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Supplemental material, sj-docx-1-ejo-10.1177_11206721231155054 for Factors affecting eye conservation and metastasis in posterior uveal melanomas by Hilal Nalcı Baytaroğlu, Ahmet Kaan Gündüz, Ibadulla Mirzayev and Funda Seher Özalp Ateş in European Journal of Ophthalmology

Iris/ciliary body tumors can broadly be classified as benign or malignant, cystic or solid, and melanocytic or non-melanocytic. This study aims to provide a systematic review of anterior segment optical coherence tomography (AS-OCT) findings in iris and ciliary body tumors. We conducted a search of published articles related to the use of AS-OCT in iris/ciliary body tumors as of December 2022. Databases that were searched included PubMed, Scopus, and Web of Science. The following search terms in various combinations were used: ‘anterior segment optical coherence tomography,’ ‘ciliary body melanoma,’ ‘ciliary body nevus,’ ‘ciliary body tumor,’ ‘iris cyst,’ ‘iris melanoma,’ ‘iris nevus,’ ‘iris tumor,’ and ‘metastasis.’ A total of 41 studies (10 original articles and 31 case reports) met the inclusion criteria. AS-OCT data pertaining to 545 iris/ciliary body lesions could be retrieved. AS-OCT has proved to be useful as a noninvasive tool for the diagnosis of solid tumors involving the iris/angle with no marked ciliary body involvement, iris pigment epithelial, and iris stromal cysts. To the best of our knowledge, this is the first systematic review in the literature evaluating the utility of AS-OCT in iris/ciliary body tumors.

Supplemental material, sj-docx-1-ejo-10.1177_11206721221100627 for Pseudoretinoblastoma: Distribution based on gender, age, and laterality by Ibadulla Mirzayev, Ahmet Kaan Gündüz, Funda Seher Özalp Ateş and Diğdem Tetik in European Journal of Ophthalmology

A 63-year-old Caucasian man with metastatic lung adenocarcinoma undergoing chemotherapy and external radiotherapy was referred for routine eye examination. Although he was asymptomatic, ocular examination revealed a relatively well-circumscribed whitish retinal lesion measuring 0.5×0.5×0.5 mm located along the inferotemporal vascular arcade in the right eye. Optical coherence tomography (OCT) showed a hyperreflective dome-shaped lesion occupying the inner retinal layers with few hyperreflective dots overlying the retina in the posterior vitreous consistent with tumor cells. Fluorescein angiography revealed early hyperfluorescence and late staining without leakage at the lesion site. A diagnosis of presumed retinal metastasis from lung adenocarcinoma was made. At 2 months follow-up after completion of chemotherapy, the retinal lesion was found to have regressed completely leaving minor irregularities in the inner retinal layers on OCT. To date, there have been only 41 cases of carcinoma metastasis to the retina reported in the literature including the current case. Despite its rarity, retinal metastasis should be considered in the differential diagnosis of a white-yellow retinal mass with/without overlying vitreous cells especially in patients with a history of systemic cancer.

Supplemental material, sj-pdf-1-ejo-10.1177_11206721211023311 for Recurrence and new tumor development after frontline intravenous chemotherapy for retinoblastoma: Risk factors and treatment results by Ahmet Kaan Gündüz, Ibadulla Mirzayev, Handan Dinçaslan and Funda Seher Özalp Ateş in European Journal of Ophthalmology

AIM: To evaluate the results and complications of secondary endoresection via pars plana vitrectomy for choroidal melanoma and review the previously reported endoresection studies on the treatment of choroidal melanoma. METHODS: The medical records of 6 patients with choroidal melanoma who underwent secondary endoresection between March 2012 and March 2020 were retrospectively reviewed. The indications for secondary endoresection were progressive or recurrent tumor and severe exudative retinal detachment after previous treatment with plaque radiotherapy/Cyberknife radiosurgery/transpupillary thermotherapy (TTT). RESULTS: Before endoresection, 2 eyes had Iodine-125 plaque radiotherapy and TTT, 1 eye had Ruthenium-106 plaque radiotherapy and TTT, 1 eye had Cyberknife radiosurgery and TTT, 1 eye had Cyberknife radiosurgery, and 1 eye had TTT only. Preoperative visual acuity ranged from 20/63 to 20/1600 (Snellen) and from 0.5 to 1.9 (mean: 1.1) on the logMAR scale. The mean tumor base diameters were 9.5×8.7 mm and the mean tumor thickness was 5.4 mm. After secondary endoresection, transient vitreous hemorrhage developed in 2 (33.3%) eyes and retinal detachment in 1 (16.7%) eye. Cytopathological examination revealed epithelioid cell melanoma in 4 (66.7%) eyes and mixed cell melanoma in 1 (16.7%). Melanoma cell type was not specified in 1 (16.7%) eye. At a mean follow-up of 49.6mo (range: 16-90mo), mean visual acuity did not improve and 1 eye was enucleated due to tumor recurrence. Final visual acuity ranged from 20/63 to 20/1600 (Snellen) and from 0.5 to 1.9 (mean: 1.2) on the logMAR scale. Two patients with choroidal melanoma developed metastasis and eventually expired. CONCLUSION: Secondary endoresection seems to be an effective treatment option for globe salvage in choroidal melanoma not responsive to conventional treatment and displaying persistent exudative retinal detachment. There was no visual acuity increase among the treated eyes but globe salvage was possible in most cases in this study.

