Retinal arteriolar macroaneurysms with supravalvular pulmonic stenosis in the United Arab Emirates.

PURPOSE: Retinal arteriolar macroaneurysms with supravalvular pulmonic stenosis (RAMSVPS) is a rare syndrome that to date has only been reported in Saudi Arabian families. All tested patients have been homozygous for a single IGFBP7 splice variant (NM_001553.2:c.830-1G>A). We report our experience with RAMSVPS in the United Arab Emirates. METHODS: Retrospective case series. RESULTS: Five affected individuals (two males and three females) from two unrelated Emirati families were known to our institution (age of first signs 6 months to 10 years of age, with one asymptomatic 6-year-old boy identified by sibling screening examination). Initial ophthalmic diagnoses had been Coats disease or traumatic retinal bleeding. Characteristic retinal arteriolar trunk beading and macroaneurysms led to the actual diagnosis of RAMSVPS. One child with esotropia at 6 months of age seemed to have unilateral Coats disease until retinal signs became apparent in the contralateral eye at 4 years old. One family consented to genetic testing, and both affected siblings were homozygous for the Saudi IGFBP7 splice variant (c.830-1G>A). The three children who underwent echocardiography were all confirmed to have cardiac valvular abnormalities (two supravalvular pulmonic stenosis and one tricuspid stenosis). DISCUSSION: The distinct ophthalmic phenotype of RAMSVPS is important to recognize because of systemic implications. Retinal findings can be misinterpreted as sequelae of trauma or Coats disease and can seem unilateral in very young children until changes in the contralateral eye become apparent years later. The homozygous IGFBP7 splice variant associated with the disease likely represents an ancestral founder effect for the Arabian Peninsula.

Adjunctive Intravitreal Triamcinolone Acetonide for Exudative Retinal Detachment in Coats Disease.

Purpose: This work aims to assess the value of intravitreal triamcinolone acetonide (IVTA) as an adjunctive therapy in advanced Coats disease with exudative retinal detachment (ERD). Methods: A retrospective review was conducted of patients with Coats disease stage 3 or higher who received IVTA to decrease subretinal fluid (SRF), facilitate retinal ablative therapy, and avoid surgical drainage. Primary outcomes were SRF resolution and avoidance of surgical SRF drainage. Results: Seventeen eyes of 17 patients (mean, [SD] age, 3.9 [3.4] years) met the inclusion criteria. ERD configuration was bullous in 7 and shallow in 10 eyes. Following a single IVTA injection, ablative therapy was achieved after a mean (SD) of 2.1 (3.0) weeks. Complete SRF resolution was observed in 13 eyes (76.4%) after a mean of 1.3 IVTA injections and a mean of 2 (SD, 1.27) laser sessions, and none of these eyes required SRF drainage up to last follow-up (mean [SD], 50.5 [26.24] months). In 4 eyes with bullous ERD at presentation, SRF persisted (P = .015) despite additional measures including surgical drainage. Final visual acuity ranged from 20/100 to no light perception. Cataract developed in 12 of the 17 eyes (70.5%). None developed an increase in intraocular pressure at final follow-up. Conclusions: IVTA injection can be a helpful adjunctive modality to address SRF in advanced Coats disease. It may obviate the need to surgically drain SRF to effectively treat the condition, particularly when the ERD is not highly bullous.

Wide-field optical coherence tomography angiography for the detection of proliferative diabetic retinopathy.

