Publications by authors named "Hatice Tuba Atalay"

22 Publications

  • Page 1 of 1

Adjuvant therapy of histopathological risk factors of retinoblastoma in Europe: A survey by the European Retinoblastoma Group (EURbG).

Pediatr Blood Cancer 2021 Jun 15;68(6):e28963. Epub 2021 Mar 15.

The Goldschleger eye institute, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.

Introduction: Advanced intraocular retinoblastoma can be cured by enucleation, but spread of retinoblastoma cells beyond the natural limits of the eye is related to a high mortality. Adjuvant therapy after enucleation has been shown to prevent metastasis in children with risk factors for extraocular retinoblastoma. However, histological criteria and adjuvant treatment regimens vary and there is no unifying consensus on the optimal choice of treatment.

Method: Data on guidelines for adjuvant treatment in European retinoblastoma referral centres were collected in an online survey among all members of the European Retinoblastoma Group (EURbG) network. Extended information was gathered via personal email communication.

Results: Data were collected from 26 centres in 17 countries. Guidelines for adjuvant treatment were in place at 92.3% of retinoblastoma centres. There was a consensus on indication for and intensity of adjuvant treatment among more than 80% of all centres. The majority of centres use no adjuvant treatment for isolated focal choroidal invasion or prelaminar optic nerve invasion. Patients with massive choroidal invasion or postlaminar optic nerve invasion receive adjuvant chemotherapy, while microscopic invasion of the resection margin of the optic nerve or extension through the sclera are treated with combined chemo- and radiotherapy.

Conclusion: Indications and adjuvant treatment regimens in European retinoblastoma referral centres are similar but not uniform. Further biomarkers in addition to histopathological risk factors could improve treatment stratification. The high consensus in European centres is an excellent foundation for a common European study with prospective validation of new biomarkers.
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http://dx.doi.org/10.1002/pbc.28963DOI Listing
June 2021

External Drainage Alone Versus External Drainage With Vitrectomy in Advanced Coats Disease.

Am J Ophthalmol 2021 02 9;222:6-14. Epub 2020 Sep 9.

Ophthalmology Department, Gazi University School of Medicine, Besevler, Ankara, Turkey.

Purpose: To evaluate the efficacy of transscleral drainage of subretinal fluid (TDSRF) with and without pars plana vitrectomy (PPV), which are performed as an adjunct to ablative therapies in advanced Coats disease.

Design: Retrospective, comparative case series.

Patient Population: A total of 31 eyes from 31 consecutive patients with advanced Coats disease at an average age of 47.8 ± 34.2 (2-156) months at the time of the surgery, who underwent TDSRF alone or in combination with PPV.

Methods: Main outcome measurements were LogMAR visual acuity values, anterior and posterior segment findings, need for further surgery, laser photocoagulation, and anti-vascular endothelial growth factor (anti-VEGF) treatment. Anatomical success was defined as the maintenance of retinal reattachment without any further surgery during follow-up.

Results: Sixteen patients underwent TDSRF alone, and 15 patients underwent combined TDSRF and PPV. Mean follow-up time was 34.8 ± 32.9 months (6-128). Anatomical success rate of combined TDSRF and PPV was higher than that of TDSRF alone (93.8% vs 75%, respectively). The incidence of epiretinal membrane formation, number of laser photocoagulation procedures, and anti-VEGF treatments were statistically significantly higher in the group that underwent TDSRF alone than in those who had combined TDSRF and PPV in the long term. There was an improvement in vision in only 4 eyes (12.9%) (all >3 years old at presentation) during the follow-up.

Conclusions: Combined TDSRF and PPV appears to be more effective in controlling the disease than TDSRF alone, as an adjunct to ablative procedures for the treatment of advanced Coats disease. The need for additional treatment is much less with the addition of PPV.
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http://dx.doi.org/10.1016/j.ajo.2020.09.006DOI Listing
February 2021

Reply to "Ultrasound evaluation of optic nerve sheath diameter to assess treatment efficacy in pediatric idiopathic intracranial hypertension".

Childs Nerv Syst 2020 05 17;36(5):887. Epub 2020 Mar 17.

Pediatric Neurology Department, Gazi University Faculty of Medicine, Ankara, Turkey.

