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Biomedical subjects

C L Shields

Publications and source records attributed to C L Shields.

At least 73 records · Page 4Linked to original sources

Combination of clinical factors predictive of growth of small choroidal melanocytic tumors.

OBJECTIVE: To better define the effect of individual risk factors and combinations thereof on the growth of small choroidal melanocytic tumors. DESIGN: Retrospective analysis. SETTING: Clinical practice of ocular oncology. PATIENTS: The study included 1287 patients with small suspicious choroidal melanocytic tumors, measuring 3 mm or less in thickness, managed with observation. RESULTS: On multivariate analysis, the clinical risk factors predictive of growth of small choroidal melanocytic tumors include tumor thickness greater than 2.0 mm, posterior tumor margin touching the disc, visual symptoms, orange pigment, and subretinal fluid. Tumor growth was detected in 4% of those patients with no risk factors. Growth was detected in approximately 36% of patients with 1 risk factor, 45% of patients with 2 risk factors, 50% of patients with 3 risk factors, 51% of patients with 4 risk factors, and 56% of patients with all 5 risk factors. The combination of risk factors offering the greatest risk for growth was tumor thickness greater than 2.0 mm, tumor margin touching disc, and subretinal fluid that was associated with tumor growth in 63% of the affected patients. The relative risk for growth was 1.9 for 1 factor, 3.8 for 2 factors, 7.4 for 3 factors, 14.1 for 4 factors, and 27.1 for all 5 risk factors combined. CONCLUSIONS: Five risk factors for growth of small choroidal melanocytic tumors have been identified. The combinations of various factors increase the risk for tumor growth from 4% if no factors are present to more than 50% if 3 or more risk factors are present. These factors may be important when counseling patients with small suspicious choroidal melanocytic tumors.

Choroid Neoplasms↗

Acquired tumors arising from congenital hypertrophy of the retinal pigment epithelium.

BACKGROUND: Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is widely recognized to be a flat, stationary condition. Although it can show minimal increase in diameter, it has not been known to spawn nodular tumor that is evident ophthalmoscopically. OBJECTIVES: To report 5 cases of CHRPE that gave rise to an elevated lesion and to describe the clinical features of these unusual nodules. METHODS: Retrospective medical record review. RESULTS: Of 5 patients with a nodular lesion arising from CHRPE, there were 4 women and 1 man, 4 whites and 1 black. Three patients were followed up for typical CHRPE for longer than 10 years before the tumor developed; 2 patients were recognized to have CHRPE and the elevated tumor concurrently. Visual acuity was decreased in 3 patients, mainly due to cystoid macular edema. The tumor was located between the equator and ora serrata in all 5 patients. There was no predilection for quadrant of the fundus. The flat part of the lesion was black and had visible lacunae in all 5 patients. The CHRPE ranged in basal diameter from 3 x 3 mm to 13 x 11 mm. The size of the elevated lesion ranged from 2 x 2 x 2 mm to 8 x 8 x 4 mm. The nodular component in all cases was supplied and drained by slightly prominent, nontortuous retinal blood vessels. Yellow retinal exudation occurred adjacent to the nodule in all 5 patients and 1 patient developed a secondary retinal detachment. Two tumors that showed progressive enlargement, increasing exudation, and progressive visual loss were treated with iodine 125-labeled plaque brachytherapy, resulting in deceased tumor size but no improvement in the visual acuity. CONCLUSIONS: Congenital hypertrophy of the retinal pigment epithelium can spawn a nodular growth that slowly enlarges, attains a retinal blood supply, and causes exudative retinopathy and chronic cystoid macular edema. Although no histopathologic evidence is yet available, we believe that the tumor probably represents either an acquired adenoma or a reactive proliferation of the retinal pigment epithelium. The best treatment of these lesions is not yet established.

Adenoma↗

Iris varix simulating an iris melanoma.

Iris varix is rare and little is known about its clinical characteristics. We treated a thrombosed iris varix that simulated an iris melanoma. A 53-year-old man developed a dark brown iris mass and hyphema in his left eye. Ultrasound biomicroscopy revealed a circumscribed mass of the iris stroma. Because of suspicion for melanoma, it was removed by sector iridectomy. Histopathologic examination disclosed an extensive focus of stromal hemorrhage, partially surrounded by endothelial cells that showed immunoreactivity to vascular markers. The histopathologic diagnosis was thrombosed iris varix. Iris varix is a rare condition that should be included in the differential diagnosis of iris melanoma.

