Coats' disease as a cause of anterior chamber cholesterolosis.
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Anterior Chamber↗
Biomedical subjects
R C Eagle
Publications and source records attributed to R C Eagle.
Histologic evidence of recurrent keratoconus seven years after keratoplasty.
PURPOSE/METHODS: A 42-year-old patient with keratoconus developed clinical signs of recurrent keratoconus in her right corneal graft seven years after the first keratoplasty was performed. She underwent successful corneal regrafting. RESULTS/CONCLUSIONS: The histopathologic examination of the corneal button disclosed typical findings of keratoconus, including breaks in Bowman's layer filled with connective tissue or epithelium. These histologic findings in the corneal graft support our clinical diagnosis of keratoconus. However, the pathogenesis of this complication is still not clear and further research is needed.
Adult↗
Multinucleate cell angiohistiocytoma of the orbit.
PURPOSE: A 31-year-old had a five-year history of painless progressive swelling of her right upper eyelid. METHOD: A mass was removed from the superonasal aspect of the right orbit. RESULTS: The orbital mass was composed of a collagenous stroma with capillary channels and irregular giant cells. The findings were characteristic of multinucleate cell angiohistiocytoma. CONCLUSION: Multinucleate cell angiohistiocytoma should be included in the differential diagnosis of circumscribed orbital tumors.
Adult↗
Natural course and histopathologic findings of lacrimal gland choristoma of the iris and ciliary body.
PURPOSE: To show a well-documented clinicopathologic correlation of lacrimal gland choristoma of the iris and ciliary body, to review the literature on the subject, and to recommend treatment of this condition. METHODS: A congenital iridociliary mass was removed by iridocyclectomy when a female infant was 19 months old because it had produced a large cyst, secondary glaucoma, a vascularized corneal pannus, focal cataract, and iris heterochromia secondary to pigment dispersion. The excised mass was studied by routine histopathologic methods. RESULTS: The mass proved histopathologically to be a lacrimal gland choristoma composed of mature lacrimal gland tissue associated with a large epithelial-lined cyst and several smaller cysts. Postoperatively, the glaucoma resolved and the child had visual acuity of 20/40 14 months after surgery. CONCLUSIONS: Lacrimal gland choristoma of the anterior uvea has typical clinical features that should indicate that correct diagnosis. It is a fleshy, often cystic, lesion with an irregular, cerebriform surface that appears identical to the lacrimal gland in the orbit. Although the mass itself is generally stable, secondary cysts in the lesion can enlarge and secondary glaucoma and other complications can develop. Consequently, affected patients should be followed up at close intervals and surgical excision performed if such complications begin to develop.
Choristoma↗
Initial management and follow-up of melanocytic iris tumors.
PURPOSES: To identify clinical variables prognostic for prompt excision of melanocytic iris tumors and to determine the clinical factors predictive of enlargement of those tumors managed initially by observation. METHODS: A retrospective study of 285 patients evaluated in a referral center over a 15-year interval (1972-1986). RESULTS: Thirty-six lesions were excised promptly, whereas 249 were observed. Eighty-nine percent of the 36 promptly excised tumors were malignant melanomas on histopathologic examination. Five clinical variables were associated strongly with prompt excision: largest basal tumor diameter greater than 3 mm; presence of pigment dispersion; prominent tumor vascularity; elevated intraocular pressure; and tumor-related ocular symptoms. In the observed group, the actuarial 5-year rate of lesion enlargement was 6.5% (standard error = 2.1%). Of the ten lesions that enlarged, six were excised and evaluated histopathologically. Five of these six lesions were malignant melanomas on histopathologic study. Largest basal tumor diameter was the only clinical variable strongly predictive of lesion enlargement. Only two patients died of metastatic uveal melanoma, and both were in the promptly treated group. CONCLUSIONS: Most melanocytic iris tumors are benign and do not enlarge appreciably when followed or lead to metastatic disease. However, even prompt locally effective treatment (excision) is insufficient to prevent metastasis in some patients. Clinical features of the iris lesion appear to enable clinicians to differentiate reasonably well between probable malignant melanomas, for which prompt treatment seems appropriate, and benign nevi, for which observation with periodic follow-up is likely to be the best management.
Female↗
Eyelid involvement in multicentric reticulohistiocytosis.
CASE REPORT: The authors report the first biopsy-confirmed ocular adnexal involvement by multicentric reticulohistiocytosis. The patient was a 28-year-old black woman with a 15-year history of well-documented multicentric reticulohistiocytosis who had multiple dermal nodules and severe mutilating arthritis that had produced a characteristic telescoping deformity of the digits. Eyelid nodules were excised to relieve pseudoptosis and for cosmesis. MATERIALS: The eyelid nodules were studied by routine light microscopy and a battery of immunohistochemical stains, including S-100 protein and several macrophage markers. RESULTS: Microscopy disclosed an infiltrate of histiocytes and giant cells with glassy eosinophilic cytoplasm and large round or oval vesicular nuclei with prominent nucleoli set in a densely fibrotic stroma consistent with multicentric reticulohistiocytosis. The histiocytes showed positive immunoreactivity for lysozyme, but were negative for alpha-1 antichymotrypsin, S-100 protein, and neuron-specific enolase. CONCLUSION: Multicentric reticulohistiocytosis should be included in the differential diagnosis of multiple eyelid nodules.
