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Biomedical subjects

Jerry A Shields

Publications and source records attributed to Jerry A Shields.

At least 19 recordsLinked to original sources

The International Classification of Retinoblastoma predicts chemoreduction success.

PURPOSE: To evaluate the reliability of the International Classification of Retinoblastoma (ICRB) for predicting treatment success with chemoreduction (CRD). DESIGN: Noncomparative interventional case series. PARTICIPANTS: Two hundred forty-nine consecutive eyes. METHODS: All eyes were treated with CRD and were classified according to the ICRB: group A included those eyes with retinoblastoma </=3 mm; group B included those eyes with retinoblastoma >3 mm, macular location, or minor subretinal fluid; group C included those eyes with retinoblastoma with localized seeds; group D included those eyes with retinoblastoma with diffuse seeds; group E included those eyes with massive retinoblastoma necessitating enucleation. The CRD regimen included vincristine, etoposide, and carboplatin for 6 cycles plus local consolidation with thermotherapy or cryotherapy. MAIN OUTCOME MEASURE: Chemoreduction success, defined as avoidance of external beam radiotherapy or enucleation. RESULTS: Of the 249 eyes, 23 (9%) were in group A, 96 (39%) were in group B, 21 (8%) were in group C, and 109 (44%) were in group D. In this series, group E eyes were managed with enucleation. Treatment success was achieved in 100% of group A, 93% of group B, 90% of group C, and 47% of group D eyes. CONCLUSIONS: The ICRB can be of assistance in predicting CRD success for retinoblastoma. Additional treatment methods are necessary to salvage more group D eyes.

Antineoplastic Combined Chemotherapy Protocols↗

Fibrous histiocytoma of the conjunctiva.

PURPOSE: To review the clinical features and course of six patients with fibrous histiocytoma (FH) of the conjunctiva. DESIGN: Retrospective, observational clinical case series. METHODS: Chart review of six consecutive patients with unilateral cases of conjunctival FH was conducted. Clinical presentation, treatment, histopathologic condition, and follow-up information were recorded. RESULTS: The mean patient age was 37 years (median, 38 years; range, 12 to 72 years). There were five white patients, one black patient, five male patients, and one female patient. The tumor was present for a mean of three months (median, five months; range, one to 12 months) and was unilateral (one right eye, five left eyes). In all cases, the tumor was a tan, dome-shaped limbal mass in the conjunctival stroma with visible intrinsic vessels. The mean tumor basal dimension was 7 mm (median, 5 mm; range, 4 to 11 mm). Surgical resection was performed in all cases, and histopathologic study demonstrated benign FH in four cases and malignant FH in two cases. Those cases with benign FH showed no recurrence over nine months (median, eight months; range, three to 18 months). One patient with malignant FH showed recurrence and required repeat excision. The other patient with malignant FH was treated with plaque radiotherapy to maintain control. There was no evidence of orbital invasion or remote metastasis in any case over the mean follow-up period of 21 months (median, 10 months; range, three to 80 months). CONCLUSION: FH is a rare conjunctival tumor that can show benign or malignant features. Complete resection is advised. Malignant FH can demonstrate recurrence that necessitates wide resection and radiotherapy.

Adolescent↗

Iodine 125 plaque radiotherapy as salvage treatment for retinoblastoma recurrence after chemoreduction in 84 tumors.

PURPOSE: To assess tumor control and complications of iodine 125 (I125) plaque radiotherapy for retinoblastoma recurrence after chemoreduction (CRD). DESIGN: Interventional retrospective case series. PARTICIPANTS: Children with retinoblastoma treated on the Oncology Service, Wills Eye Hospital, initially examined from July 1994 to April 2005. METHODS: Eighty-four retinoblastomas with solid recurrence after CRD were treated with custom designed I125 plaque radiotherapy. Eyes with vitreous or subretinal seed recurrence were excluded. The CRD regimen included vincristine, etoposide, and carboplatin for 6 cycles plus local consolidation with thermotherapy or cryotherapy. The only alternatives to plaque radiotherapy in these cases would have been external beam radiotherapy (EBRT), additional chemotherapy, or enucleation. MAIN OUTCOME MEASURES: Tumor control and radiation complications. RESULTS: Tumor control was achieved in 95% of eyes by 5 years follow-up. Of the 59 recurrent tumors after CRD alone, 56 (95%) were controlled with plaque radiotherapy. Of the 25 recurrent tumors after CRD and EBRT, 100% were controlled with plaque radiotherapy. Of the 3 failures after plaque radiotherapy, the median time to failure (tumor recurrence) was 4 months and all recurrences were detected within 1 year. There were no identifiable factors predictive of plaque radiotherapy failure. Using Kaplan-Meier analysis, radiation complications at 5 years included proliferative retinopathy (19%), nonproliferative maculopathy (24%), papillopathy (16%), transient mild vitreous hemorrhage (54%), cataract (43%), iris neovascularization (NVI; 8%), and glaucoma (4%). Nonresolving vitreous hemorrhage and scleral necrosis did not occur. Radiation complications showed minimal change at 10 and 15 years of follow-up. CONCLUSIONS: Iodine 125 plaque radiotherapy offers 95% tumor control for selected retinoblastomas that fail CRD. Complications such as radiation retinopathy and mild vitreous hemorrhage should be anticipated, depending on the location, size, and extent of the tumor.