A 43-year-old patient presented with painless proptosis, limited upgaze, and vision loss in the right eye. Funduscopic examination revealed right optic disc edema and subtle macular compression. Swept-source optical coherence tomography (SS-OCT) revealed a smooth contoured elevation of the posterior pole without any distortion of retinal structures, an appearance closely simulating dome-shaped maculopathy. Swept-source optical coherence tomography angiography (SS-OCTA) revealed normal retinal and choroidal vasculature. Orbital magnetic resonance imaging demonstrated a well-circumscribed intraconal mass compressing the globe and optic nerve in the right orbit. An anterior orbitotomy was performed, whereby the tumor was totally excised and diagnosed histopathologically as cavernous hemangioma. This case represents an orbital cavernous hemangioma touching the eyeball and producing compression of the posterior pole presenting with a dome-shaped maculopathy-like appearance on SS-OCT. SS-OCT and SS-OCTA are important noninvasive tools for evaluating the retinal and choroidal effects in orbital tumors.

A 22-year-old woman presented with progressive swelling of the nasal conjunctiva in the left eye. Anterior segment examination revealed a diffuse cystic appearance to the inferonasal bulbar conjunctiva and plica semilunaris. Anterior segment swept-source optical coherence tomography (OCT) revealed clear hyporeflective spaces demarcated by hyperreflective septae in the affected conjunctiva, consistent with the diagnosis of lymphatic malformation (LM). Magnetic resonance imaging revealed a well circumscribed intraconal mass located inferonasally in the left orbit. Systemic examination revealed a lesion similar to LM on the left hard palate. The left conjunctival mass was excised subtotally. Subsequently, a transconjunctival anterior orbitotomy was performed and the left orbital mass was completely removed intact. Histopathologically, the conjunctival mass was diagnosed as LM and the orbital mass as venous malformation (VM). This case represents a rare coexistence of histopathologically proven conjunctival LM and orbital VM as well as a presumed LM of the hard palate, all 3 lesions occurring in the ipsilateral midface area.

