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Biomedical subjects

C L Shields

Publications and source records attributed to C L Shields.

At least 91 records · Page 5Linked to original sources
PubMed2000

Vitrectomy in eyes with unsuspected retinoblastoma.

OBJECTIVE: To analyze patient management and prognosis after vitrectomy in eyes with unsuspected retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Eleven consecutive patients who had undergone vitrectomy on an eye with unsuspected retinoblastoma. MAIN OUTCOME MEASURES: The two main outcome measures were ultimate patient management and the development of retinoblastoma metastasis. RESULTS: Of more than 900 consecutive patients with retinoblastoma managed on the Ocular Oncology Service at Wills Eye Hospital in Philadelphia, 11 (1%) had prior vitrectomy in an eye with viable tumor before referral to us for suspected retinoblastoma. The main preoperative diagnoses included vitreous hemorrhage in seven patients (64%), toxocariasis in two patients (18%), toxoplasmosis in one patient (9%), and endophthalmitis in one patient (9%). In no case was retinoblastoma suspected before vitrectomy. The mean patient age at vitrectomy was 6 years. Retinoblastoma was later suspected during vitrectomy in two patients (18%), on cytologic examination of the vitrectomy specimen in eight patients (73%), and after referral in one patient (9%). The mean interval between vitrectomy and referral to us was 23 days. On examination, the globe was classified as Reese-Ellsworth group Vb in all 11 patients (100%). Anterior chamber tumor cells were clinically visible in four eyes (36%), hyphema in two eyes (18%), and iris neovascularization in two eyes (18%). Retinoblastoma cells were visualized in the vitreous in seven eyes (64%) and not visualized in four eyes (36%) that had vitreous blood. Enucleation was necessary in all 11 patients (100%). Adjuvant treatment was delivered in 10 patients (91%), using orbital radiotherapy in nine patients (82%) and chemotherapy in nine patients (82%). Histopathologic evidence of retinoblastoma invasion was documented in the episclera (two eyes; 18%), anterior chamber (seven eyes; 64%), iris (five eyes; 45%), ciliary body (five eyes; 45%), choroid (three eyes; 27%), and optic nerve (four eyes; 36%; prelaminar, two eyes; postlaminar, two eyes). The vitrectomy ports, Tenon's fascia, cut end of the optic nerve, and orbit were free of tumor. Of the 10 patients who received prophylactic chemotherapy, radiotherapy, or both in addition to enucleation for prevention of retinoblastoma metastasis, none (0%) experienced metastasis or orbital recurrence during the mean follow-up of 7 years (range, 0.2-24 years) from the time of retinoblastoma diagnosis. However, one patient was referred to us after the development of metastatic retinoblastoma, and despite aggressive chemotherapy and radiotherapy after enucleation, died 24 months later. CONCLUSIONS: Retinoblastoma may present with atypical features such as vitreous hemorrhage or signs of vitreous inflammation, particularly in older children. Vitrectomy should be avoided in these cases until the possibility of underlying retinoblastoma is excluded. If vitrectomy is performed in an eye with unsuspected retinoblastoma, enucleation combined with adjuvant chemotherapy, radiotherapy, or both without delay is advised to prevent systemic tumor dissemination.

Adolescent↗
PubMed2000

Circumscribed choroidal hemangioma: characteristic features with indocyanine green videoangiography.

OBJECTIVE: To determine the characteristic features of indocyanine green videoangiography (ICG-V) of circumscribed choroidal hemangioma. DESIGN: Prospective, observational case series. PARTICIPANTS: Twenty-five eyes of 25 consecutive patients with circumscribed choroidal hemangioma. INTERVENTION: Indocyanine green videoangiography and intravenous fluorescein angiography (IVFA) were prospectively performed and reviewed. The specific features on ICG-V were compared with features of IVFA. MAIN OUTCOME MEASURES: The behavior of circumscribed choroidal hemangioma cases was observed with ICG-V and IVFA. RESULTS: On ICG-V, earliest hyperfluorescence of circumscribed choroidal hemangioma was achieved at a mean of 27.6 seconds (range, 13-62 seconds), whereas maximum hyperfluorescence occurred at 222 seconds (range, 33-707 seconds). In the late frames, all eyes demonstrated a relative decrease in fluorescence, including 18 eyes (72%) that demonstrated "washout" of the dye. Other findings on ICG-V included intrinsic vessels in 19 eyes (76%), a late hyperfluorescent rim in 19 eyes (76%), and late frame hot spots in 14 eyes (56%). On IVFA, the earliest hyperfluorescence was achieved at a mean of 24 seconds (range, 10-66 seconds), whereas maximum hyperfluorescence occurred at a mean of 76.3 seconds (range, 21-720 seconds). Increasing hyperfluorescence in the late frames was found in all cases. Other findings included intrinsic vessels in 12 eyes (48%) and hot spots in the late frames in 9 eyes (36%). CONCLUSIONS: Circumscribed choroidal hemangioma have specific characteristics on ICG-V that are not visualized with IVFA. We believe that ICG-V may become an important noninvasive tool for the diagnosis of choroidal hemangioma.

