Search PubMed⌕ Search

Biomedical subjects

Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 37 records · Page 2Linked to original sources

Retinal nerve fiber layer analysis in the diagnosis of glaucoma.

PURPOSE OF REVIEW: The detection of optic disc and retinal nerve fiber layer damage and change is the cornerstone of glaucoma management. Assessment of the retinal nerve fiber layer for localized and diffuse damage has been traditionally based on clinical examination, with documentation of change primarily qualitative. With the latest improvements in optical imaging instruments, objective and quantitative measurements of the retinal nerve fiber layer are now possible. This review summarizes the results from recent cross-sectional studies evaluating the discriminating ability of automated retinal nerve fiber layer measurements to detect glaucoma, and from longitudinal studies assessing the ability to predict and monitor glaucomatous changes. RECENT FINDINGS: Numerous cross-sectional studies have documented good diagnostic accuracy of a scanning laser polarimeter (GDx VCC), the optical coherence tomograph (Stratus), and the Heidelberg Retina Tomograph retinal nerve fiber layer measurements for differentiating between healthy and glaucoma eyes. There are only limited data available on the ability of these retinal nerve fiber layer measurements to document change over time. SUMMARY: It is essential that the clinician understand the specific strengths and weaknesses of each technique so that only good quality retinal nerve fiber layer information will be used in conjunction with careful clinical examination and visual function testing for glaucoma management decisions. Longitudinal studies are needed to evaluate the ability of these instruments to document retinal nerve fiber layer change over time.

Diagnostic Imaging↗

Management of choroidal metastases from breast carcinomas using aromatase inhibitors.

PURPOSE OF REVIEW: The outcome of patients diagnosed with uveal metastases from breast carcinoma, all of whom were treated with one of the new generation of hormone therapies named aromatase inhibitors, is reported. RECENT FINDINGS: Charts of all patients with uveal metastases from breast cancer seen at Wills Eye Hospital between 1995 and 2005 are reviewed. Seventeen patients were diagnosed with uveal metastases from estrogen-receptor-positive breast cancer and subsequently treated with one of the aromatase inhibitors (anastrozole, letrozole, exemestane). SUMMARY: The interval between initial diagnosis of breast cancer and choroidal metastases ranged from 1 month to 17 years. Thirteen patients had received tamoxifen either at the time of breast surgery or as part of the metastases treatment. In 10 cases, both the systemic and choroidal metastases remained regressed over a mean of 20 months follow-up (4-50 months). There was no toxicity or intolerance to the medication. Aromatase inhibitors represent a new generation of hormone therapy for metastatic breast cancer. They were effective in 59% of the patients with estrogen-receptor-positive breast cancer, many of them nonresponding to prior treatment with tamoxifen. Aromatase inhibitors avoid the need for external beam radiation therapy as a treatment for the ocular metastases.

Aged↗

[Retinoblastoma update--management and classification (Part I)].

The aim of this paper is to present updated data on diagnosis, management and classification of retinoblastoma. The authors describe the growth patterns of retinoblastoma including the diffuse infiltrating type, the features of sponatneously regressed and sponatneously arrested retinoblastoma. In addition, retinoblastoma genetics including 13 q deletion syndrome, are discussed. Finally, they present the International Intraocular Retinoblastoma Classification (IIRC) and make recommendations regarding chemotherapy after enucleation based on histopathology findings.

Antineoplastic Agents↗

[Retinoblastoma update--therapy (Part II)].

The aim of this paper is to present updated data on the treatment of retinoblastoma. The authors describe current management based on the International Intraocular Retinoblastoma Classification (IIRC). They discuss the different methods of treatment, including chemoreduction, subconjunctival carboplatin, transpupillar thermotherapy, cryotherapy, laser photocoagulation, episcleral plaque radiotherapy, external-beem radiotherapy and enucleation.

Antineoplastic Agents↗

Long-term visual outcome following chemoreduction for retinoblastoma.