Diagnosis, Differential↗

Hamartomas of the iris and ciliary epithelium in tuberous sclerosis complex.

Astrocytic hamartomas of the retina are the principal ocular manifestation of tuberous sclerosis complex. Iris abnormalities are rare in tuberous sclerosis complex and include focal areas of stromal depigmentation and atypical colobomata. We describe 2 patients who were found on histopathological examination to have lesions consistent with hamartomas of the iris pigment epithelium and ciliary body epithelium. Iris abnormalities, including pupillary irregularities, were noted on clinical examination prior to the development of iris neovascularization in both patients. These observations suggest that iris abnormalities, including atypical colobomas, may be caused by hamartomas of the iris pigment epithelium and ciliary epithelium in some patients with tuberous sclerosis complex. To our knowledge, hamartomas of tissues derived from the anterior part of the neuroectodermal optic cup have not been reported in cases of tuberous sclerosis complex.

Child, Preschool↗

Iris mammillations as the only sign of ocular melanocytosis in a child with choroidal melanoma.

An 8-year-old girl had visual loss in her left eye over 2 months. Ocular examination showed that visual acuity was counting fingers in the left eye. The left iris was moderately pigmented and thickened with numerous confluent, dome-shaped elevations on its surface, consistent with iris mammillations arising from ocular melanocytosis. There was total retinal detachment and an inferiorly located large amelanotic choroidal mass compressing the optic nerve. A specimen from a fine-needle aspiration biopsy showed spindle and epithelioid melanoma cells. The eye was enucleated. Pathologic examination showed that the bland melanocytes comprising the anterior border layer of iris formed focal aggregates, corresponding to the iris mammillations observed clinically. The uvea was diffusely thickened. Arising from the posterior choroid and obscuring the optic nerve head was a moderately pigmented spindle and epithelioid cell choroidal melanoma with diffuse lymphocytic infiltration and high mitotic activity. This case demonstrates that iris mammillations can be the initial manifestation of ocular melanocytosis in the absence of scleral pigmentation.

Child↗

Choroidal metastasis as the initial manifestation of a pigmented neuroendocrine tumor.

We report the case of a 77-year-old woman in whom choroidal metastasis was the initial manifestation of a primary neoplasm presumed to be a pigmented pulmonary carcinoid tumor. The tumor initially was misdiagnosed cytologically and pathologically as a choroidal melanoma because it contained intrinsic melanin pigment. Positive immunoreactivity for cytokeratin, synaptophysin, chromogranin, and calcitonin and the presence of dense-core neurosecretory vesicles disclosed by electron microscopy established that the metastasis was a neuroendocrine tumor. Findings from systemic evaluation suggested that the primary tumor was located in the lung. The patient subsequently developed an intradural paraspinal metastasis, which also contained melanin pigment. The latter observation confirmed that the melanin in the uveal metastasis was intrinsic and did not represent secondary phagocytosis by tumor cells. Metastases from pigmented tumors of nonmelanocytic derivation are exceedingly rare but present a major diagnostic challenge to ocular pathologists and cytopathologists if the diagnosis is not suspected. Confirmatory immunohistochemical analysis should be obtained when a pigmented choroidal tumor thought to be a melanoma has atypical features. Arch Ophthalmol. 2000;118:841-845

Aged↗

Plaque radiotherapy for uveal melanoma: long-term visual outcome in 1106 consecutive patients.

