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   Table of Contents - Current issue
Coverpage
Dec 2019
Volume 67 | Issue 12
Page Nos. 1915-2113

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EDITORIAL  

Mentoring is a serious business Highly accessed article p. 1915
Santosh G Honavar
DOI:10.4103/ijo.IJO_2161_19  PMID:31755420
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GUEST EDITORIALS Top

A salute to Team Shields Highly accessed article p. 1918
Julia A Haller
DOI:10.4103/ijo.IJO_2041_19  PMID:31755421
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From Lepidoptera to uveal melanoma: Finding my career in ocular oncology p. 1922
Jerry A Shields
DOI:10.4103/ijo.IJO_1587_19  PMID:31755423
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Lessons learned on balancing home and career as a doctor, researcher, and mother: Plenty of organization, appreciation, and cheerios and goldfish Highly accessed article p. 1924
Carol L Shields
DOI:10.4103/ijo.IJO_1588_19  PMID:31755424
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Ocular oncology sans borders—A global outreach p. 1926
Fairooz P Manjandavida
DOI:10.4103/ijo.IJO_2105_19  PMID:31755422
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ONE MINUTE OPHTHALMOLOGY Top

Acquired progressive unilateral blepharoptosis p. 1929
Fairooz P Manjandavida, Shaifali Chahar
DOI:10.4103/ijo.IJO_1988_19  PMID:31755425
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REVIEW ARTICLES Top

Tumors of the conjunctiva and cornea p. 1930
Carol L Shields, Jerry A Shields
DOI:10.4103/ijo.IJO_2040_19  PMID:31755426
Tumors of the conjunctiva and cornea comprise a large and varied spectrum of conditions. These tumors are grouped into two major categories of congenital and acquired lesions. The acquired lesions are further subdivided based on origin of the mass into surface epithelial, melanocytic, vascular, fibrous, neural, histiocytic, myxoid, myogenic, lipomatous, lymphoid, leukemic, metastatic and secondary tumors. Melanocytic lesions include nevus, racial melanosis, primary acquired melanosis, melanoma, and other ocular surface conditions like ocular melanocytosis and secondary pigmentary deposition. The most frequent nonmelanocytic neoplastic lesions include squamous cell carcinoma and lymphoma, both of which have typical features appreciated on clinical examination. The caruncle displays a slightly different array of tumors compared to those elsewhere on the conjunctiva, as nevus and papilloma are most common, but oncocytoma and sebaceous gland hyperplasia, adenoma, and carcinoma can be found. In this report, we provide clinical description and illustration of the many conjunctival and corneal tumors and we discuss tumor management.
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Melanocytoma of the optic disk: A review p. 1949
Jerry A Shields, Hakan Demirci, Arman Mashayekhi, Ralph C Eagle Jr, Carol L Shields
DOI:10.4103/ijo.IJO_2039_19  PMID:31755427
Melanocytoma is a deeply pigmented variant of melanocytic nevus that classically occurs in the optic disk, sometimes with contiguous involvement of the adjacent retina or choroid. Historically, this tumor was often confused with malignant melanoma both clinically and histopathologically. Today, however, it is generally recognized by its typical clinical features that differ from most melanomas and erroneous enucleation is rarely done. Histopathologically, melanocytoma is composed of intensely pigmented round to oval nevus cells with benign features. Although traditionally believed to be a relatively stationary lesion, it is now known to exhibit minor enlargement in 10--15% of cases and can cause minor visual loss by a variety of mechanisms. In rare instance, it can induce severe visual loss due to spontaneous necrosis of the lesion or compressive optic neuropathy. More importantly, it can exhibit malignant transformation into melanoma in 1--2% of cases. Ophthalmologists should be familiar with melanocytoma of the optic disk and affected patients should be followed periodically.
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Prognostication of uveal melanoma is simple and highly predictive using The Cancer Genome Atlas (TCGA) classification: A review p. 1959
Carol L Shields, Lauren A Dalvin, Pornpattana Vichitvejpaisal, Mehdi Mazloumi, Arupa Ganguly, Jerry A Shields
DOI:10.4103/ijo.IJO_1589_19  PMID:31755428
Purpose: The cancer genome atlas (TCGA) is a comprehensive project supported by the National Cancer Institute (NCI) in the United States to explore molecular alterations in cancer, including uveal melanoma (UM). This led to TCGA classification for UM. In this report, we review the American Joint Committee on Cancer (AJCC) classification and TCGA classification for UM from the NCI's Center for Cancer Genomics (NCI CCG) (based on enucleation specimens [n = 80 eyes]) and from Wills Eye Hospital (WEH) (based on fine needle aspiration biopsy [FNAB] specimens [n = 658 eyes]). We then compare accuracy and predictability of AJCC versus (vs.) TCGA. Methods: Review of published reports on AJCC and TCGA classification for UM was performed. Outcomes based on AJCC 7th and 8th editions were assessed. For TCGA, UM was classified based on chromosomes 3 and 8 findings including disomy 3 (D3), monosomy 3 (M3), disomy 8 (D8), 8q gain (8qG), or 8q gain multiple (8qGm) and combined into four classes including Class A (D3/D8), Class B (D3/8qG), Class C (M3/8qG), and Class D (M3/8qGm). Outcomes of metastasis and death were explored and a comparison (AJCC vs. TCGA) was performed. Results: In the NCI CCG study, there were 80 eyes with UM sampled by enucleation (n = 77), resection (n = 2), or orbitotomy (n = 1) and analysis revealed four distinct genetic classes. Metastasis and death outcomes were subsequently evaluated per class in the WEH study. The WEH study reviewed 658 eyes with UM, sampled by FNAB, and found Class A (n = 342, 52%), B (n = 91, 14%), C (n = 118, 18%), and D (n = 107, 16%). Comparison by increasing class (A vs. B vs. C vs. D) revealed older mean patient age (P < 0.001), worse entering visual acuity (P < 0.001), greater distance from the optic disc (P < 0.001), larger tumor diameter (P < 0.001), and greater tumor thickness (P < 0.001). Regarding outcomes, more advanced TCGA class demonstrated increased 5-year risk for metastasis (4% vs. 20% vs. 33% vs. 63%,P < 0.001) with corresponding increasing hazard ratio (HR) (1.0 vs. 4.1, 10.1, 30.0,P= 0.01 for B vs. A andP < 0.001 for C vs. A and D vs. A) as well as increased 5-year estimated risk for death (1% vs. 0% vs. 9% vs. 23%,P < 0.001) with corresponding increasing HR (1 vs. NA vs. 3.1 vs. 13.7,P= 0.11 for C vs. A andP < 0.001 for D vs. A). Comparison of AJCC to TCGA classification revealed TCGA was superior in prediction of metastasis and death from UM. Conclusion: TCGA classification for UM is simple, accurate, and highly predictive of melanoma-related metastasis and death, more so than the AJCC classification.
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COMMENTARY Top

