Choroidal Nevi in Children: Size, Growth, and Topographic Distribution.

PURPOSE: To explore size, growth, and topographic distribution of choroidal nevi in children to gain insights into choroidal nevogenesis. DESIGN: Retrospective consecutive case series using pediatric clinic - and population - study data, comparing to adult data. METHODS: Clinical data from Cole Eye Institute (CEI) database (December 2005-January 2023) was derived from a retrospective consecutive case series of 20 children (< 20 years) with choroidal nevi. For population data, 48 children from previously reported pooled data of the participants of Sydney Pediatric Eye Disease Study, Sydney Myopia Study, Sydney Childhood Eye Study, and Sydney Adolescent Vascular and Eye Disease Study were included. Fundus photographs were reviewed and the locations of 18 choroidal nevi seen at CEI with widefield imaging were mapped on a radial scatter plot. For comparison, 100 consecutive adults with choroidal nevi were identified from CEI database. Main outcomes were size, growth, and topographic distribution of choroidal nevi. RESULTS: The median largest basal diameter was 1.6 mm (range 0.4-4.2) in children. Most choroidal nevi (75%) remained stable, and 16% demonstrated growth at follow-up. The mean growth rate was calculated as 0.12 mm/year (range 0.10-0.15). Malignant transformation was not noted during childhood. All secondary changes (drusen, orange pigment, and subretinal fluid) associated with choroidal nevi in children were less common than those in adults (p<0.05). Choroidal nevi in children were located significantly more posterior than in adults. The median distance to fovea was 2.1 mm (range 0.5-8.5) in children and 5.1 mm (range 0.4-16) in adults (p<0.0001). CONCLUSIONS: The onset and growth of choroidal nevi in children suggest active choroidal nevogenesis in childhood. A posterior topographic distribution may support the developmental framework for migration and maturation of choroidal melanoblasts.

Uveal melanoma in African Americans: Diagnostic challenges.

Uveal melanoma (UM) is uncommon in African Americans. Owing to its rarity, UM may not be suspected in African Americans leading to delayed diagnosis. In addition, socioeconomic factors may also play a role in delayed diagnosis. Clinical and ultrasonographic features may be atypical due to racial pigmentation, necessitating diagnostic fine needle aspiration biopsy. Herein, we report an illustrative case series of 12 African Americans with UM highlighting clinical features and diagnostic challenges.

Radiation Optic Neuropathy: Management Options.

Background: Radiation optic neuropathy may be one side effect of ionizing radiation exposure to the eye found in a minority of patients. It is generally devastating for visual function and has been the subject of a small but growing literature with respect to its pathophysiology, treatment, and expected outcomes. Summary: Clinical features include optic disc edema, peripapillary hemorrhages, cotton wool spots, and hard exudates. Visual acuity is generally significantly reduced. Treatment has been attempted with outcomes that have not been assessed by randomized trials. Observation may be indicated in addition to treatment. Key Messages: Radiation optic neuropathy is known to generally be devastating to vision though an uncommon side effect of radiation. Treatment has been attempted with mixed results.

Surveillance for Metastasis in High-Risk Uveal Melanoma Patients: Standard versus Enhanced Protocols.

PURPOSE: to evaluate the effectiveness of enhanced surveillance protocols (EP) utilizing high frequency (HF) or enhanced modality (EM) compared to the standard protocol (SP) in detecting metastasis and determining their impact on overall survival (OS) in high-risk uveal melanoma (UM) patients. METHODS: A total of 87 consecutive patients with Class 2 (high risk) primary UM were enrolled, with negative baseline systemic staging. The patients underwent systemic surveillance with either SP (hepatic ultrasonography [US] every 6 months) or EP (either HF [US every 3 months] or EM [incorporation hepatic computed tomography/magnetic resonance imaging]) following informed discussion. The main outcome measures were largest diameter of largest hepatic metastasis (LDLM), number of hepatic metastatic lesions, time to detection of metastasis (TDM), and OS. RESULTS: This study revealed significant differences in LDLM between surveillance protocols, with the use of EP detecting smaller metastatic lesions (HF, EM, and SP were 1.5 cm, 1.6 cm, and 6.1 cm, respectively). Patients on the EM protocol had a lower 24-month cumulative incidence of >3 cm metastasis (3.5% EM vs. 39% SP; p = 0.021), while those on the HF protocol had a higher 24-month cumulative incidence of ≤3 cm metastasis compared to SP (31% HF vs. 10% SP; p = 0.017). Hazard of death following metastasis was significantly reduced in the EP (HR: 0.25; 95% CI: 0.07, 0.84), HF (HR: 0.23; 95% CI: 0.06, 0.84), and EM (HR: 0.11; 95% CI: 0.02, 0.5) groups compared to SP. However, TDM and OS did not significantly differ between protocols. CONCLUSIONS: Enhanced surveillance protocols improved early detection of hepatic metastasis in UM patients but did not translate into a survival advantage in our study cohort. However, early detection of metastasis in patients receiving liver-directed therapies may lead to improved overall survival.