OBJECTIVE: To identify clinical predictive factors for visual outcome in a large series of patients who underwent plaque radiotherapy for uveal melanoma. DESIGN: Clinical factors, including patient data, tumor features, and radiation variables, were analyzed for their impact on visual acuity using Cox proportional hazards regression models. PARTICIPANTS: Patients with uveal melanoma and initial visual acuity of 20/100 or better in the affected eye who were treated with plaque radiotherapy between July 1976 and June 1992. MAIN OUTCOME MEASURES: Two end points were used to evaluate posttreatment visual acuity: (1) final visual acuity (good [20/20-20/100] vs poor [20/200 to no light perception]) and (2) loss of visual acuity (minimal [<5 lines Snellen visual acuity] vs moderate [> or = 5 lines Snellen visual acuity]). RESULTS: Of 1300 consecutive patients with uveal melanoma treated by plaque radiotherapy, 1106 had a visual acuity of 20/100 or better at the time of treatment. In this group, poor visual acuity was found in 34% at 5 years and 68% at 10 years of follow-up. From multivariable analysis, clinical factors that best predicted poor visual acuity were increasing tumor thickness, proximity to foveola of less than 5 mm, notched plaque shape, tumor recurrence, patient age 60 years or older, subretinal fluid, cobalt isotope, anterior tumor margin posterior to equator, and worse initial visual acuity. Moderate loss of visual acuity of 5 Snellen lines or more was found in 33% at 5 years and 69% at 10 years of follow-up. From multivariable analysis, clinical factors that best predicted moderate visual acuity loss included increasing tumor thickness, worse initial visual acuity, notched plaque shape, tumor recurrence, proximity to foveola of less than 5 mm, patient age of 60 years or older, subretinal fluid, and diabetes mellitus or hypertension. When analyzing visual outcome with regard to tumor thickness, ultimate poor visual acuity of 20/200 or worse at 5 years was found in 24% with a small melanoma (< or = 3.0 mm), 30% with a medium melanoma (3.1-8.0 mm), and 64% with a large melanoma (>8.0 mm). When analyzing visual outcome with regard to tumor proximity to visually important structures, tumors less than 5 mm from the optic disc or foveola demonstrated poor visual acuity in 35% at 5 years, whereas those 5 mm or more from the optic disc and foveola showed poor visual acuity in 25% at 5 years. CONCLUSIONS: Ultimate visual acuity after plaque radiotherapy for uveal melanoma depends on many factors, including patient age and general health, initial visual acuity, tumor location and size, subretinal fluid, radioactive isotope, and final tumor control. At 10 years' follow-up, 68% of patients demonstrate poor visual acuity. Visual acuity is most effectively preserved in eyes with small tumors outside a radius of 5 mm from the optic disc and foveola. Arch Ophthalmol. 2000;118:1219-1228

Adolescent↗

Long-term follow-up of giant nodular posterior scleritis simulating choroidal melanoma.

A 41-year-old asymptomatic woman was referred for enucleation of a 7. 5-mm-thick intraocular tumor suspected to be choroidal melanoma. The clinical findings combined with imaging studies suggested instead a diagnosis of giant nodular posterior scleritis. A scleral biopsy was performed to confirm the diagnosis. After 12 years of observation, the lesion has remained stable and visual acuity has been preserved. Nodular posterior scleritis can present with no symptoms of pain, redness, or visual disturbance and can remain quiet for many years. It must be clinically differentiated from choroidal melanoma. Arch Ophthalmol. 2000;118:1290-1292

Adult↗

Intraocular lacrimal gland choristoma involving iris and ciliary body.

PURPOSE: To describe clinical and pathologic features of an iridociliary lacrimal gland choristoma in which the diagnosis was made clinically and confirmed histopathologically. METHODS: Case report with clinical, ultrasound biomicroscopic, and histopathologic observations. RESULTS: We examined a 12-month-old male for a large iris mass that had been present since birth. We suspected it to be a lacrimal gland choristoma based on characteristic clinical features. The lesion was removed by iridocyclectomy, and the diagnosis of lacrimal gland choristoma was confirmed. CONCLUSION: Intraocular lacrimal gland choristoma has unique clinical features that should suggest the diagnosis.

Choristoma↗

Plaque radiotherapy for management of ciliary body and choroidal melanoma with extraocular extension.

