Samuray Tuncer

Autism in visually impaired individuals

Abstract  The aim of the present study was to assess the prevalence and associated risk factors of autism in a sample of visually impaired children and adolescents. A total of 257 blind children and adolescents (age range: 7–18 years) were examined for autism using a three‐stage process. The first stage estimated probable cases of autistic disorder based on the Autism Behavior Checklist and the second stage by direct observation of the subjects in different settings. In the third stage, subjects with the probable diagnosis of autistic disorder were asked to undergo psychiatric examination. A final diagnosis of autistic disorder (based on the criteria in DSM‐IV) was given after interviewing the caregivers and clinical observation. Thirty of 257 subjects met the criteria for autistic disorder. Comparison of the characteristics of the two groups (autistic and non‐autistic) with χ2‐squared and independent sample t‐tests revealed a statistically significant difference in terms of severity of blindness (P = 0.015), cerebral palsy (P = 0.02) and intellectual level (P = 0.001). The results of the present study suggest that subjects with blindness plus autism have greater neurological impairment (as suggested by the presence of lower intellectual level and cerebral palsy), and more severe visual impairment than the subjects with blindness only.

Clinical Features and Outcomes of HLA-B27-Positive and HLA-B27-Negative Acute Anterior Uveitis in a Turkish Patient Population

Purpose: To compare the clinical features and outcomes of HLA-B27-positive and HLA-B27-negative acute anterior uveitis (AAU) in a large Turkish patient population. Methods: Forty-four patients with HLA-B27-positive AAU and 65 with HLA-B27-negative AAU were reviewed retrospectively. HLA-B27 typing, gender, laterality, clinical features (fibrinous reaction, hypopyon formation), ocular complications, medical treatment, and final visual acuity were recorded. Statistical analyses included Students t-test, Fishers exact test, Yates corrected χ2 test, and Mann-Whitney U test. A p-value of < 0.05 was considered statistically significant. Results: Forty percent of all patients with AAU were found to be positive for HLA-B27 antigen. HLA-B27-positive AAU was significantly more common in males and HLA-B27-negative AAU in females. Unilateral or bilateral alternating AAU as well as fibrinous reaction and hypopyon formation were more common in HLA-B27-positive patients. Posterior iris synechiae and cataract formation were more common and systemic immunosuppressive agents were exclusively required in patients with HLA-B27-negative AAU. Visual outcome did not differ significantly between the HLA-B27-positive and HLA-B27-negative groups. Conclusions: HLA-B27 association is less common in Turkish patients with AAU than in other populations reported in the literature. Although distinct features of HLA-B27 uveitis were confirmed in our study, visual outcome did not differ from idiopathic AAU. Therefore, we believe that HLA-B27 typing in patients with AAU helps the clinician with the diagnosis. However, it has a limited value in determining the prognosis.

Preliminary Anatomical and Neurodevelopmental Outcomes of Intravitreal Bevacizumab As Adjunctive Treatment for Retinopathy of Prematurity

Abstract Purpose: To evaluate the two-year follow-up of patients with type 1 retinopathy of prematurity (ROP) who received intravitreal bevacizumab (IVB) as adjunctive treatment. Materials and methods: We conducted a longitudinal follow-up study of premature infants who received 0.625 mg IVB therapy in addition to standard laser photocoagulation therapy. For comparison of the ophthalmological and neurological assessment outcomes of these infants, a control group was formed with 13 birth weight- and gestational age-matched infants who were treated with laser therapy alone for type 1 ROP. The neurological status of the study group and the control group was examined systematically, and neurodevelopmental evaluation was assessed by the Bayley Scales of Infant Development (BSID-III). Results: A total of 18 eyes of 13 infants were included in the study. Anatomical success was obtained in 14 eyes (78%) and retinal detachment was observed in 4 eyes (22%). At two years of age, no significant difference was found in terms of spherical or cylindrical refractive errors compared to the control group. In control group, 2/13 patients’ and in study group, 3/13 patients’ neurological examinations were abnormal. No significant difference was found in the mean cognitive, language or motor BSID-III test scores of the groups. Conclusions: IVB appears to be useful for advanced ROP when laser treatment is precluded or not sufficient for preventing the progression of ROP. This pilot study indicates that IVB seems to contribute no further complications to the complications already present due to prematurity.