Iris melanoma: Prognostication for metastasis.

Uveal melanoma prognostication studies have mainly included posterior uveal melanomas located in the ciliary body and choroid, often excluding iris melanoma. In this study, we report prognostic status and survival outcomes in a series of 35 patients with biopsy-proven iris melanoma. Fluorescence in situ hybridization was performed in 10 (29%) cases and 2 (5%) underwent multiplex ligation-dependent probe amplification. In total, 9 cases demonstrated disomy 3, 2 cases with monosomy 3 (fluorescence in situ hybridization), and 1 had a technical failure. On gene expression profile testing, 20 of the 23 cases (90%) were gene expression profile class 1A, and the remaining 3 (10%) were class 1B. No patient had a Class 2 status. The median follow-up period was 49 months (mean 59, range 2-156 months). No metastasis was reported during follow-up, and metastasis-free survival was 100%. A review of the published literature revealed 47 cases with high-risk status on molecular prediction, of which only 6 (13%) developed metastasis. Ciliary body involvement was reported in 5 cases and was unknown in 2 cases. We conclude that molecular prognostication of iris melanoma demonstrates low-risk prognostic status in the majority of cases irrespective of the technique used. Even those with high-risk status do not develop metastasis unless the tumor involves the ciliary body.

Real Life Multicenter Comparison of 24-Month Outcomes of Anti-VEGF Therapy in Diabetic Macular Edema in Turkey: Ranibizumab vs. Aflibercept vs. Ranibizumab-Aflibercept Switch.

The aim of this study was to compare the outcomes of diabetic macular edema (DME) treated with aflibercept (AFB) or ranibizumab (RNB) only, and after switching from RNB to AFB. This was a retrospective, real-world, multicenter (7 cities) 24 month study. Overall, 212 eyes in the AFB group, 461 in the RNB group, and 141 in the RNB to AFB group were included. The primary endpoints were differences in visual acuity (VA) and central macular thickness (CMT) from baseline to the final visit. The secondary outcomes were the percentage of eyes that achieved ≥10 letters gain and ≥10 letters loss in vision at month 12 and 24, and the percentage of eyes that achieved a thinning of ≥20% in CMT at month 3 and month 6. The results showed that VA did not significantly differ at baseline (AFB: 0.62 ± 0.38, RNB: 0.61 ± 0.36, RNB to AFB: 0.61 ± 0.38), at checkpoints, or at the final visit (AFB: 0.46 ± 0.38, RNB: 0.5 ± 0.37, RNB to AFB: 0.53 ± 0.36) (p > 0.05). Though the mean CMT at baseline was significantly thicker in the RNB to AFB group (479 ± 129.6 µm) when compared to the AFB (450.5 ± 122.6 µm) and RNB (442 ± 116 µm) groups (p < 0.01), similar measurements were obtained after 12 months. The percentages of eyes that gained or lost ≥10 letters in the AFB, RNB, and RNB to AFB groups at year 1 and 2 were similar, as was the percentages of eyes that demonstrated ≥20% CMT thinning at month 3 and 6. Our study showed similar visual improvements in non-switchers (AFB and RNB groups) and switchers (RNB to AFB group) through 2 years follow-up, however, AFB patients required fewer injections, visits, or need for additional treatments.

