Carol L Shields

L V Prasad Eye Institute, Hyderābād, State of Andhra Pradesh, India

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Publications (387)1155.57 Total impact

  • Article: American Joint Committee on Cancer Classification of Posterior Uveal Melanoma (Tumor Size Category) Predicts Prognosis in 7731 Patients.
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    ABSTRACT: PURPOSE: To evaluate the clinical features and prognosis of posterior uveal (ciliary body and choroid) melanoma based on the American Joint Committee on Cancer (AJCC) classification (7th edition) of primary tumor (T). DESIGN: Retrospective, interventional case series. PARTICIPANTS: Seven thousand seven hundred thirty-one patients. INTERVENTION: Ocular management including plaque radiotherapy, enucleation, local resection, or laser therapy. MAIN OUTCOME MEASURES: Melanoma-related metastasis and death. RESULTS: Of 7731 patients with posterior uveal melanoma, the AJCC classification based on T was category T1 in 3557 (46%), T2 in 2082 (27%), T3 in 1599 (21%), and T4 in 493 (6%). Based on tumor categories T1, T2, T3, and T4, respectively, features that showed significant increase with tumor category included patient age at presentation (57, 58, 58, and 61 years; P<0.001), tumor base (8, 12, 15, and 20 mm; P<0.001), tumor thickness (3.5, 5.2, 8.9, and 11.4 mm; P<0.001), mushroom configuration (8%, 20%, 38%, and 39%; P<0.001), associated subretinal fluid (64%, 80%, 82%, and 83%; P<0.001), intraocular hemorrhage (5%, 12%, 17%, and 18%; P<0.001), rupture of Bruch's membrane (9%, 24%, 40%, and 40%; P<0.001), and extraocular extension (1%, <1%, 4%, and 12%; P<0.001). After therapy, Kaplan-Meier estimates of metastasis at 5, 10, and 20 years were 8%, 15%, and 25% for category T1, 14%, 25%, and 40% for category T2, 31%, 49%, and 62% for category T3, and 51%, 63%, and 69% for category T4, respectively (P<0.001). Kaplan-Meier estimates of death at 5, 10, and 20 years were 4%, 8%, and 11% for category T1, 8%, 13%, and 24% for category T2, 19%, 27%, and 36% for category T3, and 30%, 43%, and 51% for category T4, respectively (P<0.001). Compared with category T1, the hazard ratio for metastasis and death for T2 was 1.8 and 1.9, respectively, that for T3 was 4.5 and 4.7, respectively, and that for T4 was 8.2 and 8.8, respectively. CONCLUSIONS: Based on the AJCC classification, increasing tumor category was associated with older age, larger tumor, and greater incidence of subretinal fluid, hemorrhage, and extraocular extension. Compared with uveal melanoma classified as T1, the rate of metastasis and death was 2 times greater for T2, 4 times greater for T3, and 8 times greater for T4. The risk for metastasis and death increased 2-fold with each increasing melanoma category. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 05/2013; · 5.45 Impact Factor
  • Article: Choroidal Hemorrhage Simulating Choroidal Melanoma.
    Retina (Philadelphia, Pa.) 04/2013; · 2.93 Impact Factor
  • Article: DIFFUSE VERSUS NONDIFFUSE SMALL (≤3 MM THICKNESS) CHOROIDAL MELANOMA: Comparative Analysis in 1,751 Cases. The 2012 F. Phinizy Calhoun Lecture.