Systemic Rituximab Immunotherapy in the Management of Primary Ocular Adnexal Lymphoma: Single Institution Experience

Abstract Purpose: To evaluate the efficacy of systemic rituximab immunotherapy in the management of primary ocular adnexal lymphomas (OAL). Materials and methods: Clinical records of 10 consecutive patients (11 eyes) with biopsy-proven OAL managed with systemic anti-CD20 monoclonal antibody (rituximab; 375 mg/m2 intravenously once every three weeks for 6–8 cycles) between June 2008–March 2013 were evaluated retrospectively. Orbital magnetic resonance imaging and positron emission tomography were performed to evaluate any orbital and systemic involvement, respectively. Clinical response was classified as complete or partial. Results: The age of patients ranged between 27–85 (median, 55) years. Nine patients (90%) presented with unilateral and one (10%) with bilateral conjunctival involvement. Orbit was affected in 4 patients (40%), one of which had also choroidal involvement (10%). None of the patients had systemic involvement at initial presentation. All patients received an average of 7 cycles (range, 6–8) of systemic immunotherapy. After a median follow-up of 31 months (range, 10–61 months), complete response without recurrence could be achieved in 4 eyes (36%) with rituximab monotherapy. No systemic or ocular side effects were observed in any patient. Additional radiotherapy was required in 6 patients (7 eyes; 64%) with partial response or recurrence. Conclusions: Complete regression of primary OALs without recurrence was observed in about one-third of eyes after systemic rituximab monotherapy. Adjunctive radiotherapy was required in remaining two-thirds of the cases to achieve complete response. Thus, considering the balance between high rate of local control and potential ocular complications of radiotherapy, systemic rituximab can be considered as a first-line therapeutic option in the management of primary OAL.

Dramatic Regression of Amelanotic Choroidal Melanoma With PDT Following Poor Response to Brachytherapy

Photodynamic therapy (PDT) has been used for treatment of choroidal neovascular membrane from exudative macular degeneration. Other applications include treatment of some intraocular tumors, such as choroidal hemangioma, vasoproliferative tumor, and choroidal osteoma. The authors report the effect of PDT for amelanotic choroidal melanoma. A 40-year-old woman with an amelanotic choroidal melanoma of 6.5 mm thickness showed poor response to iodine brachytherapy (80 Gy apical dose) with no reduction in thickness at 16 months of follow-up. There was prominent residual tumor. The amelanotic tumor was treated with verteporfin PDT using three overlapping spots (8,600 microns), with avoidance of the optic disc using standard treatment parameters. Dramatic tumor regression over 2 months to a completely flat scar (1.3 mm thickness) was documented and remained stable at 50 months of follow-up. Amelanotic choroidal melanoma with incomplete response following conventional plaque radiotherapy can be treated with verteporfin PDT for consolidation.

Evaluation of material heterogeneity dosimetric effects using radiochromic film for COMS eye plaques loaded with 125I seeds (model I25.S16)

PURPOSE (1) To measure absolute dose distributions in eye phantom for COMS eye plaques with (125)I seeds (model I25.S16) using radiochromic EBT film dosimetry. (2) To determine the dose correction function for calculations involving the TG-43 formalism to account for the presence of the COMS eye plaque using Monte Carlo (MC) method specific to this seed model. (3) To test the heterogeneous dose calculation accuracy of the new version of Plaque Simulator (v5.3.9) against the EBT film data for this seed model. METHODS Using EBT film, absolute doses were measured for (125)I seeds (model I25.S16) in COMS eye plaques (1) along the plaques central axis for (a) uniformly loaded plaques (14-20 mm in diameter) and (b) a 20 mm plaque with single seed, and (2) in off-axis direction at depths of 5 and 12 mm for all four plaque sizes. The EBT film calibration was performed at (125)I photon energy. MC calculations using MCNP5 code for a single seed at the center of a 20 mm plaque in homogeneous water and polystyrene medium were performed. The heterogeneity dose correction function was determined from the MC calculations. These function values at various depths were entered into PS software (v5.3.9) to calculate the heterogeneous dose distributions for the uniformly loaded plaques (of all four sizes). The dose distributions with homogeneous water assumptions were also calculated using PS for comparison. The EBT film measured absolute dose rate values (film) were compared with those calculated using PS with homogeneous assumption (PS Homo) and heterogeneity correction (PS Hetero). The values of dose ratio (film∕PS Homo) and (film∕PS Hetero) were obtained. RESULTS The central axis depth dose rate values for a single seed in 20 mm plaque measured using EBT film and calculated with MCNP5 code (both in ploystyrene phantom) were compared, and agreement within 9% was found. The dose ratio (film∕PS Homo) values were substantially lower than unity (mostly between 0.8 and 0.9) for all four plaque sizes, indicating dose reduction by COMS plaque compared with homogeneous assumption. The dose ratio (film∕PS Hetero) values were close to unity, indicating the PS Hetero calculations agree with those from the film study. CONCLUSIONS Substantial heterogeneity effect on the (125)I dose distributions in an eye phantom for COMS plaques was verified using radiochromic EBT film dosimetry. The calculated doses for uniformly loaded plaques using PS with heterogeneity correction option enabled were corroborated by the EBT film measurement data. Radiochromic EBT film dosimetry is feasible in measuring absolute dose distributions in eye phantom for COMS eye plaques loaded with single or multiple (125)I seeds. Plaque Simulator is a viable tool for the calculation of dose distributions if one understands its limitations and uses the proper heterogeneity correction feature.