PURPOSE: To report the results of plaque radiotherapy of ciliary body and choroidal melanoma with extraocular extension. METHODS: This retrospective study of 17 eyes of 17 patients with ciliary body and choroidal melanoma demonstrated extraocular extension that was clinically evident but less than 3 mm in thickness. In 14 eyes, the extraocular extension was located anteriorly and in three posteriorly. The outcome of plaque radiotherapy in terms of intraocular and extraocular tumor responses and the development of metastasis were analyzed. RESULTS: At a median follow-up of 63 months (range, 23 to 164 months) after plaque radiotherapy, all patients showed control of both the intraocular and extraocular components of ciliary body and choroidal melanoma. In no case did the intraocular or extraocular portion of the tumor relapse. The initial median thickness of the intraocular component was 4.9 mm, and the final median thickness was 2.4 mm. The initial median thickness of the extraocular component was 2.0 mm, and the final median thickness was 1.0 mm. The initial median basal diameter of the extraocular component was 3.5 mm, and the final median basal diameter was 2.0 mm. Of 17 patients, 11 (10 with anterior and one with posterior extraocular extension) showed partial regression in base and thickness of the extraocular extension, and in six there was little or no change. In those with anterior extrascleral extension, the sclera remained intact without clinically observable necrosis, and the scleral fibers were gradually more visible as the tumor regressed. Three patients (18%) died of metastatic melanoma at a median interval of 38 months after plaque radiotherapy. CONCLUSIONS: Plaque radiotherapy appears to be a reasonable treatment option for selected cases of ciliary body and choroidal melanoma with clinically visible extraocular extension less than 3 mm in thickness.

Adult↗

Congenital orbital varices causing extreme neonatal proptosis.

PURPOSE: To report a 6-week-old male with extreme proptosis caused by thrombosed orbital varices. METHODS: A 6-week-old male presented with advanced proptosis of his left eye. Computed tomography and magnetic resonance imaging studies of the orbits revealed a heterogeneous cystic mass that filled the entire left orbit. Extreme proptosis and corneal exposure prompted urgent surgical excision of the mass. Histopathologic review of the lesion was consistent with orbital varices. CONCLUSIONS: Although orbital varices usually are found in adults, they should be considered in the differential diagnosis of orbital lesions in children. Observation is usually warranted, but surgical intervention may be necessary in advanced cases.

Exophthalmos↗

Compound nevus of the cornea simulating a foreign body.

PURPOSE: To report a corneal nevus, without conjunctival involvement, that clinically simulated a metallic foreign body. METHODS: Case report with clinicopathologic correlation. RESULTS: A 22-year-old man developed a superficial pigmented lesion of his left cornea. It was suspected to be a chronically retained foreign body, but a melanocytic neoplasm could not be excluded. The lesion was removed by lamellar keratectomy and proved histopathologically to be a compound nevus of the cornea, without conjunctival involvement. CONCLUSIONS: Melanocytic nevus can rarely occur in the cornea. Involvement of the cornea by a melanocytic lesion does not necessarily imply a malignant melanoma.

Adult↗

Systemic amyloidosis presenting as a mass of the conjunctival semilunar fold.

PURPOSE: To report a case of systemic amyloidosis that presented as a mass of the plica semilunaris (semilunar fold) of the conjunctiva. METHODS: Case report. RESULTS: A healthy 56-year-old man developed a fleshy, hemorrhagic lesion of the semilunar fold of the left conjunctiva. Excisional biopsy revealed an acellular lesion of the conjunctival stroma that stained positively for Congo red and showed apple-green birefringence and dichroism with polarization microscopy. The diagnosis was amyloidosis of the semilunar fold of the conjunctiva. Subsequent evaluation disclosed systemic amyloidosis. The patient remains asymptomatic. CONCLUSIONS: Although conjunctival amyloidosis usually occurs as a localized lesion without systemic involvement, it can be the initial sign of systemic amyloidosis.

Amyloidosis↗

Concurrent choroidal melanoma in son and father.

PURPOSE: To report the concurrent occurrence of choroidal melanoma in a son and father. METHODS: Case reports. The likelihood of familial occurrence of uveal melanoma in which father and son are affected was estimated using the principles of probability. RESULTS: Unilateral choroidal melanoma was diagnosed in a 41-year-old man. Three months later, his 68-year-old father presented with a pigmented choroidal mass that greatly increased in size during the next 6 months and was diagnosed a choroidal melanoma. No personal or family history of atypical moles, cutaneous melanoma, or other malignancy existed. The likelihood of familial occurrence of uveal melanoma using principles of probability was estimated to be 1 in 10 million. CONCLUSION: Considering the low prevalence of uveal melanoma in the general population, the likelihood of uveal melanoma occurring in a family setting is remote.

Adult↗

Localized infection by Serratia marcescens simulating a conjunctival neoplasm.