Anti-vascular endothelial growth factor therapy in diabetic macular edema: real-life outcomes from a multicenter study in Turkey over 36 months.

PURPOSE: This study aimed to report the visual and anatomical outcomes of intravitreal anti-VEGF treatment for diabetic macular edema (DME) in a real-world clinical setting from Turkey over 36 months. METHODS: This is a retrospective, multicenter (7 sites) study. The medical records of 1072 eyes (both previously treated and naive eyes) of 706 consecutive patients with visual impairment due to center-involving DME treated with intravitreal anti-VEGF injections between April 2007 and February 2017 were reviewed. The eyes were divided into mutually exclusive three groups based on the duration of follow-up (12, 24, or 36 months). Primary outcome measures were changes in visual acuity (VA) and central macular thickness (CMT) from baseline to final visit in each cohort, frequency of visits and intravitreal anti-VEGF injections. As secondary endpoints, VA outcomes were assessed in subgroups stratified by baseline VA [<70 ETDRS letters and ≥70 ETDRS letters] and loading dose status of anti-VEGF injections. RESULTS: VA increased by a mean of 8.2 letters (12-month cohort, p < 0.001), 5.3 letters (24-month cohort, p < 0.001), and 4.4 letters (36-month cohort, p = 0.017) at final visits. The eyes with <70 VA letters achieved more significant VA improvement at final visits in all cohorts compared with eyes with >70 VA letters (p < 0.001). The mean decreases in CMT from baseline to last visits at 12-, 24-, and 36- month cohorts were -100.5 µm, -107.7 µm, and -114.3 µm, respectively (p < 0.001). The mean number of injections given were 4.6, 2.3, and 1.8 during years 1 to 3, respectively. Patients who received loading dose showed greater VA gains than those who did not in all follow-up cohorts. CONCLUSION: Our study revealed that anti-VEGF treatment improved VA and CMT over a follow-up of 36 months. Although these real-life VA outcomes following anti-VEGF therapy for DME were similar to other real-life studies, they were inferior to those noted in randomized controlled trials, mainly due to undertreatment.

A Myopic Normative Database for Retinal Nerve Fiber Layer Thickness Using Optical Coherence Tomography.

PRCIS: The purpose of this study was to determine changes in optical coherence tomography (OCT) color codes after applying a myopic normative database. The diagnostic performance of the retinal nerve fiber layer analysis improved with the use of this database. PURPOSE: To evaluate the peripapillary retinal nerve fiber layer (pRNFL) OCT color codes based on a newly generated myopic normative database in comparison to the built-in normative database. MATERIALS AND METHODS: A total of 371 subjects were included in this validation study in an attempt to generate a myopic normative database. Eighty myopic glaucomatous and 80 myopic healthy eyes were evaluated to determine the diagnostic performance of this database. The distribution of the color codes was investigated among the groups with reference to the built-in and myopic normative databases, and the 2 databases were compared in terms of abnormal color code frequency. The diagnostic performance of the myopic database was presented with sensitivity, specificity and area under the receiver operating characteristic curve values. RESULTS: The agreement between the databases decreased with increasing myopia degree. The distribution of the color codes of the built-in software significantly differed among the study groups in all sectors ( P =0.009 for the temporal sector and P <0.001 for the remaining sectors). When the myopic database was used, there were no longer significant differences among the groups for the temporosuperior, temporoinferior, temporal, and nasal sectors ( P =0.561, 0.299, 0.201, and 0.089, respectively). After applying the myopic normative database, the specificity of the pRNFL color codes increased from 70.1% to 90.2%, and the area under the receiver operating characteristic curve value from 0.851 to 0.945. CONCLUSIONS: The use of a myopic normative database for pRNFL using SD-OCT significantly decreased differences among myopia severity groups, and may help to more reliably assess glaucoma in myopic eyes.

Alterations in the Retinal Nerve Fiber Layer Thickness Color Map in Non-Glaucomatous Eyes with Myopia.

