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Purpose: To report wide-field fundus imaging and fluorescein angiography findings in conditions mimicking retinoblastoma (pseudoretinoblastoma). Methods: The clinical and imaging records of 28 patients (36 eyes) imaged with RetCam 3 (Clarity Medical Systems, Inc) wide-field fundus photography and fluorescein angiography who were diagnosed as having various pseudoretinoblastoma disorders between February 2020 and August 2021 were retrospectively evaluated. Results: Most patients were referred with suspicion of retinoblastoma. Other patients had unresolved eye conditions and received initial treatment elsewhere and they were referred later for exclusion of retinoblastoma. On RetCam fundus photography and fluorescein angiography, pseudoretinoblastoma conditions were diagnosed as Coats disease (9 eyes), congenital glaucoma (3 eyes), persistent fetal vasculature (3 eyes), familial exudative vitreoretinopathy (2 eyes), retinopathy of prematurity (2 eyes), myelinated retinal nerve fibers (2 eyes), optic nerve hypoplasia (2 eyes), oculocutaneous albinism (2 eyes), meridional fold (2 eyes), combined hamartoma of retina and retinal pigment epithelium (2 eyes), grouped congenital hypertrophy of the retinal pigment epithelium (1 eye), retinal astrocytic hamartoma (1 eye), morning glory syndrome (1 eye), optic glioma (1 eye), giant choroidal nevus (1 eye), vasculitis (1 eye), and chorioretinitis (1 eye). RetCam imaging and fluorescein angiography demonstrated telangiectasias, microaneurysms, submacular exudation/fibrosis, capillary non-perfusion, and exudation posterior to telangiectasias in Coats disease. Hypofluorescent optic discs, peripheral non-perfusion, and vascular leakage were observed in congenital glaucoma. Large areas of capillary non-perfusion, poor foveal morphology/formation, elongated ciliary processes with hyperfluorescent central core, and hyperfluorescent fibrovascular stalk at the posterior pole were hallmarks of persistent fetal vasculature. Conclusions: RetCam wide-field fundus imaging and fluorescein angiography play an important role in the diagnosis of conditions simulating retinoblastoma. [ J Pediatr Ophthalmol Strabismus . 2023;60(1):60–74.]

A 71-year-old male presented with painless and slowly progressive proptosis of the right eye. The patient had no history of cancer and was in good health. Orbital magnetic resonance imaging showed a well-demarcated, slightly lobulated intraconal orbital mass hypo-isointense on T1- weighted and isointense on T2-weighted images, demonstrating contrast enhancement in the right orbit. The lesion had a central cavitary region. An anterior orbitotomy with lower lid crease incision was performed and the lesion was excised in toto. Histopathologic examination revealed diffuse infiltration of monomorphic population of small lymphoid cells. Immunohistochemical staining demonstrated diffuse and strong positivity for CD20, CD5, and cyclin D1. Histopathological and immunohistochemical findings were consistent with mantle cell lymphoma. Positron emission tomography-computed tomography did not show systemic involvement. The patient underwent orbital external beam radiotherapy (36 Gy). There was no recurrence and/or systemic involvement at 2.5 years follow-up.

Purpose To investigate the distribution of pseudoretinoblastoma (PSRB) cases based on gender, age, and laterality Materials and Methods The clinical records of 607 patients (851 eyes) who were referred for diagnosis of retinoblastoma or simulating conditions between October 1998 and May 2021 were retrospectively evaluated. Patients were stratified by age as follows: ≤1 year, >1–3 years, >3–5 years, and >5 years. Results Of 190/607 PSRB patients, 129 (67.9%) were males and 61 (32.1%) females ( p = 0.001). The 3 most common diagnoses in males were Coats disease (20.2%), persistent fetal vasculature (PFV, 14.0%), and chorioretinal coloboma (6.2%). In females, the 3 most common diagnoses included PFV (21.3%), retinal dysplasia, congenital glaucoma, and combined hamartoma (each 6.6%). PFV was the most common diagnosis in ≤1 year old patient group (26.6%). Coats disease and PFV were the most common diagnoses in >1–3 years old patient group (each 16.7%). Coats disease was the most common diagnosis in >3–5 years old (30.8%) and >5 years old patient groups (13.1%). PSRBs were unilateral in 121/190 (63.7%) patients. Coats disease usually presented unilaterally (p Discussion PSRB diagnoses show differences depending on gender, age, and laterality. In our study, the most common PSRB lesions were Coats disease in males and PFV in females. PFV was the most frequent diagnosis in ≤3 years and Coats disease in >3 years of age groups. Coats disease and PFV were the most common unilateral and bilateral PSRB diagnoses respectively.

Surgery in intraocular tumors is done for excision/biopsy and the management of complications secondary to the treatment of these tumors. Excision/biopsy of intraocular tumors can be done via fine-needle aspiration biopsy (FNAB), transretinal biopsy (TRB), partial lamellar sclerouvectomy (PLSU), and endoresection. FNAB, TRB, and PLSU can be used in tumors that cannot be diagnosed by clinical examination and other ancillary testing methods. PLSU is employed in tumors involving the iridociliary region and choroid anterior to the equator. Excisional PLSU is performed for iridociliary and ciliary body tumors with less than 3 clock hours of iris and ciliary body involvement and choroidal tumors with a base diameter less than 15 mm. However, for biopsy, PLSU can be employed with any size tumor. Endoresection is a procedure whereby the intraocular tumor is excised using vitrectomy techniques. The rationale for performing endoresection is based on the fact that irradiated uveal melanomas may cause complications such as exudation, neovascular glaucoma, and intraocular pigment and tumor dissemination (toxic tumor syndrome), and removing the dead tumor tissue may contribute to better visual outcome. Endoresection is recommended 1-2 weeks after external radiotherapy. Pars plana vitrectomy is also used in the management of complications including vitreous hemorrhage, retinal detachment, and epiretinal membrane that can occur after treatment of posterior segment tumors using radiotherapy and transpupillary thermotherapy. It is important to make sure the intraocular tumor has been eradicated before embarking on such treatment.