PURPOSE: To compare the ability of wide-field optical coherence tomography angiography (WF-OCTA) to that of ultra-wide field fluorescein angiography (UWF-FA) and ultra-wide-field color fundus photography (UWF-CP) to detect retinal neovascularization (NV) in eyes with proliferative diabetic retinopathy (PDR). METHODS: In this cross-sectional study, naïve patients with active PDR underwent UWF-FA and UWF-CP using the Optos 200Tx and WF-OCTA with 12 × 12 mm fields of five visual fixations using the PLEX Elite 9000. NV was defined on OCTA when the co-registered B-scan with flow overlay of the vitreoretinal interface (VRI) segmentation showed extraretinal proliferation. Three masked readers examined the UWF-FA, UWF-CP, and WF-OCTA independently for the presence of NV. Statistical analysis was performed to compare the diagnostic accuracy of the 3 wide-field imaging modalities using OCT B-scan as the reference standard. RESULTS: In 82 eyes with PDR, neovascularization of the disc (NVD) was detected in 13 eyes by UWF-CP, 35 eyes with UWF-FA, and 37 eyes with OCTA using the VRI slab. Upon review of the 2500 OCT B-scans with superimposed flow overlay of each eye, NVD was confirmed in 37 eyes. The sensitivity and specificity of NVD detection were 35.1% and 97.8%, respectively for UWF-CP; 94.6% and 100%, respectively, for UWF-FA; and 100% and 100% for WF-OCTA. One hundred ninety-six foci of neovascularization elsewhere (NVE) were identified with the OCT B-scan with superimposed flow overlay. UWF-CP analysis was able to detect 62 foci of NVE of the 196 confirmed by B-scan (31.6% detection rate). An additional 11 foci of NVE seen on UWF-CP were not confirmed by B-Scan (15% false positive rate). There were 182 foci of NVE identified by UWF-FA (detection rate 91.3%), while WF-OCTA detected 196 retinal NVEs (detection rate 100%). The rate of false positives for both UWF-FA and WF-OCTA was low (< 2%). CONCLUSION: WF-OCTA can identify NV that is not evident in UWF-CP and represents a faster and safer alternative to UWF-FA for surveillance of PDR with comparable diagnostic accuracy.

Inner retinal toxicity due to silicone oil: a case series and review of the literature.

PURPOSE: Cases of sudden loss of central vision in eyes with silicone oil in situ and after oil removal have been described. The aim of this review is to present current data on silicone oil toxicity to the neuronal aspects of the retina as well as the Cleveland Clinic Abu Dhabi experience with this retinopathy (maculopathy). METHODS: A PubMed review using the terms "silicon oil" and/or "toxicity" and/or "ganglion cell" and/or "nerve fiber" was conducted to identify case reports, case series, and original articles presenting toxicity from silicon oil. Timing of visual loss, as well as SD-OCT data and RNFL/GCC analysis, was collected. Selected cases were pooled from Cleveland Clinic Abu Dhabi Vitreoretinal database to further reinforce the findings. RESULTS: Twenty-four papers were identified (case/series/articles). The earliest papers that met our criteria seemed to report vision loss at the time of or after silicone oil removal; however, more recent studies have described such toxicity from 1 to 6 months after tamponade with silicone oil in situ. Since the first description of central visual loss from silicone oil, all researchers describe a thinning of perifoveal ganglion cells measured with SD-OCT, while there is no concordance as far as RNFL changes, with some authors describing a thinning and others a thickening, but neither was ever clearly associated with visual loss. The correlation between SD-OCT hyperreflective goblets in the inner retina and histological description of intraretinal oil droplets migration seems to suggest instances of silicone oil penetration in the retinal layers. CONCLUSION: In a small percentage of cases who underwent retinal tamponade with silicone oil, ganglion cells can suffer a direct damage either from particles of oil that migrate in the retina or from direct contact with it. Indirect damage may be caused by phototoxicity due to the transparent nature of silicon oil or by inflammatory damage from cytokines sandwiched between oil and retina.

Phenotypic delineation of the retinal arterial macroaneurysms with supravalvular pulmonic stenosis syndrome.

Retinal arterial macroaneurysms with supravalvular pulmonic stenosis (RAMSVPS), also known as Familial Retinal Arterial Macroaneurysms (FRAM) syndrome, is a very rare multisystem disorder. Here, we present a case series comprising ophthalmologic and systemic evaluation of patients homozygous for RAMSVPS syndrome causative IGFBP7 variant. New clinical details on 22 previously published and 8 previously unpublished patients are described. Age at first presentation ranged from 1 to 34 years. The classical feature of macroaneurysms and vascular beading involving the retinal arteries was universal. Follow up extending up to 14 years after initial diagnosis revealed recurrent episodes of bleeding and leakage from macroaneurysms in 55% and 59% of patients, respectively. The majority of patients who underwent echocardiography (18/23) showed evidence of heart involvement, most characteristically pulmonary (valvular or supravalvular) stenosis, often requiring surgical correction (12/18). Four patients died in the course of the study from complications of pulmonary stenosis, cerebral hemorrhage, and cardiac complications. Liver involvement (usually cirrhosis) was observed in eight patients. Cerebral vascular involvement was observed in one patient, and stroke was observed in two. We conclude that RAMSVPS is a recognizable syndrome characterized by a high burden of ocular and systemic morbidity, and risk of premature death. Recommendations are proposed for early detection and management of these complications.