Çalışmaya dahil edilen 108 olgunun 64'ü (%59.3) kadın, 44'ü (%40.7) erkekti. Ortalama tanı yaşı 50.8 (12-83) idi. 57 (%52.8) hastada sol, 51 (%47.2) hastada sağ göz tutulumu mevcuttu. Olguların 56'sına (%51.9) PLSU, 40'ına (%37.0) PPV ile TRB, 8'ine (%7.4) PPV ile endorezeksiyon, 4'üne (%3.7) İİAB uygulandı. PLSU, TRB, endorezeksiyon ve İİAB gurubunda ortalama tümör taban çapı sırasıyla 5.8x3.4 mm, 12.0x9.8 mm, 7.8x7.7 mm ve 12.0x11.8 mm ve ortalama tümör kalınlığı sırasıyla 3.3 mm, 4.9 mm, 3.9 mm ve 8.3 mm idi. PLSU uygulanan grupta preoperatif görme keskinliği logMAR skalasına göre 0-1.9 (ortalama 0.56) idi. Tümörlerin 23'ü (%41.1) iridosiliyer, 18'i (%32.1) iris, 8'i (%14.3) siliyer cisim, 4'ü (%7.1) siliyokoroid, 3'ü (%5.4) ise iridosiliyokoroid tutulumu göstermekteydi. 15 (%26.8) olguda superior, 8 (%14.3) olguda inferotemporal, 8 (%14.3) olguda inferonazal, 7 (%12.5) olguda nazal, 6 (%10.7) olguda temporal, 6 (%10.7) olguda inferior, 4 (%7.1) olguda superonazal, 2 (%3.6) olguda superotemporal kadranda yerleşimli tümör vardı. Tüm olgularda kitle total eksize edildi. Histopatolojik tanı 30 (%53.6) olguda melanom, 13 (%23.3) olguda nevüs, 4 (%7.1) olguda melanositom, 6 (%10.7) olguda stromal kist, 2 (%3.6) olguda Fuchs adenomu, 1 (%1.8) olguda invaziv meme kanseri metastazı olarak raporlandı. Postoperatif dönemde 21 (%37.5) olguda katarakt, 15 (%26.8) olguda vitreus hemorajisi, 10 (%17.9) olguda skleral incelme, 6 (%10.8) olguda hifema, 3 (%5.4) olguda sekonder glokom, 3 (%5.4) olguda geçici göz içi basınç artışı, 3 (%5,4) olguda periferik ön sineşi, 1 (%1.8) olguda büllöz keratopati gelişti. PLSU sonrası 5 olguya adjuvan Ru-106 plak radyoterapisi yapıldı. Postoperatif dönemde son muayenedeki görme keskinliği logMAR skalasına göre 0-1.9 (ortalama 0.67) idi. Ortalama 40.4 aylık izlem boyunca, PLSU uygulanan 1 olguda karaciğer metastazı, 1 olguda ise nüks izlendi.TRB uygulanan gruptaki olguların preoperatif görme keskinliği logMAR skalasına göre 0-1.9 (ortalama 0.64) idi. Sitopatolojik tanı 29 (%72.5) olguda melanom, 2 (%5.0) olguda küçük hücreli dışı akciğer kanseri metastazı, 1 (%2.5) olguda adenom, 1 (%2.5) olguda küçük hücreli akciğer kanseri metastazı, 1 (%2.5) olguda invaziv meme kanseri metastazı, 1 (%2.5) olguda retinal astrositik hamartom, 1 (%2.5) olguda da pseudoneoplastik gliozis olarak raporlandı. 4 (%10.0) olgunun sitopatolojik inceleme sonucunda yaşa bağlı makula dejenerasyonu ile uyumlu bulgular saptandı. Postoperatif dönemde görülen komplikasyonlar 16 (%40.0) olguda vitreus hemorajisi, 2 (%5.0) olguda retina dekolmanı, 1 (%2.5) olguda hifema, 1 (%2.5) olguda glokom, 1 (%2.5) olguda maküler hol idi. Ortalama 11.1 (1-55) aylık izlem süresi boyunca 9 (%22.5) göze enükleasyon uygulandı. 1 olguda karaciğer metastazı gelişti. Hiçbir olguda ölüm görülmedi. Endorezeksiyon yapılan olguların preoperatif görme keskinliği logMAR skalasına göre 0.5-1.9 (ortalama 1.1) idi. Endorezeksiyon öncesi 5 olguya TTT, 3 olguya Cyberknife radyocerrahisi, 2 olguya İyod-125 plak radyoterapisi ve 1 olguya Ru-106 radyoterapisi uygulandı. Sitopatolojik inceleme sonrası 6 olguda melanom, 1 olguda retina kapiller hemanjomu, 1 olguda ise vazoproliferatif tümör tespit edildi. Olguların son muayenedeki görme keskinliği logMAR skalasına göre 0-1.9 (ortalama 1.2) arasında idi. Postoperatif dönemde sitopatoloji raporu melanom olarak raporlanmış 1 olguda total retina dekolmanı gelişti, USG'de nüks düşündüren bulgu görüldüğü için bu olguya enükleasyon, daha sonra orbita dokusunda nüks geliştiği için ekzenterasyon yapıldı, takiplerde karaciğer ve kemik metastazı gelişti. Yazım sırasında bu hasta hayattaydı. 