Commentary: Prognostication of uveal melanoma based on molecular diagnosis - Are we there yet? p. 1964
Yamini Attiku, Vikas Khetan
DOI:10.4103/ijo.IJO_1899_19  PMID:31755429
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REVIEW ARTICLE Top

Circumscribed choroidal hemangioma: An overview of clinical manifestation, diagnosis and management p. 1965
Mrittika Sen, Santosh G Honavar
DOI:10.4103/ijo.IJO_2036_19  PMID:31755430
Circumscribed choroidal hemangioma is a benign vascular tumor which presents in middle-aged adults with progressive diminution of vision, metamorphopsia, floaters, and visual field defects. Diagnosis is based on the characteristic clinical features. It is an orange-red, usually solitary, tumor situated in the posterior pole. The visual symptoms are because of the associated subretinal fluid, cystoid macular edema, and, in long-standing cases, retinal pigment epithelium changes, subretinal fibrosis and retinoschisis. It must be distinguished from the more ominous amelanotic melanoma and choroidal metastasis. Diagnostic tools such as ultrasound, fundus fluorescein angiography, indocyanine green angiography, and optical coherence tomography are helpful in cases with diagnostic dilemma. Treatment is indicated in symptomatic cases. The management of choroidal hemangioma has evolved over the years beginning with laser photocoagulation to transpupillary thermotherapy, photodynamic therapy, plaque brachytherapy and external beam radiotherapy. No one therapeutic option holds superiority over the other. In this article, we review the epidemiology, clinical manifestations and treatment of the circumscribed variant of choroidal hemangioma.
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ORIGINAL ARTICLE Top

To compare intralesional and oral propranolol for treating periorbital and eyelid capillary hemangiomas p. 1974
Aditi Mehta, Mandeep S Bajaj, Neelam Pushker, Bhavna Chawla, Amar Pujari, Sartaj S Grewal, Satinder Pal Singh Grewal, Simar Rajan Singh, Alisha Kishore, Neha Singh Yadav
DOI:10.4103/ijo.IJO_59_19  PMID:31755431
Purpose: A pilot randomized control trial to compare the efficacy and side effects of intralesional and oral propranolol in periorbital and eyelid capillary hemangiomas. Methods: Twenty patients were prospectively randomized to two groups of ten each. Group 1 was initiated on oral propranolol 1 mg/kg/day titrated to final dose of 3 mg/kg/day over 1 week which was continued for 6 months and then tapered over 1 week; Group 2 received 3 doses of direct intralesional propranolol hydrochloride 1 mg/ml; 0.2 ml/cm 4–6 weeks apart. Hemangioma area and corneal astigmatism were measured. Results: Within each group at 6 months there was a significant reduction in area (group 1: 83.48 ± 11.67%,P= 0.0019; group 2: 67.78 ± 21.71%,P= 0.0019) and improvement in astigmatism (pre, post: group 1: 2.98D @ 179.8°, 1.13D @ 179.8°,P= 0.0045; group 2: 1.62D @ 90.16°, 0.75D @ 179.9°,P= 0.0001). There was no difference in area reduction (P = 0.056), change in appearance (P = 0.085), ptosis (P = 0.23) and side effects (lethargy, poor feeding;P= 0.171) between the two groups. Conclusion: Efficacy and side effects with intralesional propranolol are comparable to oral propranolol for periorbital and eyelid lesions.
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COMMENTARY Top

Commentary: Propranolol for infantile hemangiomas — The intralesional route p. 1981
Devjyoti Tripathy
DOI:10.4103/ijo.IJO_1085_19  PMID:31755432
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ORIGINAL ARTICLES Top

