before-PPV had 23, 29, 17, and 20 eyes, respectively. The proportion of eyes gaining ≥2 lines was substantially higher in the IVB-before-PPV and PPV groups (90% and 77%, respectively) compared with IVB and observation groups (41% and 22%, respectively). Surgical treatment was associated with a 2.38 times higher likelihood of gaining ≥2 lines than the non-surgical one (incidence ratio: 2.38, 95% CI 1.19, 4.78 P = 0.015) after adjusting for age, hyperglycemia and BCVA at presentation. Less invasive treatment such as IVB injections did not result in the same amount of improvement in vision as did PPV. Prospective randomized studies are needed to better define the role of IVB injections in the management of diabetic VH.

Retinal capillary hemangioblastoma and hemiretinal vein occlusion in a patient with primary congenital glaucoma: A case report.

The presence of retinal capillary hemangioblastoma and cerebellar hemangioblastoma in the context of Von Hippel-Lindau syndrome (VHL) is not characteristically associated with other ophthalmologic conditions. Here, we report the case of a 22-yearold female with a history of bilateral primary congenital glaucoma who presented with a right juxtapapillary retinal capillary hemangioblastoma and an old hemiretinal vein occlusion in which the retinal capillary hemangioblastoma was likely the contributing factor. Her systemic work up was positive for VHL syndrome and revealed the presence of a fatal large brainstem hemangioblastoma. To our knowledge, the association of VHL and congenital glaucoma and/or retinal venous occlusion has not been reported.

Long-term follow-up of retinal function and structure in TRPM1-associated complete congenital stationary night blindness.

Purpose: TRPM1-associated congenital stationary night blindness (CSNB) is characterized by nystagmus and high myopia. We assessed retinal function and structure over long-term follow-up up to 10 years in two siblings from a family with the homozygous deletion c.2394delC in exon 18 that we previously identified. In addition, we describe retinal function and structure in two other siblings with the novel homozygous c.1394T>A (p.Met465Lys) missense mutation. Methods: Clinical examination included full-field electroretinography, axial length measurements, and multimodal retinal imaging. Molecular genetic tests included next-generation sequencing and Sanger sequencing. Results: All patients had non-recordable rod responses and electronegative configuration of the rod-cone responses at presentation. There was a median of 26% reduction in the dark- and light-adapted electroretinographic (ERG) amplitudes over 4 years. Myopia progressed rapidly in childhood but showed only a mild progression after the teenage years. Visual acuities were stable over time, and there was no sign of progressive retinal thinning. All patients had axial myopia. A novel homozygous c.1394T>A (p.Met465Lys) missense mutation in TRPM1 was identified in two siblings. Conclusions: Further prospective study in larger samples is needed to establish whether there is progressive retinal degeneration in TRPM1-associated CSNB. The associated myopia was found to be mainly axial, which has not been described previously. The mechanism of myopia development in this condition remains incompletely understood; however, it may be related to altered retinal dopamine signaling and amacrine cell dysfunction.

Serous retinal detachment after panretinal photocoagulation for proliferative diabetic retinopathy: a case report.

BACKGROUND: Proliferative diabetic retinopathy is a major cause of visual impairment in working-age adults worldwide. Panretinal photocoagulation is a cornerstone in its management; however, it may include a range of side effects and complications, one of these being serous retinal detachment. To the best of our knowledge, this is the first report of the use of intravitreal injection of bevacizumab for serous retinal detachment after panretinal photocoagulation. CASE PRESENTATION: A 24-year-old Saudi man with poorly controlled type 1 diabetes presented with bilateral progressive proliferative retinopathy in spite of several sessions of panretinal photocoagulation. After one additional such session, he developed bilateral serous retinal detachment and vision loss, which was managed with a single bilateral intravitreal bevacizumab injection. The serous retinal detachment subsided with partial recovery of vision. CONCLUSIONS: Serous retinal detachment after panretinal photocoagulation for proliferative diabetic retinopathy is a rare complication nowadays. In this case, it seems that excessive photocoagulation exceeded the energy-absorbing capacity of the retinal pigment epithelium, leading to a disruption of the blood-retinal barrier. A single injection of bilateral intravitreal bevacizumab was sufficient to control the serous retinal detachment. This effect may have been due to a reduction of vascular leakage resulting from the mechanism of action of this drug. No complications were noted from the injection. Caution should be exerted when attempting bilateral panretinal photocoagulation.

Update on Clinical Trials in Dry Age-related Macular Degeneration.

This review article summarizes the most recent clinical trials for dry age-related macular degeneration (AMD), the most common cause of vision loss in the elderly in developed countries. A literature search through websites https://www.pubmed.org and https://www.clinicaltrials.gov/, both accessed no later than November 04, 2015, was performed. We identified three Phase III clinical trials that were completed over the recent 5 years Age-Related Eye Disease Study 2 (AREDS2), implantable miniature telescope and tandospirone, and several other trials targeting a variety of mechanisms including, oxidative stress, complement inhibition, visual cycle inhibition, retinal and choroidal blood flow, stem cells, gene therapy, and visual rehabilitation. To date, none of the biologically oriented therapies have resulted in improved vision. Vision improvement was reported with an implantable mini telescope. Stem cells therapy holds a potential for vision improvement. The AREDS2 formulas did not add any further reduced risk of progression to advanced AMD, compared to the original AREDS formula. Several recently discovered pathogenetic mechanisms in dry AMD have enabled development of new treatment strategies, and several of these have been tested in recent clinical trials and are currently being tested in ongoing trials. The rapid development and understanding of pathogenesis holds promise for the future.