1 hastada daha karaciğer metastazı görüldü ve bu hasta ex oldu. Endorezeksiyon grubunda melanom tanısı almış 6 olgudan 5'inin (%83.3) gözü korundu. Bu grupta toplam göz korunma ve sağkalım oranı %87.5 idi.Hastaların 4'üne İİAB uygulandı. Bu olguların preoperatif görme keskinliği logMAR skalasına göre 0.1-1.0 (ortalama 0.6) arasında idi. Olguların sitopatolojik inceleme sonucu 2 olguda melanom, 1 olguda koroid hemanjiomu, 1 olguda ise melanositom olarak raporlandı. 3 olguya enükleasyon uygulandı. Olguların hiç birinde nüks, metastaz ve ölüm görülmedi.Çalışmamızda ortalama 28.6 aylık izlem süresi boyunca toplam göz korunma oranı %87.0, sağkalım oranı ise %99.1 idi. Of the 108 cases included in the study, 64 (59.2%) were female and 44 (40.7%) were male. The mean age at diagnosis was 50.8 (12-83) years. PLSU was done in to 56 patients (51.9%), TRB with PPV in 40 (37.0%), endoresection with PPV in 8 (7.4%), and fine needle aspiration biopsy (FNAB) in 4 (3.7%). The mean tumor base diameters in PLSU, TRB, endoresection and FNAB groups were 5.8x3.4 mm, 12.0x9.8 mm, 7.8x7.7 mm and 12.0x11.8 mm; and the mean tumor thicknesses were 3.3 mm, 4.9 mm, 3.9 mm and 8.3 mm, respectively. Preoperative visual acuity in the PLSU group was 0-1.9 (mean 0.56) according to the logMAR scale. There were iridociliary involvement in 23 (41.1%) tumors, only iris in 18 (32.1%), only ciliary body in 8 (14.3%), ciliochoroidal in 4 (7.1%), and iridociliocoroidal in 3 (5.4%). With respect to location, 15 (26.8%) tumors were superior, 8 (14.3%) were inferotemporal, 8 (14.3%) were inferonasal, 7 (12.5%) were nasal, 6 (10.7%) were temporal, 6 (10.7%) were inferior, 4 (10.7%) were superonasal, and 2 (%3.6) were superotemporal. The lesion was totally excised in all cases. Histopathologic diagnoses were melanoma in 30 (53.6%), nevus in 13 (23.3%), Fuchs adenoma in 2 (3.6%), melanocytoma in 4 (7.1%), stromal cyst in 6 (10.7%), invasive breast cancer metastasis in 1 (1.8%). Postoperative complications were cataract in 21 (37.5%) cases, vitreous hemorrhage in 15 (26.8%), scleral thinning in 10 (17.9%), hyphema in 6 (10.8%), secondary glaucoma in 3 (5.4%), temporary increase in intraocular pressure in 3(5.4%), peripheral anterior synechia in 3 (5.4%), and bullous keratopathy in 1 (1.8%). Adjuvant Ru-106 plaque radiotherapy was performed in 5 patients after PLSU. Postoperative visual acuity was 0-1.9 (mean 0.67) according to the logMAR scale. During a mean follow-up of 40.4 months, 1 patient had liver metastasis and 1 patient had recurrence. In the TRB group, preoperative visual acuity was 0-1.9 (mean 0.64) according to the logMAR scale. According to cytopathologic diagnosis, 29 (72.5%) cases had melanoma, 2 (5.0%) had non-small cell lung cancer metastasis,1 (2.5%) had adenoma, 1 (2.5%) had small cell lung cancer metastasis, 1 (2.5%) had invasive breast cancer metastasis, 1 (2.5%) had retinal astrocytic hamartoma, and 1 (2.5%) had pseudoneoplastic gliosis. Cytopathological examination of 4 (10.0%) cases revealed findings consistent with age-related macular degeneration. Postoperative complications were vitreous hemorrhage in 16 (40.0%) cases, retinal detachment in 2 (5%), hyphema in 1 (2.5%), glaucoma in 1 (2.5%), and macular hole in 1 (2.5%). During the mean follow-up period of 11.1 (1-55) months, 9 (22.5%) eyes were enucleated. One patient developed liver metastasis. There were no deaths. Preoperative visual acuity was 0.5-1.9 (mean 1.1) according to the logMAR scale in the endoresection group. Before endoresection, 5 patients underwent TTT, 3 patients underwent Cyberknife radiosurgery, 2 patients underwent Iodine-125 plaque radiotherapy, and 1 patient underwent Ru-106 radiotherapy. Cytopathology revealed melanoma in 6 cases, retinal capillary hemangioma in 1 case, and