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http://dx.doi.org/10.1007/s00381-020-04576-9DOI Listing
May 2020

Rose Bengal-Mediated Photodynamic Antimicrobial Treatment of Keratitis.

Curr Eye Res 2020 10 27;45(10):1205-1210. Epub 2020 Feb 27.

Departments of Ophthalmology, Gazi University , Ankara, Turkey.

: To evaluate the in vivo efficacy of rose bengal (RB)-mediated photodynamic antimicrobial therapy (PDAT) for treatment of keratitis (AK). : An animal (rabbit) AK model was successfully achieved via intrastromal inoculation of a suspension of cells and trophozoites. Prior to RB-PDAT (pre-treatment, day-5), the severity of the induced corneal infection was graded numerically for epithelial defects, stromal edema, neovascularity, and stromal opacity/infiltration. The right eyes of rabbits (n = 18) were divided equally into three groups (n = 6/group): control (no treatment); 0.1% RB+518 nm irradiation (5.4 J/cm); and 0.2% RB+518 nm irradiation (5.4 J/cm). On post-treatment day-5, animals were euthanized, after which corneal buttons were excised and submitted for real-time polymerase chain reaction (RT-PCR) analysis. : Post-treatment clinical scores of the 0.1 and 0.2% RB groups indicated significant improvement compared to control group scores (pre-treatment clinical scores; 5.17 ± 0.98, 7.50 ± 0.62, and 6.17 ± 0.70 and post-treatment clinical scores; 4.50 ± 0.56, ( = .043), 3.50 ± 0.99 ( = .039), 6.83 ± 1.66 ( = .34), respectively). RT-PCR analysis revealed that the mean cycle threshold (Ct) values were significantly higher in treated-group corneas compared to control-group corneas, with no significant differences between treated-groups (Mean Ct values; 34.33, 34.5, and 29.67 for 0.1 and 0.2% RB, and control groups). There was a statistically significant negative correlation between post-treatment clinical scores and Ct values ( = -0.474, -value 0.047). : Our results demonstrate that RB-PDAT is effective in decreasing the parasitic load and clinical severity of AK.
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http://dx.doi.org/10.1080/02713683.2020.1731830DOI Listing
October 2020

Real-life comparison of the viewing angle and the image quality of two commonly used viewing systems for vitreoretinal surgery

Turk J Med Sci 2020 06 23;50(4):689-696. Epub 2020 Jun 23.

Department of Ophthalmology, Gazi University, Ankara, Turkey

Background/aim: To compare the clinical use, image quality and viewing angle of a commonly used contact wide angle viewing (WAV) system (Advanced Visual Instruments (AVI) Panoramic Imaging Systems, NY, USA) with a commonly used noncontact WAV system (Leica RUV800, Leica Microsystems, Switzerland).

Materials And Methods: Images of 42 consecutively operated eyes were obtained with both systems at the same surgical stages and were compared for image quality using the Imatest Master 4.5.13 (Imatest LLC, Boulder, USA) software. The viewing angles of the images were calculated using the optic disc sizes measured from the OCT and infrared fundus images. The 68-degree AVI lens was compared with the 90-dioptre (D) Leica RUV800 lens, while the 130-degree AVI lens was compared with the 132-D Leica RUV800 lens. The surgical assistants were asked to grade the difficulty of holding the lens in place from 1 to 10, 1 being the easiest and 10 being the most difficult.

Results: The contact system provided wider viewing angles with higher quality compared to the noncontact system both under fluid and air media. The difference was clinically significant in eyes with impaired corneal clarity, very high myopia, or small pupil. The difficulty of holding the lens in place ranged from 4 to 7, and decreased gradually with practice.

Conclusions: Both WAV systems provided high image quality and adequate viewing angles in most cases. However, the contact system appeared to provide a superior image quality and/or a wider viewing angle in more challenging situations. The difficulty of holding the contact lens in place was found to be moderate.
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http://dx.doi.org/10.3906/sag-1910-11DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7379410PMC
June 2020

The Effect of Sildenafil on Selenite-Induced Cataract in Rats.

Curr Eye Res 2020 09 11;45(9):1082-1088. Epub 2020 Feb 11.

Department of Medical Biochemistry, Gazi University Medical School , Ankara, Turkey.

Purpose: To investigate the effect of sildenafil on an experimental sodium selenite-induced cataract model in rats.