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    ABSTRACT: OBJECTIVE:: To determine prognosis of small choroidal melanoma (≤3 mm thickness) comparing diffuse versus nondiffuse variants. METHODS:: Retrospective chart review of 1,751 patients with small choroidal melanoma classified as diffuse (thickness/base ≤20%) versus nondiffuse (thickness/base >20%). RESULTS:: Of 1,751 patients with small choroidal melanoma, 297 (17%) were diffuse and 1,454 (83%) nondiffuse. Features with statistical differences (diffuse vs. nondiffuse) included mean distance to optic disk (3 vs. 4 mm), mean tumor base (12 vs. 8 mm), and mean tumor thickness (1.9 vs. 2.5 mm). Using Kaplan-Meier estimates, melanoma-related metastasis (diffuse vs. nondiffuse) was 8% versus 4% at 5 years, 16% versus 10% at 10 years, and 19% versus 16% at 15 years (P = 0.0344). Melanoma-related death was 6% versus 2% at 5 years, 11% versus 4% at 10 years, and 16% versus 6% at 15 years (P < 0.0001). In the subgroup of thin melanoma 2 mm or less in thickness, melanoma-related death was 7% versus 2% at 5 years, 10% versus 2% at 10 years, and 16% versus 4% at 15 years (P = 0.0077). By multivariate analysis, factors predictive of metastasis from diffuse melanoma included larger tumor basal dimension (P = 0.0027) and plateau/flat tumor configuration (P = 0.0257). CONCLUSION:: Of 1751 patients with small (≤3 mm thickness) choroidal melanoma, those with diffuse tumor show higher probability of metastasis and death than those with nondiffuse tumor. This finding is evident even in the thinnest melanomas (≤2 mm thickness).
    Retina (Philadelphia, Pa.) 04/2013; · 2.93 Impact Factor
  • Article: Ciliochoroidal metastasis as the initial manifestation of an occult soft-tissue extraosseous sarcoma in a 10-year-old girl.
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    ABSTRACT: Uveal metastases are rare in children and metastasis from sarcoma is rare at any age. We report a purportedly healthy 10-year-old girl who developed ciliochoroidal metastasis from an occult primary extraosseous sarcoma of the ankle region. The patient died from widespread metastases 6 months after enucleation despite intensive chemotherapy.
    Journal of AAPOS: the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus 04/2013; 17(2):217-20. · 1.07 Impact Factor
  • Article: Conjunctival Papilloma: Features and Outcomes Based on Age at Initial Examination.
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    ABSTRACT: IMPORTANCE Conjunctival papilloma is a benign epithelial tumor occurring in both children and adults with varying clinical features and outcomes. In this article, we describe our experience regarding the difference in the clinical features and outcomes of conjunctival papilloma based on age at initial examination. OBJECTIVE To evaluate the clinical features, treatment, and outcomes in patients with conjunctival papilloma based on age at initial examination. DESIGN Retrospective study. SETTING Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania. PARTICIPANTS Ten children and adolescents (aged ≤20 years) and 63 adults (aged >20 years) with conjunctival papilloma. INTERVENTIONS Excisional biopsy, cryotherapy, oral cimetidine, topical or injection interferon alfa-2b, and photodynamic therapy. MAIN OUTCOME MEASURE Tumor response. RESULTS A comparison of conjunctival papillomas between age groups revealed significant differences in the mean number of tumors per eye (children and adolescents vs adults, 2 vs 1; P = .05), tumor basal dimension (8 vs 6 mm; P = .05), and associated feeder vessels (20% vs 47%; P = .05). Primary treatment included sole treatment with oral cimetidine (15% vs 5%), topical interferon alfa-2b (0% vs 1%), cryotherapy (0% vs 3%), photodynamic therapy (0% vs 1%), excisional biopsy and cryotherapy (38% vs 65%), excisional biopsy and cryotherapy with adjuvant oral cimetidine (8% vs 9%), and excisional biopsy and cryotherapy with adjuvant topical or injection interferon alfa-2b (38% vs 15%). Significant differences in age groups in treatment outcome during the follow-up period (mean, 24 vs 38 months) included complete regression with single treatment (38% vs 95%; P < .01) and tumor recurrence (15% vs 1%; P = .05). CONCLUSIONS AND RELEVANCE Conjunctival papillomas are larger and more likely to be multiple in children and adolescents than in adults. Excisional biopsy and cryotherapy with or without adjuvant oral cimetidine and/or topical interferon alfa-2b provide satisfactory tumor control. Papilloma recurrence is more common in children and adolescents than in adults.
    JAMA ophthalmology. 03/2013;
  • Article: Metastasis from Uveal Melanoma Associated with Congenital Ocular Melanocytosis: A Matched Study.