Evaluation of PD-L1 and PD-1 expression in aggressive eyelid sebaceous gland carcinoma and its clinical significance p. 1983
Perumal Jayaraj, Seema Sen
DOI:10.4103/ijo.IJO_2056_18  PMID:31755433
Purpose: Eyelid sebaceous gland carcinoma (SGC) is an aggressive but rare malignancy of ocular region. Over-expression of PD-L1 and PD-1 has been demonstrated in a variety of solid tumors including conjunctival melanoma. PD-L1 is an immunoinhibitory molecule that suppresses the effective T cells response against tumor antigen leading to the progression of tumors. Inhibitors of the interaction of PD-L1 and PD-1 are associated with good clinical response various carcinomas. The prognostic value of the PD-1/PD-L1 axis in SGC remains unexplored. The purpose of this study was to evaluate expressions of PD-1 and its ligand PD-L1 in SGC and correlate its expression with clinicopathological features and patients survival. Methods: The immunohistochemical expression of PD-L1 and PD-1 was evaluated in 30 SGC cases. Results: PD-L1 immunopositivity was detected in 41.9% of the SGC cases. PD-1 expression in tumor infiltrative lymphocytes (TILs) was observed in 53.3% samples. Tumor PD-L1 positivity, PD-1 expression in TILs and tumor size (>10 mm) was associated with reduced disease-free survival. On multivariate analysis only tumor size (>10 mm) and a combined positivity of PD-L1 in tumor cells and PD-1 in TILs with an odds ratio of 5.212 (95% confidence interval 1.449-18.737) continued to be significantly associated with SGC recurrence. Conclusion: PD-L1 is overexpressed in 50% of SGC cases. The combined tumor PD-L1 positivity and TILs showing PD-1 expression within the same SGC patient's samples predict high-risk SGC, suggesting that the up-regulation of PD-L1 in tumor cells and PD-1 positivity within the same SGC patient may aggravate tumor recurrence.
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Sensitivity and specificity of frozen section diagnosis in orbital and adnexal malignancies p. 1988
Md Shahid Alam, Andrea Tongbram, Subramanian Krishnakumar, Jyotirmay Biswas, Bipasha Mukherjee
DOI:10.4103/ijo.IJO_2096_18  PMID:31755434
Purpose: To analyze the diagnostic accuracy of frozen section in orbital and adnexal malignancies. Methods: A total of 55 cases between January 2006 and December 2011 for which intraoperative frozen section was performed for various orbital and adnexal lesions were included in the study. The frozen section diagnosis was compared with the permanent section diagnosis. Margin clearance was also compared between the two. Data were analysed using SPSS version 14. Odds ratio and cross-tabulation was used to perform the analysis. Results: The mean age at presentation was 51.46 ± 20 years. Eyelid was the most common site of involvement. Out of 55 cases, diagnosis was deferred in four cases (7.27%) on frozen section. Among 51 cases, 44 (86%) cases were concordant, whereas 7 (13%) cases were discordant. The sensitivity and specificity of frozen section compared to permanent section for diagnosis of malignancy was found to be 87.2% and 87.5%, respectively. The sensitivity and specificity of frozen section for diagnoses of basal cell carcinoma was found to be 100%, while it was 83.3% and 100% respectively for sebaceous gland carcinoma and 87.5% and 94.9% respectively for squamous cell carcinoma. Conclusion: Frozen section had high sensitivity and specificity when compared with permanent section for all three parameters studied. It is an important intraoperative tool that is increasingly being used in histopathological examination of ophthalmic lesions. However, it should not be used as a substitute for the permanent section and critical decisions based on it are best avoided.
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Idiopathic inflammatory diseases of orbit and ocular adnexa: Histopathological and immunochemical analysis p. 1993
Dipankar Das, Panna Deka, Kasturi Bhattacharjee, Jayanta Kumar Das, Ganesh Chandra Kuri, Harsha Bhattaacharjee, Nilutparna Deori, Saurabh Deshmukh, Rammohan Paidi, Apurba Deka
DOI:10.4103/ijo.IJO_2120_18  PMID:31755435
Purpose: To present histopathological and immunohistochemical analysis of idiopathic inflammatory diseases of orbit and ocular adnexa. Methods: Design- A retrospective laboratory-based study. The study was carried out in an ocular pathology laboratory in a tertiary institute of northeast India where analysis of 93 cases was done in 5 years, during the period from 2011 to 2016. Hematoxylin--eosin and special stains were done for the diagnoses. Immunohistochemistry (IHC) panel was also carried out. For infectious pathology, Grocott's methenamine silver (GMS) stain for fungus, tissue Gram's stain for bacteria's, and acid-fast stains for tubercular bacilli were done. IHC panels were done for CD 20 (B-cells), CD-3 (T-cells), CD-45 (Leukocyte common antigen, LCA), BCL-2, CD-138 (Plasma cells), Kappa, Lambda, IgG-4 in tissue, IgG-4 in serum, etc. IHCs were done using kit methods (standardized) and adequate controls were taken for each sample. Results: 93 cases of nonspecific orbital inflammation were reported out of 1,467 specimens. Orbital pseudotumors (idiopathic orbital inflammatory disease, IOID) were seen in 27 cases (sclerosing variety-6); benign lymphoid hyperplasia in two cases; reactive lymphoid hyperplasia in 10 cases; atypical plasma lymphoproliferative reactive (polyclonal immunophenotypically, IgG4 negative) lesions in four cases; IgG-4 related disease in one case; nonspecific inflammatory reactions (conjunctiva, sclera, and lid) in 49 cases. In all the diagnoses, infections and lymphomas were excluded. Conclusion: Biopsy supported study on nonspecific orbital inflammation was important to know the pattern.
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COMMENTARY Top

Commentary: As is our pathology, so is our practice p. 1996
Jyotirmay Biswas
DOI:10.4103/ijo.IJO_888_19  PMID:31755436
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ORIGINAL ARTICLE Top

Outcomes of neonatal retinoblastoma in pre-chemotherapy and chemotherapy eras p. 1997
David A Camp, Lauren A Dalvin, Rachel Schwendeman, Li-Anne S Lim, Carol L Shields
DOI:10.4103/ijo.IJO_634_19  PMID:31755437
Purpose: To quantify outcomes for neonatal retinoblastoma patients treated during the pre-chemotherapy (1980–1994) and chemotherapy (1995–2018) eras. Methods: Retrospective review of retinoblastoma patients diagnosed within the first 28 days of life between 1/1/1980 and 11/30/2018. Student's t-test, Chi-square, and Fisher's exact test were performed to compare treatments and outcomes by era. Results: There were 68 patients with neonatal retinoblastoma (12% unilateral and 88% bilateral). According to era (pre-chemotherapy vs. chemotherapy), the number of treated patients was 26 (38%) vs. 42 (62%). Primary treatment was external beam radiotherapy (50% vs. 1%,P < 0.001), plaque radiotherapy (17% vs. 0%,P < 0.001), focal treatment (transpupillary thermotherapy or cryotherapy) only (21% vs. 14%,P= 0.33), intravenous chemotherapy (0% vs. 81%,P < 0.001), enucleation (10% vs. 4%,P= 0.26), or exenteration (2% vs. 0%,P= 0.37). Outcomes included tumor control (79% vs. 94%,P= 0.02), globe salvage (75% vs. 91%,P= 0.02), final gross visual acuity for salvaged eyes 20/200 or better (66% vs. 89%,P < 0.01), and death (19% vs. 0%,P < 0.01). Conclusion: Chemotherapy advancements for neonatal retinoblastoma have improved tumor control, globe salvage, visual acuity, and patient survival.
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COMMENTARY Top