Materials And Methods: Twenty-six young Wistar rats were separated into four groups. On postpartum day 10, six rats received only selenite (group 1, selenite-induced cataract), seven rats received selenite and high dose oral sildenafil (group 2, high-dose sildenafil-treated), seven rats received selenite and low dose oral sildenafil (group 3, low-dose sildenafil-treated), and six rats received only saline (group 4, controls). On postpartum day 30, cataract formation was graded and recorded using an operating microscope. The rats were sacrificed, lens tissues were isolated, and serum samples were collected. Nitrite oxide metabolites (NOx), advanced oxidative protein products (AOPP), and total sulfhydryl (TSH) levels were assessed in both serum and lenticular samples.

Results: The rats treated with low-dose sildenafil showed lower levels of AOPP and NOx, and the higher levels of TSH than the rats in other experimental groups. Otherwise, the rats treated with high-dose sildenafil, similar to the selenite-induced cataract group, showed higher levels of AOPP and serum NOx than rats in the low-dose sildenafil-treated group. The rats treated with low-dose sildenafil also showed less cataract development than rats in the other experimental groups.

Conclusion: Low doses (0.7 mg/kg) of oral sildenafil might show a protective effect on cataract development by lowering oxidative stress.
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http://dx.doi.org/10.1080/02713683.2020.1726405DOI Listing
September 2020

Rhegmatogenous retinal detachment secondary to isolated retinal metastasis from small cell lung carcinoma: An uncommon complication.

Eur J Ophthalmol 2020 Jan 21:1120672120902022. Epub 2020 Jan 21.

Department of Ophthalmology, Gazi University School of Medicine, Ankara, Turkey.

Purpose: To report a case of rhegmatogenous retinal detachment associated with isolated retinal metastasis from lung carcinoma.

Methods: Multimodal imaging, including wide-field retinal imaging, ultrasonic imaging, and magnetic resonance imaging.

Results: Systemic chemotherapy and cranial prophylactic radiotherapy resulted in shrinkage of these lesions and retinal breaks making them much smaller and preventing progression of retinal detachment transiently.

Conclusion: This is the first reported case of rhegmatogenous retinal detachment secondary to retinal metastasis from a lung cancer.
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http://dx.doi.org/10.1177/1120672120902022DOI Listing
January 2020

Optic nerve ultrasonography in monitoring treatment efficacy in pediatric idiopathic intracranial hypertension.

Childs Nerv Syst 2020 07 15;36(7):1425-1433. Epub 2020 Jan 15.

Pediatric Neurology Department, Gazi University Faculty of Medicine, Ankara, Turkey.

Purpose: Measurement of optic nerve sheath diameter (ONSD) with ocular ultrasonography (USG) is a noninvasive technique that can be readily used to determine clues of increased intracranial pressure. In this study, we aimed to determine the role of optic nerve sheath diameter measurements in the diagnosis and follow-up of pediatric patients with idiopathic intracranial hypertension (IIH).

Methods: Eight patients with a diagnosis of IIH with a median age of 11.7 (range 4.5-17) years were examined prospectively. During follow-up, orbital ultrasonography (USG) was performed immediately prior to lumbar puncture (LP) and at 24 h, at 1 week, and between 1 and 18 months after LP. Cranial MRI examinations and automated visual field assessments were performed at baseline and at 3 months, and both measurements were compared with each other.

Results: The mean cerebrospinal fluid opening pressure (37.75 ± 12.64 cm HO) and the mean ONSD (5.94 ± 0.46 mm) were correlated. The median follow-up was 16 (range, 12-18 months), and ONSD regressed gradually consistent with clinical and radiologic improvement during follow-up.

Conclusions: To the best of our knowledge, this is the first prospective pilot study performed on pediatric patients with IIH using orbital USG for ONSD measurements. Despite the small sample size, the present study indicated that orbital USG may be used as a promising noninvasive tool to diagnose increased intracranial pressure and for monitoring treatment efficacy in this special patient population.
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http://dx.doi.org/10.1007/s00381-019-04497-2DOI Listing
July 2020

Intravitreal Dexamethasone Implant in the Treatment of Non-infectious Uveitis

Turk J Ophthalmol 2019 Oct;49(5):250-257

Gazi University Faculty of Medicine, Department of Ophthalmology, Ankara, Turkey

Objectives: To evaluate the long-term results of intravitreal dexamethasone implant (DEX) for noninfectious uveitis.