This study aims to systematically review the reported literature on the use of anterior segment optical coherence tomography (AS-OCT) in ocular surface tumours and simulating lesions. A systematic literature search was done using PubMed, Scopus, and Web of Science databases between January 2002 and December 2021. On AS-OCT, ocular surface squamous neoplasia typically demonstrate epithelial thickening, epithelial hyperreflectivity, and an abrupt transition between normal and abnormal epithelium. Conjunctival nevi usually show mildly hyperreflective epithelium of normal thickness, internal hyperreflectivity, and intralesional cysts which is the hallmark of this tumour. Primary acquired melanosis presents with normal thickness epithelium, basal epithelial hyperreflectivity, and absence of cysts. Conjunctival melanoma demonstrates hyperreflective normal/thickened epithelium, hyperreflective basal epithelium, internal hyperreflectivity, and absence of intralesional cysts. Conjunctival lymphoma shows homogenous, low-medium reflective subepithelial lesions with smooth borders, and dot-like infiltrates. Benign reactive lymphoid hyperplasia findings are similar to lymphoma but the infiltrates are more hyperreflective compared to lymphoma. Pterygium shows thickened conjunctival epithelium, epithelial hyperreflectivity, and subepithelial wedge-shaped hyperreflective tissue separated from the overlying epithelium by a cleavage plane. Pinguecula demonstrates mildly thickened epithelium and similar findings with pterygium but does not extend beyond the corneal limbus. This review shows that AS-OCT, as a noninvasive tool, has potential uses in the differential diagnosis of ocular surface tumours and simulating lesions. Major limitations of AS-OCT include limited visualization of the posterior border of thick, keratinized, and pigmented tumours and lack of assessment of large conjunctival tumours in a single cut.摘要: 本研究旨在系统性综述已发表的有关前节光学相干断层扫描技术(AS-OCT)用于眼表肿瘤和模拟病变中的文献。我们使用 PubMed、Scopus和Web of Science数据库对发表于2002年1月至2021年12月期间的文献进行了系统检索。眼表鳞状肿瘤在AS-OCT上通常表现为上皮增厚、上皮高反射性, 以及正常和异常上皮之间的突变。结膜痣通常以为厚度正常的上皮轻度高反射灶, 内部高反射性, 以及病灶内囊肿为特征。原发性获得性黑色素瘤表现为上皮厚度正常, 基底上皮高反射性灶及无囊肿。结膜黑色素瘤表现为正常/增厚上皮的高反射, 基底上皮高反射, 内部呈高反射, 无病灶内囊肿。结膜淋巴瘤表现为均匀的、中-低度反射的上皮下病灶, 边界光滑并伴随点状浸润。良性反应性淋巴增生的表现与淋巴瘤相似, 但与淋巴瘤相比, 其浸润病灶表现为更高的反射性。翼状胬肉显示结膜上皮增厚, 上皮反射亢进, 上皮下楔形高反射组织与被覆上皮被裂隙面分开。睑裂斑表现为上皮轻度增厚, 与翼状胬肉类似, 但病灶不超过角膜边缘。本综述显示, AS-OCT作为一种无创工具, 可应用于眼表肿瘤的鉴别诊断。AS-OCT的局限性主要包括对厚的、角质化和色素性的肿瘤后缘的可视化有限, 以及单次断扫缺乏对大型结膜肿瘤的评估能力。.

We report a case of bilateral optic nerve head drusen complicated by choroidal neovascularization (CNV) in the left eye at presentation. The presence of optic disc and macular edema in addition to exudation led to the misdiagnosis of neuroretinitis at an outside medical center. Swept-source optical coherence tomography (SS-OCT) and SSOCT angiography were critical in establishing the diagnosis and follow-up in a noninvasive manner. Secondary CNV associated with optic nerve head drusen responded well to intravitreal injections of anti-vascular endothelial growth factor in the left eye. Asymptomatic nonexudative CNV that developed in the right eye during follow-up regressed spontaneously without treatment. [ Ophthalmic Surg Lasers Imaging Retina 2022;53:518–521.]

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 measuring0.5×0.5×0.5 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.