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    ABSTRACT: PURPOSE: To determine the rate of metastasis resulting from uveal melanoma associated with congenital ocular melanocytosis (COM) and to compare it with the rate of metastasis resulting from uveal melanoma not associated with COM. DESIGN: Matched retrospective study. PARTICIPANTS: Fifty-seven patients with uveal melanoma associated with ocular melanocytosis (melanocytosis group). Each patient in the melanocytosis group was matched with 2 patients with uveal melanoma not associated with ocular melanocytosis (no melanocytosis group) for age, gender, location of anterior tumor margin, location of tumor epicenter, tumor basal diameter, and tumor thickness. METHODS: Review of medical records. MAIN OUTCOME MEASURES: Metastasis resulting from uveal melanoma. RESULTS: The mean basal diameter and thickness of tumors in the melanocytosis group were 10 and 4.9 mm, respectively, and most were located between the macula and equator (70%). The melanocytosis and no melanocytosis groups were similar with regard to all patient, ocular, and tumor features studied. Nineteen (33%) of 57 patients in the melanocytosis group and 18 (16%) of 114 patients in the no melanocytosis group demonstrated systemic metastasis during mean follow-up periods of 77 months (range, 1-402) and 64 months (range, 1-252), respectively (P = 0.013). The Kaplan-Meier estimates for systemic metastasis in the melanocytosis group at 5 and 15 years were 27% and 59%, respectively, compared with 15% and 33%, respectively, in the no melanocytosis group (hazard ratio, 1.99; 95% confidence interval, 1.16-3.41). CONCLUSIONS: In this matched study, patients with uveal melanoma associated with COM were twice as likely to have systemic metastasis compared with patients with uveal melanoma not associated with COM. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 03/2013; · 5.45 Impact Factor
  • Article: Tumor-related lipid exudation and associated tumor-related complications after plaque radiotherapy of posterior uveal melanoma.
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    ABSTRACT: Objective. To identify complications associated with tumor-related lipid exudation (TRLE) after plaque radiotherapy of posterior uveal melanoma. Methods. Comparative study including 294 patients with posterior uveal melanoma who developed TRLE after plaque radiotherapy (TRLE group) and 294 patients who did not develop TRLE (non-TRLE group). Patients in the TRLE group were matched with patients in the non-TRLE group for age, gender, and initial tumor thickness. Results. The TRLE group was associated with significantly higher incidence of postradiation complications including early transient increase of serous retinal detachment (p and lt;0.001), iris neovascularization (NVI) (p and lt;0.001), neovascular glaucoma (NVG) (p and lt;0.001), posterior synechia (p and lt;0.001), vitreous hemorrhage (p and lt;0.001), subretinal hemorrhage (p and lt;0.001), and retinoschisis (p and lt;0.001). A significant relationship was found between extent of TRLE and NVI (p and lt;0.001), NVG (p and lt;0.001), posterior synechia (p = 0.007), and retinoschisis over tumor (p = 0.010). Radiation-induced retinopathy, maculopathy, and papillopathy were not associated with presence or extent of TRLE. Tumor-related lipid exudation was also associated with worse final visual acuity (p = 0.011), higher rate of secondary enucleation (p and lt;0.001), and lower rate of systemic metastasis (p = 0.006). Conclusions. Tumor-related lipid exudation following plaque radiotherapy of posterior uveal melanoma is associated with major intraocular complications and portends poor prognosis for final visual acuity and eye retention. These findings provide evidence for the presence of 2 distinct pathogenic mechanisms, one radiation-induced and one tumor-related, in development of postradiation complications in eyes with posterior uveal melanoma.
    European journal of ophthalmology 03/2013; · 0.96 Impact Factor
  • Article: Retinal Vasoproliferative Tumors: Comparative Clinical Features of Primary vs Secondary Tumors in 334 Cases.