Trimethoprim/Sulfamethoxazole and azithromycin combination therapy for ocular toxoplasmosis.

PURPOSE: To evaluate the efficacy and safety of trimethoprim/sulfamethoxazole and azithromycin combination for the treatment of ocular toxoplasmosis. METHODS: Nineteen ocular toxoplasmosis patients treated with trimethoprim/sulfamethoxazole and azithromycin +/- corticosteroid combination were retrospectively reviewed. Demographic data, clinical findings, the time interval until resolution of inflammation, recurrences, and drug side effects were collected. RESULTS: The mean follow-up time of the patients was 25.0 +/- 22.5 (range; 6 -66) months. Final visual acuity improved with a mean of 6 +/- 4 lines in 15 patients (78.9%). Inflammatory findings began to subside within 14.8 +/- 10.0 days. Three patients (15.8%) had recurrent attack. Only 1 patient (5.3%) had side effects from therapy. CONCLUSIONS: Trimethoprim/sulfamethoxazole and azithromycin combination is an effective and safe treatment modality for the treatment of ocular toxoplasmosis.

An orthotopic model for human uveal melanoma in SCID mice.

The microcirculation of primary uveal melanomas, their precursors, and their metastases is distinctive. Medium-sized and even large primary uveal melanomas typically lack significant zones of necrosis, suggesting that either these tumors are relatively well perfused or they are capable of growth in a severely blood-deprived microenvironment. In addition to normal choroidal vessels that are incorporated into nevi and most primary uveal melanomas, aggressive primary and metastatic uveal melanomas tend to contain patterns of extracellular matrix that surround spheroidal or cylindrical packets of tumor cells. Some components of this branching, looping, and interconnected system of matrix may be perfused. It is now known that the generation of this patterning is a characteristic of genetically dysregulated melanoma cells (nonaggressive tumor cells do not form these patterns and melanomas lacking branching, looping, or interconnected matrix patterns tend to follow a relatively indolent course). We developed an orthotopic model of an aggressive human uveal melanoma by injecting suspensions of the primary human choroidal melanoma cell line (OCM1) into the subretinal space of one eye of 20 SCID mice. All mice were examined daily for tumor growth and tumors developed in every eye within 3 weeks of injection. The tumors were characterized by extraocular extension and the development of looping matrix patterns characteristic of those seen in aggressive human uveal melanoma. As in human uveal melanomas, these patterns were perfused by blood in areas. The orthotopic injection of human uveal melanoma cells into the SCID mouse eye generates a model reproducing the matrix-associated microcirculatory patterns of aggressive primary human uveal melanomas. This model can be used to explore the molecular pathogenesis and modulation of this novel circulation in vivo, to facilitate our understanding of the blood flow to these tumors providing insight into perfusion and drug delivery, to enable testing of pharmacologic modulation of pattern formation and intratumoral blood flow, and to refine noninvasive methods such as confocal scanning laser ophthalmoscopy to detect the presence of these patterns by which ophthalmologists might assess the biological behavior of tumors as noninvasive substitute for biopsy.

Long-term therapy with low dose cyclosporin A in ocular Behçet's disease.

Ocular complications of Behçet's disease can be severe and lead to blindness in 90% of untreated patients. We aimed to evaluate the long-term use of low dose cyclosporin-A (CsA) which is a potent immunomodulatory agent in the treatment of ocular Behçet's disease. Fifty-two patients (104 eyes) with ocular Behçet's disease using CsA for at least 1 year were included in this study. All the patients underwent complete ophthalmological and systemic examination. Five mg/kg/per day CsA was started to the patients with severe posterior uveitis and/or frequent anterior inflammatory attacks unresponsive to the conventional therapeutic agents. According to ocular response and adverse effects, the dose was tapered gradually over 2 months to a maintenance dose of 3 mg/kg/per day. Prednisone (0.2-0.8 mg/kg/per day) was added when necessary. Forty-six of the patients (88.5%) were males and six (11.5%) were females. The mean age was 33.65 +/- 7.75 (range, 19-53) years. The mean ocular involvement period was 64.1 (range, 12-180) months. Posterior uveitis was present in 49 (94.21%) and severe, recurrent anterior uveitis in three (5.8%) patients. The mean CsA administration period was 38 +/- 18.1 months. Visual acuity improved in 31(29.8%), deteriorated in 32 (30.8%) and unchanged in 41(39.4%) of the 104 eyes. No ocular attacks occurred in 50% of the eyes during therapy. Nine (17.3%) of the patients had to stop the therapy because of the adverse effects of the CsA and the others tolerated well for a long-term period. CsA is not the ideal therapeutic agent in ocular Behçet's disease because it can not completely eliminate the disease, but it is currently one of the most effective and efficient drug to control the uveitis and its complications until better treatment modalities are developed.