OBJECTIVES: To investigate the results of partial lamellar sclerouvectomy (PLSU) for anteriorly located uveal tumours. METHODS: We reviewed the tumour features, histopathologic findings, complications, visual acuity outcomes, eye preservation, metastasis, and mortality data of 56 cases with uveal tumours who underwent PLSU between February 1999 and February 2019. RESULTS: The mean largest tumour base diameters were 5.8 × 3.4 mm and the mean tumour thickness was 3.3 mm. Histopathologically, 30 (53.6%) eyes had malignant melanoma, 13 (23.2%) had nevus, 5 (8.9%) had iris stromal cyst, 4 (7.1%) had melanocytoma, 2 (3.6%) had Fuchs’ adenoma, 1 (1.8%) had iris pigment epithelial cyst, and 1 (1.8%) had invasive breast cancer metastasis. The most common postoperative complications included cataract in 21 (37.5%) eyes, vitreous haemorrhage in 15 (26.8%), scleral thinning in 10 (17.9%), and hyphema in 6 (10.7%). At a mean follow-up of 40.4 (range: 10–201) months, tumour recurrence was observed in 2/30 (6.7%) eyes with melanoma and 1/5 (20.0%) eye with iris stromal cyst. Eyes with recurrent melanoma were treated with enucleation. Liver metastasis developed in only 1 (3.3%) melanoma case. All patients were alive at the end of follow-up. CONCLUSIONS: PLSU is a successful treatment method for many anteriorly located uveal tumours. In our series, the overall tumour recurrence and globe salvage rates were 5.4% and 96.4% respectively. Among melanoma cases, the metastasis rate was 3.3% and survival rate was 100%.

Purpose To report RetCam fluorescein angiographic (FA) changes and correlation with clinical findings in the follow-up of retinoblastoma (RB) after frontline intravenous chemotherapy and other treatments. Methods Patients having RB who underwent post-treatment RetCam fundus photography and FA under general anesthesia between February 2020 and February 2022 were retrospectively analyzed. Results 78 eyes of 70 patients with RB were included. In 55 (70.5%) eyes with type 1, 2 or 3 regression patterns, the main tumor started to show hyperfluorescence in arterial phase in 24 eyes (43.6%), in early venous phase in 24 (43.6%) eyes, and in late venous phase in 7 (12.7%) eyes. Of thirty-six (46.2%) eyes with chorioretinal scars developing after focal treatments (cryotherapy and transpupillary thermotherapy), window defects and visible choroidal vessels were found in all eyes, scleral staining in 10 (27.8%), and gliosis on scar tissue in 8 (22.2%). Vitreous seeds showed hyperfluorescence if they were calcified (6 eyes, 7.7%) and hypofluorescence if non-calcified (11 eyes, 14.1%). Retrohyaloid seeds (11 eyes, 14.1%) were hypo/isofluorescent while subretinal seeds (6 eyes, 7.7%) were hyperfluorescent. Leakage from peripheral retinal vessels was detected in 14 (17.9%) eyes and non-perfusion in 6 (7.7%) eyes. Conclusion RetCam wide-angle FA is useful to evaluate the tumor and associated vascular and retinal changes after treatment in RB. Regressed tumors demonstrate later fluorescein uptake in venous phases. Fluorescein angiographic changes in chorioretinal scars include window defects, visibility of choroidal vessels, scleral staining, and leakage from vessels. Retinal vascular leakage and peripheral non-perfusion can be seen in eyes with regressed stable tumors.

Purpose To determine the effect of patient and tumor features and different treatments on eye removal (enucleation or exenteration) and metastasis in posterior uveal melanoma (PUM). Methods Retrospective analysis. Patient age (≤60 vs >60 years), sex (female vs male), visual acuity (VA, ≤20/40 vs >20/40), largest tumor basal diameter (LTBD), tumor thickness, tumor stage according to American Joint Committee on Cancer (AJCC) 8th edition, ciliary body involvement, distance to optic disc (OD)/fovea (≤3 mm vs >3 mm), OD involvement, and histopathology were evaluated. Primary treatment options were transpupillary thermotherapy, plaque radiotherapy, Cyberknife radiosurgery, exoresection, and eye removal. Risk factors for primary eye removal were determined using logistic regression test and those for secondary eye removal and metastasis with Cox regression analysis. Results Of 387 cases, 153 (39.5%) underwent primary eye removal. Multivariable risk factors for primary eye removal included AJCC tumor stage (p = 0.001, OR:4.586; p < 0.001, OR:34.545; p < 0.001, OR:103.468 for stages T2, T3, and T4 vs stage T1, respectively), and VA≤20/40 (p = 0.014, OR:2.597). Multivariable risk factors for secondary eye removal were VA≤20/40 (p = 0.019, RR:2.817) and AJCC stage T3 vs T1 (p = 0.021, RR:2.666). Eye preservation rates in patients undergoing eye-conserving treatments were 80.3%, 69.6%, and 51.5% at 5, 10, and 15 years, respectively. Metastasis-free survival rates were 81.0%, 73.0%, and 56.7% at 5, 10, and 15 years, respectively. Multivariable risk factors for metastasis included eye removal as primary treatment (p = 0.005, RR:2.828) and mixed type histopathology (p < 0.001, RR:4.804). Discussion Early diagnosis is crucial for both eye preservation and survival in PUM. Increasing AJCC tumor stage and lower VA were risk factors for eye removal in this study. Mixed type histopathology and primary eye removal were risk factors for metastasis.