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    ABSTRACT: OBJECTIVE To compare the clinical features of primary vs secondary retinal vasoproliferative tumors (VPTs). METHODS Retrospective case series of 334 tumors in 295 eyes of 275 patients. RESULTS Of 275 patients with VPT, 41% (n = 113) were male and 59% (n = 162) were female, with a mean age of 44 years at presentation. Primary VPT occurred in 80% (n = 219) and secondary VPT, in 20% (n = 56) of patients. Secondary VPT (n = 67) occurred in eyes with retinitis pigmentosa (n = 15, 22%), pars planitis (n = 14, 21%), Coats disease (n = 11, 16%), previous retinal detachment surgery (n = 8, 12%), idiopathic peripheral retinal vasculitis (n = 4, 6%), familial exudative vitreoretinopathy (n = 3, 4%), and others (n = 12, 18%). The mean interval between diagnosis of underlying ocular condition and secondary VPT was 160 months. Statistically significant differences (P < .05) in clinical features (primary vs secondary VPTs) included mean age at presentation (46 vs 38 years), visual symptoms (74% vs 87%), poor visual acuity worse than 20/200 (15% vs 28%), bilaterality (4% vs 20%), multifocality (5% vs 15%), postequatorial tumor location (20% vs 33%), tumor basal dimension (6 vs 7 mm), anterior chamber cells (16% vs 30%), and vitreous cells (19% vs 48%). CONCLUSIONS Retinal vasoproliferative tumor can be primary (80%) or secondary (20%). Compared with primary VPT, secondary VPT is more often bilateral, multiple, and larger and occurs at an earlier age associated with poorer visual acuity.
    JAMA ophthalmology. 03/2013; 131(3):328-334.
  • Article: Autofluorescence of intraocular tumours.
    Ana Almeida, Swathi Kaliki, Carol L Shields
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    ABSTRACT: PURPOSE OF REVIEW: Fundus autofluorescence is a noninvasive technique for evaluation of intrinsic autofluorescence of the tissues within the eye. In recent years, autofluorescence has become an important diagnostic tool for the assessment of various ocular diseases such as age-related macular degeneration and retinal dystrophies. In this report, we review the recent literature on autofluorescence of intraocular tumours. RECENT FINDINGS: The autofluorescence features of intraocular tumours range from bright hyperautofluorescence to dark hypoautofluorescence. The fundus autofluorescence generally represents the status of the overlying retinal pigment epithelium (RPE). Choroidal nevi typically have overlying hypoautofluorescence from chronic RPE atrophy as opposed to choroidal melanoma that exhibits hyperautofluorescence from overlying lipofuscin within RPE (orange pigment) and free fluorophores within fresh subretinal fluid. Choroidal metastases demonstrate overlying hyperautofluorescence that correlates to focal RPE accumulation of lipofuscin as well as subretinal fluid, particularly on the fresh advancing tumour margin. Choroidal haemangioma displays overlying hyperautofluorescence from lipofuscin within RPE and fresh subretinal fluid, but when choroidal haemangioma is chronic with resolved fluid, there is often overlying hypoautofluorescence from RPE atrophy. Congenital hypertrophy of the RPE is characterized by marked hypoautofluorescence of the RPE lesion and trace hyperautofluorescence within the lacunae. SUMMARY: Autofluorescence is a noninvasive, valuable diagnostic tool for assessment of intraocular tumours, based primarily on the effects on the overlying RPE. Some findings are strongly characteristic of certain tumours, particularly the bright hyperautofluorescence overlying small choroidal melanoma and the dark hypoautofluorescence of congenital hypertrophy of the RPE.
    Current opinion in ophthalmology 02/2013; · 2.49 Impact Factor
  • Article: High-Risk Retinoblastoma Based on International Classification of Retinoblastoma: Analysis of 519 Enucleated Eyes.