Commentary: The challenges of treating retinoblastoma in India p. 2004
Parag K Shah
DOI:10.4103/ijo.IJO_1274_19  PMID:31755438
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Management of retinoblastoma in older children (>5 years) using intra-arterial chemotherapy: Comparison of outcomes to prechemotherapy and intravenous chemotherapy eras p. 2005
Evan B Selzer, R Joel Welch, Pascal Jabbour, Ann M Leahey, Carol L Shields
DOI:10.4103/ijo.IJO_642_19  PMID:31755439
Purpose: Intra-arterial chemotherapy (IAC) has emerged as an effective treatment for retinoblastoma (RB) however, little information exists regarding its use in older patients (>5 years). In the present study, we evaluate the use of IAC (2008–2018) for RB in older patients and compare the outcomes to those in the prechemotherapy (<1994) and intravenous chemotherapy (IVC) (1994–2007) eras. Methods: A retrospective analysis of all patients older than 5 years treated with IAC for RB from 2008–2018. Comparisons were made to 26 active RB cases in older children treated in the prechemotherapy era and to 12 active RB cases treated in the IVC era. Results: There were 13 eyes with RB in 13 older patients treated in the IAC era. The median patient age was 6.8 years. Tumor response was achieved in all 13 eyes at a median interval of 1.1 months from first IAC. Globe salvage was achieved in eight eyes with five eyes requiring enucleation. At 14 months, median follow-up after IAC, there was no metastasis or death. Compared to the prechemotherapy era, those in the IAC era demonstrated significant reduction in need for enucleation (P < 0.001) and EBRT or enucleation (P < 0.001). Compared to the IVC era, there was significant reduction in need for EBRT (P = 0.02) and EBRT or enucleation (P = 0.03) and similar avoidance of metastasis (P > 0.99) and death (P > 0.99). Conclusion: Older patients with RB managed in the IAC era demonstrated reduced need for EBRT or enucleation compared to those managed in the IVC or prechemotherapy eras, with no instance of metastasis or death.
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COMMENTARY Top