Purpose: To evaluate the results of secondary intra-arterial chemotherapy (IAC) and/or intravitreal chemotherapy (IVC) as a salvage treatment for retinoblastoma (RB). Materials and Methods: The medical records of 31 (20 male, 11 female) cases (with 38 eyes) who underwent secondary IAC and/or IVC between February 2010 and June 2019 were retrospectively reviewed. Results: Thirteen (41.9%) cases had unilateral and 18 (58.1%) had bilateral RB. According to the International Classification of RB, 6 (15.8%) eyes had group B, 9 (23.7%) eyes had group C, 16 (42.1%) eyes had group D, and 7 (18.4%) eyes had group E RB at diagnosis. All patients underwent six-cycle intravenous chemotherapy as primary treatment and 8 eyes received external radiotherapy before IAC/IVC. Secondary IAC was performed in 21 (55.3%) eyes, IVC in 10 (26.3%) eyes, and IAC + IVC in 7 (18.4%) eyes. External radiotherapy was applied in 2 (5.3%) eyes after IAC/IVC, one of which was later enucleated. In total, 17 (44.7%) eyes undergoing secondary IAC/IVC treatments were enucleated. Metastasis and death were not observed in any case during the mean follow-up period of 59.3 (median 61, range: 10–98) months. Discussion: Although 60.5% of the eyes undergoing IAC/IVC consisted of groups D and E RB, globe salvage and survival rates were 55.3% and 100.0%, respectively. External radiotherapy was required in 5.3% of the eyes after IAC/IVC. In conclusion, IAC and IVC are safe and effective treatment methods in eyes with RB unresponsive to other eye-preserving treatments and those demonstrating recurrence.

OBJECTIVES: To report the swept-source optical coherence tomography angiography (SS-OCTA) findings in choroidal and retinal tumors. METHODS: A retrospective noncomparative interventional case series of 60 eyes having various choroidal and retinal tumors imaged with SS-OCTA (Topcon DR1 Triton Plus, Tokyo, Japan) between September 2018 and February 2020 was conducted. Inclusion criteria were tumor thickness

Aim To evaluate the demographic features, location, and histopathologic results in primary eyelid tumors. Methods We retrospectively reviewed the records of patients with primary eyelid tumor diagnosed and treated between November 1997 and June 2016 on our service. Results Nine hundred and eleven lesions from 874 patients were included in this study. Four hundred and forty-six (51.0%) of the patients were females and 428 (49.0%) were males. The mean age was 51.0y (range: 3mo-94y). The lesions were located in the upper eyelid (418 cases, 45.9%), lower eyelid (378 cases, 41.5%), medial canthus (89 cases, 9.8%), and lateral canthus (26 cases, 2.8%). Four hundred and seventy (51.6%) lesions were located on the right side and 441 (48.4%) on the left. Of the eyelid lesions, 666 (73.1%) were benign, 230 (25.2%) malignant, and 15 (1.6%) premalignant. When the eyelid tumors were classified according to their tissue or cell of origin, 527 (57.8%) of the lesions were found to be epidermal, 171 (18.8%) adnexal, 131 (14.4%) inflammatory and infectious, and 65 (7.1%) stromal. The most common benign lesions were squamous cell papilloma (139 lesions, 15.2%), intradermal nevus (97 lesions, 10.6%), epidermoid inclusion cysts (78 lesions, 8.6%), seborrheic keratosis (60 lesions, 6.6%), and inflammatory masses (59 lesions, 6.5%). Basal cell carcinoma was the most common malignant tumor (191 lesions, 21.0%) followed by squamous cell carcinoma (16 lesions, 1.8%), sebaceous carcinoma (14 lesions, 1.5%), and malignant melanoma (5 lesions, 0.5%). Conclusion In this study, 73.1% of eyelid lesions are benign and the remaining 26.9% are premalignant and malignant. Basal cell carcinoma is the most common tumor among all histopathological diagnosis followed by squamous papilloma.

To describe clinical, anterior segment optical coherence tomography (AS-OCT), in vivo confocal microscopy (IVCM), histopathologic, and immunohistochemical findings in a patient with multiple endocrine neoplasia type 2b (MEN 2b) syndrome.Retrospective case report of a patient with MEN 2b.A 31-year-old male diagnosed with MEN 2b presented with eye redness, burning, and visible conjunctival mass in both eyes. The patient's past medical history revealed that he underwent bilateral adrenalectomy and total thyroidectomy. Genetic testing revealed M918T heterozygous mutation in the RET proto-oncogene. Corrected visual acuity was 20/20 in both eyes. Anterior segment examination revealed bilateral thickened lid margins, ectropion, blepharitis, conjunctival injection, temporal and inferonasal subconjunctival lesions with corneal invasion, corneal neovascularization, and marked corneal nerves. AS-OCT showed a subepithelial mixed reflective lobular mass in both eyes. Hyperreflective and noticeable thickened stromal corneal nerves were observed on IVCM in the left eye. After incisional biopsy of the right perilimbal lesions, histopathological examination revealed that lesions consisted of spindle cells in hypocellular bundles with no atypia and mitosis. Immunohistochemical examination revealed diffuse staining with S100, focal staining with synaptophysin, and no staining with neurofilament protein. These findings were considered compatible with a benign nerve sheath tumor, probably schwannoma.We present clinical, AS-OCT, IVCM, histopathological, and immunohistochemical findings in a patient with MEN 2b. To our knowledge, this is the first case of a conjunctival schwannoma diagnosed histopathologically in MEN 2b.