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    ABSTRACT: PURPOSE: To determine the correlation between the International Classification of Retinoblastoma (ICRB) and histopathologic high-risk retinoblastoma. DESIGN: Retrospective study. PARTICIPANTS: A total of 519 patients. INTERVENTION: Primary enucleation. MAIN OUTCOME MEASURES: High-risk retinoblastoma, metastasis, and death. RESULTS: Of 519 primarily enucleated eyes, 87 (17%) were classified as group D and 432 (83%) were classified as group E on the basis of the ICRB. High-risk retinoblastoma was identified in 23% (117/519) of enucleated eyes, including 17% (15/87) group D and 24% (102/432) group E eyes. High-risk histopathologic features of retinoblastoma included anterior chamber involvement (5/15 [33%] group D eyes, 31/102 [30%] group E eyes), isolated massive posterior uveal invasion ≥3 mm (7/15 [47%] group D eyes, 22/102 [22%] group E eyes), isolated post-laminar optic nerve invasion (2/15 [13%] group D eyes, 46/102 [45%] group E eyes), and any combination of posterior uveal invasion and optic nerve involvement (7/15 [47%] group D eyes, 37/102 [36%] group E eyes). On logistic regression analysis, massive posterior uveal invasion ≥3 mm was more common in group D eyes (P = 0.0442), and post-laminar optic nerve invasion was more common in group E eyes (P = 0.0390). Of 117 patients with high-risk retinoblastoma, systemic adjuvant chemotherapy was administered in 83 patients (71%). Systemic metastasis developed in 0% (0/15) of those with high-risk group D retinoblastoma and 10% (10/102) of those with high-risk group E retinoblastoma over a mean follow-up period of 78 months (median, 62 months; range, 1-419 months). There was no metastasis in any patient (n = 402) classified with non-high-risk retinoblastoma. Of the 10 patients who developed metastasis, 4 had received prior adjuvant chemotherapy and 6 had no prior adjuvant chemotherapy. There was no metastasis in high-risk patients treated with vincristine sulphate, etoposide phosphate, and carboplatin (VEC). Death from metastasis occurred in 4% of high-risk patients (5/117). CONCLUSIONS: On the basis of the ICRB, 17% of group D and 24% of group E eyes are at increased risk for metastatic disease. In this study, 8% of patients developed metastasis. There was no metastasis in any patient classified with non-high-risk retinoblastoma. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 02/2013; · 5.45 Impact Factor
  • Article: Pigmented choroidal nevus in a child with oculocutaneous albinism.
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    ABSTRACT: We report the case of an 8-year-old white girl with albinism and a flat pigmented choroidal lesion in the left eye measuring 0.5 mm in diameter. There was no subretinal fluid, lipofuscin, or drusen. The patient later displayed 10 lightly-pigmented cutaneous nevi on her upper chest, left arm, and right leg at 8 months' follow-up. The choroidal nevus showed minimal change over 2 years.
    Journal of AAPOS: the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus 01/2013; · 1.07 Impact Factor
  • Article: Scleral Necrosis after Plaque Radiotherapy of Uveal Melanoma: A Case-Control Study.
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    ABSTRACT: PURPOSE: To identify risk factors and outcome of scleral necrosis after plaque radiotherapy of uveal melanoma. DESIGN: Case-control study. PARTICIPANTS: A total of 73 cases with scleral necrosis and 73 controls without necrosis after plaque radiotherapy. Controls were matched for anteroposterior tumor epicenter and follow-up duration. INTERVENTION: Plaque radiotherapy with iodine-125, cobalt-60, iridium-192, or ruthenium-106. MAIN OUTCOME MEASURE: Scleral necrosis. RESULTS: Of 5057 patients treated with plaque radiotherapy for uveal melanoma, 73 (1%) developed radiotherapy-induced scleral necrosis. Scleral necrosis occurred in <1% of patients (3/1140) when plaque radiotherapy was used for tumors <3 mm in thickness, 1% of patients (33/3155) with 3- to 8-mm tumor thickness, and 5% of patients (37/762) with >8-mm-thick tumors. On the basis of tumor location, scleral necrosis was detected after plaque radiotherapy of iris melanoma in 0% of patients (0/91), ciliary body melanoma in 29% of patients (67/235), and choroid melanoma in <1% of patients (6/4731). The mean time interval between plaque radiotherapy and scleral necrosis was 32 months (median, 23 months; range, 4-126 months). The mean basal dimension of scleral necrosis was 4 mm (median, 3 mm; range, 1-15 mm), equivalent to 29% of mean tumor base (median, 24%; range, 6%-100%) and 22% of mean plaque size (median, 19%; range, 5%-75%). Multivariate analysis of factors that predicted clinically evident scleral necrosis included ciliary body (P = 0.0001) and pars plana to ora serrata (P < 0.0001) locations of anterior tumor margin, tumor thickness ≥6 mm (P = 0.0001), and radiation dose ≥400 Gy to the outer sclera (P = 0.0455). Scleral necrosis remained stable in 48% of patients (35/73), increased in size/severity in 48% of patients (35/73), or progressed to scleral perforation in 4% of patients (3/73) over a mean follow-up of 79 months (median, 54 months; range, 5-351 months). Treatment of scleral necrosis included observation in 81% of patients (59/73), scleral patch graft in 14% of patients (10/73), and enucleation in 5% of patients (4/73). CONCLUSIONS: Scleral necrosis after plaque radiotherapy of uveal melanoma was detected in 1% of cases. Factors predictive of scleral necrosis included increasing tumor thickness, ciliary body and peripheral choroidal location, and higher radiation dose to sclera. Most patients (81%) did not require treatment, and 4% evolved to full-thickness perforation. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 01/2013; · 5.45 Impact Factor
  • Article: Iris Nevus Growth into Melanoma: Analysis of 1611 Consecutive Eyes: The ABCDEF Guide.