Spare some internal limiting membrane for later: free ILM patch and neurosensory retina graft.

PURPOSE: Editorial to De Giacinto et al case report on free autologous neurosensory retina patch. METHODS: Literature review and experts' opinion RESULTS: In the present issue, De Giacinto et al describe a free autologous neurosensory retina patch to close a chronic macular hole. This new technique was made necessary by an extended internal limiting membrane peeling during the first surgery, that prevented grafting a patch of internal limiting membrane when the hole did not close. We hereby review pros and cons of patching a chronic macular hole with an internal limiting membrane patch, as well as the importance of not over-enlarging a peeling. DISCUSSION: Internal limiting membrane patch can be considered in chronic macular holes. It may not be an option in cases of over-enlargement of a previous peel; free autologous neurosensory retina patch may be a valid alternative in such cases.

Cilioretinal artery hypoperfusion and its association with paracentral acute middle maculopathy.

BACKGROUND/AIMS: To study the multimodal imaging findings of a large series of eyes with cilioretinal artery obstruction (CILRAO) and describe the systemic associations. METHODS: Multicentre, retrospective chart review from 12 different retina clinics worldwide of eyes with CILRAO, defined as acute retinal whitening in the distribution of the cilioretinal artery, were identified. The clinical, systemic information and multimodal retinal imaging findings were collected and analysed. RESULTS: A total of 53 eyes of 53 patients with CILRAO were included in the study. In 100% of eyes, fundus photography illustrated deep retinal whitening corresponding to the course of the cilioretinal artery. Twenty-eight patients (52.8%) presented with isolated CILRAO (baseline best-corrected visual acuity (BCVA) 20/50, final BCVA 20/25) associated with nocturnal hypotension, 23 patients (43.4%) with CILRAO secondary to central retinal vein occlusion (CRVO) (baseline BCVA 20/40, final BCVA 20/20) and two patients with CILRAO due to biopsy-proven giant cell arteritis (GCA) (baseline BCVA 20/175, final BCVA 20/75). With spectral domain optical coherence tomography (SD-OCT), a hyper-reflective band involving the inner nuclear layer (ie, paracentral acute middle maculopathy or PAMM) was noted in 51 eyes (28/28 eyes with isolated CILRAO and 23/23 eyes with CILRAO+CRVO) corresponding to the retinal whitening. In the two eyes with CILRAO+GCA, SD-OCT illustrated hyper-reflective ischaemia of both the middle and inner retina. CONCLUSIONS: Isolated CILRAO and CILRAO secondary to CRVO are the result of hypoperfusion or insufficiency, rather than occlusion, of the cilioretinal artery and are associated with PAMM or selective infarction of the the inner nuclear layer. With GCA, there is complete occlusion of the cilioretinal artery producing ischaemia involving both the middle and inner retina associated with worse visual outcomes.

Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain.