Materials And Methods: The study included 62 eyes of 44 patients treated with DEX implant due to noninfectious uveitis and followed up for at least a year. Best-corrected visual acuity (BCVA), central foveal thickness, intraocular pressure (IOP), vitreous haze score, indications, immunomodulatory therapy and steroid usage before/after injection, number of injections, and adverse events were analyzed retrospectively.

Results: Average follow-up was 20 months (range 12-64 months). The female/male ratio was 29/15. Mean age was 50 years (range 22-75 years). The most frequent uveitis etiologies were idiopathic (25 patients, 40.3%) and Behçet’s uveitis. (17 patients, 27.4%) The most common indication for DEX injection was cystoid macular edema together with resistant vitreous haze (26 eyes, 41.9%). Twenty-two eyes (30%) received more than one DEX injection. Mean BCVA was improved from 0.55 logMAR at baseline to 0.38, 0.32, and 0.35 after 1, 3, and 6 months, respectively (p<0.001 for each). Mean CFT was decreased from 386 μm at baseline to 288, 311, and 302 μm after 1, 3, and 6 months, respectively (p<0.001 for each). Mean IOP did not change significantly during follow-up. Five eyes (8%) received topical anti-glaucoma medication (IOP ≥25 mmHg). Eighteen (46%) of 39 phakic eyes underwent cataract surgery during follow-up. Similar efficacy of the DEX implant was observed in eyes that received multiple injections. Systemic immunomodulatory therapy did not change significantly during follow-up.

Conclusion: Intravitreal DEX injection does not alter systemic immunomodulatory therapy, but may facilitate the management of noninfectious uveitis by suppressing local intraocular inflammation. Multiple injections yielded comparable visual and anatomical outcomes to single injections. Follow-up for ocular hypertension and cataract formation are important, especially in eyes receiving multiple injections.
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http://dx.doi.org/10.4274/tjo.galenos.2019.81594DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6823586PMC
October 2019

Elevated serum ferritin level with cataract of spectacular morphology: Hyperferritinemia-cataract syndrome.

Pediatr Hematol Oncol 2019 Sep 14;36(6):390-393. Epub 2019 Sep 14.

Department of Ophthalmology, Faculty of Medicine, Gazi University , Ankara , Turkey.

Hyperferritinemia-cataract syndrome, characterized by high serum ferritin concentration and cataracts in early life, remains a less-known rare disease, with fewer than 100 families reported worldwide. Though benign, high ferritin levels frequently result in misdiagnosis with iron storage disease, and patients can be exposed to unnecessary, even invasive, evaluation and treatment procedures. The presence of cataract together with isolated serum ferritin elevation should alert clinicians to consider this syndrome. We herein present a new family with hyperferritinemia-cataract syndrome to increase clinical awareness.
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http://dx.doi.org/10.1080/08880018.2019.1621972DOI Listing
September 2019

Results of surgery for late sequelae of cicatricial retinopathy of prematurity.

Indian J Ophthalmol 2019 06;67(6):908-911

Department of Ophthalmology, Gazi University, School of Medicine, Ankara, Turkey.

Purpose: To report anatomical and functional results of vitreoretinal surgery in our case series of late cicatricial retinopathy of prematurity (ROP) patients with subtotal retinal detachment.

Methods: This is a retrospective, consecutive case series. Eleven eyes of 10 patients presented with partial tractional retinal detachment secondary to late cicatricial ROP (cicatricial stage 4B) who underwent vitreoretinal surgery were retrospectively reviewed. Anatomical and functional outcomes were evaluated.

Results: The mean gestational age at birth was 28.6 (26-32) weeks. The mean age at surgery was 79 (4-213) months. Patients were followed up for 21.7 (6-40) months. Six eyes (55%) had lens-sparing vitrectomy and five eyes (45%) had lensectomy + vitrectomy. Anatomical success was achieved in 10 eyes (91%). Improvement in visual acuity was noted in nine eyes (82%).

