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Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 127 records · Page 7Linked to original sources

CME review: sclerochoroidal calcification: the 2001 Harold Gifford Lecture.

Sclerochoroidal calcification is a recently recognized ocular condition that is characterized by typical geographic yellow-white fundus lesions that usually occur bilaterally in adults. It can clinically simulate a number of intraocular tumors like choroidal metastasis, choroidal melanoma, and choroidal osteoma. Clinical evaluation with ultrasonography, computed tomography, and some histopathologic studies have supported the fact the condition represents calcium deposition in the sclera and choroid. Early reports suggested that sclerochoroidal calcification was usually associated with hypercalcemic conditions such as hyperparathyroidism and pseudohypoparathyroidism. Subsequently, many patients with sclerochoroidal calcification have had no apparent systemic associations and have been classified as idiopathic. However, very recent studies have suggested that this ocular condition can also be associated with Gitelman syndrome or Bartter syndrome. These are autosomal recessive conditions of hypokalemic alkalosis associated with hypomagnesemia and deposition of calcium salts in various tissues. Ophthalmologists should be familiar with fundus features and systemic associations of sclerochoroidal calcification.

Calcinosis↗

Ring melanoma of the ciliary body: report on twenty-three patients.

BACKGROUND: Ring melanoma of the ciliary body is a rare variant of uveal melanoma that has a poor prognosis. Only isolated cases have been reported in the literature. METHODS: A retrospective review of the clinical features, management, histopathology, and prognosis of 23 consecutive patients with ring melanoma of the ciliary body was performed. RESULTS: Of 8,800 patients with uveal melanoma treated on the Oncology Service at Wills Eye Hospital over a 25-year period, only 23 patients (0.3%) were found to have ring melanoma of the ciliary body. The mean patient age was 59 years (median, 63 years; range, 8-81 years). The only visual symptom was blurred vision in 17 patients (74%), and the other six patients (26%) were asymptomatic. At the initial presentation elsewhere, melanoma was recognized in 13 cases (57%). In the remaining 10 cases (43%), the initial clinical diagnosis was glaucoma in three (13%), iris nevus in three (13%), cataract in two (9%), ciliary body detachment in one (4%), and iridocyclitis in one (4%). The patients were observed elsewhere for a mean of 3 months before the diagnosis of melanoma was suspected and referral to Oncology Service was made. On examination, the tumor involved a mean of eight clock hours of the ciliary body. Seven patients (30%) had complete circumferential (360 degrees) involvement of the ciliary body, and 16 (70%) had incomplete (180-360 degrees) ring involvement. The mean tumor thickness was 8 mm. Associated findings included neovascularization of the iris in one case (4%), shallow anterior chamber in 11 (48%), anterior chamber inflammation in five (22%), cataract in nine (39%), lens indentation in eight (35%), and lens subluxation in three (13%). A prominent episcleral (sentinel) vessel (17 cases, 74%), multilobulation of the mass (19 cases, 83%), blockage of light on transillumination (23 cases, 100%), and ultrasonographic hollowness with intrinsic pulsations (23 cases, 100%) were important features differentiating this tumor from simulating conditions. The tumor was managed with enucleation in all 23 patients. Histopathologic studies revealed epithelioid cell-type melanoma in two patients (9%), mixed cell-type in 17 (74%), and spindle cell-type in four (17%). Extraocular extension was present in eight patients (35%). Metastasis developed in 12 of 23 patients (52%) after a mean follow-up of 55 months. CONCLUSIONS: Ring melanoma of the ciliary body is a rare tumor that can remain hidden from ophthalmoscopic examination. Certain features, such as prominent episcleral sentinel vessels, multilobulation of the mass, light blockage on transillumination, and ultrasonographic hollowness with intrinsic vascular pulsations, suggest the diagnosis. The life prognosis is poor.

Adult↗

Clinical features of small choroidal melanoma.