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    ABSTRACT: PURPOSE: To determine clinical features predictive of growth of iris nevus into melanoma. DESIGN: Retrospective, comparative case series. PARTICIPANTS: A total of 1611 consecutive patients referred to an ocular oncology center with iris nevus. INTERVENTION: Observation and photographic documentation. MAIN OUTCOME MEASURES: Growth into melanoma. RESULTS: The mean age at referral for iris nevus was 51 years (median, 54; range, <1-94 years). At presentation, the mean tumor basal diameter was 3 mm (median, 3 mm; range, <1-12 mm) and mean tumor thickness was 0.8 mm (median, 0.5 mm; range, 0-5 mm). All patients were initially diagnosed with benign iris nevus. Growth of iris nevus to melanoma was confirmed in 2% of eyes (n = 27) over a mean follow-up of 68 months (median, 46 months; range, 3-465 months). By Kaplan-Meier estimates, iris nevus growth to melanoma occurred in <1%, 3%, 4%, 8%, and 11% at 1, 5, 10, 15, and 20 years, respectively. Factors predictive of iris nevus growth to melanoma by multivariable analysis included age ≤40 years at presentation (hazard ratio [HR], 3), episode of hyphema (HR, 9), 4:00 to 9:00 clock hour location of tumor (HR, 9), diffuse tumor (involving entire iris surface) (HR, 14), ectropion uveae (HR, 4), and feathery tumor margins (HR, 3). Additional important factors by univariable analysis included tumor seeding on the iris or in the anterior chamber angle, feeder vessels, and nodule formation. These factors can be remembered using the mnemonic ABCDEF, representing A = age young, B = blood, C = clock hour inferior, D = diffuse, E = ectropion, and F = feathery margin. CONCLUSIONS: In an analysis of 1611 cases of iris nevus referred for evaluation at an ocular oncology center, growth into melanoma occurred in 8% by 15 years. Risk factors for growth, identified by ABCDEF included Age young, Blood (hyphema), Clock hour inferior, Diffuse configuration, Ectropion uveae, and Feathery tumor margin. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 01/2013; · 5.45 Impact Factor
  • Article: Transient increased exudation after photodynamic therapy of intraocular tumors.
    Arman Mashayekhi, Carol L Shields, Jerry A Shields
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    ABSTRACT: To report transient increased exudation after photodynamic therapy (PDT) of three different intraocular tumors (retinal hemangioblastoma, retinal astrocytoma, amelanotic choroidal melanoma). PDT with verteporfin (6 mg/m(2) body surface area) was delivered at a dose of 50 J/cm(2) and intensity of 600 mW/cm(2) over 83 s. All patients experienced decreased vision within a few days following PDT. Optical coherence tomography showed development of subfoveal fluid in all cases and noncystoid intraretinal edema in the eye with juxtapapillary retinal hemangioblastoma. There was complete absorption of retinal/subretinal fluid with improvement of visual acuity to 20/20 in all cases between 3 weeks to 4 months after PDT.
    Middle East African journal of ophthalmology 01/2013; 20(1):83-6.
  • Article: Solitary Idiopathic Choroiditis: Findings on Enhanced Depth Imaging Optical Coherence Tomography in 10 Cases.