Objectives: To report treatment results and complications of stereotactic radiosurgery in uveal malignant melanoma and to identify risk factors for development of radiation retinopathy. Materials and Methods: This was a retrospective study of 36 patients diagnosed with uveal melanoma between 2014 and 2019. Best corrected visual acuity, funduscopic findings, basal tumor diameter and tumor thickness were recorded at baseline and at follow-up visits at 3-month intervals. The response of tumors to stereotactic radiosurgery and complications were determined. Results: The mean basal diameter of tumor was 10.2 (range: 4.0-19.4, standard deviation [SD]: ±3.3) mm x 9.7 (range: 4.5-18.0, SD: ±3.3), tumor thickness was 5.1 (range: 2.0-11.0, ±2.4) mm at baseline. The mean follow-up period was 17.2 (range: 6-48, SD: ±10.43) months. The mean visual acuity was 0.5 (SD: ±0.3) logMAR before treatment and 0.6 (SD: ±0.3) logMAR after the mean follow-up period. The most common complications after stereotactic radiosurgery were cataract (38.9%) and radiation retinopathy (27.7%). There was a statistically significant relation between radiation retinopathy development and tumor distance from the optic disc (p=0.04). The rate of eye salvage was 83.3% in this study. Conclusion: Our short-term results show stereotactic radiosurgery was an effective and sustained treatment modality among the other eye conservation therapies.

OBJECTIVES To evaluate the long-term treatment outcomes in intraocular retinoblastoma (RB) including the associated factors for eventual treatment with external beam radiotherapy (EBRT) and enucleation as well as to analyse the risk factors for metastasis and death in extraocular RB. METHODS Retrospective analysis of 390 eyes from 256 (89.8%) intraocular RB and 29 (10.2%) extraocular RB cases diagnosed and treated between October 1998 and May 2018 at one of the largest tertiary care centers in Turkey. RESULTS Of 351 intraocular RB eyes, 53.3% had group D/E disease at presentation. 75 (21.4%) of 351 eyes underwent primary enucleation. Of the remaining 276 eyes undergoing eye-conserving treatments, 201 (72.8%) were salvaged. Most of these eyes were treated using intravenous chemotherapy and/or focal treatments [transpupillary thermotherapy (TTT) and cryotherapy] initially. EBRT was eventually required in 48 (17.4%) eyes and secondary enucleation in 75 (27.2%) eyes. At mean follow-ups of 76.7 and 39.7 months for intraocular and extraocular RB cohorts, respectively, 180 (46.2%) eyes underwent primary/secondary enucleation and exenteration. Overall, 13 cases developed metastasis and 9 died. Two patients with trilateral RB also expired. Multivariable risk factors for enucleation were the presence of vitreous seeds (p

This study investigates the demographic and clinical features of conjunctival tumours.Conjunctival tumours include a large spectrum of conditions ranging from benign lesions to aggressive, life-threatening malignancies. Knowing the distribution of conjunctival tumours by age and gender is important for reducing cancer morbidity.The clinical records of 375 patients (410 eyes) diagnosed with a conjunctival mass at a tertiary referral centre between February 1999 and November 2020 were retrospectively evaluated.Two-hundred-seventeen (57.9%) patients were male and 158 (42.1%) were female. Of 410 conjunctival tumours, 159 (38.8%) were benign, 106 (25.9%) premalignant, and 145 (35.4%) malignant. Overall, the 3 most common diagnoses were squamous cell carcinoma (SCC, 19.5%), conjunctival intraepithelial neoplasia (CIN, 18.3%), and naevus (17.8%). The most common benign, premalignant, and malignant tumours were naevus (n = 73/159, 45.9%), CIN (n = 75/106, 70.8%), and SCC (n = 80/145, 55.2%) respectively. Naevus was the most common tumour in ≤20 years and 20-40 years old patient groups (56.2% and 25.4% respectively). CIN was the most frequent tumour in patients aged 40-60 years (25.7%). SCC was the most common tumour in 60-80 years and 80 years old patient groups (44.3% and 80.0% respectively). The median patient age was greater in patients with malignant tumours (64.5 years) compared to patients with premalignant (55.5 years, p = 0.011) and benign tumours (22.0 years, p 0.001). Malignant tumours displayed larger base diameter, greater thickness, and intrinsic vessels compared to premalignant or benign lesions (p 0.001 for each parameter). Malignant tumours also displayed more amelanotic vs melanotic appearance (p 0.001) and limbal vs extralimbal bulbar location compared to benign lesions (p 0.001).Premalignant and malignant tumours comprised 61.2% of all conjunctival tumours and were usually detected in patients40 years of age in this study.

We report a case of unilateral varicella-zoster virus (VZV) related keratouveitis and papillitis. A 54-year-old male presented with headache, left forehead rash, and decreased vision in the left eye. Examination of the left eye showed visual acuity (VA) of 0.15, relative afferent pupillary defect, anterior stromal corneal edema, nongranulomatous keratic precipitates, a swollen and hyperemic optic disc. The patient could read 6/12 of the Ishihara plates in the left eye. Optical coherence tomography (OCT) showed optic disc edema and OCT angiography (OCTA) revealed increased peripapillary vascularity. Computerized visual field testing demonstrated an enlarged blind spot and lower altitudinal defect. Serum anti-VZV IgM antibody level was elevated. The patient was put on oral acyclovir and prednisone treatment. At 1-month follow-up visit, VA increased to 0.9 and color vision was 11/12 Ishihara plates. OCT showed resolution of optic disc edema and OCTA demonstrated normalization of the increased peripapillary vascularity. Computerized visual field test demonstrated improvement in the lower altitudinal visual field defect and normalization of the enlarged blind spot. Optic nerve involvement in VZV infection occurs rarely, especially in immunocompetent patients as in our case.