The detection and treatment of choroidal melanoma early in its natural course is critical to providing the patient with the best prognosis. Patients with small choroidal melanoma (< 4 mm thickness) develop metastasis in 16% of cases at 5 years follow up, whereas those with medium choroidal melanoma (4-8 mm thickness) and large choroidal melanoma (> 8 mm thickness) develop metastasis in 32 and 53%, respectively. The difficulty with early detection of choroidal melanoma relates to its clinical similarity to benign choroidal nevus. Factors that differentiate small choroidal melanoma from choroidal nevus can be remembered using the mnemonic TFSOM, indicating To Find Small Ocular Melanoma. The letters in this mnemonic represent T (Thickness >2 mm), F (subretinal Fluid), S (Symptoms), O (Orange pigment), and M (Margin touching optic disc). Choroidal melanocytic tumors that display 0 factors have 3% chance for growth at 5 years and most likely represent choroidal nevi. Tumors that display one factor have a 38% chance for growth and those with two or more factors show growth in over 50% of cases at 5 years. Most of those tumors with two or more risk factors probably represent small choroidal melanoma and early treatment is generally indicated. Therefore, ophthalmologists should be aware of the important factors that identify small choroidal melanoma so that early treatment and better life prognosis can be achieved for their patients.

Choroid Neoplasms↗

Factors predictive of recurrence of retinal tumors, vitreous seeds, and subretinal seeds following chemoreduction for retinoblastoma.

OBJECTIVE: To identify the clinical features of eyes with retinoblastomas that predict the recurrence of retinal tumors, vitreous seeds, and subretinal seeds following treatment with chemoreduction. DESIGN: Prospective nonrandomized single-center clinical trial. SETTING: Ocular oncology service at Wills Eye Hospital of Thomas Jefferson University (Philadelphia, Pa) in conjunction with the division of oncology at Children's Hospital of Philadelphia. PARTICIPANTS: There were 158 eyes with 364 tumors in 103 consecutive patients with retinoblastoma managed with chemoreduction between June 1994 and August 1999. INTERVENTION: All patients received treatment for retinoblastoma with 6 cycles of chemoreduction using vincristine, etoposide, and carboplatin combined with focal treatment (cryotherapy, thermotherapy, or plaque radiotherapy) for each retinal tumor. MAIN OUTCOME MEASURES: The 3 main outcome measures included recurrence of retinal tumors, recurrence of vitreous seeds, and recurrence of subretinal seeds. The clinical features at the initial examination were analyzed for their association with the main outcome measures using a series of Cox proportional hazards regressions. RESULTS: All retinal tumors, vitreous seeds, and subretinal seeds showed an initial favorable response of regression during this treatment regimen. Using Kaplan-Meier estimates, at least 1 retinal tumor recurrence per eye was found in 37% of eyes at 1 year, 51% at 3 years, and no further increase at 5 years. By multivariate analysis, the only factor predictive of retinal tumor recurrence was the presence of tumor-associated subretinal seeds at the initial examination. Of the 54 eyes that had vitreous seeds at the initial examination, vitreous seed recurrence was found in 26% of eyes at 1 year, 46% at 3 years, and 50% at 5 years. By univariate analysis, the only factor predictive of vitreous seed recurrence was the presence of tumor-associated subretinal seeds at the initial examination. Of the 71 eyes that had subretinal seeds at the initial examination, subretinal seed recurrence was detected in 53% of eyes at 1 year, 62% at 3 years, and no further increase at 5 years. By multivariate analysis, factors predictive of subretinal seed recurrence included a tumor base greater than 15 mm and a patient age of 12 months or younger at diagnosis. There were no patients who developed retinoblastoma metastasis, pinealoblastoma, or second malignant neoplasms. CONCLUSIONS: Chemoreduction combined with focal therapy is effective for selected eyes with retinoblastomas. Eyes with subretinal seeds at initial examination are at particular risk for recurrence of retinal tumor and vitreous seeds. Younger patients with large tumors are at risk for recurrence of subretinal seeds. Retinal tumor and subretinal seed recurrence seems to manifest within 3 years of follow-up. Close follow-up of all patients treated with chemoreduction is warranted.

Antineoplastic Combined Chemotherapy Protocols↗