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    ABSTRACT: OBJECTIVE: To describe the imaging characteristics of solitary idiopathic choroiditis (SIC). DESIGN: Retrospective, observational case series. PARTICIPANTS: Ten eyes in 10 patients with SIC. METHODS: Review of chart, fundus photography, ultrasonography, fundus autofluorescence (FAF), infrared reflectance (IR) imaging, and enhanced depth imaging optical coherence tomography (EDI OCT). MAIN OUTCOME MEASURES: Scleral, choroidal, and retinal features of SIC as analyzed by EDI OCT. RESULTS: The mean age at diagnosis was 47 years (range, 7-76 years). There were 4 male patients and 6 female patients. The mean best-corrected visual acuity was 20/25 (range, 20/20-20/150). The SIC lesions were postequatorial (n = 10), with a mean basal diameter of 2.6 mm (range, 1.0-4.0 mm), yellow hue (n = 10), and surrounding orange halo (n = 6). Ultrasonography revealed acoustic solidity (n = 10) with a mean thickness of 1.7 mm (range, 1.4-2.1 mm), FAF disclosed hyperautofluorescence (n = 9), and IR imaging displayed hyperreflectivity (n = 9). On EDI OCT, all 10 lesions were dome shaped with a smooth surface and arose with a gentle slope from the sclera. A more abruptly elevated volcanic configuration was seen in 2 lesions. The overlying choroid was thinned (mean thickness, 32 μm; range, 0-52 μm). The lesions were moderately reflective with an optically bright anterior band and deep shadowing (n = 8). The posterior margin of the lesion could be ascertained in only 1 case. By EDI OCT, the mean diameter was 2942 μm (range, 1887-3809 μm). In no case was there disturbance of the inner retina or subretinal fluid. CONCLUSIONS: Solitary idiopathic choroiditis generally displays ultrasonographic solidity, hyperautofluorescence, and hyperreflectivity on IR imaging. On EDI OCT, the dome-shaped lesion arises from the sclera, outer choroid, or both and the overlying choroidal vasculature is thinned. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 12/2012; · 5.45 Impact Factor
  • Article: Lesions Simulating Retinoblastoma (Pseudoretinoblastoma) in 604 Cases: Results Based on Age at Presentation.
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    ABSTRACT: PURPOSE: To determine the types and frequency of ocular conditions that simulate retinoblastoma (pseudoretinoblastoma) based on age at presentation. DESIGN: Retrospective case series. PARTICIPANTS: Two thousand seven hundred seventy-five patients. METHODS: Chart review. MAIN OUTCOME MEASURES: Conditions simulating retinoblastoma. RESULTS: Of 2775 patients referred for management of retinoblastoma, 2171 patients (78%) had confirmed retinoblastoma and 604 patients (22%) had simulating lesions (pseudoretinoblastomas). In the pseudoretinoblastoma cohort, the mean patient age at presentation was 4 years (median, 2 years). There were 27 different pseudoretinoblastoma conditions, and the 10 most common included Coats' disease (n = 244; 40%), persistent fetal vasculature (PFV; n = 158; 28%), vitreous hemorrhage (n = 27; 5%), ocular toxocariasis (n = 22; 4%), familial exudative vitreoretinopathy (FEVR; n = 18; 3%), rhegmatogenous retinal detachment (n = 18; 3%), coloboma (n = 17; 3%), astrocytic hamartoma (n = 15; 2%), combined hamartoma of retina and retinal pigment epithelium (n = 15; 2%), and endogenous endophthalmitis (n = 10; 2%). Simulating lesions differed based on age at presentation, and children younger than 1 year were most likely to have PFV (49%), Coats' disease (20%), or vitreous hemorrhage (7%); those 2 to 5 years of age were most likely to have Coats' disease (61%), toxocariasis (8%), or PFV (7%); and those older than 5 years were most likely to have Coats' disease (57%), toxocariasis (8%), or FEVR (6%). CONCLUSIONS: The most common pseudoretinoblastomas include Coats' disease, PFV, and vitreous hemorrhage, but the spectrum varies depending on patient age. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
    Ophthalmology 10/2012; · 5.45 Impact Factor
  • Article: Influence of age on prognosis of young patients with uveal melanoma: a matched retrospective cohort study.
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    ABSTRACT: Purpose. To determine the influence of patient age on life prognosis in patients with uveal melanoma.
Design. Matched retrospective cohort study of 122 patients in each age category (young [≤20 years], mid-adults [21-60 years], older adults [>60 years]).