Purpose: To evaluate the risk factors leading to recurrence and new tumor (NT) development in patients with retinoblastoma after intravenous chemotherapy (IVC) and to review the treatment outcomes. Materials and methods: The records of 166 retinoblastoma cases (having 246 affected eyes) who underwent six-cycle IVC (vincristine, etoposide, and carboplatin) as primary treatment between October 1999 and August 2020 were reviewed retrospectively. Results: The mean ages at presentation were 9.0 (median: 8.0) and 9.2 (median: 8.5) months in cases with recurrence and NTs respectively. Recurrence was detected in 40 (16.3%) eyes, NTs in 29 (11.8%), and both recurrence/NTs in 24 (9.8%). The mean time elapsed till recurrence and NT was 10.7 months. Multivariable analysis showed that the factors predictive of recurrence were largest tumor base diameter (LTBD) >12 mm ( p = 0.039) and presence of subretinal seeds at diagnosis ( p = 0.043). Multivariable risk factors for the development of NTs were bilateral familial retinoblastoma ( p = 0.001) and presence of subretinal seeds at diagnosis ( p = 0.010). Mean follow-up was 80.1 (median: 72.5) months. By Kaplan-Meier analysis, the 1-, 3-, and 6-year recurrence and NT rates were 21.2%, 28.1%, and 28.7% and 14.9%, 22.6%, and 23.9% respectively. The most common treatment methods used for recurrent and/or NTs included cryotherapy, transpupillary thermotherapy, and intra-arterial chemotherapy. Enucleation was eventually required in 24/93 (25.8%) eyes. No patient developed metastasis. Discussion: Development of recurrence and/or NT after IVC was noted in 38% of all retinoblastoma eyes. Bilateral familial disease, LTBD >12 mm, and presence of subretinal seeds at baseline were risk factors for recurrence and NTs in this study.

Purpose: To review the authors' experience in the diagnosis of retinoblastoma and to explore the frequency of intraocular conditions that mimic this malignancy according to patient age at presentation. Methods: This was a retrospective observational study including 549 patients (769 eyes) who were referred for confirmation and/or management of retinoblastoma between October 1998 and June 2019 at a single tertiary center. A detailed ocular examination was done by the same ocular oncologist under general anesthesia for every patient. Results: Of 549 patients referred for diagnostic confirmation or management of retinoblastoma, 393 (71.6%) patients were found to have retinoblastoma and 156 (28.4%) patients received the diagnosis of pseudoretinoblastoma. The mean patient age at presentation was 52.1 months, ranging from 1 to 276 months. The most common diagnoses among patients with pseudoretinoblastoma younger than 1 year were persistent fetal vasculature (PFV) (n = 19; 28.8%), Coats disease (n = 7; 10.6%), chorioretinal coloboma (n = 4; 6.1%), retinal dysplasia (n = 4; 6.1%), and retinal detachment (n = 4; 6.1%). In patients with pseudoretinoblastoma who were 1 to 5 years old, the most common diagnoses were Coats disease (n = 10; 25.6%), PFV (n = 7; 17.9%), and optic disc hypoplasia (n = 3; 7.7%). Patients older than 5 years were most likely to have Coats disease (n = 8; 15.7%), optic disc drusen (n = 5; 9.8%), retinopathy of prematurity (n = 4; 7.8%), and combined hamartoma (n = 4; 7.8%). Conclusions: This study shows that 28.4% of patients referred for suspicion or management of retinoblastoma were classified as having pseudoretinoblastoma. The most common conditions causing diagnostic confusion with retinoblastoma included PFV and Coats disease, similar to previous publications from both high-and low-income countries. [ J Pediatr Ophthalmol Strabismus . 2021;58(3):161–167.]

Purpose To discuss the evolution of noninvasive diagnostic methods in the identification of choroidal nevus and determination of risk factors for malignant transformation as well as introduce the novel role that artificial intelligence (AI) can play in the diagnostic process. Methods White paper. Results Longstanding diagnostic methods to stratify benign choroidal nevus from choroidal melanoma and to further determine the risk for nevus transformation into melanoma have been dependent on recognition of key clinical features by ophthalmic examination. These risk factors have been derived from multiple large cohort research studies over the past several decades and have garnered widespread use throughout the world. More recent publications have applied ocular diagnostic testing (fundus photography, ultrasound examination, autofluorescence, and optical coherence tomography) to identify risk factors for the malignant transformation of choroidal nevus based on multimodal imaging features. The widespread usage of ophthalmic imaging systems to identify and follow choroidal nevus, in conjunction with the characterization of malignant transformation risk factors via diagnostic imaging, presents a novel path to apply AI. Conclusions AI applied to existing ophthalmic imaging systems could be used for both identification of choroidal nevus and as a tool to aid in earlier detection of transformation to malignant melanoma. Translational relevance Advances in AI models applied to ophthalmic imaging systems have the potential to improve patient care, because earlier detection and treatment of melanoma has been proven to improve long-term clinical outcomes.