OBJECTIVE: To evaluate long-term visual outcome following chemoreduction. DESIGN: Interventional case series evaluating 54 eyes of 40 children with retinoblastoma successfully treated with chemoreduction, consisting of a combination of intravenous carboplatin, etoposide phosphate, and vincristine sulfate plus focal therapy without external beam radiotherapy or enucleation. All patients were followed up for at least 5 years. Patient and tumor data were analyzed for their effect on the main outcome measures (final visual acuities of 20/40 or better and of 20/200 or better) using univariate and multivariate regression models. Patients who failed chemoreduction were excluded. RESULTS: There were 4 eyes in Reese-Ellsworth group I, 7 in group II, 3 in group III, 15 in group IV, and 25 in group V. The mean distance from the posterior tumor margin to the optic disc was 2 mm, and from the tumor margin to the foveola it was 3 mm. After a mean follow-up of 68 months, 27 eyes (50%) had a final visual acuity of 20/40 or better, and 36 eyes (67%) had final visual acuity of 20/200 or better. Of 33 eyes with macular tumor, only 8 (24%) had a final visual acuity of 20/40 or better, and 15 (45%) had a final visual acuity of 20/200 or better. Of 21 eyes with extramacular tumor, 19 (90%) had a final visual acuity of 20/40 or better, and all (100%) had a final visual acuity of 20/200 or better. CONCLUSION: The clinical factors that predicted visual acuity of 20/40 or better were a tumor margin at least 3 mm from the foveola and optic disc and an absence of subretinal fluid.

Antineoplastic Combined Chemotherapy Protocols↗

Factors predictive of tumor growth, tumor decalcification, choroidal neovascularization, and visual outcome in 74 eyes with choroidal osteoma.

OBJECTIVE: To evaluate choroidal osteoma for tumor growth, tumor decalcification, related choroidal neovascularization, visual acuity loss, and poor visual acuity. DESIGN: Retrospective nonrandomized single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa. PARTICIPANTS: There were 74 eyes of 61 patients with choroidal osteoma evaluated between January 1, 1977, and January 1, 2003. MAIN OUTCOME MEASURES: The 5 outcome measures included tumor growth, tumor decalcification, related choroidal neovascularization, visual acuity loss of 3 or more Snellen lines, and poor visual acuity of 20/200 or worse. RESULTS: At 5 and 10 years, Kaplan-Meier analysis revealed tumor growth in 22% and 51% of eyes, tumor decalcification in 28% and 46% of eyes, choroidal neovascularization in 31% and 31% of eyes, visual acuity loss in 26% and 45% of eyes, and poor visual acuity in 45% and 56% of eyes, respectively. The clinical factor predictive of tumor growth was absent overlying retinal pigment epithelial alterations. The factor predictive of decalcification was irregular tumor surface. Of the 15 tumors that showed partial decalcification at the first visit, there was no further tumor growth in any case. Of the remaining 12 tumors that later developed decalcification, tumor growth, if it occurred, was along the margin opposite the decalcification. No tumor showed growth in the region of decalcification. Factors predictive of choroidal neovascularization included irregular tumor surface and subretinal hemorrhage. In 6 eyes that had both choroidal neovascularization and tumor decalcification, the neovascularization was detected prior to or at the same time as the decalcification. The factor predictive of visual acuity loss was presence of subretinal fluid whereas the factors predictive of poor visual acuity included symptoms and tumor decalcification. A comparison of eyes with subfoveal vs extrafoveal choroidal osteoma showed poor visual acuity in 15 (34%) of 44 eyes and 3 (10%) of 30 eyes, respectively. Eyes with decalcified choroidal osteomas manifested poor visual acuity in 13 (48%) of 27 eyes whereas those with nondecalcified tumors showed poor visual acuity in 5 (11%) of 47 eyes. CONCLUSIONS: Choroidal osteoma showed evidence of growth in 51% of eyes and decalcification in nearly 50% of eyes by 10 years. Tumors with any degree of decalcification at the initial visit showed no further growth. Overall, poor visual acuity of 20/200 or worse was found in 56% of eyes by 10 years, and decalcified subfoveal choroidal osteomas displayed a particularly poor visual prognosis.