Choroid Neoplasms↗
PubMed2000

Clinical features predictive of orbital exenteration for conjunctival melanoma.

PURPOSE: Conjunctival malignant melanoma can sometimes be difficult to control locally, and orbital exenteration may be necessary. To our knowledge, the risk factors that portend exenteration have not been previously identified. The purpose of this study was to review patients who underwent orbital exenteration for advanced disease and to identify retrospectively the clinical factors predictive of ultimate exenteration. METHODS: The records of patients with conjunctival melanoma who were managed on the Oncology Service at Wills Eye Hospital from 1974 to 1997 were reviewed. Factors predictive of orbital exenteration were analyzed using Cox proportional hazards regression models. RESULTS: Of 151 consecutive patients with conjunctival melanoma, 20 (13%) required orbital exenteration for advanced disease. The number of melanoma excisions performed before referral ranged from 0 to 11, with a mean of 2. The clinical factors predictive of orbital exenteration on multivariable analysis were visual acuity of 20/200 or worse, lack of tumor pigmentation, and extralimbal tumor location. All exenterations were performed using an eyelid-sparing technique. At mean follow-up of 51 months after exenteration, 4 patients (20%) had died of metastasis, 3 (15%) were alive with metastasis, and 13 (65%) had not developed metastasis. CONCLUSIONS: Conjunctival melanoma can show aggressive local behavior, and orbital exenteration is necessary in up to 13% of cases at a tertiary referral center. Patients with risk factors such as poor visual acuity, amelanotic tumor, and extralimbal tumor location may require wider excision and radiation therapy to control the tumor and avoid orbital exenteration. It is hoped that modern surgical approaches will decrease the need for orbital exenteration.

Adult↗
PubMed2000

Chemoreduction and local ophthalmic therapy for intraocular retinoblastoma.

PURPOSE: To study the effectiveness of combined systemic chemotherapy and local ophthalmic therapy for retinoblastoma with the goal of avoiding enucleation and external-beam radiation therapy (EBRT). PATIENTS AND METHODS: This was a prospective, nonrandomized, single-arm clinical trial. Seventy-five eyes were followed in 47 children. Patients were treated with a six-cycle protocol of vincristine, etoposide, and carboplatin. Most (83%) also received ophthalmic treatment (cryotherapy, laser photocoagulation, thermotherapy, or plaque radiation therapy) during and/or after the chemotherapy. RESULTS: With a median follow-up of 13 months, event-free survival was 74%, with an event defined as enucleation and/or EBRT. Six children required EBRT in seven eyes (9%); five required enucleation of one eye (7%); five required a combination of EBRT and enucleation in six eyes (8%). Reese-Ellsworth groups 1, 2, and 3 eyes had excellent results, with avoidance of EBRT or enucleation in all 39. Treatment of groups 4 and 5 was less successful, with 33% of six eyes and 53% of 30 eyes, respectively, requiring EBRT and/or enucleation. Toxicities from chemotherapy were mild and included cytopenias (89%), fever and neutropenia (28%), infection (9%), and gastrointestinal symptoms, dehydration, and vincristine neurotoxicity (40%). No patients developed a second malignancy, metastatic disease, renal disease, or ototoxicity. CONCLUSION: In retinoblastoma patients with Reese-Ellsworth eye groups 1, 2, or 3, systemic chemotherapy used with local ophthalmic therapies can eliminate the need for enucleation or EBRT without significant systemic toxicity. More effective therapy is required for Reese-Ellsworth eye groups 4 and 5.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

Uveal melanoma in young patients.