Commentary: The shift to intra-arterial chemotherapy – Relevance in Indian context p. 2011
P Mahesh Shanmugam
DOI:10.4103/ijo.IJO_1193_19  PMID:31755440
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Swept-source optical coherence tomography features of regressed macular retinoblastoma p. 2013
Sourav Damodaran, Mandeep S Bajaj, Pradeep Sharma, Atul Kumar, Rohan Chawla, Amar Pujari, Gaurav Garg, Shreyas Temkar
DOI:10.4103/ijo.IJO_533_19  PMID:31755441
Purpose: To describe the swept-source optical coherence tomography (SS-OCT) features of regressed macular retinoblastoma (RB). Methods: A cross-sectional observational study was carried out in 13 patients with regressed macular RB with good fixation in at least one eye. Fundus photography and SS-OCT were documented. High-resolution scans with good signal strength were selected. The types of clinical regression and SS-OCT characteristics of the regressed lesions (presence of vitreous detachment, intratumor schisis/cavitation, calcification, foveal dip, and OCT pattern) were noted. Results: Of the 13 eyes, 7 (53%) were group B, 4 (30%) were group C, and 2 (17%) were group D. Lesion involving fovea was seen in seven eyes (53%). On SS-OCT, the lesion was isodense to hyperdense in all cases. Three patterns of regressed RB were noted on OCT. Intralesion calcification was noted in eight cases. Subretinal fluid was not detected in any of the cases. Conclusion: SS-OCT is a useful technology to image and analyze cases of regressed macular RB including large lesions. SS-OCT system helps in successful imaging even in smaller children.
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Accuracy of preoperative imaging in predicting optic nerve invasion in retinoblastoma: A retrospective study p. 2019
Usha Kim, Gunjan Rathi, Gunja Chowdhary, KG Srinavasan, R Shanthi, R S Prabhu Krishna
DOI:10.4103/ijo.IJO_1611_18  PMID:31755442
Purpose: Optic nerve invasion is an important cause of mortality in retinoblastoma. We aimed correlate preoperative imaging and surgical histopathology findings in enucleated eyes with retinoblastoma to determine the efficacy of preoperative imaging in predicting optic nerve invasion in retinoblastoma. Methods: A retrospective review of records of all patients undergoing primary enucleation for retinoblastoma at a tertiary eyecare system between March 2013 and December 2017 with all patients having undergone preoperative imaging, either CT scan or MRI. Data was analyzed statistically to determine the correlation between preoperative CT scan/MRI and histopathology. Results: Totally, 97 eyes of 97 patients were included in the study who underwent primary enucleation for unilateral retinoblastoma. The average age at presentation was 27.8 months with the chief complaint being leukocoria in all the cases. 14 patients (14.43%) had evidence of optic nerve involvement in preoperative imaging. 30 patients had optic nerve invasion on histopathology (laminar and retrolaminar). Spearman's rank correlation test revealed a significant correlation between MRI findings and HP and an insignficant correlation between CT findings and HP. The CT scan had a sensitivity of 20%, specificity of 88.89%, 50% positive predictive value and 66.67% negative predictive value. MRI had a sensitivity of 40%, specificity of 93.55%, positive predictive value of 66.67% and a negative predictive value of 82.86%. Conclusion: MRI showed significant moderate correlation with surgical histopathology for predicting optic nerve invasion in retinoblastoma whereas CT shows insignificant correlation with HPE. Therefore, we recommend MRI for predicting optic nerve invasion in cases of retinoblastoma.
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Understanding the structural changes following photodynamic and transpupillary thermotherapy for choroidal hemangioma using optical coherence tomography and optical coherence tomography angiography p. 2023
Vishal Raval, Mudit Tyagi, Jay Chhablani, Swathi Kaliki, Rajeev Reddy, Taraprasad Das
DOI:10.4103/ijo.IJO_962_19  PMID:31755443
Purpose: To study optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A) features of circumscribed choroidal hemangioma (CCH) following treatment with photodynamic therapy (PDT) and transpupillary thermotherapy (TTT). Methods: A retrospective chart review of consecutive patients treated for CCH over 2 years (May 2016–April 2018). The investigations, in addition to comprehensive eye examination, included color fundus photography, B-scan ultrasonography, OCT, and OCT-A. Results: The study included 16 eyes of 16 patients (9 males and 7 females). The mean age at presentation was 43.5 ± 9 years (range 33–62 years). Macula (n = 6) and superior arcade (n = 5) were the common tumor locations. Twelve eyes received multiple treatment sessions: TTT (seven eyes; mean 2.4 sessions) and PDT (five eyes; mean 2 sessions). Four eyes were observed because vision was not threatened. Pretreatment OCT features were Bruch's membrane atrophy (15 eyes), retinal pigment epithelial atrophy (13 eyes), outer retinal abnormalities (12 eyes), and macular subretinal fluid (12 eyes). Pretreatment OCT-A features were complete loss of choriocapillaris (16 eyes), irregularly arranged fine arborizing vessels (11 eyes), and more than 50% signal void hyporeflective areas (12 eyes). Posttreatment OCT-A showed persistence of choriocapillaris loss, flat scar with fibrosis and thinning of choroid in all eyes treated with TTT, and persistence of deeper choroidal vessels and no loss of choriocapillaris in eyes treated with PDT. Conclusion: OCT and OCT-A help understand the structural outcome following PDT and TTT in circumscribed choroidal hemangioma.
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Insights into retinal hemangioblastoma using ultra widefield imaging p. 2029
Pradeep Kumar, Raghav Ravani, Sahil Agarwal, Suman Dhanda, Vinod Kumar
DOI:10.4103/ijo.IJO_802_19  PMID:31755444
Purpose: Retinal hemangioblastomas (RHs) are characteristic of von Hippel-Lindau (VHL) disease. Early diagnosis of retinal lesions may aid in systemic diagnosis. Early identification of VHL is life-saving and also prevents vision loss. Fundus fluorescein angiography (FFA) is a useful tool in the diagnosis and management of RHs. The aim of this study is to report FFA features of RH using ultra-widefield (UWF) imaging. Methods: A retrospective cross-sectional study of consecutive patients of RH who underwent UWF FFA at a tertiary eye care center. Images were analyzed and assessed by authors. The main outcome measures were (a) the number and size of RH in each eye and (b) vascular characteristics of the retina. UWF-FFA characteristics in each eye were tabulated. The number of clock hours involved by these characteristics and their correlation with the number and size of RH were analyzed. Results: The study evaluated 24 eyes of 13 patients. The mean age was 28.4 years. The median number of RHs in an eye was 3.5 (range 1–16), and the size of RHs varied from 0.1 to 4 disc diameters. Novel UWF-FFA findings noted in this study were the presence of abnormal capillary network in 22 of 24 eyes (91.7%), capillary leakage in 15 of 24 eyes (62.5%), and capillary telangiectasia in 7 of 24 eyes (29.2%). In addition, feeder arterioles and venules showed bulbous projections in 8 of 24 eyes (33.3%). Conclusion: The UWF-FFA characteristics of RH, which have not been described before, were identified. These add to our understanding of the pathogenesis of the disease and may pave the way for future therapeutic targets.
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Malignant transformation of choroidal nevus according to race in 3334 consecutive patients p. 2035
Charlotte L Marous, Carol L Shields, Michael D Yu, Lauren A Dalvin, David Ancona-Lezama, Jerry A Shields
DOI:10.4103/ijo.IJO_1217_19  PMID:31755445
Purpose: To evaluate choroidal nevus demographics, clinical features, imaging features, and the rate of transformation into melanoma by race. Methods: In this observational case series, There were 3334 participants (3806 choroidal nevi) at a single tertiary-referral center evaluated between January 2, 2007, and August 7, 2017. Retrospective chart and multimodal imaging review was performed. Patient demographics, tumor features, and outcomes were compared between different races using Chi-squared test, Fisher's exact test, t-test, and analysis of variance. The main outcome measure was clinical features of choroidal nevus and the rate of transformation into melanoma by race. Results: Of the 3334 patients, there were Caucasian (n = 3167, 95%) and non-Caucasian (n = 167, 5%). The non-Caucasian races included African-American (n = 27, <1%), Hispanic (n = 38, <1%), Asian (n = 15, <1%), Asian Indian (n = 2, <1%), Middle Eastern (n = 4, <1%), and unknown (n = 83, 3%). By comparison (Caucasian versus vs. non-Caucasian), there were differences in the mean age at presentation (61 vs. 56 years,P < 0.0001), female sex (63% vs. 52%,P < 0.01), dysplastic nevus syndrome (<1% vs. 1%,P < 0.01), and previous cutaneous melanoma (5% vs. 1%,P= 0.03). A comparison of tumor features revealed differences in presence of symptoms (12% vs. 20%,P < 0.01) and ≥3 nevi per eye (3% vs. <1%,P= 0.04). A comparison of imaging features showed no differences. A comparison of outcome of nevus transformation into melanoma revealed no difference (2% vs. 3%,P= 0.29). However, of those nevi exhibiting growth to melanoma, ultrasonographic hollowness was less frequent in Caucasians (29% vs. 67%,P= 0.04). Conclusion: In this analysis of 3334 patients with choroidal nevus, we found differences in the mean age of presentation, sex, dysplastic nevus syndrome, previous cutaneous melanoma, presence of symptoms, and multiplicity of nevus per eye by race. However, there was no difference in the rate of transformation into melanoma by race.
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PHOTO ESSAY Top