Conclusion: Eye grows but fibrotic tissue does not grow with age, and during this period retinal traction may get worse. Relieving these tractions may lead to good anatomical and visual outcomes in selected late cicatricial ROP cases.
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http://dx.doi.org/10.4103/ijo.IJO_1083_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552626PMC
June 2019

Anterior elongation of the retina in persistent fetal vasculature: emphasis on retinal complications.

Eye (Lond) 2019 06 6;33(6):938-947. Epub 2019 Feb 6.

Gazi University School of Medicine, Ankara, 06500, Turkey.

Purpose: To identify the characteristics of peripheral retinal anomalies associated with persistent fetal vasculature (PFV) and evaluate the complications and outcomes.

Methods: Retrospective, noncomparative case series of patients with PFV who underwent surgery. Type of PFV, presence of peripheral retinal anomalies, extent of the retrolental membrane, complications, and functional and anatomical results were evaluated.

Results: Twenty-nine eyes of 28 patients were enrolled in the study: 14 eyes (48.3%) with anterior PFV, 10 eyes (34.5%) with posterior PFV, and 5 eyes (17.2%) with combined PFV. The retina was found to elongate anteriorly as finger-like projections beyond the ora serrata, incorporating into retrolental fibrovascular tissue in 81.8% of the anterior PFV cases. Cases with more extensive retrolental tissue had a higher risk of retinal complications (p = 0.009) and anterior segment complications (p = 0.026) than those with localized disease. Leaving the peripheral part of the fibrovascular tissue in place led to complications by later contraction. A total of 35.7% of the anterior PFV cases had 20/200 or better vision at the final follow-up versus 6.7% of the cases with posterior involvement. Retinal attachment was achieved in 12 eyes (80%) with posterior involvement. Four eyes (13.7%) resulted in total retinal detachment at final follow-up.

Conclusions: The high incidence of peripheral retinal anomalies that are found in anterior PFV patients and their relation to retinal complications warrant careful examination of the ora serrata-ciliary body area. Limbal approach may be preferred as a safer method in cases with an extensive fibrovascular membrane obscuring the view. Leaving the peripheral part of the fibrovascular tissue may result in severe postoperative complications, which should be avoided.
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http://dx.doi.org/10.1038/s41433-019-0345-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6707181PMC
June 2019

Parameters affecting postoperative success of surgery for stage 4A/4B ROP.

Br J Ophthalmol 2019 11 18;103(11):1624-1632. Epub 2019 Jan 18.

Gazi University School of Medicine, Ankara, Turkey.

Purpose: To describe the long-term anatomical and functional results of surgery for retinal detachment (RD) associated with stage 4 retinopathy of prematurity (ROP) and patient and surgery-related factors affecting postoperative success.

Design: Retrospective case series at a single tertiary referral paediatric vitreoretinal practice.

Methods: One hundred and twenty-one eyes of 82 infants (40 female/42 male) who underwent lens-sparing vitrectomy (LSV) or lensectomy with vitrectomy surgery for stage 4A and 4B ROP at Gazi University Department of Ophthalmology between 2011 and 2016 were enrolled in this study. Patient characteristics including gestational age, birth weight, gender, stage of ROP at presentation, preoperative treatment (laser, anti-vascular endothelial growth factor (VEGF) or combined), anatomical and functional outcome and complications were recorded. The effect of birth weight, gestational age, presence of plus disease, preoperative treatment status, surgically induced posterior hyaloid detachment, postoperative vitreous haemorrhage and iatrogenic retinal tear formation on anatomical and functional results was evaluated.

Results: 61.2% of the eyes were stage 4A and 38.8% were stage 4B ROP. The mean follow-up was 24.5 months. 18.2% of the eyes had no preoperative treatment. Anatomical success was 86.5% for stage 4A and 68.1% for stage 4B at the first year, 91.7% for stage 4A and 69.4% for stage 4B at the second year, and 95.8% for stage 4A and 57.9% for stage 4B at the third year. Functional success was 85.1% for stage 4A and 65.9% for stage 4B at the first year, 89.6% for stage 4A and 61.1% for stage 4B at the second year, and 87.5% for stage 4A and 57.8% for stage 4B at the third year. The mean visual acuity was 1.12±0.34 logarithm of the minimum angle of resolution (logMAR) for stage 4A and 1.34±0.32 logMAR at the 3-year follow-up duration (p>0.05). There was preoperative plus disease in 59.5% of the eyes. Subsequent retinal surgeries were required in 17.4% of the eyes. Presence of plus disease and absence of preoperative treatment, iatrogenic retinal tear formation and postoperative vitreous haemorrhage were found to have significant negative effects, while surgical induction of posterior hyaloid detachment and sparing the lens intraoperatively affected the anatomical and functional results positively.