vasoproliferative tumor in 1 case. Visual acuity at the final examination was between 0-1.9 (mean 1.2) according to the logMAR scale. In the postoperative period, one patient whose cytopathology report was reported as melanoma developed total retinal detachment. Because of the findings suggesting tumor recurrence on USG, enucleation was done in this case. Subsequently, exenteration was performed because of orbital tumor recurrence. During follow-up period, liver and bone metastasis developed in this patient. This patient was alive with metstasis at the time of this writing. One patient developed liver metastasis and eventually expired. In the endoresection group, 5 (83.3%) eyes of 6 cases with melanoma were preserved. Total eye protection and survival rate was 87.5% in this group. FNAB was applied to 4 patients. The preoperative visual acuity of these cases was between 0.1-1.0 (mean 0.6) according to the logMAR scale. Cytopathologic examination revealed melanoma in 2 cases, choroidal hemangioma in 1 case and melanocytoma in 1 case. Enucleation was performed in 3 cases. There was no recurrence, metastasis or death.In our study, total globe salvage rate was 87.0% and survival rate was 99.1% during the mean follow-up period of 28.6 months. 87

Amaç: Koroid, retina pigment epiteli, retina ve optik disk tümörlerinin tanısında optik koherans tomografi (OKT) ve fundus otoflöresans görüntüleme (FOF) bulgularını tanımlamak.Gereç ve Yöntem: Ankara Üniversitesi Tıp Fakültesi Göz Hastalıkları Anabilim Dalı Tümör Servisi'nde Mart 2016 – Mart 2019 tarihleri arasında retina ve koroid tümörü tanısı alan 110 hastanın 113 gözü çalışmaya dahil edildi. Hastaların tanı anında çekilen OKT ve FOF görüntüleri retrospektif olarak incelendi.Bulgular: Koroid nevüsü, koroid malign melanomu (KMM), sınırlı koroid hemanjiomu (SKH) ve koroid osteomunda, OKT'de kubbe şekilli kontur izlenirken, koroid metastazlarında inişli çıkışlı kontur izlendi. SKH hariç diğer koroid tümörlerinde koroid kompresyonu izlendi. KMM, FOF görüntülerde genellikle hiperotoflöresans gösterirken, diğer koroid tümörleri çoğunlukla hipootoflöresans ya da izootoflöresans gösterdi. Retina pigment epitelinin konjenital hamartomunda OKT'de düz kontur, lezyon sınırında başlayan elipsoid zon atrofisi ve retina pigment epitel hipertrofisi, FOF görüntülerde ise hipootoflöresans izlendi. Retina ve retina pigment epitelinin kombine hamartomunda OKT'de preretinal fibrozis, vitreoretinal traksiyon ve retina disorganizasyonu, FOF görüntüde ise hipootoflöresans saptandı. Optik disk melanositomunda OKT'de parmak şekilli kontur izlendi. Vitreoretinal lenfoma OKT'de farklı retina katlarında infiltrat şeklinde tutulum gösterdi. Retinal astrositik hamartomda lezyon içinde güve yeniği şeklinde multiple kistik boşluklar ya da kaviteler izlendi.Sonuç: OKT ve FOF görüntüleme arka segment tümörlerinin ayırıcı tanısında yol göstericidir. Purpose: To describe the findings of optical coherence tomography (OCT) and fundus autofluorescence (FAF) imaging in choroidal tumor, tumors of the retina pigment epithelium, retinal and optic disc tumors.Material and Methods: 113 eyes of 110 patients diagnosed as retinal and choroid tumors between March 2016 and March 2019 in Ankara University School of Medicine, Department of Ophthalmology were included in this study. OCT and FAF images taken at the time of diagnosis were reviewed retrospectivelyResults: In choroidal nevi, choroidal malignant melanoma (CMM), circumscribed choroidal hemangiomas (CCH) and choroidal osteoma, dome-shaped contour was observed, while choroidal metastases showed lumpy bumpy contour on OCT. Choroidal compression was observed in all choroidal tumors except CCH. While CMM