Objectives: To determine the normal values for retinal nerve fiber layer thickness (RNFLT) in myopic patients without glaucoma and analyze the changes in their color map. Materials and Methods: A total of 245 eyes without glaucoma were included in the study. According to the degree of myopia, the cases were divided into 4 groups: control group (+1.00/-1.00 D; n=70), Group 1 (-1.00/-3.00 D; n=50), Group 2 (-3.00/-6.00 D; n=75), and Group 3 (>-6.00 D; n=50). Intra-group comparisons were performed in terms of superotemporal, superonasal, nasal, inferonasal, inferotemporal, temporal, and global RNFLT (Heidelberg Spectralis, Optic Coherence Tomography, Germany) and the color coding of these quadrants (green: within normal limits, yellow: borderline, red: outside normal limits). Results: All groups were similar in age and gender (p>0.05). As the degree of myopia increased, RNFLT became thinner in the upper and lower temporal and upper and lower nasal quadrants (p<0.01). The rate of measurements considered borderline and outside normal limit in at least 1 quadrant was higher in groups with higher myopia for all quadrants (p<0.05). This rate was found to be 8/70 (11.4%) for the control group, 9/50 (18.0%) for Group 1, 21/75 (28.0%) for Group 2, and 33/50 (66.0%) for Group 3 (p<0.01). Conclusion: The high rate of RNFLT classified as borderline or outside normal limits in myopic patients is a finding to which clinicians should pay attention in order not to make a misdiagnosis, especially in cases of suspected glaucoma.

Effect of blunt ocular trauma on retinal microvasculature: An optical coherence tomography angiography study.

BACKGROUND: To evaluate the retinal vascular changes and foveal avascular zone area in patients with unilateral blunt ocular trauma using optical coherence tomography angiography. METHODS: This retrospective study consisted of 50 traumatized and 50 contralateral sound eyes of the patients with blunt ocular trauma. The foveal avascular zone area, choriocapillaris flow area, foveal, parafoveal, and perifoveal vessel densities in both superficial capillary plexus and deep capillary plexus and central macular thickness were evaluated. RESULTS: There were no significant differences between traumatized and sound eyes in the foveal avascular zone area (p:0.36), choriocapillaris flow area (p:0.43), central macular thickness (p:0.67), and in vessel densities of superficial capillary plexus over all regions (p > 0.05 for all). However, the vessel densities of deep capillary plexus were significantly lower in traumatized eyes (p < 0.05 for all). CONCLUSION: Optical coherence tomography angiography demonstrated a significant decrease in retinal deep capillary plexus vessel density of the eyes effected by blunt ocular trauma, even with no evident findings on fundus examination or structural spectral domain-optical coherence tomography changes.

Comparative 2-year outcomes of conventional and accelerated corneal collagen crosslinking in progressive keratoconus.

AIM: To compare the safety and efficacy of conventional versus accelerated (9 mW/cm2) corneal collagen crosslinking (CXL) in progressive keratoconus at the 2-year follow-up. METHODS: In this prospective study, consecutive progressive keratoconus patients were randomized to receive either conventional CXL (CCXL) or accelerated CXL (ACXL; using hydroxypropyl methylcellulose-assisted riboflavin imbibition for 10min at 9 mW/cm2). Visual, refractive, keratometric, topographic, and aberrometric outcomes and stromal demarcation line depth (DLD) measurements were compared at the end of a 2-year follow-up. RESULTS: Thirty-two eyes from 32 patients in the CCXL and 27 eyes from 27 patients in the ACXL groups completed 2-year follow-up. At 2y post-CXL, both uncorrected and corrected visual acuities improved significantly in both groups. The improvements in keratometric readings, flattening rate (flattening of the maximum keratometry more than 1 D), 3 topographic indices, and vertical coma were significantly better in the CCXL group compared to the ACXL group (P<0.05). The DLD as measured by anterior segment optical coherence tomography or in vivo confocal microscopy was better detectable and significantly deeper in the CCXL group compared to the ACXL group. The deeper DLD was found to be significantly correlated with improvements in the mean keratometry measurements. Progression was noted in 11.1% of eyes in the ACXL group, whereas progression was not observed in any patient eye in the CCXL group. CONCLUSION: In this prospective randomized study, ACXL is less effective in halting the progression of keratoconus at a 2-year follow-up compared to CCXL.