PURPOSE: To report a Serratia marcescens infection that clinically simulated a conjunctival neoplasm. METHOD: Case report. RESULTS: A healthy 80-year-old man without contact lenses presented with a pink-yellow conjunctival mass that resembled a solid neoplasm. Stains and cultures of material that exuded from the mass during surgery revealed S. marcescens. Histopathology disclosed an epithelial-lined cyst with macrophages containing S. marcescens. CONCLUSION: Although S. marcescens usually affects the eye as a keratoconjunctivitis in patients with contact lenses, it can also present as a mass simulating a neoplasm in a patient who does not wear contact lenses.

Aged↗

Cutaneous capillary hemangiomas of the eyelid, scalp, and digits in premature triplets.

PURPOSE: To document the unique occurrence of multiple cutaneous capillary hemangiomas in healthy triplets. METHODS: Case reports. RESULTS: Triplets born 6 weeks prematurely were noted at birth to have classic cutaneous capillary hemangiomas that enlarged in the first few months of life. At 3 months of age, triplet #1 manifested a small capillary hemangioma on the left index finger and a large hemangioma that involved the entire left upper eyelid and completely occluded the visual axis. Triplet #2 had a small hemangioma on the thumb and a large hemangioma on the scalp of the forehead. Triplet #3 displayed only a minor hemangioma of the right upper eyelid that did not threaten her vision. The parents of the triplets admitted having a history of periocular capillary hemangioma that spontaneously resolved. Triplet #1 was managed with oral corticosteroids, with dramatic regression of the mass and exposure of the visual axis. CONCLUSIONS: Cutaneous capillary hemangioma is usually a sporadic condition. We found this tumor in premature triplets whose parents had a history of a similar tumor. This suggests that hereditary and environmental factors may play a role in the development of this tumor. We advise that medical and family histories be obtained in all children with cutaneous capillary hemangioma.

Eyelids↗

Juxtapapillary capillary hemangiomas. Clinical features and visual acuity outcomes.

OBJECTIVE: To describe the clinical features, association with von Hippel-Lindau (VHL) disease and visual acuity outcomes of patients with a juxtapapillary capillary hemangioma. DESIGN: Retrospective observational case series. PARTICIPANTS: Seventy-two eyes of 68 patients identified with a juxtapapillary capillary hemangioma. Follow-up data of at least 6 months duration were available for 60 eyes. METHODS: A retrospective chart review of patients diagnosed with a juxtapapillary capillary hemangioma examined at four medical centers. MAIN OUTCOME MEASURES: Age at diagnosis, visual acuity (VA) at first examination and at last follow-up, tumor growth pattern and location, associated clinical features, type of treatment, association with VHL, and presence of peripheral hemangiomas were recorded for each patient. RESULTS: On initial examination, VA was >/=20/40 in 43 of 70 eyes (61%) and was >/=20/200 in 60 eyes (86%). At an average follow-up of 5.4 years (range, 0.5-19 years), VA of >/=20/40 was achieved in 21 eyes (35%) and >/=20/200 in 33 eyes (55%). Patients with VHL had poorer initial VA (48% vs. 70% with VA >/=20/40, and 74% vs. 93% with VA >/=20/200) and final VA (26% vs. 41% with VA >/=20/40, and 39% vs. 65% with VA >/=20/200) compared with patients without VHL. Patients with VHL more commonly were seen at an earlier age (average, 20 vs. 44 years, P: < 0.001), with bilateral (17% vs. 0%), and/or peripheral (39% vs. 0%) (P: < 0.001) tumors that more often had an endophytic growth pattern (63% vs. 22%, P: = 0.001) compared with patients without VHL. Patients selected for laser treatment generally had poorer initial (52% vs. 74% with VA >/=20/40, 79% vs. 96% with VA >/=20/200) and final VAs (18% vs. 56% with VA >/=20/40, 45% vs. 67% with VA >/=20/200) compared with patients not treated with laser. CONCLUSIONS: On long-term follow-up of patients with a juxtapapillary capillary hemangioma, the VA generally worsens. Patients with VHL and a juxtapapillary hemangioma more often present at a younger age, have tumors with an endophytic growth pattern, and have bilateral, multiple tumors. Treatment with laser photocoagulation results in variable VA outcomes.

Adolescent↗