Adult↗
Orbital varix presenting as a subconjunctival mass.
A 39-year-old woman presented with a vermiform superior conjunctival mass that progressively enlarged during 4 years of follow-up. Although magnetic resonance imaging suggested that the lesion was located in the superior forniceal conjunctiva, surgical exploration disclosed a vascular anomaly (varix) that extended deep into the orbit. The clinical features and management of orbital varices are discussed.
Adult↗
Benign mixed tumor arising from an accessory lacrimal gland of Wolfring.
We report a 59-year-old man who had a slowly enlarging mass of the central upper eyelid that proved histopathologically to be a benign mixed tumor (pleomorphic adenoma) arising from an accessory lacrimal gland of Wolfring. The tumor was totally separate from the main lacrimal gland, and its deep location in the lid excluded origin from dermal sweat glands. Benign mixed tumors of the accessory lacrimal glands are exceedingly rare tumors of the ocular adnexa.
Eyelid Neoplasms↗
Uveal melanoma and familial atypical mole and melanoma (FAM-M) syndrome.
We conducted this study to determine whether occurrence of primary uveal melanoma in the setting of familial atypical mole and melanoma (F A M-M) syndrome (an autosomal dominant cutaneous preneoplastic syndrome) follows a pattern of a hereditary cancer predisposition syndrome. A retrospective review of 4600 consecutive patients with primary uveal melanoma revealed eight patients with biopsy-proven F A M-M syndrome. The clinical profile of these patients was studied and their kindreds analyzed. In patients with F A M-M syndrome, the uveal melanoma occurred at a relatively young age (mean 40 years; range 10-52 years). The diagnosis of F A M-M syndrome preceded or followed the diagnosis of uveal melanoma by as much as 10 years. None of the patients had an associated nonmelanocytic malignancy. Three of the eight patients had a positive family history of melanoma (cutaneous melanoma (2) and uveal melanoma (1). The authors conclude that the occurrence of primary uveal melanoma in the setting of F A M-M syndrome does not follow a clear pattern of a hereditary cancer predisposition syndrome.
Adult↗
Localized transscleral staphylococcal abscess simulating a neoplasm.
A 47-year-old woman with a history of breast cancer developed a focal epibulbar mass in the right eye. The initial diagnosis was nodular episcleritis, but the lesion failed to respond to local and systemic corticosteroids. The rule out an epibulbar metastatic carcinoma an excisional biopsy was performed, which demonstrate a localized staphylococcal abscess that had locally eroded the sclera and exposed the underlying uveal tract. Covering the defect with a scleral patch graft and promptly instituting oral amoxicillin/clavulanic acid and topical cefazolin treatment resulted in an uncomplicated recovery. Infectious scleral abscess in an uncommon entity and the differential diagnosis includes nodular episcleritis and epibulbar metastatic tumor.
Abscess↗
Endogenous endophthalmitis simulating retinoblastoma. The 1993 David and Mary Seslen Endowment Lecture.
BACKGROUND: Among conditions that can simulate retinoblastoma, endogenous endophthalmitis is quite rare. METHODS: Case records of six children with unusual forms of endogenous endophthalmitis, all of whom were referred to the authors because retinoblastoma was a strong diagnostic consideration, were reviewed. The clinical features that may help differentiate atypical endophthalmitis from retinoblastoma were investigated. RESULTS: The final diagnosis in these cases included idiopathic subretinal abscess, streptococcal endophthalmitis, idiopathic retinovitreal abscess, cytomegalovirus endophthalmitis, Candida endophthalmitis, and meningococcal endophthalmitis. All of the affected children presented primarily with ocular findings without serious systemic infection. Although these conditions closely simulated retinoblastoma, they were more likely to have signs of concurrent or prior inflammation. CONCLUSION: Differentiation between infectious conditions and retinoblastoma can sometimes be difficult, but clues as to the diagnosis can be obtained from careful clinical examination.
Abscess↗
Iris melanoma in ocular melanocytosis.
A pigmented, circumscribed iris lesion thought to be a nevus was noted in the right eye of a 64-year-old man with congenital ocular melanocytosis. Although the patient had excess episcleral and choroidal pigmentation, the iris in the affected eye was blue. Two years later, growth was documented, suggesting that the lesion was malignant. The tumor was resected by a sector iridectomy and studied by light microscopy. Histopathologically, the tumor was shown to be a malignant melanoma of the mixed-cell type. No evidence of congenital melanocytosis was found in the adjoining normal iris. This is a well-documented case of a malignant melanoma of the iris arising in a blue, uninvolved iris in a patient with sectoral congenital ocular melanocytosis. Although this specific association has not been previously reported, we believe that any pigmented uveal tumor occurring in the setting of congenital ocular melanocytosis should be observed closely for growth that can signal malignant transformation.
Choroid Neoplasms↗
Observations on seven cases of intraocular leiomyoma. The 1993 Byron Demorest Lecture.