Conclusions: Surgery for stage 4 ROP-associated RD resulted in encouraging anatomical and functional outcomes and the results are even better in eyes with preoperative (laser/anti-VEGF) treatment, LSV and surgically induced posterior hyaloid detachment.
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http://dx.doi.org/10.1136/bjophthalmol-2018-312922DOI Listing
November 2019

Effect of Riboflavin/Rose Bengal-Mediated PACK-CXL on Acanthamoeba Trophozoites and Cysts in Vitro.

Curr Eye Res 2018 11 7;43(11):1322-1325. Epub 2018 Aug 7.

a Department of Ophthalmology, School of Medicine , Gazi University , Ankara , Turkey.

Purpose: To evaluate the antiamoebic properties of photo-activated chromophore for keratitis (PACK)-corneal cross-linking (CXL) (PACK-CXL), in combination with riboflavin (0.1 and 0.25%) or rose bengal (0.1 and 0.2%), for treatment of Acanthamoeba trophozoites and cysts.

Materials And Methods: Cultures of Acanthamoeba castellanii were grown in a fluid medium at a concentration of 2.7 × 10 cell/ml. PACK-CXL was used on A. castellani cells in combination with either riboflavin (0.1 and 0.25%) or rose bengal (0.1 and 0.2%). Riboflavin-containing wells were irradiated with ultraviolet-A (UVA) light (365-nm wavelength). Rose bengal-containing wells were irradiated with green light (523-nm wavelength). A power density of 9 mW/cm for 10 min and total irradiation dose of 5.4 J/cm was used for both riboflavin and rose bengal. After UVA and green light irradiation, cell viabilities were evaluated, and percentage of dead cells calculated.

Results: No significant amoebicidal activity was observed following PACK-CXL/riboflavin at either concentration. PACK-CXL/rose bengal, however, was observed to be highly effective in eradicating Acanthamoeba cells at either concentration, with no significant difference observed between the two concentrations. The percentage of dead cells was 63% following treatment at either rose bengal concentration.

Conclusion: PACK-CXL with rose bengal demonstrated pronounced antiamoebic activity against A.castellanii. Further in vitro and in vivo studies are needed to confirm this finding.
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http://dx.doi.org/10.1080/02713683.2018.1501074DOI Listing
November 2018

Prediction of Descemet Membrane Perforation During Deep Anterior Lamellar Keratoplasty in Patients With Keratoconus With Stromal Scar.

Eye Contact Lens 2018 Nov;44 Suppl 2:S176-S179

Department of Cornea, Gazi University Hospital, Ankara, Turkey.

Objectives: To report big-bubble deep anterior lamellar keratoplasty (DALK) in patients with keratoconus with corneal stromal scar, and to investigate factors that can influence intraoperative Descemet membrane (DM) perforation.

Methods: A retrospective study included patients with keratoconus with central stromal scar that underwent DALK using the big-bubble technique. Best spectacle-corrected visual acuity (BSCVA), keratometry, minimum corneal thickness (MCT), stromal scar depth, and endothelial cell density (ECD) were recorded. Receiver operating characteristic (ROC) curves were analyzed to predict an intraoperative perforation related to stromal scar.

Results: Thirty-eight eyes of 38 patients with keratoconus (mean age: 30.0±9.2 years) were included in this study. Thirty-four eyes had successful DALK; in four eyes, the procedure was converted to penetrating keratoplasty because of DM macroperforations. Successful big-bubble formation was achieved in 24 of 38 (63.2%) eyes, whereas in 10 eyes, manual dissection was used to complete the DALK. Mean preoperative and postoperative second year BSCVA were 1.3 (±0.4) and 0.5 (±0.2) logarithm of the minimum angle of resolution (P<0.001), keratometry values were 65.5±7.4 and 42.8±7.8 diopters (P<0.001), and ECD were 2,740±359 and 2,279±452 cells/mm (P<0.001), respectively. Using ROC analysis, the area under curve value to predict DM perforation was found to be 0.792 (scar/MCT ratio, P=0.04) and the best cutoff point for scar depth/MCT ratio was 53%.

