Search PubMed⌕ Search

Biomedical subjects

R C Eagle

Publications and source records attributed to R C Eagle.

At least 37 records · Page 2Linked to original sources

Central retinal vascular obstruction secondary to melanocytoma of the optic disc.

A 35-year-old black man developed abrupt visual loss in his left eye. Ophthalmic examination revealed a deeply pigmented mass obscuring the optic disc, hemorrhagic retinopathy, and signs of central retinal vascular obstruction. Fluorescein angiography disclosed sluggish filling of the retinal blood vessels; ultrasonography disclosed an acoustically solid mass in the optic nerve head. Cytopathologic findings of a fine needle aspiration biopsy specimen demonstrated probable benign tumor cells, but melanoma could not be excluded. Histopathologic findings in the enucleated eye revealed a large, necrotic melanocytoma of the optic disc and hemorrhagic necrosis of the retina secondary to obstruction of the central retinal artery and vein. Melanocytoma of the optic nerve can undergo spontaneous necrosis and induce central retinal vascular obstruction. Abrupt visual loss in a patient with a melanocytoma does not necessarily imply malignant transformation.

Adult↗

Congenital corneal opacification in De Barsy syndrome.

A newborn male was noted to have bilateral congenital corneal opacification. Findings from examination disclosed a variety of dysmorphic features, including cutis laxa, progeroid aspect, short stature, multiple hyperextensible subluxated joints, muscular hypotonia, and hyperreflexia. Bilateral penetrating keratoplasties were performed; histopathologic examination revealed diffuse epithelial thickening, loss of the Bowman layer, and stromal attenuation with anterior stromal scarring. Special stains showed no deposition of abnormal material in the corneas. Electron microscopy demonstrated absence of Bowman layer differentiation with a paucity of collagen fibers, as well as extensive small elastic fibers in the anterior stroma. The diagnosis of De Barsy syndrome was made, a rare progeroid syndrome associated with characteristic ocular, facial, skeletal, dermatologic, and neurologic abnormalities. De Barsy syndrome should be included in the differential diagnosis of congenital corneal opacification; its distinctive clinical features enable the clinician to easily differentiate it from other causes of congenitally cloudy corneas.

Abnormalities, Multiple↗

Adenoma of the retinal pigment epithelium simulating a juxtapapillary choroidal neovascular membrane.

We report a case of an adenoma of the retinal pigment epithelium that clinically simulated a juxtapapillary choroidal neovascular membrane in a 60-year-old man. Fluorescein angiography supported the diagnosis of a juxtapapillary choroidal neovascular membrane in his left eye. After 13 years the lesion become slightly pigmented and the optic disc became swollen. The possibility of choroidal melanoma with optic disc invasion was considered, and the eye was enucleated. The lesion proved histopathologically to be an adenoma of the retinal pigment epithelium.

Adenoma↗

Malignant melanoma arising from unusual conjunctival blue nevus.

Cellular blue nevus is an uncommon pigmented tumor in the conjunctiva, where it generally appears as a deep, circumscribed, pigmented conjunctival mass. We report a case of conjunctival blue nevus that clinically resembled primary acquired melanosis and gave rise to conjunctival melanoma. A 41-year-old man developed a diffuse pigmented mass in the inferior fornix of his left eye. Over a 20-year period, he noted slight progression of the pigment. Foci of epibulbar pigmentation were also present. The lesion resembled primary acquired melanosis. Excisional biopsy and adjuvant cryotherapy were performed. Histopathologic examination disclosed an intense infiltrate of heavily pigmented dendritic melanocytes with aggregates of less pigmented plump cells in the substantia propria. The conjunctival epithelium was normal. Malignant cellular features consistent with melanoma were observed in some foci. Cellular blue nevus of the conjunctiva can simulate primary acquired melanosis and can give rise to malignant melanoma. Arch Ophthalmol. 2000;118:1581-1584

Adult↗

Observations on 17 patients with retinocytoma.

OBJECTIVE: To study the clinical features and natural history of 17 patients with retinocytoma. DESIGN: A retrospective case series. SETTING: Tertiary referral center. PATIENTS: Data on 17 patients with retinocytoma were reviewed for clinical features. The natural history of retinocytoma and its risk for malignant transformation were also evaluated. RESULTS: Among 920 consecutive patients who had retinoblastoma, retinocytoma, or both, we identified 24 tumors in 17 patients (1.8%) with clinical features compatible with retinocytoma. The median age at diagnosis was 15 years (range, 4-45 years). Of the 24 tumors, the retinocytoma was bilateral in 3 cases (13%) and the family history of retinoblastoma was positive in 3 cases (13%). Seventeen (71%) of the tumors were extramacular in location, and 7 (29%) were located in the macular area. Ophthalmoscopic features characteristic of retinocytoma included the presence of a translucent retinal mass in 21 (88%), calcification in 15 (63%), and retinal pigment epithelial alteration in 13 (54%) of the 24 tumors. A combination of all 3 features was observed in 8 (33%) of the 24 tumors. In 13 (54%) of the tumors, a zone of chorioretinal atrophy could be observed. In 1 patient, subtle tumor regression was documented photographically. Only 1 retinocytoma (4%) underwent malignant transformation into retinoblastoma. At the last follow-up visit, none of the patients had developed a pineoblastoma or another second malignant neoplasm. CONCLUSIONS: Retinocytoma is a rare benign retinal tumor that has characteristic clinical features. The areas of chorioretinal atrophy were suggestive of tumor regression. In our series, the risk for malignant transformation of retinocytoma into retinoblastoma was 4%; therefore, patients with a presumed diagnosis of retinocytoma should be closely observed.

Adolescent↗

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↗

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↗

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↗

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↗