Purpose: To investigate the alterations in the retinal pigment epithelium (RPE) in the crest and trough portions of chorioretinal folds (CRFs) induced by an orbital vascular tumor. Methods: Review of multimodal imaging in two eyes of two patients with globe compression and CRFs from an orbital vascular tumor. Results: Fundus photography demonstrated obliquely extending CRFs with alternating hyperpigmented and hypopigmented linear alterations in both eyes. Fundus autofluorescence (AF) imaging showed obliquely oriented hypoAF lines, incompletely alternating with hyperAF lines. In Case 1, the hyperAF lines had interspersed hypoAF segments and Case 2 had peripapillary mottling of AF. Fluorescein angiography (FA) showed alternating hyper and hypofluorescent lines in the late phase in Case 1. Optical coherence tomography (OCT) documented relative thinning of RPE at the folded crests in Case 1 and preservation of RPE in Case 2. Swept-source OCT angiography (SS-OCTA) demonstrated oblique hyporeflective lines in the outer retina and choriocapillaris layers in Case 2. These findings suggest that the crest of a CRF represents thinned or rarified RPE with hypoAF, transmission hyperfluorescence (FA), partially attenuated RPE layer (OCT), and isoreflectivity (SS-OCTA) while the trough represents compressed RPE with irregular hyperAF, transmission hypofluorescence (FA), thickened RPE layer (OCT), and hyporeflectivity (SS-OCTA). Conclusion: The anatomic and functional status of the RPE in CRFs based on multimodal imaging reveals normal to attenuated RPE with hypofunctionality at the fold crest and compacted, thickened RPE at the trough with segmental functional impairment on AF imaging. Anatomic information regarding CRFs is evident on OCT, FA, and SS-OCTA while the functional status is depicted on AF.

Objectives To evaluate the long-term treatment outcomes in intraocular retinoblastoma (RB) including the associated factors for eventual treatment with external beam radiotherapy (EBRT) and enucleation as well as to analyse the risk factors for metastasis and death in extraocular RB. Methods Retrospective analysis of 390 eyes from 256 (89.8%) intraocular RB and 29 (10.2%) extraocular RB cases diagnosed and treated between October 1998 and May 2018 at one of the largest tertiary care centers in Turkey. Results Of 351 intraocular RB eyes, 53.3% had group D/E disease at presentation. 75 (21.4%) of 351 eyes underwent primary enucleation. Of the remaining 276 eyes undergoing eye-conserving treatments, 201 (72.8%) were salvaged. Most of these eyes were treated using intravenous chemotherapy and/or focal treatments [transpupillary thermotherapy (TTT) and cryotherapy] initially. EBRT was eventually required in 48 (17.4%) eyes and secondary enucleation in 75 (27.2%) eyes. At mean follow-ups of 76.7 and 39.7 months for intraocular and extraocular RB cohorts, respectively, 180 (46.2%) eyes underwent primary/secondary enucleation and exenteration. Overall, 13 cases developed metastasis and 9 died. Two patients with trilateral RB also expired. Multivariable risk factors for enucleation were the presence of vitreous seeds (p

To evaluate the cytopathological results, surgical complications, and patient outcomes after transretinal biopsy (TRB) for diagnosis of retinal/choroidal tumors METHODS: Records of 40 cases who underwent TRB via 23-gauge (23 G) pars plana vitrectomy between March 2011 and March 2020 were reviewed.Retrospective.Twenty-six (65.0%) cases were women and 14 (35.0%) were men. The mean age at diagnosis was 57.2 (range: 18-83) years. The mean tumor base diameter was 12.0×9.8 mm and the mean tumor thickness was 4.9 mm. According to cytopathological examination, 29 (72.5%) cases had choroidal melanoma, 2 (5.0%) had non-small cell lung cancer metastasis, 1 (2.5%) had adenoma of retinal pigment epithelium, 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 macular and extramacular degeneration. Postoperative complications were mild vitreous hemorrhage in 16 (40.0%) cases, gradually worsening cataract in 4 (11.8%), retinal detachment in 1 (2.5%), hyphema in 1 (2.5%), glaucoma in 1 (2.5%), and macular hole in 1 (2.5%). During the mean 11.1 (range: 1-55) months follow-up, 1 (3.4%) patient with choroidal melanoma developed liver metastasis. All patients were alive at the end of follow-up.TRB using 23 G pars plana vitrectomy can be used to make the cytopathologic diagnosis of retinal/choroidal tumors whenever the clinical diagnosis is not certain or in cases with known diagnosis to obtain information on cell type/cytogenetics. In our series, the most common diagnosis after cytopathologic examination was choroidal melanoma.

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.

The goal of this article is to review the key clinical and radiological features, treatment strategies, and prognosis of lacrimal gland tumors.Debulking of the orbital lobe of the lacrimal gland may offer improved control rates in dacryoadenitis without compromise of tear film function. Contrary to previous belief, careful biopsy of the lacrimal gland prior to excision does not appear to increase the risk of recurrence in cases with suspected pleomorphic adenoma. Low-dose radiation (4 Gy) in two 2-Gy fractions appears to be effective and well tolerated in indolent non-Hodgkin lymphoma of the ocular adnexa with high local control rate. Eye-sparing surgery for adenoid cystic carcinoma (ACC) leaving minimal or no tumor residual in the orbit followed by adjuvant radiation therapy or chemoradiotherapy may provide good local control and long-term survival outcomes. Intra-arterial chemotherapy has been found to decrease recurrence and improve survival in ACC and can also be used as part of an eye-sparing treatment strategy. The development of targeted drugs may offer palliation for patients with unresectable or metastatic disease in lacrimal gland carcinoma.This article offers an update on diagnosis, management, and prognosis of the major lacrimal gland lesions.