Adolescent↗

The association between host susceptibility factors and uveal melanoma: a meta-analysis.

OBJECTIVE: To conduct a meta-analysis, using observational studies, to examine the association between host susceptibility factors and uveal melanoma. METHODS: A review of 132 published reports on risk factors for uveal melanoma revealed 10 case-control studies that provided enough information to calculate odds ratios (ORs) and standard errors for host susceptibility factors. Data from these studies were extracted and categorized. Summary statistics were calculated for all risk factors reported by at least 4 independent studies. RESULTS: Summary statistics using meta-analysis are presented as ORs and their 95% confidence intervals (CIs). Statistically significant risk factors include light eye color (OR, 1.75 [95% CI, 1.31-2.34]), using 10 studies (1732 cases); fair skin color (OR, 1.80 [95% CI, 1.31-2.47]), using 5 studies (586 cases); and ability to tan (OR, 1.64 [95% CI, 1.29-2.09]), using 6 studies (1021 cases). Blond or red hair color, using 7 studies (1012 cases), was not a statistically significant independent risk factor (OR, 1.02 [95% CI, 0.82-1.26]). CONCLUSION: This meta-analysis yielded strong evidence associating the host susceptibility factors of iris color, skin color, and ability to tan with uveal melanoma.

Case-Control Studies↗

Arteriovenous malformation of the iris in 14 cases.

OBJECTIVE: To report the clinical and fluorescein angiography features of arteriovenous (AV) malformation of the iris. PATIENTS AND METHODS: We reviewed the medical records and photographs of our patients with iris AV malformation. The iris lesion was classified as simple if the blood vessel made a loop only and complex if it made intertwining convolutions. We reviewed systemic and ocular findings in each case. RESULTS: Fourteen patients had an iris AV malformation. The mean age at diagnosis was 49 years (median age, 50 years; range, 16-79 years). All lesions were unilateral, without predilection for either eye or significant systemic associations. The lesion was classified as simple in 5 cases and complex in 9. The full extent of the lesion was not easily visualized with routine slitlamp examination. However, it was apparent with fluorescein angiography, which showed the vascular lesions to be uniformly hyperfluorescent in the early phases, with minimal or no late leakage of dye. There were typical areas of capillary nonperfusion in the iris stroma between the large abnormal vessels. A dilated episcleral blood vessel ("sentinel vessel") was noted in the quadrant of the iris lesion in 7 (50%) of the 14 cases. On follow-up ranging from 6 months to 14 years, none of the lesions changed or produced complications. CONCLUSIONS: Iris AV malformation has characteristic clinical and fluorescein angiographic features. It appears to be a benign stationary condition that has no apparent systemic associations and no local complications.

Adolescent↗

Orbital-conjunctival glomangiomas involving two ocular rectus muscles.

PURPOSE: To report two glomangiomas in one orbit, each isolated to a rectus muscle. DESIGN: Clinicopathologic correlation. METHODS: A 12-year-old boy developed two separate vascular tumors, near the insertions of the medial rectus and superior rectus muscles, respectively. A biopsy of one tumor was studied by light microscopy and immunohistochemistry. RESULTS: Histopathology revealed blood vessels surrounded by cuboidal cells characteristic of glomangioma. The cells showed immunoreactivity for smooth muscle actin and vimentin, supporting the diagnosis. CONCLUSIONS: Glomangioma can involve the rectus muscles in the conjunctiva and orbit, and should be considered in differential diagnosis of vascular tumors in the ocular region.