Culture-Proven Candida Albicans Endogenous Endophthalmitis in a Patient with Onychomycosis.

Purpose: To report a case of Candida albicans endogenous endophthalmitis in an immunocompetent patient with onychomycosis.Methods: Retrospective case report.Results: A 40-year-old man with onychomycosis presented with C. albicans subretinal abscess in the left eye. Systemic and intravitreal injections did not prevent further progression of the infection. The patient underwent pars plana vitrectomy. One month after surgery, the intraocular inflammation gradually subsided. However, his visual acuity stayed at counting fingers as a result of macular scarring.Conclusion: The aim of this case presentation is to emphasize that endogenous fungal endophthalmitis can be seen in an immunocompetent patient. The use of systemic steroids in the past was the main reason for the progression of the disease in this case. In these situations, when the clinical findings suggest a fungal etiology, it should keep in mind that endogenous candida endophthalmitis can be a result of fungal infections from distant sites such as the toenails and systemic steroids should not be started before definite diagnosis.

Correction of Irregular Astigmatism With New-Generation Hybrid Contact Lenses.

PURPOSE: To evaluate the safety, efficacy, and on-eye performance of new-generation hybrid contact lenses (HCLs) in visual rehabilitation of eyes with irregular astigmatism and rigid gas-permeable (RGP) contact lens intolerance or failure. METHODS: The charts of patients who had been fit with new-generation HCLs were retrospectively reviewed. The reason for HCL fit and previous method of correction were noted. After the initial on-eye evaluation, visual and refractive outcomes, slit-lamp biomicroscopy, and lens comfort were evaluated at the last follow-up examination. RESULTS: Forty-seven eyes of 33 patients could be fit successfully with EyeBrid silicone (LCS, Cane, France) or Airflex (SwissLens, Prilly, Switzerland) lenses. The mean number of trial lenses required for ideal fit was 1.4±0.6 (range; 1-3) lenses. The reason for fit was either centration problems with RGP lenses or RGP intolerance. Twenty-nine eyes of 20 patients (72.5%) continued wearing their lenses more than 10 hr a day and for more than 3 months. In these successful wearers, the visual acuity (VA) improved significantly compared with the baseline uncorrected and spectacle-corrected VA (P<0.01), and no serious adverse events were encountered during the mean follow-up period of 10.1±6.4 months. At the last follow-up examination, patients preferred the new-generation HCLs over their habitual correction in regard to both VA and quality (P<0.05). CONCLUSION: New-generation HCLs seem to provide a viable alternative for visual rehabilitation of irregular astigmatism in selected eyes with RGP intolerance or RGP failure. The ease of the fitting process similar to fitting soft toric lenses and high patient satisfaction seem to be major advantages of these designs.

Severe upper eyelid mass in a patient with mycosis fungoides.

Mycosis fungoides is a cutaneous T-cell lymphoma that has been rarely reported to involve ocular structures. Ophthalmic manifestations usually appear in advanced disease. A case of a 58-year-old man presenting with progressive, full thickness, giant upper eyelid mass is presented. The patient had a long history of recurrent tumoral lesions on the trunk and limbs, previously diagnosed as mycosis fungoides. The histopathological examinations of eyelid tumor supported the diagnosis of mycosis fungoides. The mycosis fungoides was stage as IIB (T3N0M0B0) by TNMB classifications and referred to the Hematology and Radiation Oncology clinics. The importance of ophthalmic involvement is being seen in advanced or refractory cases, and there is a possible relation between mycosis fungoides and poor prognosis by being an early indicator of systemic involvement.

Choroidal Nevus With Outer Retinal Atrophy Simulating Solar Retinopathy.