Importance: Congenital retinal macrovessel (CRM) is a rarely reported venous malformation of the retina that is associated with venous anomalies of the brain. Objective: To study the multimodal imaging findings of a series of eyes with congenital retinal macrovessel and describe the systemic associations. Design, Setting, and Participants: In this cross-sectional multicenter study, medical records were retrospectively reviewed from 7 different retina clinics worldwide over a 10-year period (2007-2017). Patients with CRM, defined as an abnormal, large, macular vessel with a vascular distribution above and below the horizontal raphe, were identified. Data were analyzed from December 2016 to August 2017. Main Outcomes and Measures: Clinical information and multimodal retinal imaging findings were collected and studied. Pertinent systemic information, including brain magnetic resonance imaging findings, was also noted if available. Results: Of the 49 included patients, 32 (65%) were female, and the mean (SD) age at onset was 44.0 (20.9) years. A total of 49 eyes from 49 patients were studied. Macrovessel was unilateral in all patients. Color fundus photography illustrated a large aberrant dilated and tortuous retinal vein in all patients. Early-phase frames of fluorescein angiography further confirmed the venous nature of the macrovessel in 40 of 40 eyes. Optical coherence tomography angiography, available in 17 eyes (35%), displayed microvascular capillary abnormalities around the CRM, which were more evident in the deep capillary plexus. Of the 49 patients with CRM, 39 (80%) did not illustrate any evidence of ophthalmic complications. Ten patients (20%) presented with retinal complications, typically an incidental association with CRM. Twelve patients (24%) were noted to have venous malformations of the brain with associated magnetic resonance imaging. Of these, location of the venous anomaly in the brain was ipsilateral to the CRM in 10 patients (83%) and contralateral in 2 patients (17%), mainly located in the frontal lobe in 9 patients (75%). Conclusions and Relevance: Our study has identified an association between macrovessels in the retina and venous anomalies of the brain (24% compared with 0.2% to 6.0% in the normal population). Thus, we recommend new guidelines for the systemic workup of patients with CRM to include brain magnetic resonance imaging with contrast. These lesions may be more accurately referred to as retinal venous malformations, which may raise awareness regarding potential cerebral associations.

Fundus autofluorescence imaging in hereditary retinal diseases.

Fundus autofluorescence (FAF) is a non-invasive retinal imaging modality used in clinical practice to non-invasively map changes at the level of the retinal pigment epithelium (RPE)/photoreceptor complex and alterations of macular pigment distribution. This imaging method is based on the visualization of intrinsic fluorophores and may be easily and rapidly used in routine patient care. Excessive accumulation of lipofuscin granules in the lysosomal compartment of RPE cells represents a common downstream pathogenic pathway in various hereditary and complex retinal diseases. The clinical applications of FAF continue to expand. It is now an essential tool for evaluating macular dystrophies and various hereditary retinal disorders. Fundus autofluorescence (FAF) may detect abnormalities beyond those detected on funduscopic examination, fluorescein angiography (FA) or optical coherence tomography (OCT). Fundus autofluorescence (FAF) imaging is particularly helpful for differential diagnosis, detection and extent delineation of involved retinal areas, genotype-phenotype correlations and monitoring of changes overtime. Given its ease of use, non-invasive nature and value in characterizing retinal disease, FAF enjoys increasing clinical relevance. This review summarizes basic principles and FAF findings in various hereditary retinal diseases.

OUTCOMES OF PARS PLANA VITRECTOMY IN THE MANAGEMENT OF REFRACTORY AQUEOUS MISDIRECTION SYNDROME.

PURPOSE: To determine the efficacy and complications of pars plana vitrectomy (PPV) and adjunct surgeries for aqueous misdirection refractory to medical therapy. METHODS: A retrospective review of consecutive eyes with refractory aqueous misdirection at the King Khaled Eye Specialist Hospital between 2002 and 2010. Patients underwent two-port and three-port pars plana vitrectomy (PPV) with adjunct procedures including pars plana lensectomy combined with posterior capsulectomy, hyaloido-zonulo-iridectomy, and synechiolysis. Main outcome measures included anatomical success, functional success, and factors associated with the outcomes. RESULTS: Sixty-nine eyes were evaluated over a mean follow-up period of 17.6 ± 3.8 months (3-156 months). Anatomical success was achieved in 62 eyes (90%) and functional success in 54 eyes (78%) that underwent PPV as a primary surgery. The factors associated with the altering misdirected aqueous flow and reducing intraocular pressure significantly associated with a two-line improvement of best-corrected visual acuity included surgical treatment within 4 weeks of presentation (P = 0.004) and preoperative intraocular pressure (P = 0.001). The success of two-port PPV and standard three-port PPV was similar (P = 0.7). The intraoperative and postoperative complications included retinal detachment in two eyes and endophthalmitis in one eye. CONCLUSION: The PPV was effective for managing aqueous misdirection refractory to medical therapy. Two-port or three-port PPV did not change the success rate but early surgery improved both anatomical and functional outcomes.