Results. Kaplan-Meier estimates of tumor-related metastasis at 3, 5, and 10 years were 1%, 8%, and 8% in young; 8%, 11%, and 26% in mid-adults; and 13%, 16%, and 24% in older adults. After exclusion of iris melanoma, Kaplan-Meier estimates of tumor-related metastasis at 3, 5, and 10 years were 2%, 11%, and 18% in young; 9%, 14%, and 21% in mid-adults; and 9%, 34%, and 33% in older adults. Risk factors for metastasis based on multivariate analysis included increasing age in young (p=0.05, hazard ratio [HR] 1.33), male gender in mid-adults (p=0.046, HR 4.23), and larger tumor basal diameter in mid-adults (p=0.002, HR 1.37) and older adults (p=0.001, HR 1.30). After adjusting for tumor diameter, the metastatic rate was lower in young patients compared to mid-adults (0=0.042, HR 3.00) and older adults (p=0.007, HR 4.20). 
Conclusions. Younger patient age at the time of diagnosis of uveal melanoma is associated with lower rate of metastasis compared to mid-adults and older adults.
    European journal of ophthalmology 10/2012; · 0.96 Impact Factor
  • Article: Morning glory disc anomaly with peripheral retinal nonperfusion in 4 consecutive cases.
    Archives of ophthalmology 10/2012; 130(10):1327-30. · 3.86 Impact Factor
  • Article: Safety of pars plana vitrectomy in eyes with plaque-irradiated posterior uveal melanoma.
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    ABSTRACT: OBJECTIVE To determine the long-term safety of pars plana vitrectomy (PPV) in eyes with plaque-irradiated posterior uveal melanoma. METHODS In this retrospective case series, patients with plaque-irradiated posterior uveal melanoma subsequently underwent PPV for vitreous hemorrhage. The main outcome measures are the rates of intraocular melanoma dissemination, extrascleral extension of melanoma, local melanoma recurrence, and systemic melanoma metastasis after PPV. RESULTS Forty-seven eyes of 47 patients underwent PPV for vitreous hemorrhage after iodine 125-labeled plaque radiotherapy for choroidal melanoma. The mean interval between the onset of vitreous hemorrhage and PPV was 13 (median, 10; range, 0-52) months. The mean time from PPV to last follow-up was 5 (range, 0.5-16) years. There were no cases of intraocular melanoma dissemination or extrascleral extension of melanoma. One patient (2%) developed local choroidal melanoma recurrence (2 years after PPV and 5 years after initial plaque radiotherapy) and was successfully managed with transpupillary thermotherapy. Systemic melanoma metastasis occurred in 4 patients (9%) during a mean interval of 5 years after plaque radiotherapy. During follow-up, 43 patients (91%) were alive without systemic metastasis and 4 patients (9%) were alive with metastasis. CONCLUSION Management of vitreous hemorrhage by PPV in eyes with previously irradiated uveal melanoma appears to be safe and without increased risk for intraocular, local, orbital, or systemic dissemination of the tumor.
    Archives of ophthalmology 10/2012; 130(10):1285-90. · 3.86 Impact Factor
  • Article: Transformation of optic disc melanocytoma into melanoma over 33 years.
    Archives of ophthalmology 10/2012; 130(10):1344-7. · 3.86 Impact Factor

Institutions

  • 2013
    • L V Prasad Eye Institute
      Hyderābād, State of Andhra Pradesh, India
    • Hospital Egas Moniz
      Lisbon, Lisbon, Portugal
  • 2003–2013
    • Wills Eye Institute
      Philadelphia, PA, USA
    • Thomas Jefferson University
      • Wills Eye Institute
      Philadelphia, PA, USA
  • 2012
    • Istanbul University
      • Department of Family Medicine (Istanbul Medical Faculty)
      İstanbul, Istanbul, Turkey
    • Thomas Jefferson University Hospitals
      Philadelphia, PA, USA
  • 2011
    • The Philadelphia Center
      Philadelphia, PA, USA
    • The Children's Hospital of Philadelphia
      Philadelphia, PA, USA
    • Duke University
      • Department of Ophthalmology
      Durham, NC, USA
    • St. Jude Children's Research Hospital
      Memphis, TN, USA
  • 2010
    • Royal Victoria Eye and Ear Hospital
      Dublin, L, Ireland (Republic of Ireland)
    • Yale-New Haven Hospital
      New Haven, CT, USA
    • Saint Vincent Hospital
      Worcester, MA, USA
  • 2005–2009
    • Harvard University
      • Department of Epidemiology
      Boston, MA, USA