OBJECTIVE: To study the clinical profile of young patients with uveal melanoma. DESIGN: Retrospective case-control series. SETTING: Tertiary referral center. PATIENTS: Data on 63 patients aged 20 years or younger with uveal melanoma were reviewed for clinical profile and association with oculo(dermal) melanocytosis, familial uveal melanoma, dysplastic nevus syndrome, cutaneous melanoma, and other second malignant neoplasms. RESULTS: Of 8000 patients with uveal melanoma, 63 (0.8%) were found in patients who were 20 years of age or younger. The median age at diagnosis was 16 years, and the youngest patient was 3 years old. Sixty-two patients (98%) were white, and uveal melanoma was unilateral in all cases. Seven patients (11%) had oculo(dermal) melanocytosis. Two patients (3%) had dysplastic nevi syndrome, and personal history of cutaneous melanoma was observed in 1 patient (2%). No other second cancers were present in any patient. The 5- and 15-year posttreatment survival estimates were 0.95 (95% confidence interval, 0.87-1.00) and 0.77 (95% confidence interval, 0.52-1.00), respectively. CONCLUSIONS: Uveal melanoma is rare in children or teenagers. It occurs in a heterogeneous group displaying various associations, especially with oculo(dermal) melanocytosis. Oculo(dermal) melanocytosis is 9 times (95% confidence interval, 3.6-22.8) more common in young patients with uveal melanoma than in the general population with uveal melanoma. Young patients with uveal melanoma have short-term (5-year) survival better than that of adults, but the long-term (15-year) survival is similar to that of adults. Arch Ophthalmol. 2000;118:918-923

Adolescent↗
PubMed2000

Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients.

OBJECTIVE: To identify the risk factors of conjunctival malignant melanoma that predict local tumor recurrence, orbital exenteration, distant metastasis, and tumor-related mortality. DESIGN: The study group consisted of 150 consecutive patients with a diagnosis of conjunctival melanoma. The clinical parameters of the patient, tumor, and treatment were analyzed in a nonrandomized fashion for their relation to 4 main outcome measures using Cox proportional hazards regression models. RESULTS: The Kaplan-Meier estimate of local tumor recurrence was 26% at 5 years, 51% at 10 years, and 65% at 15 years. The mean number of recurrences per patient was 1 (median, 0). Ninety-eight patients (65%) had no recurrences, 28 patients (19%) had 1, 11 patients (7%) had 2, 5 patients (3%) had 3, and 8 patients (5%) had 4 or more recurrences. According to multivariate analysis, the factors that predicted local tumor recurrence were the location of the melanoma (not touching the limbus) (P = .01) and tumor-margin pathology (lateral margin involved) (P = .02). Multivariate analysis for features predictive of ultimate exenteration included initial visual acuity (20/40 or worse) (P = .0007), melanoma color (red) (P = .01), and melanoma location (not touching the limbus) (P = .02). Tumor metastasis occurred in 16% of patients at 5 years, 26% at 10 years, and 32% at 15 years. Metastasis was initially in the regional lymph nodes in 17 cases, brain in 4, liver in 3, lung in 2, and disseminated in 1 case. Risks for metastases with use of multivariate analysis included tumor-margin pathology (lateral margin involved) (P = .002) and melanoma location (not touching limbus) (P = .04). Tumor-related death occurred in 7% of patients at 5 years and 13% at 8 years. Risk factors for death with use of multivariate analysis included initial symptoms (lump) (P = .004) and pathologic findings (de novo melanoma without primary acquired melanosis) (P = .05). In a series of univariate analyses, the technique of initial surgery was shown to be an important factor in preventing eventual tumor recurrence (P = .07), metastasis (P = .03), and death (P = .006). Patients who were managed with excisional biopsy using the "no-touch technique" plus alcohol corneal epitheliectomy and supplemental cryotherapy fared far better than those treated with excisional biopsy alone. In addition, the surgical technique used before referral to us was critical. Those patients who had an incisional diagnostic biopsy prior to referral were at risk for more than 1 recurrence (P = .04), and those who had excisional biopsy alone without supplemental cryotherapy were at risk for eventual exenteration (P = .0006) and death (P = .04). CONCLUSIONS: Conjunctival malignant melanoma is a potentially deadly tumor. In this study, metastasis was detected in 26% of patients and death occurs in 13% at 10 years. The surgical technique of tumor management was found to be possibly related to tumor metastases and death. Meticulous surgical planning, use of wide microsurgical excisional biopsy with the no-touch technique, and supplemental alcohol corneal epitheliectomy and conjunctival cryotherapy performed by experienced surgeons are advised. Incisional biopsy should be avoided.