Adolescent↗

Macular retinoblastoma managed with chemoreduction: analysis of tumor control with or without adjuvant thermotherapy in 68 tumors.

OBJECTIVE: To evaluate the effectiveness of chemoreduction alone and chemoreduction with thermotherapy for macular retinoblastoma. DESIGN: Prospective, nonrandomized, single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at the Children's Hospital of Philadelphia (Pa). PARTICIPANTS: There were 68 macular retinoblastomas in 62 eyes of 49 patients managed with chemoreduction from January 1995 through January 2003. Intervention All patients received 6 cycles of intravenous chemoreduction using vincristine, etoposide, and carboplatin. The patients were then treated according to 1 of 2 approaches: chemoreduction alone with no adjuvant focal therapy (group A) or chemoreduction combined with adjuvant foveal-sparing thermotherapy to each macular retinoblastoma (group B). Main Outcome Measure Tumor recurrence. RESULTS: Of the 68 tumors, 28 were in group A and 40 were in group B. A comparison of both groups revealed that the tumors were similar with regard to clinical features. The mean tumor basal dimension was 12.3 mm for group A and 12.1 mm for group B, and the mean tumor thickness was 6.8 mm for group A and 6.1 mm for group B. Tumors in group A occupied a mean of 71% of the macula, and those in group B occupied 74% of the macula. Following treatment, Kaplan-Meier estimates revealed that group A tumors showed recurrence in 25% by 1 year and 35% by 4 years whereas those in group B showed recurrence in 17% by 1 year and 17% by 4 years. All recurrences were treated with additional focal thermotherapy, cryotherapy, or plaque radiotherapy except for 1 that required external beam radiotherapy and 1 that required enucleation, both in group A. Univariate analysis revealed that predictors of tumor recurrence were intraretinal growth pattern (vs endophytic); small tumor basal dimension (less than 3 mm and occupying a smaller percentage of the macula); absence of subretinal fluid, subretinal seeds, and vitreous seeds; and chemoreduction response with less tumor calcification and tumor regression of type 0 (complete disappearance without a scar). By multivariate analysis, the most important factors predictive of tumor recurrence were smaller macular tumor size (judged by percentage of the macula occupied by the tumor), absence of subretinal or vitreous seeds, and unilateral disease. CONCLUSIONS: Treatment of macular retinoblastoma with chemoreduction plus adjuvant foveal-sparing thermotherapy provides tumor control of 83% by 4 years, and this is slightly more favorable than chemoreduction alone, which provides control of 65% by 4 years. Tumors most destined for recurrence are small tumors.

Antineoplastic Combined Chemotherapy Protocols↗

New ophthalmic manifestations of branchio-oculo-facial syndrome.

PURPOSE: To report new ocular manifestations of branchio-oculo-facial (BOF) syndrome. DESIGN: Case report. METHODS: A 10-year-old girl with known BOF syndrome was referred because of a fundus lesion in her left eye. RESULTS: She had undergone excision of a left orbital dermoid cyst at age 18 months and a branchial cleft fistula from the right side of neck at age 4 years. Examination disclosed openings of sinus tracts on each side of the nose connecting the lacrimal sac to skin. In the right eye, an iris pigment epithelial cyst was confirmed with ultrasound biomicroscopy. In the left eye, there was a combined hamartoma of the retina and retina pigment epithelium. CONCLUSION: BOF syndrome can display mild to severe craniofacial, auricular, oral, and ophthalmic anomalies. In this case, the ophthalmic manifestations included lacrimal sac fistula, orbital dermoid cyst, iris pigment epithelial cyst, and combined hamartoma of the retina and retinal pigment epithelium.

Branchio-Oto-Renal Syndrome↗

Sclerochoroidal calcification in a patient with classic Bartter's syndrome.