Solitary Fibrous Tumor of the lacrimal sac p. 2043
Avriel I Gudkar, Bipasha Mukherjee, Subramanian Krishnakumar
DOI:10.4103/ijo.IJO_875_19  PMID:31755446
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Conjunctival keratoacanthoma/keratoacanthoma like squamous cell carcinoma: Err on the side of caution p. 2044
Anasua Kapoor, VS Vijitha, Priya Mittal, Ruchi Mittal
DOI:10.4103/ijo.IJO_314_19  PMID:31755447
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Metastatic carcinoma masquerading as lens matter p. 2046
Somanath Anjana, Sreedhar Rekha, Sivakumar R Rathinam
DOI:10.4103/ijo.IJO_674_19  PMID:31755448
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Iridocilary tuberculoma mimicking melanoma p. 2048
Neethu Latiff, Sridharan Sudharshan, Aditya Verma, Vikas Khetan, Jyotirmay Biswas
DOI:10.4103/ijo.IJO_1271_19  PMID:31755449
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“String of beads” appearance on fundus fluorescein angiography as a clinical clue for leukemia-related proliferative retinopathy p. 2049
BV Priya, Kushagra Jain, Padmamalini Mahendradas, Bhujang K Shetty
DOI:10.4103/ijo.IJO_233_19  PMID:31755450
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Neoplastic lymphomatous submaculopathy p. 2051
Ilaria Testi, Aniruddha Agarwal, Vishali Gupta, for the PGI Ocular Lymphoma Study Group
DOI:10.4103/ijo.IJO_1697_19  PMID:31755451
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Presumed solitary circumscribed retinal astrocytic proliferation p. 2052
Devashish Dubey, Mahesh Shanmugam, Rajesh Ramanjulu
DOI:10.4103/ijo.IJO_894_19  PMID:31755452
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Neurofibromatosis type-1 with retinal microvascular corkscrew tortuosity p. 2054
Adrian J Battiston, Lauren A Dalvin, Carol L Shields
DOI:10.4103/ijo.IJO_1796_19  PMID:31755453
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Multicolor imaging of optic disc melanocytoma p. 2056
Arpitha Pereira, Sherina Thomas, Naresh Kumar Yadav, Ramesh Venkatesh
DOI:10.4103/ijo.IJO_1954_19  PMID:31755454
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Ultra-widefield angiographic imaging of albumin-bound paclitaxel-induced cystoid macular edema p. 2058
Junhyuck Lee, Ho Ra, Jiwon Baek
DOI:10.4103/ijo.IJO_734_19  PMID:31755455
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Central retinal artery occlusion in a child with angiolymphoid hyperplasia with eosinophilia orbit p. 2059
VS Vijitha, Anasua Ganguly Kapoor, Ruchi Mittal, Aditya Kapoor
DOI:10.4103/ijo.IJO_1422_19  PMID:31755456
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OPHTHALMIC IMAGES Top

Cavernous hemangioma of the conjunctiva p. 2061
Bipasha Mukherjee, Sayeed Moosa, Rama Rajagopal
DOI:10.4103/ijo.IJO_1783_18  PMID:31755457
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Bilateral iris cysts in an infant with retinopathy of prematurity p. 2062
Simar Rajan Singh, Mohit Dogra, Deeksha Katoch, Mangat Ram Dogra
DOI:10.4103/ijo.IJO_857_19  PMID:31755458
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Doughnut osteoma: A rare case of circumpapillary choroidal osteoma p. 2063
Manit Sanjay Agrawal, Madhu Kumar, Vinaya Kumar Konana, Jayamadhury Gudimetla, Ashok Kanakamedla, Parag Anil Bagad
DOI:10.4103/ijo.IJO_454_19  PMID:31755459
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CASE REPORTS Top