Primary orbital leiomyosarcoma.

A 79-year-old woman was presented with a 6-month history of painless proptosis in the left eye. On examination, there was a palpable superotemporal mass displacing the left eye inferomedially. Orbital CT revealed a heterogeneous, lobulated mass occupying the left orbital space with no bone erosion or destruction. MRI showed an extraconal, heterogenous, lobulated orbital mass in the lacrimal gland region of the left eye. The lesion was excised, and the diagnosis of leiomyosarcoma was made by histopathologic examination and immunohistochemistry. Systemic examinations were negative for a primary or a metastatic tumor. There was no evidence of tumor recurrence after 12 months of follow-up. Primary orbital leiomyosarcoma is a rare tumor that mainly occurs in older women and presents with painless proptosis. It should be considered in the differential diagnosis of superotemporal extraconal lesions.

An international survey of classification and treatment choices for group D retinoblastoma

AIM To determine which IIRC scheme was used by retinoblastoma centers worldwide and the percentage of D eyes treated primarily with enucleation versus globe salvaging therapies as well as to correlate trends in treatment choice to IIRC version used and geographic region. METHODS An anonymized electronic survey was offered to 115 physicians at 39 retinoblastoma centers worldwide asking about IIRC classification schemes and treatment patterns used between 2008 and 2012. Participants were asked to record which version of the IIRC was used for classification, how many group D eyes were diagnosed, and how many eyes were treated with enucleation versus globe salvaging therapies. Averages of eyes per treatment modality were calculated and stratified by both IIRC version and geographic region. Statistical significance was determined by Chi-square, ANOVA and Kruskal-Wallis tests using Prism. RESULTS The survey was completed by 29% of physicians invited to participate. Totally 1807 D eyes were diagnosed. Regarding IIRC system, 27% of centers used the Childrens Hospital of Los Angeles (CHLA) version, 33% used the Childrens Oncology Group (COG) version, 23% used the Philadelphia version, and 17% were unsure. The rate for primary enucleation varied between 0 and 100% and the mean was 29%. By IIRC version, primary enucleation rates were: Philadelphia, 8%; COG, 34%; and CHLA, 37%. By geographic region, primary enucleation rates were: Latin America, 57%; Asia, 40%; Europe, 36%; Africa, 10%, US, 8%; and Middle East, 8%. However, systemic chemoreduction was used more often than enucleation in all regions except Latin America with a mean of 57% per center (P<0.0001). CONCLUSION Worldwide there is no consensus on which IIRC version is used, systemic chemoreduction was the most frequently used initial treatment during the study period followed by enucleation and primary treatment modality, especially enucleation, varied greatly with regards to IIRC version used and geographic region.

Intravitreal Lower-Dose (20 µg) Melphalan for Persistent or Recurrent Retinoblastoma Vitreous Seeds

BACKGROUND AND OBJECTIVE The major cause of failure in the management of retinoblastoma is the persistence/recurrence of vitreous seeds (VS). This study reports the efficacy and complications of standard lower-dose (20 µg) intravitreal melphalan for VS. PATIENTS AND METHODS Retrospective review of all patients with active VS treated with lower-dose intravitreal melphalan (20 µg/0.1 mL) on a monthly basis until complete VS regression was achieved. RESULTS A total of 14 injections were delivered to seven eyes of seven patients (range: 1-4; median: 2). At a median follow-up of 20 months (range: 12-32 months), complete regression of VS was achieved in all cases (100%), and globe salvage was achieved in six cases (86%). One eye required enucleation for solid tumor recurrence. Side effects of retinal pigment epithelium mottling at the site of injection was noted in two eyes (29%). CONCLUSION The 2-year results of this study suggest that standard lower-dose (20 µg) intravitreal melphalan is safe and highly effective for the management of viable VS from retinoblastoma.

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.