The outcomes and prognostic factors of vitrectomy in chronic diabetic traction macular detachment.

PURPOSE: To investigate the outcomes of pars plana vitrectomy (PPV) for chronic diabetic traction macular detachment (CTMD). METHODS: Ninety-six eyes that underwent PPV for CTMD of at least 6 months duration were retrospectively analyzed. Retinal reattachment rate, final vision, and prognostic factors for poor visual outcome were the main outcome measures. RESULTS: All eyes had long-standing TMD (median 12, range: 6-70 months). The median postoperative follow-up was 15 (range: 3-65) months. Eighty-seven eyes (90.6%) had their retina and macula reattached after one PPV. At final examination, 84 eyes (87.5%) had stable vision or at least one line improvement, and three had no light perception. Seventeen (17.7%) and 41 (43%) eyes had preoperative visual acuity of ≥20/200 and ≥5/200 as compared to 40 (41.6%; P=0.0005) and 64 (66.7%; P=0.0014) eyes at final follow-up, respectively. Age >50 years (Odds ratio [OR] =5.84, 95% confidence interval [CI] =1.53-22.19, P=0.01), preoperative vision <20/400 (OR =7.012, 95% CI =1.82-26.93, P=0.005), and ischemic macula (OR =14.13, 95% CI =3.61-55.33, P<0.001) were significantly associated with final vision <20/400. CONCLUSION: PPV for CTMD may be beneficial particularly in patients who are relatively younger and have good baseline vision and no macular ischemia.

Treatment of retinitis pigmentosa due to MERTK mutations by ocular subretinal injection of adeno-associated virus gene vector: results of a phase I trial.

MERTK is an essential component of the signaling network that controls phagocytosis in retinal pigment epithelium (RPE), the loss of which results in photoreceptor degeneration. Previous proof-of-concept studies have demonstrated the efficacy of gene therapy using human MERTK (hMERTK) packaged into adeno-associated virus (AAV2) in treating RCS rats and mice with MERTK deficiency. The purpose of this study was to assess the safety of gene transfer via subretinal administration of rAAV2-VMD2-hMERTK in subjects with MERTK-associated retinitis pigmentosa (RP). After a preclinical phase confirming the safety of the study vector in monkeys, six patients (aged 14 to 54, mean 33.3 years) with MERTK-related RP and baseline visual acuity (VA) ranging from 20/50 to <20/6400 were entered in a phase I open-label, dose-escalation trial. One eye of each patient (the worse-seeing eye in five subjects) received a submacular injection of the viral vector, first at a dose of 150 µl (5.96 × 10(10)vg; 2 patients) and then 450 µl (17.88 × 10(10)vg; 4 patients). Patients were followed daily for 10 days at 30, 60, 90, 180, 270, 365, 540, and 730 days post-injection. Collected data included (1) full ophthalmologic examination including best-corrected VA, intraocular pressure, color fundus photographs, macular spectral domain optical coherence tomography and full-field stimulus threshold test (FST) in both the study and fellow eyes; (2) systemic safety data including CBC, liver and kidney function tests, coagulation profiles, urine analysis, AAV antibody titers, peripheral blood PCR and ASR measurement; and (3) listing of ophthalmological or systemic adverse effects. All patients completed the 2-year follow-up. Subretinal injection of rAAV2-VMD2-hMERTK was associated with acceptable ocular and systemic safety profiles based on 2-year follow-up. None of the patients developed complications that could be attributed to the gene vector with certainty. Postoperatively, one patient developed filamentary keratitis, and two patients developed progressive cataract. Of these two patients, one also developed transient subfoveal fluid after the injection as well as monocular oscillopsia. Two patients developed a rise in AAV antibodies, but neither patient was positive for rAAV vector genomes via PCR. Three patients also displayed measurable improved visual acuity in the treated eye following surgery, although the improvement was lost by 2 years in two of these patients. Gene therapy for MERTK-related RP using careful subretinal injection of rAAV2-VMD2-hMERTK is not associated with major side effects and may result in clinical improvement in a subset of patients.