A rare case of benign fibrous histiocytoma of the cornea p. 2064
Sudesh Arya, Tanu Singh, Ranjeev Bhagat, Vinisha Bansal
DOI:10.4103/ijo.IJO_1648_18  PMID:31755460
Fibrous histiocytoma (FH) commonly occurs in the superficial layers of the skin. Orbit and limbus are documented ophthalmic sites of involvement but isolated corneal FH has never been reported in literature. We present the first case of FH exclusively involving the cornea where a 10-year-old male child presented with a 3-month history of a painless growth on the superior cornea of the right eye with deterioration of vision. Tumor excision with therapeutic penetrating keratoplasty was done and the histopathological examination confirmed the diagnosis. There was no recurrence and the corneal graft was clear at 1 year.
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Severe necrotising inflammatory skin reaction to topical 5-fluorouracil p. 2066
Nikhil S Gokhale
DOI:10.4103/ijo.IJO_373_19  PMID:31755461
I report two patients who developed a severe necrotising inflammatory skin reaction with ulceration and hemorrhagic crusting during the first cycle of chemotherapy with 1% 5-Fluorouracil eye drops for Ocular surface squamous neoplasia. The skin reaction subsided on stoppage of the drops and the use of a steroid ointment. 5 fluorouracil therapy was terminated and the patients were shifted to interferon therapy subsequently.
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Ipsilateral presentation of ocular surface squamous neoplasia and conjunctival melanoma in xeroderma pigmentosum: A rare occurrence p. 2068
Deepanjali Arya, Sima Das, Arpan Gandhi
DOI:10.4103/ijo.IJO_1449_19  PMID:31755462
A case of a 16-year-old male with xeroderma pigmentosum (XP) who presented with multiple pigmented and nonpigmented conjunctival lesions in both eyes is reported. He had a keratinized lesion at the limbus and a pigmented lesion in bulbar conjunctiva in the left eye and multiple pigmented bulbar conjunctival lesions and a keratinized limbal nodule in the right eye. Excision biopsy confirmed the diagnosis of ocular surface squamous neoplasia (OSSN) and conjunctival intraepithelial melanocytic neoplasia-2 (CIMN-2) in the right eye and OSSN and conjunctival melanoma in situ (CIMN-5) in the left eye. Two malignant conjunctival lesions occurring simultaneously in the same eye of a patient with XP have not been reported earlier.
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Conjunctival melanoma with orbital invasion and liver metastasis managed with systemic immune checkpoint inhibitor therapy p. 2071
Michael Chang, Sara E Lally, Lauren A Dalvin, Marlana M Orloff, Carol L Shields
DOI:10.4103/ijo.IJO_663_19  PMID:31755463
A 60-year-old Caucasian female was referred for biopsy-proven amelanotic orbito-conjunctival melanoma. Map biopsies revealed residual invasive melanoma on the deep tarsal margin at the site of previous surgery. Repeat excisions were required after recurrence was detected following 3 months and 7 months. Positron emission tomography scan detected liver metastasis and additional orbito-conjunctival melanoma recurrence. Biomarker testing showed NRAS mutation without BRAF or c-KIT mutations and without PD-L1 expression. Systemic checkpoint inhibitor therapy was initiated with regression of both the orbito-conjunctival melanoma and liver metastasis. Invasive, non resectable orbito-conjunctival melanoma with liver metastasis can demonstrate a response to systemic checkpoint inhibitor therapy.
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A novel side effect of mitogen-activated protein kinase inhibitor cobimetinib: Acute corneal decompensation p. 2073
Mahmut Asfuroglu, Yonca Asfuroğlu
DOI:10.4103/ijo.IJO_2025_18  PMID:31755464
A 38-year-old man with a diagnosis of BRAF-mutated metastatic melanoma was referred to our clinic. He had been under treatment with 60-mg oral cobimetinib daily for 21 days/7 day off in combination with 960 mg vemurafenib twice daily. The patient had symptoms of blurred vision and photophobia in his right eye. A slit-lamp examination revealed bilateral central corneal stromal opacity and epithelial microcystic edema Involvement was more severe in the right eye compared with the left eye. Fourteen days after the first visit, the patient's symptoms and slit-lamp findings were largely resolved. We suggest that endothelium pump failure was involved in this acute corneal decompensation case similar to the mechanism in retinal pigment epithelium.
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Case report: Bilateral uveitis and papillitis secondary to treatment with pembrolizumab p. 2075
Carmen Navarro-Perea, Javier Garcia-Gonzalez, Eugenio Perez-Blazquez
DOI:10.4103/ijo.IJO_1161_19  PMID:31755465
Pembrolizumab is a programmed cell death protein 1 (PD-1) monoclonal antibody used in the treatment of metastatic melanomas. Severe ocular complications appear in less than 1% of the patients and require early treatment. We present the case of a patient diagnosed with a BRAF mutated metastatic melanoma. Ocular pain and a blurred vision appeared after treatment and the patient visited the ophthalmology emergency room, where he was diagnosed with acute anterior uveitis (AAU), synechiae, and bilateral papillitis. The patient was treated with topical corticosteroids, prednisone, and mydriatics, which immediately improved the patient's status. Therefore, when an ocular inflammatory disease exists, immune checkpoint inhibitor treatments must be ruled out as possible causes.
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Intraocular amyloidosis with multifocal iris and anterior chamber translucent spherules p. 2078
Sima Das, Sweety Tiple, Julie Pegu, Suneeta Dubey, Umang Mathur, Kaustabh Mulay, Carol L Shields
DOI:10.4103/ijo.IJO_812_19  PMID:31755466
Ocular amylodosis, although a rare entity, is known to affect the conjunctiva, extraocular muscles, orbit, lacrimal gland, and skin around the eyes. Intraocular deposition of amyloid mainly confines to the vitreous and cornea. In this report, we describe two cases of intraocular amyloidosis presenting as multiple iris and anterior chamber cysts. Histopathological examination with special stain like Congo Red and Transmission Electron Microscopy confirmed the diagnosis of amyloidosis. Systemic investigations ruled out systemic association confirming the diagnosis of primary ocular amyloidosis.
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Spontaneous microscopic hyphema secondary to iris vascular tufts: Case report with video documentation p. 2080
Nicolas A Blanco, Alejandro Tello, Virgilio Galvis, Maria Fernanda Acuña, Angelica Pedraza-Concha
DOI:10.4103/ijo.IJO_1200_19  PMID:31755467
A 71-year-old woman presented with spontaneous microhyphema in her left eye, causing blurry vision. Bleeding stopped spontaneously shortly after several cycles of digital compression on the upper eyelid, (which were documented in video), and therefore, did not require laser photocoagulation, a possible approach previously explained to the patient. A microhemangioma at the edge of the iris was identified to be the cause of the condition. The hemorrhage did not recur during the follow-up period (9 months).
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Vitrectomy as a treatment modality in vitreous seeding secondary to ciliary body melanocytoma p. 2083
P Mahesh Shanmugam, Ishfaq A Sofi, Pradeep Sagar, Vinaya K Konana, Rajesh Ramanjulu
DOI:10.4103/ijo.IJO_439_19  PMID:31755468
Melanocytoma is a locally invasive intraocular tumor usually located in the optic nerve head, iris, ciliary body and choroid. Melanocytoma can undergo necrosis and lead to pigment dispersion. We report a case of melanocytoma of the ciliary body with vitreous seeds filling the vitreous cavity. A sub conjunctival pigmented lesion was seen due to extra scleral extension of the tumor. The diagnosis of melanocytoma was confirmed by biopsy of the sub conjunctival lesion. Pars plana vitrectomy was performed to clear the vitreous cavity with good visual recovery.
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COMMENTARY Top

Commentary: Vitrectomy as a treatment modality in vitreous seeding secondary to ciliary body melanocytoma p. 2085
Pukhraj Rishi
DOI:10.4103/ijo.IJO_1119_19  PMID:31755469
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CASE REPORTS Top