Actins↗

Photoreceptor loss overlying congenital hypertrophy of the retinal pigment epithelium by optical coherence tomography.

OBJECTIVE: To describe the cross-sectional retinal anatomy overlying congenital hypertrophy of the retinal pigment epithelium (CHRPE) using optical coherence tomography (OCT). DESIGN: Retrospective, observational, noncomparative case series. PARTICIPANTS: Ten consecutive patients with CHRPE. MAIN OUTCOME MEASURES: The main outcome measures were retinal and retinal pigment epithelium (RPE) thickness relative to normal tissue and the status of the retinal layers, in particular the photoreceptor layer. RESULTS: The median patient age was 36 years (mean, 34 years; range, 7-56 years). The CHRPE was within 6 mm of the optic disc or foveola in all patients. All patients showed retinal thinning and complete photoreceptor loss overlying the CHRPE. The sensory retina measured a mean of 68% of normal thickness (range, 47%-87%). Of the 8 patients with pigmented CHRPE, the CHRPE was 52% thicker than adjacent RPE. All 8 patients with pigmented CHRPE showed moderate relative shadowing of the underlying choroid. The 2 patients with nonpigmented CHRPE, presumed to represent large lacunae with thin CHRPE rim, showed absent RPE and increased transmission of light, not shadowing, into the choroid by OCT. Both patients with visual field testing showed a visual field defect corresponding to the photoreceptor loss. CONCLUSIONS: Using OCT, overlying retinal thinning and photoreceptor loss directly over CHRPE was found in 10 consecutive patients. These findings likely account for related visual field loss.

Adolescent↗

Optical coherence tomography of retinal astrocytic hamartoma in 15 cases.

OBJECTIVE: To describe the features of retinal astrocytic hamartoma using optical coherence tomography (OCT). DESIGN: Retrospective, observational, noncomparative case series. PARTICIPANTS: Fifteen consecutive eyes from 14 patients with retinal astrocytic hamartoma. MAIN OUTCOME MEASURES: Optical coherence tomography characteristics of the tumor. RESULTS: The mean patient age at OCT was 33 years, and tuberous sclerosis was present in 3 patients (21%). By ophthalmoscopy, the mean basal diameter of the tumor was 3.6 mm. By OCT, the tumor showed hyperreflectivity at its surface, internal retinal disorganization, and a gradual gently sloping transition from a normal retina into a tumorous retina in all 15 cases (100%). By OCT, the retinal disorganization was limited to the inner retina in 3 (20%), outer retina in 0 (0%), full retina in 5 (33%), and inner retina with no view of deeper layers due to shadowing in 7 (47%). On OCT, there was mild retinal traction on the surface of the tumor in 4 (27%), discrete internal moth-eaten optically empty spaces representing intralesional calcification or intratumoral cavities in 10 (67%), and optical shadowing posterior to the tumor in 14 (93%). Other retinal findings on OCT included shallow elevation of the adjacent retina in 2 (13%), adjacent retinal edema in 4 (27%), and macular edema in 3 (20%). CONCLUSIONS: Retinal astrocytic hamartoma shows characteristic features on OCT, including a gradual transition from a normal retina into an optically hyperreflective mass with retinal disorganization, characteristic moth-eaten spaces, and posterior shadowing.

Adolescent↗

Fine-needle aspiration biopsy of iris tumors in 100 consecutive cases: technique and complications.

OBJECTIVE: To evaluate the technique and complications of fine-needle aspiration biopsy (FNAB) for iris tumors. DESIGN: Retrospective, nonrandomized, single-center case series. PARTICIPANTS: One hundred eyes of 100 patients with diagnostically challenging iris tumors evaluated with FNAB. MAIN OUTCOME MEASURES: Biopsy technique and complications. RESULTS: Of more than 1400 patients referred with an iris tumor over a 24-year period, 100 underwent FNAB for diagnostic purposes. The median patient age was 44 years. The median basal dimension of the lesion was 9.0 mm and median thickness was 2.5 mm. A limbal entry with transaqueous approach parallel to the iris was used in 100% of patients. Entry was from the superotemporal (52%) or inferotemporal (42%) direction. Needle gauge size was 22 (9%), 25 (55%), 27 (25%), or 30 (9%), depending on the friability and vascularity of the tumor. Adequate tumor sample was achieved in 99 eyes (99%). At the time of FNAB, the only immediate complication was partial hyphema (34%). Two weeks after FNAB, persistent hyphema was found in 6 eyes (6%), requiring surgical washout in 1 patient. There were no cases of recurrent hyphema, vitreous hemorrhage, prolonged hypotony, lens damage, endophthalmitis, or extraocular tumor seeding. At mean follow-up of 5 years, there was no incident of tumor recurrence on the iris, along the needle tract, or on the corneal or epibulbar surface. CONCLUSIONS: Using our technique, FNAB for diagnostically challenging iris tumors can achieve high yield with relatively few complications.