Adolescent↗
PubMed1999

Adenoma of nonpigmented ciliary epithelium with smooth muscle differentiation.

A 35-year-old man underwent successful iridocyclectomy for a ciliary body mass that was subluxating the lens and causing a secondary cataract. Histopathologically the mass proved to be a spindle cell tumor, but leiomyoma and melanoma were initially considered to be diagnostic possibilities. However, further studies and immunohistochemical studies revealed that the tumor probably arose from the nonpigmented ciliary epithelium. The spindle cells represented smooth muscle differentiation within the mass. The final diagnosis was adenoma of the nonpigmented ciliary epithelium with smooth muscle differentiation.

Adenoma↗
PubMed1999

Plaque radiotherapy of uveal melanoma with predominant ciliary body involvement.

BACKGROUND: There are several options for management of ciliary body melanoma, including plaque radiotherapy, charged particle irradiation, local resection, and enucleation. The choice of therapy depends on many factors, and plaque radiotherapy is often used. OBJECTIVES: To determine the outcome of plaque radiotherapy in the management of ciliary body melanoma and to identify the risk factors associated with the development of radiation complications, tumor recurrence, metastasis, and melanoma-related death after plaque radiotherapy of ciliary body melanoma. METHODS: We analyzed the clinical records of 136 patients with ciliary body melanoma who were treated with plaque radiotherapy between July 1976 and June 1992. RESULTS: The median follow-up period was 70 months. Using Kaplan-Meier survival estimates, the most frequent radiation complication at 5 years' follow-up was cataract, developing in 48% of the patients, followed by neovascular glaucoma (21%), retinopathy (20%), scleral necrosis (12%), and vitreous hemorrhage (11%). Visual acuity decrease (by > or =3 Snellen lines) was noted in 40% of the patients at 5 years. Kaplan-Meier estimates showed that 8% of the patients developed recurrence, 28% had metastasis, and 22% died of melanoma-related causes by 5 years. Univariate analysis demonstrated that the factors predictive of radiation cataract were superonasal (P = .003) and inferior tumor meridian (P = .02) compared with inferonasal meridian and apex dose rate greater than 57 cGy/h (P = .05). The development of neovascular glaucoma was significantly related to iris involvement with the ciliary body tumor (P<.001). The factors predictive of development of radiation retinopathy were base dose rate greater than 230 cGy/h (P = .03) and the presence of diabetes mellitus (P = .05). The only predictor of metastasis was tumor thickness greater than 7 mm (P = .02). The risk factors for melanoma-related death were the presence of metastasis (P<.001), tumor thickness greater than 7 mm (P = .02), and recurrence (P = .02). Multivariate analyses showed that the most significant variables predictive of the development of scleral necrosis were intraocular pressure greater than 15 mm Hg (P<.001) and tumor thickness greater than 7 mm (P = .007). The most significant predictive factors for vitreous hemorrhage were visual acuity of 20/40 to 20/200 (P = .02) and intraocular pressure greater than 15 mm Hg (P = .02). The best subset of independent predictors of vision decrease were mushroom tumor shape (P = .002), age older than 61 years (P = .006), and superonasal meridian (P = .04). The risks for melanoma-related death were presence of metastasis (P<.001) and tumor thickness greater than 7 mm (P = .01). There was no group of significant variables predictive for radiation cataract, neovascular glaucoma, retinopathy, tumor recurrence, and metastasis in multivariate analysis. CONCLUSIONS: Plaque radiotherapy offers 92% 5-year local control rate for ciliary body melanoma. Metastasis occurs in 28% of the patients treated with this method by 5 years. Patients with tumors greater than 7 mm in thickness are at greater risk than patients with thinner tumors for metastatic disease and melanoma-related death. Major radiation complications include radiation cataract, neovascular glaucoma, retinopathy, and scleral necrosis.

Adult↗