Optical coherence tomography angiography–based analysis of intrinsic vasculature in juxtapapillary melanoma after ruthenium-106 plaque brachytherapy p. 2086
P Mahesh Shanmugam, Vinaya Kumar Konana, Rajesh Ramanjulu, K C Divyansh Mishra, Pradeep Sagar, Dilip Kumar
DOI:10.4103/ijo.IJO_1928_18  PMID:31755470
In this case report, we demonstrate the use of optical coherence tomography angiography (OCTA) as a tool to evaluate intrinsic vasculature in a case of juxtapapillary melanoma which underwent ruthinium.106 plaque brachytherapy. In this case, OCTA could demonstrate a decrease in caliber and density of the intrinsic vasculature of the tumor post brachytherapy.
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Multifocal choroidal melanoma in oculodermal melanocytosis in an Asian Indian p. 2089
Raksha Rao, Santosh G Honavar, Kaustubh Mulay
DOI:10.4103/ijo.IJO_1773_18  PMID:31755471
A 51-year-old Asian Indian male with right oculodermal melanocytosis presented with a blurred visual acuity of 20/40. Upon fundus examination, he was discovered to have two independent pigmented choroidal melanomas in the temporal juxtapapillary region and inferiorly, in the region of trace clinical sectoral choroidal melanocytosis. The patient underwent enucleation, and on histopathology the two tumors were found to be in the area of choroidal melanocytosis, in continuity with each other. Multifocal choroidal melanoma is an exceedingly rare diagnosis. Ocular melanocytosis is a known predisposing factor for both unifocal and multifocal melanomas. Lifelong monitoring for uveal melanomas must be done in all patients with ocular melanocytosis.
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Trifocal choroidal melanoma in an eye with oculodermal melanocytosis: A case report p. 2092
Samuel J Fallon, Charlotte N Shields, Maura Di Nicola
DOI:10.4103/ijo.IJO_493_19  PMID:31755472
We report a case of trifocal choroidal melanoma (three separate tumors) in a 48-year-old Caucasian female who had been followed for oculodermal melanocytosis since childhood. At presentation, no tumor was present and annual examination was advised. Seventeen years later, three choroidal melanocytic lesions were detected in the right eye. Growth of each was documented, enucleation was performed, and histopathology revealed three independent choroidal melanomas. The patient developed extensive liver and bone metastases and subsequently died. Oculodermal melanocytosis is a risk factor for the development of uveal melanoma and a potential marker for worse prognosis. Careful long-term follow-up is required.
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Bilateral diffuse uveal melanocytic proliferation secondary to thyroid carcinoma p. 2094
Vishal Raval, Avinash Pathengay, Raja Narayanan
DOI:10.4103/ijo.IJO_445_19  PMID:31755473
We present a rare case of a bilateral diffuse uveal melanocytic proliferation (BDUMP), which occurred secondary to recurrence of carcinoma of thyroid in a 79-year-old gentleman who was initially misdiagnosed to have age related macular degeneration and/or chronic central serous chorioretinopathy. In spite of being treated with anti-VEGF injection and photodynamic therapy there was progressive loss of vision. Multimodal imaging like autoflourescence, infrared imaging, fluorescein angiography, indocyanine angiography, and OCT angiography helped us in clinching the final diagnosis.
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Sequential imaging of a case of choroidal osteoma using swept-source OCT and optical coherence tomography angiography: A 4-year follow-up study p. 2097
Thirumalesh M Basavaraj, Sugaranjini Galiyugavaradhan
DOI:10.4103/ijo.IJO_919_19  PMID:31755474
A 33-year-old gentleman was presented with metamorphopsia in the left eye due to choroidal osteoma (CO) complicated by choroidal neovascular membrane (CNVM). Optical coherence tomography angiography (OCTA) proved to be a valuable, noninvasive tool in monitoring treatment response of CNVM. The tumor subsequently underwent decalcification over a period of 4 years. In addition, SS-OCT scans were instrumental in documenting the natural course of the tumor and focal choroidal excavations (FCE), which were found in correspondence with tumor decalcification. Close follow-up is warranted in FCE, secondary to decalcification of CO, as CNVM has been documented to occur on the slope or bottom of eyes with FCE.
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Retinal cafe-au-lait macules: A rare retinal finding in a patient with neurofibromatosis type 1 p. 2101
Ramesh Venkatesh, Kushagra Jain, Arpitha Pereira, Shreya Dass Jain, Aditya Aseem, Padmamalini Mahendradas, Naresh Kumar Yadav
DOI:10.4103/ijo.IJO_925_19  PMID:31755475
We report the retinal and choroidal manifestations using multimodal imaging in a patient with Neurofibromatosis type 1 (NF-1). In this report, we describe the occurrence of a new retinal finding which we label as retinal café-au-lait macules. Also, we describe the superiority of multicolour imaging in comparison to colour fundus photography for identifying the retinal manifestations in NF-1.
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Laser photocoagulation, intravitreal anti-VEGF, and vitreous surgery for the treatment of juxtapapillary retinal capillary hemangioma p. 2104
Gamze Ucan Gunduz, Oner Gelisken, Ozgur Yalcinbayir, Kazim Guler
DOI:10.4103/ijo.IJO_1799_18  PMID:31755476
Juxtapapillary retinal capillary hemangiomas (JRCHs) are benign vascular tumors located on or adjacent to the optic nerve head. A 19-year-old girl presented with epiretinal membrane (ERM) associated with an elevated and round vascular tumoral mass located in the juxtapapillary region of her left eye. She was subsequently diagnosed with isolated JRCH. A combined approach with laser photocoagulation and intravitreal bevacizumab injection was used to facilitate shrinkage of the tumor preoperatively and pars plana vitrectomy was used to remove the tumor and ERM. A small remnant of tumoral mass remained intact and did not show any growth for 7 years.
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Kimura's disease involving bilateral lacrimal glands and extraocular muscles along with ipsilateral face: A unique case report p. 2107
Chandana Chakraborti, Ajoy Kumar Saha, Ankita Bhattacharjee, Rupanjili Lakra
DOI:10.4103/ijo.IJO_810_19  PMID:31755477
A 23 year female presented with bilateral recurrent swelling of eyelids along with ptosis and proptosis for last 3 years. She also had swellings over the right cheek, parotid gland, and retro auricular area along with regional lymphadenopathy. Systemic laboratory workup revealed raised serum IgE and a high peripheral eosinophil count. Computed tomography and magnetic resonance imaging showed bilateral enlargement of extraocular muscles, lacrimal glands, and ipsilateral parotid gland. Excision biopsy of the retro-auricular lymph node was suggestive of Kimura's disease (KD). The patient responded well to systemic corticosteroid. KD rarely affects orbit, but it should be included in the differential diagnosis of inflammatory diseases of the orbit. To our knowledge, this is the first reported case of KD from India involving the orbit, lacrimal gland, extraocular muscles, parotid gland and buccal area.
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Primary chondromyxoid fibroma of the orbit: An orbital mass with calcification p. 2110
Aditi Mehta Grewal, Manpreet Singh, Vikarn Vishwajeet, Umang Thakur, Ashim Das, Pankaj Gupta
DOI:10.4103/ijo.IJO_1275_19  PMID:31755478
Primary orbital chondromyxoid fibroma is a rarely reported entity. A 34-year-old lady presented with painless, non-axial proptosis of the left eye of 6 months duration. Orbital imaging showed a supero-temporal mass with calcific foci and bone erosion. The mass caused globe compression resulting in choroidal folds. Anterior orbitotomy with complete mass excision was performed. The histopathology revealed a chondromyxoid fibroma. At 12-months follow-up, the patient is doing fine with no clinical recurrence. Chondromyxoid fibroma is an important differential diagnosis for bony orbital tumors.
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