Purpose To compare the efficacy of transpupillary thermotherapy (TTT), indocyanine green-enhanced TTT (ICG-TTT), and photodynamic therapy (PDT) in the management of circumscribed choroidal hemangioma (CCH) and to investigate the factors affecting treatment outcomes METHODS: Retrospective review of clinical records of 60 eyes with CCH which underwent TTT (25), ICG-TTT (22), or PDT (13). Main study outcomes were 1) final visual acuity (VA) ≤20/200, 2) ˂2 Snellen lines VA increase, 3) persistent subretinal fluid (SRF), and 4) ˂20% decrease in tumor thickness. Results Multivariable factors associated with final VA ≤20/200 included longer duration of symptoms (p=0.015), lower initial VA (0.030), and presence of retinoschisis overlying the tumor (0.047). Multivariable factors for ˂2 Snellen lines VA increase were longer duration of symptoms (p=0.018) and previous failed treatment (p=0.003). By multivariable analysis, the only significant factor for persistent SRF was the presence of retinoschisis (p=0.001). Multivariable factors associated with a decrease in tumor thickness by ˂20% were smaller initial tumor thickness (p=0.045) and presence of retinoschisis (p=0.014). By Pearson Chi-Square/Fisher Exact Test, final VA ≤20/200 rates (48.0%, 13.6%, 30.8% respectively, p=0.041) and VA improvement by ˂2 lines rates (64.0%, 27.3%, 38.5% respectively, p=0.036) were significantly different between TTT, ICG-TTT, and PDT groups. However, by post hoc analysis, ICG-TTT yielded statistically better outcomes compared to TTT with respect to final visual acuity (VA) ≤20/200 (p=0.012) and ˂2 Snellen lines VA increase (p=0.012). Conclusions Longer symptom duration, previous failed treatment, lower initial VA, and presence of retinoschisis were risk factors for worse visual outcomes. Eyes with retinoschisis displayed more SRF persistence after treatment. Smaller initial tumor thickness and presence of retinoschisis were risk factors for ˂20% decrease in tumor thickness.

Anterior segment optical coherence tomography (AS-OCT) is a relatively new imaging modality that allows assessment of anterior segment structures. AS-OCT enables the differentiation of benign and malignant tumors through the evaluation of lesion size, internal structure, degree of vascularity, and anterior and posterior surfaces. Herein, we discuss the AS-OCT findings of a patient with spindle type iridociliary melanoma diagnosed in pathologic examination.

The orbit is a relatively small anatomical area. Space-occupying lesions generally increase orbital volume and can result in proptosis of the globe and adversely affect visual acuity and extraocular muscle function, leading to decreased vision and diplopia. Orbital tumors comprise a group of diverse tumors from benign to malignant diseases. Orbital tumors can be classified into pediatric (≤18 years) or adult (>18 years) orbital tumors. The most common pediatric orbital tumors are dermoid cyst, capillary hemangioma and rhabdomyosarcoma. The top adult orbital tumors are lymphoid tumors, cavernous hemangioma, lacrimal gland tumors and idiopathic orbital inflammation. Several important developments have occurred both in clinical management and pathologic evaluation of orbital tumors. A new classification system (ISSVA) was devised for orbital vascular tumors. Capillary hemangiomas are now treated preferentially with oral β-blocker agents, and lymphangiomas can be reduced with sildenafil.

Objectives: To report the clinical, magnetic resonance imaging (MRI), and treatment features in cases of orbital cavernous hemangioma. Materials and Methods: TThe records of 32 patients with orbital cavernous hemangioma operated in the Department of Ophthalmology at Ankara University School of Medicine from June 1998 to April 2013 were reviewed retrospectively. Results: Twenty-three patients were female and 9 patients were male. The mean age was 42 years, ranging from 9 to 62 years. In MRI of 21 patients, all tumors were isointense to muscle in T1A-weighted images, hyperintense to muscle in T2A-weighted images, and demonstrated heterogeneous contrast enhancement. The tumor was intraconal in 13 patients, superomedial in 3 patients, superotemporal in 3 patients, and inferomedial in 2 patients. In all patients, orbitotomy was performed and the orbital mass was totally removed in one piece. The endoscopic approach used was as follows; upper temporal in 13 patients, inferior temporal in 10, upper nasal in 8, and transnasal orbitotomy in 1 patient. In all patients, no residual/recurrent mass was found after surgery. In eighteen patients, visual acuity remained the same before and after surgery. In fourteen patients, mean visual acuity at first month after surgery increased to 0.10±0.14 logMAR, while mean visual acuity before surgery was 0.25±0.21 LogMAR (p

Idiopathic orbital inflammation (IOI) is a benign inflammatory condition usually confined to the orbit but extraorbital extension can also occur. IOI has been classified into categories including anterior, diffuse, posterior or apical, myositis, and dacryoadenitis. Other rare types of IOI include periscleritis, perineuritis, and focal mass. Diagnosis is based on careful history, clinical findings, computed tomography, and magnetic resonance imaging findings. An orbital biopsy is usually done for accessible orbital lesions such as dacryoadenitis. For other types such as myositis and apical IOI where surgery is difficult or dangerous, orbital biopsy is not initially considered. The mainstay of therapy consists of systemic corticosteroids, but other options including external beam radiotherapy, antimetabolites, alkylating agents, T-cell/calcineurin inhibitors, lymphocyte inhibitors, tumor necrosis factor-α inhibitors, and surgical debulking have also been used.