Exome-based case-control association study using extreme phenotype design reveals novel candidates with protective effect in diabetic retinopathy.

UNLABELLED: Diabetic retinopathy (DR) is a common clinical expression of diabetes mellitus-induced vasculopathy and is a major cause of vision loss. Significant gaps remain in our understanding of the molecular pathoetiology of DR, and it is hoped that human genetic approaches can reveal novel targets especially since DR is a heritable trait. Previous studies have focused on genetic risk factors of DR but their results have been mixed. In this study, we hypothesized that the use of the extreme phenotype design will increase the power of a genomewide search for "protective" genetic variants. We enrolled a small yet atypical cohort of 43 diabetics who did not develop DR a decade or more after diagnosis (cases), and 64 diabetics with DR (controls), all of similar ethnic background (Saudi). Whole-exome sequencing of the entire cohort was followed by statistical analysis employing combined multivariate and collapsing methods at the gene level, to identify genes that are enriched for rare variants in cases vs. CONTROLS: Three genes (NME3, LOC728699, and FASTK) reached gene-based genome-wide significance at the 10(-08) threshold (p value = 1.55 × 10(-10), 6.23 × 10(-10), 3.21 × 10(-08), respectively). Our results reveal novel candidate genes whose increased rare variant burden appears to protect against DR, thus highlighting them as attractive candidate targets, if replicated by future studies, for the treatment and prevention of DR. Extreme phenotype design when implemented in sequencing-based genome-wide case-control studies has the potential to reveal novel candidates with a smaller cohort size compared to standard study designs.

Expanding the clinical, allelic, and locus heterogeneity of retinal dystrophies.

PURPOSE: Retinal dystrophies (RD) are heterogeneous hereditary disorders of the retina that are usually progressive in nature. The aim of this study was to clinically and molecularly characterize a large cohort of RD patients. METHODS: We have developed a next-generation sequencing assay that allows known RD genes to be sequenced simultaneously. We also performed mapping studies and exome sequencing on familial and on syndromic RD patients who tested negative on the panel. RESULTS: Our panel identified the likely causal mutation in >60% of the 292 RD families tested. Mapping studies on all 162 familial RD patients who tested negative on the panel identified two novel disease loci on Chr2:25,550,180-28,794,007 and Chr16:59,225,000-72,511,000. Whole-exome sequencing revealed the likely candidate as AGBL5 and CDH16, respectively. We also performed exome sequencing on negative syndromic RD cases and identified a novel homozygous truncating mutation in GNS in a family with the novel combination of mucopolysaccharidosis and RD. Moreover, we identified a homozygous truncating mutation in DNAJC17 in a family with an apparently novel syndrome of retinitis pigmentosa and hypogammaglobulinemia. CONCLUSION: Our study expands the clinical and allelic spectrum of known RD genes, and reveals AGBL5, CDH16, and DNAJC17 as novel disease candidates.Genet Med 18 6, 554-562.

Full-Thickness Macular Hole Secondary to High-Power Handheld Blue Laser: Natural History and Management Outcomes.

PURPOSE: To report the natural history and management outcomes of full-thickness macular hole (MH) caused by momentary exposure to a high-power handheld blue laser device and highlight the dangers of such easily available devices. DESIGN: Retrospective consecutive case series. METHODS: A chart review of all patients presenting with full-thickness MH from exposure to blue-light high-powered lasers from January 2012 to May 2014 at 2 institutions was performed. Evaluation included a full ophthalmic examination, fundus photography, macular spectral-domain optical coherence tomography, and fundus fluorescein angiography. The main and secondary outcomes were MH closure and final visual acuity, respectively. RESULTS: There were 17 eyes of 17 patients with full-thickness MH. Best-corrected Snellen visual acuity (BCVA) at presentation ranged from 20/30 to 2/200 (mean: 20/210). The MH minimum diameter ranged from 168 µm to 620 µm (mean: 351 µm). Fourteen eyes underwent pars plana vitrectomy, internal limiting membrane peeling, and gas or silicone oil tamponade. Eleven of the 14 (78.6%) operated eyes had complete closure of the macular hole. Of the 3 unoperated eyes, only 1 eye with the smallest macular hole (minimum diameter: 168 µm) closed spontaneously with observation. Final BCVA in all cases had a mean of 20/62 (range: 20/20-4/200). CONCLUSION: Full-thickness MH can result from momentary exposure to high-power handheld laser devices. While spontaneous closure may occur in rare cases, most cases require early surgical intervention. Vitrectomy may be successful in closing the macular hole with visual acuity improvement in most of the cases.