Adolescent↗

Multifocal hypopigmented retinal pigment epithelial lesions in incontinentia pigmenti.

BACKGROUND: Incontinentia pigmenti (IP) can manifest with retinal detachment in children and can simulate retinoblastoma. The authors report a clinicopathologic correlation of IP and illustrate the retinal pigment epithelium (RPE) alterations that may be useful in establishing the diagnosis. METHODS: A 30-month-old reportedly healthy girl developed strabismus and was discovered to have total retinal detachment in the left eye. Intraocular calcification was found on ultrasonography and computed tomography, suggestive of retinoblastoma. RESULTS: Ophthalmoscopy of the right eye revealed sector distribution of approximately 70 elongated, linear hypopigmented lesions of the RPE located in the peripheral superotemporal, inferotemporal, and superonasal quadrants and measuring 300 microm or less in basal dimension. The left eye had a total retinal detachment, vitreous traction, perivascular exudation, and iris neovascularization. Despite the lack of medical history in this patient, skin examination revealed classic cutaneous findings of IP The enucleated left globe displayed tractional retinal detachment with vitreoretinal neovascularization and nonspecific RPE papillary proliferation. Scattered minor foci of intraretinal calcification were observed. There was no sign of retinoblastoma. CONCLUSIONS: IP can closely simulate retinoblastoma with retinal detachment and minor intraocular calcification. In this case, the classic cutaneous manifestations combined with the fine linear hypopigmented RPE foci were important in suggesting the diagnosis of IP.

Calcinosis↗

Progressive enlargement of cavity within melanoma masquerading as iris cyst.

PURPOSE: To describe progressive enlargement of intralesional cavities within a mass that masqueraded clinically as an iris pigment epithelial cyst for 2 years but later proved to be a melanoma. METHODS: An 81-year-old woman developed asymptomatic focal elevation of the iris, and ultrasound biomicroscopy showed a fluid-filled mass that was interpreted as an iris pigment epithelial cyst. Over a 2-year period of observation, the cavity enlarged from 0.7 to 10 mm, and the solid component enlarged from approximately 6 to 16 mm basal dimension. RESULTS: On referral, visual acuity was 20/400 in the right eye and 20/25 in the left eye. The affected right eye displayed a pigmented mushroom-shaped ciliary body mass with anterior chamber invasion, distorting the pupil and occupying 40% of the anterior chamber. Ultrasound biomicroscopy and B-scan ultrasonography revealed many cavities within the 14-mm-thick mass, suggestive of ciliary body melanoma. After enucleation, histopathology disclosed multiple cavities, some with granular eosinophilic material, within a mushroom-shaped mixed-cell melanoma. CONCLUSION: Cavitation can occur in ciliary body melanoma and can lead to misinterpretation as a cyst. Cavitary melanoma should be considered in the differential diagnosis of cystic lesions of the anterior segment.

Aged, 80 and over↗

Sebaceous carcinoma of the caruncle.

PURPOSE: To report a case of sebaceous carcinoma of the caruncle. METHODS: A 68-year-old woman developed a slowly enlarging mass of her left caruncle. Incisional biopsy elsewhere was interpreted histopathologically as invasive squamous cell carcinoma. On the basis of the clinical findings, however, we suspected sebaceous carcinoma. Excisional biopsy with frozen section control was undertaken and the lesion was studied histopathologically. RESULTS: Histopathological examination disclosed infiltration of the caruncular stroma by lobules of a malignant neoplasm that had prominent cytoplasmic vacuoles, typical of sebaceous carcinoma. CONCLUSION: Although it is best known to arise in the meibomian glands of the tarsus, sebaceous carcinoma can rarely arise in the caruncle and can be misinterpreted histopathologically as squamous cell carcinoma.

Adenocarcinoma, Sebaceous↗