Conclusion: Big-bubble DALK is effective in patients with keratoconus with stromal scars. Scar depth/MCT ratio seems to predict the DM perforation.
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http://dx.doi.org/10.1097/ICL.0000000000000434DOI Listing
November 2018

Intravitreal bevacizumab for retinopathy of prematurity in infants ineligible for laser therapy.

Turk J Med Sci 2016 Apr 19;46(3):764-8. Epub 2016 Apr 19.

Department of Ophthalmology, Faculty of Medicine, Gazi University, Ankara, Turkey.

Background/aim: To evaluate refractive and strabismic results and the efficacy of intravitreal bevacizumab in retinopathy of prematurity (ROP) ineligible for laser therapy.

Materials And Methods: Thirty-nine eyes of 20 consecutive infants with high-risk prethreshold ROP (11 infants with Zone I and 9 infants with Zone II disease) who were ineligible for laser therapy due to systemic and/or ocular conditions were treated with intravitreal bevacizumab. Recurrent retinopathy was treated with laser ablation. The final follow-up examination was performed at 29.8 ± 6.0 months of corrected age.

Results: All eyes responded to the initial treatment with intravitreal bevacizumab. ROP recurred in 8 eyes (36%) with initial Zone I disease and in only 2 eyes (11%) with initial Zone II disease, which were successfully treated with laser ablation. No eye developed myopia higher than 5.0 diopters. At 2.5 years, the Zone I eyes that had received laser treatment appeared to be more myopic than the Zone I eyes treated only with intravitreal bevacizumab (P = 0.038). A tendency for a higher incidence of strabismus after additional laser therapy was also noted, but was not significant (P = 0.22).

Conclusion: Avoidance or even deferral of laser ablation with intravitreal bevacizumab may lead to less myopization in ROP compared with conventional laser treatment.
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http://dx.doi.org/10.3906/sag-1502-65DOI Listing
April 2016

Bi-allelic Mutations in KLHL7 Cause a Crisponi/CISS1-like Phenotype Associated with Early-Onset Retinitis Pigmentosa.

Am J Hum Genet 2016 Jul;99(1):236-45

Department of General Pediatrics, Münster University Children's Hospital, 48149 Münster, Germany.

Crisponi syndrome (CS)/cold-induced sweating syndrome type 1 (CISS1) is a very rare autosomal-recessive disorder characterized by a complex phenotype with high neonatal lethality, associated with the following main clinical features: hyperthermia and feeding difficulties in the neonatal period, scoliosis, and paradoxical sweating induced by cold since early childhood. CS/CISS1 can be caused by mutations in cytokine receptor-like factor 1 (CRLF1). However, the physiopathological role of CRLF1 is still poorly understood. A subset of CS/CISS1 cases remain yet genetically unexplained after CRLF1 sequencing. In five of them, exome sequencing and targeted Sanger sequencing identified four homozygous disease-causing mutations in kelch-like family member 7 (KLHL7), affecting the Kelch domains of the protein. KLHL7 encodes a BTB-Kelch-related protein involved in the ubiquitination of target proteins for proteasome-mediated degradation. Mono-allelic substitutions in other domains of KLHL7 have been reported in three families affected by a late-onset form of autosomal-dominant retinitis pigmentosa. Retinitis pigmentosa was also present in two surviving children reported here carrying bi-allelic KLHL7 mutations. KLHL7 mutations are thus associated with a more severe phenotype in recessive than in dominant cases. Although these data further support the pathogenic role of KLHL7 mutations in a CS/CISS1-like phenotype, they do not explain all their clinical manifestations and highlight the high phenotypic heterogeneity associated with mutations in KLHL7.
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http://dx.doi.org/10.1016/j.ajhg.2016.05.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5005468PMC
July 2016

Sector iris hemangioma in association with diffuse choroidal hemangioma.

J AAPOS 2015 Feb;19(1):83-6

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.

Two patients referred for iris lesions were found to have sector hemangioma of the iris stroma in contiguity with diffuse choroidal hemangioma. Neither patient had other manifestations of Sturge-Weber syndrome.
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http://dx.doi.org/10.1016/j.jaapos.2014.09.012DOI Listing
February 2015

Augmented surgical amounts for intermittent exotropia to prevent recurrence.