A 58-year-old man presented with visual distortion in the right eye for 2 years. His best-corrected visual acuity was 20/25 in each eye. Fundus examination revealed a macular pigmented lesion with central retinal pigment epithelial (RPE) atrophy and drusen in the right eye. Enhanced depth imaging optical coherence tomography (EDI-OCT) of the right eye showed a slightly elevated choroidal lesion with choroidal vascular compression, optical shadowing, and trace cystoid macular edema. In addition, there was notable, prominent focal outer retinal atrophy simulating solar retinopathy, but the patient denied sun-gazing, laser pointer-gazing, and alkyl nitrate (popper) medications. The final diagnosis was choroidal nevus with focal extensive outer retinal atrophy, giving a pseudosolar retinopathy appearance. Imaging with EDI-OCT provides indispensable information concerning retinal and RPE alterations overlying choroidal nevus and other choroidal lesions. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:586-588.].

Idiopathic Orbital Inflammation: Review of Literature and New Advances.

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.

Lacrimal gland tumors in Turkey: types, frequency, and outcomes.

AIM: To evaluate the clinical, radiological, and treatment features of lacrimal gland tumors. METHODS: Retrospective review of 99 eyes of 92 patients with lacrimal gland tumors diagnosed and managed in a single institution between January 1999 and March 2017. Clinical and radiological features, histopathology, treatment methods, and prognosis were evaluated. RESULTS: The mean patient age was 40.3 (range: 7-80)y. The diagnosis was made histopathologically in 91 (91.9%) tumors and on a clinical and radiological basis in 8 (8.1%) tumors. Final diagnoses included idiopathic orbital inflammation (pseudotumor) in 46 (46.5%) lesions, pleomorphic adenoma in 14 (14.1%), adenoid cystic carcinoma in 12 (12.1%), granulomatous inflammation in 10 (10.1%), lymphoma in 5 (5.0%), benign reactive lymphoid hyperplasia in 3 (3.0%), dacryops in 3 (3.0%), carcinoma ex pleomorphic adenoma in 2 (2.0%), adenocarcinoma in 1 (1.0%), dermoid cyst in 1 (1.0%), cavernous hemangioma in 1 (1.0%), and leukemic infiltration in 1 (1.0%). Non-epithelial tumors comprised 64.6% (n=64) of all lacrimal gland tumors, epithelial tumors 32.3% (n=32), dermoid cyst 1% (n=1), cavernous hemangioma 1% (n=1), and leukemic infiltration 1% (n=1). There were in total 78 (78.8%) benign and 21 (21.2%) malignant tumors. CONCLUSION: Overall, 65% of lacrimal gland tumors were of non-epithelial origin and 32% of epithelial origin. By histopathology and clinical evaluation, 79% of lacrimal gland tumors were benign. The most common lacrimal gland tumors include idiopathic orbital inflammation (46.5%), epithelial (32.3%), and lymphoproliferative (8.1%) lesions.

Overview of benign and malignant lacrimal gland tumors.

PURPOSE OF REVIEW: The goal of this article is to review the key clinical and radiological features, treatment strategies, and prognosis of lacrimal gland tumors. RECENT FINDINGS: 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. SUMMARY: This article offers an update on diagnosis, management, and prognosis of the major lacrimal gland lesions.

Ectopic rhabdoid meningioma of the orbit in a child: case report and review of the literature.

In August 2016, an 11-year-old boy presented to the authors' institution with a right orbital tumor that was located superotemporally (superolaterally) and adherent to the sclera. The patient's past medical history revealed that he had undergone 2 previous craniotomies elsewhere in June 2008 and July 2010 for a superomedially located orbital lesion that had been histopathologically diagnosed as a neurothekeoma. After the second craniotomy, the patient underwent adjuvant intensity modulated radiotherapy (IMRT) to the right medial orbit. At the authors' institution, total excision of the orbital tumor was performed via an anterior conjunctival orbitotomy. Histopathological examination revealed a rhabdoid meningioma. Review of the histopathology obtained at the time of previous tumor excisions showed that the lesion was misdiagnosed as neurothekeoma and instead represented a meningioma from the beginning. The patient was started on a regimen of oral sunitinib and remained free of recurrence at 1.5 years of follow-up. Ectopic meningioma of the orbit is a rare entity. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%-3% of all intracranial meningiomas. To the best of the authors' knowledge, this is the first case of an ectopic orbital rhabdoid meningioma reported in the literature. They suspect that tumor seeding during the previous surgeries might have played a role in the occurrence of the tumor in an orbital location not targeted by IMRT.