Nine years of retained perfluoro-n-octane in the anterior chamber after retinal detachment repair with no signs of ocular toxicity.

Perfluoro-n-octane (PFO) is commonly used in vitreoretinal surgery. In this case report, we present a 35-year-old patient with retained PFO up to 9 years after par plana vitrectomy. Post-operatively, PFO bubbles occupied 15% of the anterior chamber (AC). Follow-up over 9 years, consistently showed a quiet AC, normal intraocular pressure and endothelial cell counts remained stable. Until date, the patient has been under observation and there have been no ocular symptoms or side-effects. Residual PFO that inadvertently remains in the AC can be well- tolerated, without side-effects for up to 9 years.

Role of Intravitreal Antivascular Endothelial Growth Factor Injections for Choroidal Neovascularization due to Choroidal Osteoma.

We treated 26 eyes of 25 young patients having a mean age of 30 years with intravitreal vascular endothelial growth factor (VEGF) inhibitor for choroidal new vessel (CNV) formation overlying choroidal osteoma over a mean follow-up of 26 months. Mean number of injections was 2.4 at 6 months, 3.2 at 12 months, and 5.5 at 24 months. CNV was subfoveal in 14 eyes, juxtafoveal in 5, extrafoveal in 5, and peripapillary in 2. By paired comparison, mean decrease from baseline was 119.7 microns at 6 months (n = 15; P = 0.001), 105.3 microns at 1 year (n = 10; P = 0.03), and 157.6 microns at 2 years (n = 7; P = 0.08). BCVA improved by 3.3 lines at 6 months after therapy (n = 26; P < 0.001), 2.8 lines (n = 20; P = 0.01) at 1 year, and 3.1 lines (n = 13; P = 0.049) at 2 years. We conclude that intravitreal anti-VEGF injections improve vision in majority of eyes with CNV from choroidal osteoma.

Ocular malignant tumors. Review of the Tumor Registry at a tertiary eye hospital in central Saudi Arabia.

OBJECTIVE: To present the epidemiologic profile and magnitude of ocular malignant tumors (MT) representative of the Saudi population from the Tumor Registry (TR) at King Khaled Eye Specialist Hospital (KKESH). METHODS: This study evaluated the demographic information, clinical features including tumor laterality, ocular tissue of origin, and diagnosis of patients from the TR registry between 1983 and 2012 at KKESH, Riyadh, Kingdom of Saudi Arabia. The incidence of MT among Saudi adults (>/= 15 years old), and children (<15 years old) was estimated. RESULTS: The TR recorded 4,146 neoplasms (2,509 [60.5%] benign tumors, and 1,637 [39.5%] MT). The incidence of MT in children was 3.6 per million/year (M/Y), and 2.4/M/Y for adults. Retinoblastoma (Rb) (n=763, 91%) was the most common ocular malignancy in children. In adults, the most common MT was squamous cell carcinoma (SCC) (n=363, 45.8%), basal cell carcinoma (BCC) (n=186, 23%), uvealmelanoma (n=94, 11.9%), sebaceous gland carcinoma (n=54, 6.8%), lymphomas (orbital, adnexal) (n=46, 5.8%), and others (n=53, 6.8%). The Rb (7.7/M/Y in <5 years old Saudi children) was less frequent than that reported in some Gulf countries, but higher than that reported from the West. The SCC was less frequent in countries with comparable sun exposure than in other continents, but the incidence remained unchanged over 3 decades. There was a significant increase in BCC between 1983-1992 and 2003-2012. CONCLUSION: The rates of all cancers remained stable over 3 decades except BCC, which showed a significant rise.