A review of seven cases of intraocular leiomyoma personally managed by the authors disclosed clinical and histopathologic characteristics that serve to differentiate this uncommon tumor from uveal melanoma. Leiomyoma generally occurs in younger patients and has a definite predilection for females. It tends to affect the ciliary body and peripheral choroid rather than the posterior choroid. In contrast to melanoma, which is located in the uveal stroma, leiomyoma usually is located in the supraciliary or suprachoroidal space. During transillumination, leiomyoma usually transmits light readily, whereas most melanomas cast a shadow. If intraocular leiomyoma is suspected clinically, the best management seems to be removal by a modified lamellar sclerouvectomy. In contrast to melanoma, leiomyoma shows positive immunoreactivity for muscle markers and negative immunoreactivity for melanoma-specific antigen and neural markers.
Adult↗
Adenocarcinoma of retinal pigment epithelium arising from a juxtapapillary histoplasmosis scar.
A 66-year-old woman underwent a transocular fine-needle aspiration biopsy for an enlarging mass arising from a juxtapapillary histoplasmosis scar in the left eye. The cytologic study revealed pigmented malignant cells, suggesting that the lesion was either a malignant choroidal melanoma or an adenocarcinoma of the retinal pigment epithelium. The eye was enucleated and histopathologic studies revealed findings suggestive of an adenocarcinoma of the retinal pigment epithelium. Immunohistochemical stains were positive for cytokeratin and were negative for melanoma-specific antigen, supporting an epithelial origin of the neoplasm. It is possible that the tumor developed as a neoplastic transformation of a reactive proliferation of the retinal pigment epithelium in a histoplasmosis scar. The possible association between a chorioretinal scar and the development of a tumor of the pigment epithelium is discussed herein.
Adenocarcinoma↗
Fresh-frozen cucumber as a mount for conjunctival and corneal tissue in cryomicrotomy.
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Carcinoma in Situ↗
Melanotic schwannoma of the choroid. Immunohistochemistry and electron microscopic observations.
PURPOSE: The purpose of this article is to describe what is probably the first reported case of a melanotic schwannoma of the choroid and to discuss the clinical and histopathologic features that serve to differentiate this rare intraocular tumor from a malignant melanoma of the choroid. PATIENT AND METHODS: A 21-year-old woman underwent enucleation of the right eye for a slowly enlarging pigmented choroidal mass that was associated with ipsilateral blindness. The tumor was studied by light microscopy, immunohistochemistry, and electron microscopy. FINDINGS: The tumor was composed of fascicles and whorls of pigmented and nonpigmented plump spindle cells that had light microscopic features of a schwannoma. The tumor cells showed immunoreactivity to S-100 protein and HMB-45. Electron microscopy showed cytoplasmic processes surrounded by a continuous basement membrane and abundant extracellular collagen. The pigmented spindle-shaped cells contained melanosomes in different stages of melanogenesis. The findings were compatible with a melanotic schwannoma. CONCLUSION: Melanotic schwannoma has been reported in soft tissues, peripheral nerves, and in the orbit. To the authors' knowledge, this is the first report of a melanotic schwannoma of the choroid. This extremely rare pigmented choroidal tumor may be very difficult to differentiate clinically from choroidal melanoma.
Adult↗
Squamous cell carcinoma of the eyelid.
A total of 24 patients (12 men and 12 women) with squamous cell carcinoma of the eyelid were identified from pathology records at Wills Eye Hospital from 1978 through 1987. Squamous cell carcinoma accounted for 24 of 648 (3.7%) malignant eyelid lesions submitted during the 10-year study period. The median age of patients at the time of diagnosis was 72 years (range, 55 to 96 years). Initial therapy of all tumors involved surgical excision. Four patients developed clinical recurrence of squamous cell carcinoma in a median of 7.5 months after surgery. There were no tumor recurrences when frozen section techniques were applied during the initial surgical excision. The data suggest that the risk of persistence or recurrence of tumor was increased for patients who delayed seeking medical care after the lesion was first noticed and when frozen section control was not used during surgery. None of the patients were known to have developed metastases, and there were no tumor-related deaths.
Aged↗
Mucosa specific lymphocytes in the human conjunctiva, corneoscleral limbus and lacrimal gland.
Conjunctiva associated lymphoid tissue shows several similarities to mucosa associated lymphoid tissue of the gut and respiratory tract. These similarities have been described in relation to lymphocyte subpopulations and epithelial cell morphology. However, unlike the lymphoid tissue of the gut and respiratory tract, mucosa specific lymphocytes have not been described in the ocular mucosa. In this study we demonstrated the presence of mucosa specific lymphocytes bearing the Human Mucosal Lymphocyte-1 antigen (beta 7 integrin), in the human conjunctiva, limbus and lacrimal gland. The distribution of this subset of lymphocytes corresponded to the distribution of CD8+ T-cells and was found maximally in the epithelium of the epibulbar conjunctiva and in the lacrimal gland. The Human mucosal lymphocyte antigen may function to determine mucosal homing of this particular subset of CD8+ T-cells, which in turn, may have special function in immunological defense and tolerance mechanisms occurring at mucosal surfaces.
Adolescent↗