generally showed hyperotofluorescence on FAF images, other choroid tumors mostly showed hypootofluorescence or isootofluorescence. Congenital hamartoma of the retinal pigment epithelium revealed a flat contour, ellipsoid zone atrophy immediately at the lesion border and retinal pigment epithelial hypertrophy on OCT and hypootofluorescence on FOF images. Pretetinal fibrosis, vitreoretinal traction and retinal disorganization were detected on OCT in the combined hamartoma of the retina and retinal pigment epithelium, and hypootofluorescence was prominent in the FOF images. Optic disc melanocytoma showed thumb-shaped contour on OCT. Vitreoretinal lymphoma showed inftrates at the different layers of retina. In retinal astrocytic hamartoma, moth-eaten appearance of multiple cystic spaces or cavities were observed on OCT.Conclusion: Characteristic and common findings of posterior segment tumors in OCT and FAF guide clinicans in differential diagnosis. 106

Retinoblastomu taklit eden lezyonların türü (pseudoretinoblastom), sıklığı ve uygulanan tedavi yöntemlerini araştırmaktır.Gereç ve YöntemRetrospektif olarak Ekim 1998- Ağustos 2014 tarihleri arasında 16 yıllık bir süre içinde retinoblastom şüphesi ile oftalmik onkoloji servisine yönlendirilen 335 hasta değerlendirildi. Detaylı anamnez, fizik muayene, göz ile görülür oküler bulgular, biyomikroskopik muayene, ve genel anestezi altında skleral çökertme ile binoküler indirekt fundus muayenesi tüm olgulara uygulandı.BulgularRetinoblastom şüphesi ile yönlendirilen 335 hastadan 262'sinde (%78) retinoblastom ve 73'ünde (%22) pseudoretinoblastom saptandı. Pseudoretinoblastom olan olgularda 20 farklı hastalık saptandı ve bunların içinde en yaygın 5 hastalık persistan hiperplastik primer vitreus(PHPV; n =18 ;%24.3),Coats hastalığı(n = 17;%23.0), retinal distrofi (n= 6 ;%8.1), astrositik hamartom ( n= 6; %8.1),koroidal kolobomu ( n=4; %5.4) idi.Pseudoretinoblastom tanısı ile tedavi yapılan hastalara kriyoterapi (n=11; %13.5), laser fotokoagülasyon (n=9; %10.8), enükleasyon (n=7; %9.5), intravitreal Anti-VEGF (n=4; %5.4), fotodinamik tedavi (n=2; %2.8), kemoterapi (n=2; %2.8), fakoemülsifikasyon (n=2; %2.8), transpupiller termoterapi (n=1; %1.4), orbitotomi (n=1; %1.4) ve vitrektomi (n=1; %1.4)uygulandı.SonuçPseudoretinoblastomda en yaygın hastalıklar PHPV ,Coats hastalığı ve retinal distrofidir. Pseudoretinoblastom tanısı alan 73 hastanın 45'sine(%63) izlem ile takip önerildi ve 28'sına(%37) tedavi uygulandı. 8.SUMMARYPurposeTo determine the types and frequency of ocular conditions that simulate retinoblastoma (pseudoretinoblastoma) and treatment methods used inthe management of these conditions.Material- MethodRetrospective chart review of 335 retinoblastoma-related patients seen by the ophthalmic oncology service during a 16-year period from October 1998 to August 2014. For all patients detailed history, external ocular examination, slit lamp biomicroscopy and binocular indirect ophthalmoscopy with scleral indentation were performed under anesthesia in order to diagnose the lesion.ResultsOf 335 patients referred for management of retinoblastoma, 262 patients (78%) had retinoblastoma and 73 patients (22%) had simulating lesions (pseudoretinoblastomas).There were 20 different pseudoretinoblastoma conditions, and the 5 most common included persistent hyperplastic primery vitreous (PHPV; n=18;%24.3), Coats disease (n=17; %23.0), retinal dystrophy (n=6;%8.1), astrosytic hamartoma (n=6; %8.1) and choroidal coloboma (n=4; %5.4).The patients with pseudoretinoblastomas were treated with cryotherapy (n=11; %13.5), laser photocoagulation (n=9; %10.8), enucleation ( n=7; %9.5), intravitreal Anti-VEGF (n=4; %5.4), photodynamic therapy (n=2; %2.8), chemotherapy (n=2; %2.8), phacoemulsification (n=2; %2.8) transpupillary thermotherapy (n=1; %1.4), orbitotomy (n=1; %1.4) and vitrectomy (n=1; %1.4).ConclusıonsThe most common pseudoretinoblastomas include PHPV, Coats disease, and retinal dystrophy. Of 73 patients with pseudoretinoblastoma 45 (%63) were followed up with serial examinations while 28 (%37) underwent treatment. 85