Indian J Ophthalmol 2014 Nov;62(11):1056-1059

University Hospitals Case Medical Center and Rainbow Babies and Children's Hospital, Department of Ophthalmology, Cleveland, USA, .

Purpose: The purpose was to evaluate the results of bilateral lateral rectus (BLR) recession which is based on augmented surgical amounts of classical surgical table of Parks' for basic and pseudo-divergence excess type intermittent exotropia [X(T)]. Materials and Methods: Patients with X(T) operated by the same surgeon and followed-up for at least 6 months were included. Patients with prior surgery, neurobehavioral and musculoskeletal conditions, strabismus different from that mentioned above X(T) were excluded. All the patients received BLR only. The amount of the recession was increased by the amount needed to correct 5 prism diopters (PD) more X(T) than what was measured. After the operation, 1 st week, 2 nd and 6 months measurements were recorded. The patients were grouped according to their 1 st week (3-7 days) postoperative examination as: >10 PD esotropia (Group 1), ≤10 PD esotropia (Group 2), exotropia (Group 3), and orthotropic (Group 4), respectively. Final surgical outcomes were classified as "good" (≤10 PD exotropia and ≤5 PD esotropia), "recurrence" (>10 PD exotropia) and "overcorrected" (>5 esotropia). Results: Thirty-seven patients were included. The mean age was 6.78 ± 2.87 years (range: 2-12 years). Mean preoperative deviation was 29.72 ± 8.07 PD (range: 15-45 PD) at distance and 20.94 ± 11.65 PD (range: 10-45 PD) at near (P < 0.0001). There were 21 (56.8%) patients in Group 1, 9 (24.3%) patients in Group 2, 1 (2.7%) patient in Group 3 and 6 (16.2%) patients in Group 4. Initial esotropia was achieved in 30 (30/37) of the patients. Twenty-eight of them had good results at the end of the 6 months. Overall "motor surgical" success rate was found to be 89.2% (33/37 patients), with 1 (2.7%) overcorrection and 3 (8.1%) recurrences at the end of the 6 months. Conclusion: This study demonstrated that early overcorrection of 10-20 PD after X(T) surgery can achieve acceptable motor outcomes in the first 6 months postoperative period.
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http://dx.doi.org/10.4103/0301-4738.146710DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4290193PMC
November 2014

Uveal melanoma in children and teenagers.

Saudi J Ophthalmol 2013 Jul 5;27(3):197-201. Epub 2013 Jul 5.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, United States.

Purpose: To review the features and prognosis of uveal melanoma in children.

Methods: Retrospective case series.

Results: Of 122 children with uveal melanoma, there were 53 (43%) male and 69 (57%) female patients. In this group, the mean age at presentation was 15 years (median 16 years, range 3-20 years). Age at presentation was 0 to 5 years in 4 (3%), 5.1 to 10 years in 14 (11%), 10.1 to 15 years in 43 (35%), and 15.1 to ⩽20 in 61 (50%). Associated ocular melanocytosis was present in 4 (3%). The melanoma was primarily located in the iris (n = 30, 25%), ciliary body (n = 10, 8%), or choroid (n = 82, 67%). The mean tumor basal dimension was 9.8 mm and mean thickness was 5.0 mm. The tumor color was pigmented (brown) (n = 102, 84%), nonpigmented (yellow) (n = 19, 16%), or mixed (n = 25, 21%). Subretinal fluid (n = 66, 54%) and hemorrhage (n = 9, 7%) were noted. Primary treatment involved laser photocoagulation (n = 3, 2%), transpupillary thermotherapy (n = 17, 14%), local tumor resection (n = 26, 21%), plaque radiotherapy (n = 42, 34%), or enucleation (n = 54, 44%). Kaplan Meier 5, 10, and 20-year estimates for uveal melanoma-related metastasis were 9%, 9%, and 20%, respectively, for children compared to 15%, 25%, and 36% for all ages.

Conclusion: Uveal melanoma in children tends to occur most often in the teenage years as a pigmented tumor involving the choroid or iris and with mean thickness of 5 mm. Prompt treatment is advised.
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http://dx.doi.org/10.1016/j.sjopt.2013.06.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3770213PMC
July 2013