PURPOSE: To report sclerochoroidal calcification in a patient with classic Bartter's syndrome. DESIGN: Observational case report. METHODS: A 42-year-old woman with a 26-year history of classic Bartter's syndrome was found to have bilateral fundus tumors. The patient presented initially with quivering lips and hand stiffness at age 6 years but was not diagnosed until age 16 years. Treatment included magnesium and potassium supplementation and Amiloride therapy. RESULTS: On ocular examination, there were multifocal, yellow-white, geographic, solid choroidal lesions along the superior and inferior retinal vascular arcades in both eyes. Ultrasonography showed echogenic, placoid calcified lesions at the level of the sclera and choroid, consistent with bilateral sclerochoroidal calcification. CONCLUSIONS: Sclerochoroidal calcification can be associated with classic Bartter's syndrome.

Adult↗

Iris melanocytoma: clinical features and natural course in 47 cases.

PURPOSE: To describe the clinical features, natural course, management and histopathologic features of iris melanocytoma. DESIGN: Single-center retrospective case series. METHODS PATIENT POPULATION: Forty-seven consecutive patients (47 eyes) with iris melanocytoma. INTERVENTION PROCEDURE: Data regarding patient and tumor features were analyzed for their impact on the main outcome measures using univariate and multivariate regression models. Kaplan-Meier estimates were used to analyze the main outcomes as a function of time. MAIN OUTCOME MEASURES: Increased intraocular pressure (IOP), tumor seeding, and tumor growth. RESULTS: Associated findings at initial presentation included iris stromal seeds in 20 patients (43%), and anterior chamber angle seeds in 12 (26%). Intrinsic vascularization and sector cataract were not seen in any eyes. The management at presentation included observation in 39 patients (83%), tumor removal by sector iridectomy/iridocyclectomy in 7 (15%), and enucleation for blind painful eye with secondary increased IOP in 1 (2%). The diagnosis was confirmed by histopathologic examination in 11 patients (23%). The mean follow-up was 58 months. Using Kaplan-Meier estimates, clinical evidence of growth was observed in 23% at 5 years, 48% at 10 years, and 74% at 15 years. New tumor seeds developed in 34% at 5 years, 63% at 10 years, and 75% at 15 years. Increased IOP was observed in 11% at 5 years, 11% at 10 years, and 55% at 15 years. CONCLUSIONS: Iris melanocytoma represented only 3% of all iris nevi. Related iris stromal and anterior chamber angle seeds were common, and secondary glaucoma occurred in 11% at 5 years. Growth was observed in 23% at 5 years but no malignant transformation was found.

Adolescent↗

Cancer-associated nummular loss of the pigment epithelium.

PURPOSE: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) that had areas of retinal pigment epithelial loss. DESIGN: Observational case report. METHODS: A 67-year-old woman with a history of uterine cancer presented with 4 months of bilateral visual loss. RESULTS: Although B-scan ultrasonography revealed both small shallow serous retinal and choroidal detachments in the periphery, the choroid was normal in thickness. Fluorescein angiography revealed numerous nummular-shaped areas of transmission defects suggesting retinal pigment epithelium loss. Autofluorescence photography showed complete absence of autofluorescence in these nummular areas, and optical coherence tomography showed segmental areas with lack of any signal from affected areas of the retinal pigment epithelium, suggesting complete loss of retinal pigment epithelium cells. CONCLUSIONS: Although the fundus findings in BDUMP have been attributed to the proliferation of uveal melanocytic cells in the outer choroid in previous papers, our patient had nummular areas of loss of the retinal pigment epithelium as the apparent reason for visual decline.

Aged↗

Total blindness from presumed optic nerve melanocytoma.

PURPOSE: To describe total blindness secondary to presumed optic nerve melanocytoma. DESIGN: Interventional case report. METHODS: A 12-year-old African-American girl, who developed loss of light perception secondary to a pigmented lesion of the optic disk, underwent transvitreal fine-needle aspiration biopsy (FNAB) of the lesion. RESULTS: Cytopathology revealed cells with features of melanocytoma and melanophages but no cells compatible with melanoma. The patient is being followed without treatment. CONCLUSIONS: Optic nerve melanocytoma can cause total blindness in the affected eye. Although it has limitations, FNAB can assist in diagnosis and management of melanocytoma with visual loss.

Blindness↗