Tiroid oftalmopati, Graves Hastalığı'nın en önemli tiroid dışı bulgusu olup göz tutulumu sıklıkla tiroid fonksiyon bozukluğuna eşlik eder, ancak klinik oftalmopati ötroid graves varlığında da ortaya çıkabilir. Tiroid oftalmopati, otoimmün bir hastalık olup tiroide ve retroorbital dokuya ait ortak bir antijene karşı antikor cevabı oluşmaktadır. Retroorbital dokudaki fibroblastlar bu antijenin primer bölgesi gibi görünmektedir. Otoimmün tiroid hastalığı bulunan kişilerde göz şikayetleri ile sık karşılaşılmakta, ancak olguların çoğunda hafif veya orta şiddette klinik oftalmopati görülmektedir. Az sayıda olguda ise optik nöropati veya kornea perforasyonu neticesinde ciddi görme kaybına neden olabilen ciddi oftalmopati izlenmektedir. Tanı genellikle klinik bulgulara dayanılarak konulur; tiroid fonksiyon testleri, tiroid antikorları ve orbita görüntüleme yöntemleri ise tanıda yardımcıdır. Günümüzde oftalmopati gelişimini engelleyecek bir tedavi yönteminin bulunmaması hastalığın tedavisinde özellikle hafif olgularda semptomatik yaklaşımı gündemde tutmaktadır. Ancak, orta veya ağır TO olgularında birden fazla tıbbi ve/veya cerrahi tedavi gerekebilir. Bu hastaların yakından takip edilmesi ve tedavilerinin doğru sırayla uygulanması çok önemlidir. Bu çalışmada, tiroid oftalmopati tanısı alan ve üçüncü basamak referans merkezi olarak endokrinoloji ve diğer göz kliniklerinden kliniğimize refere edilen olguların takip ve tedavi sonuçlarının tartışılması amaçlandı.Hastaların kayıtları retrospektif olarak incelendi ve EUGOGO kriterlerine göre bilimsel detayların tamamlanabildiği 168 olgunun 306 gözü çalışmaya dahil edildi. Olguların yaş ortalaması 42,3 ± 12,4 yıl idi. Olguların 105'i kadın, 63'ü erkek idi. Olguların %54,2'sinde sigara kullanım öyküsü mevcuttu. Olguların %39,2'sinin birinci veya ikinci derece akrabasında tiroid hastalığı bulunmaktaydı. Tiroid hastalıkları incelendiğinde, 135 olguda (%80,4) graves hastalığı saptanmış olup, kalan 33 olguda diğer tiroid hastalıkları (hashimoto tiroiditi %8,3, multinodüler guatr %8,3 ve tiroid kanseri %3) bulunmaktaydı. Göz tutulum ve tiroid hastalığı süreleri birlikte değerlendirildiğinde, 91 olguda (%54,2) klinik oftalmopati ile tiroid hastalığı tanılarının eş zamanlı olduğu görüldü. Gözlerin %68'inde kapak retraksiyonu, %54,8'inde aşağı bakışta kapak gecikmesi, %6,9'unda lagoftalmus ve %2'sinde pitozis saptandı. Ortalama proptozis ölçümü 20,3 ± 3,2 mm olup, proptosis gözlerin %47'sinde mevcuttu. Gözlerin %32,4'ünde hareket kısıtlılığı görülürken, olguların sadece %7,1'inde manifest kayma (4 hT, 3 ET+hT, 2 ET, 3 XT) tespit edildi. Kompresif optik nöropati %2 oranında saptanırken, ciddi korneal tutulum hiçbir olguda mevcut değildi. Hastalık şiddeti değerlendirildiğinde, 197 gözde (%64,4) hafif, 103 gözde (%33,6) orta-ciddi ve 6 gözde (%2) ise çok ciddi tutulum izlendi. Yüz gözde (%32,6) aktif TO bulguları izlenirken, 206 gözde (%67,4) TO inaktif fazda idi. Hastaların %20'sine (34 olgu) tedavi uygulandı. En çok uygulanan tedavi sistemik steroidler (%12) olup bunu orbita dekompresyonu (%5), orbital radyoterapi (%2) ve topikal guanetidin uygulaması (%1) izledi.Sonuçta, göze yönelik tedaviler hastalığın şiddet ve aktivitesi yanında, hastaların ek sistemik hastalıkları, tedavi uyumu ve endokrinoloji bölümünün önerileri de göz önünde bulundurularak planlanmalıdır. Hastalarda spontan regresyon olabileceği unutulmamalı ve tedavi aşamasında fazla proaktif davranılmamalıdır.Anahtar kelimeler: klinik aktivite skorlaması, orbital dekompresyon, orbital radyoterapi, sigara, tiroid oftalmopati. Thyroid ophthalmopathy, is the most important extra thyroidal finding of Graves disease. Ocular involvement often accompanies with thyroid dysfunction but ophthalmopathy can also occur in the presence of euthyroid Graves.Thyroid ophtalmopathy, is an autoimmune disorder that an antibody response was seen against a common antigen of thyroid gland and retroorbital tissue. Orbital fibroblasts seem to be the primary site of this antigen.Eye problems are common in cases with autoimmune thyroid diseases, but mild or moderate clinical ophthalmopathy is seen mostly. In a few cases there is severe ophthalmopathy that corneal perforation or optic neuropathy leads to severe vision loss. Diagnosis is based on the clinical findings, however thyroid function tests, thyroid autoantibodies and orbital imaging methods are useful.Symptomatic approach is preferred especially in mild cases since there is no treatment modality that can prevent ophtalmopathy development. However, many medical and/or surgical treatments may be necessary in cases with moderate or severe TO. These patients should be followed up frequently and treated in the correct order.The aim of this study was to discuss the follow up and treatment results of cases who were referred to our clinic as the tertiary reference center from endocrinology and other eye clinics.Medical records of the patients were evaluated retrospectively and 306 eyes of 168 patients that scientific details can be completed according to the EUGOGO criteria were included in the study. The mean age of the patients was 42.3 ± 12.4 years. Sixty three men and 105 women participated to the study. Smoking habits were noted in 54.2% of the cases. There was a thyroid disease in the first or second degree relatives in 39,2% of the cases. Graves disease was found in 135 cases (80.4%), the remaining 33 cases had other thyroid diseases (Hashimoto's thyroidits 8.3%, multinodular goiter 8.3% and thyroid cancer 3%). When the durations of eye and thyroid diseases were evaluated together, it was observed that the times of diagnosis for both eye and thyroid diseases were simultaneous in 54,2% of the cases. There was lid retraction in 68%, lid lag in 54.8%, lagophtalmus in 6.9% and pitosis in 2% of the eyes. The mean proptosis was 20.3 ± 3.2 mm and proptosis was present in 47% of the eyes. Movement was limited in 32.4% of the eyes, but manifest deviation (4 ht, 3 hT+ET, 2 ET, 3 XT) was detected only in 7.1% of the cases. While compressive optic neuropathy was seen in 2% of the cases, severe corneal involvement was not available in any case. Considering the severity of the disease, TO is mild in 197 eyes (64.4%), moderate to severe in 103 eyes (33.6%) and very severe in 6 eyes (2%). While TO is active in 100 eyes (32.6%), it was inactive in 206 eyes (67.4%). Treatment was applied to 20% of the cases. Systemic steroid was the most common treatment modality and followed by orbital decompression (5%), orbital radiotherapy (2%) and topical guanethidine (1%) respectively.As a result, treatment should be planned taking account of patients' compliances and other systemic diseases besides the severity and activity of TO. Patients should be followed up in a collaboration with endocrinology clinic. Patients should not be treated very proactively remembering the possibility of spontanenous regression.Key words: clinical activity scoring, orbital decompression, orbital radiotherapy, smoking, thyroid ophtalmopathy. 83