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Biomedical subjects

R C Eagle

Publications and source records attributed to R C Eagle.

At least 109 records · Page 6Linked to original sources

Non-African Burkitt lymphoma presenting with oral thrush and an orbital mass in a child.

PURPOSE: To report an unusual case of orbital involvement with non-African Burkitt lymphoma in a child. METHODS: A 26-month-old boy developed oral thrush and painless right proptosis with eyelid swelling. Magnetic resonance imaging disclosed a diffuse mass involving the inferior, lateral, and superior right orbit and both maxillary sinuses. Incisional biopsy of the orbital mass was performed. RESULTS: Histopathology disclosed diffuse infiltration of the orbital tissues by a high-grade lymphoid neoplasm that showed positive immunoreactivity for B-cell markers. Immunoglobulin gene rearrangement studies disclosed a characteristic t(8;14) translocation consistent with a small, noncleaved malignant lymphoma of the Burkitt type. CONCLUSION: Non-African Burkitt lymphoma may manifest with orbital involvement.

Antineoplastic Combined Chemotherapy Protocols↗

Ocular manifestations of the organoid nevus syndrome.

BACKGROUND: The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. Based on ophthalmoscopy and computed tomography (CT), a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some authors or a choroidal osteoma by others. This study was undertaken to review the authors' personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. METHODS: The authors reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the authors. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. RESULTS: The authors identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area and two had seizures and arachnoid cysts. All five patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the four patients with clear ocular media, was a flat, yellow discoloration of the posterior fundus, of variable size and shape, that appeared to correlate with a dense plaque noted on ultrasonography and CT. In one case, histopathologic studies showed that this posterior lesion contained intrascleral cartilage. CONCLUSIONS: The authors' observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Just like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal, yellow discoloration in the fundus, probably related to intrascleral cartilage.

Adolescent↗

Effect of topical anti-transforming growth factor-beta on corneal stromal haze after photorefractive keratectomy in rabbits.

PURPOSE: To determine the relationship between anti-transforming growth factor-beta (anti-TGF-beta) antibodies and the amount of corneal stromal haze after excimer laser photorefractive keratectomy (PRK). SETTING: Wills Eye Hospital, Philadelphia, Pennsylvania, USA. METHODS: Nineteen rabbits had bilateral PRK. Dichlorotriazinyl fluorescein was used to stain the exposed stroma; all rabbits were then treated with antibiotic ointment for 4 days. Ten rabbits were randomized to treatment with topical anti-TGF-beta1, -beta2, and -beta3 antibody 50 microg three times a day for 4 days; the others received diluent three times a day for 4 days. Stromal haze was graded weekly for 8 weeks on a 0 to 4+ scale. At the end of the study, all corneas were examined histopathologically. RESULTS: All treated eyes developed appreciable haze. Seven control rabbits and one antibody-treated rabbit had an epithelial erosion (P = .00001). Antibody-treated rabbits had significantly less haze at 3, 4, and 5 weeks (right eyes) and 3, 4, 5, 7 and 8 weeks (left eyes) (P < .05). Histopathology and fluorescence microscopy showed subepithelial collagen deposition consistent with clinical haze. CONCLUSIONS: Topical anti-TGF-beta antibody reduced stromal haze after PRK in the rabbit model and may be clinically beneficial in humans.

Administration, Topical↗

Chewing-induced visual impairment from a dumbbell dermoid cyst.

A 29-year-old woman presented with a 1-year history of blurred vision in her left eye while chewing. Examination revealed a rubbery mass with ill-defined borders in the superotemporal brow region. Imaging studies demonstrated a cystic lesion extending from the temporal fossa into the orbit through a bony defect in the lateral orbital wall, assuming a dumbbell configuration. Histopathology of the excised specimen confirmed the clinical diagnosis of a dermoid cyst of cutaneous origin. We believe that the patient's visual disturbance was caused by transient displacement of orbital contents by the dermoid cyst as it was compressed into the orbit during mastication. Dermoid cysts are frequently associated with bony defects, which may require preoperative imaging and may produce unexpected symptoms.

Adult↗

Iris juvenile xanthogranuloma studied by immunohistochemistry and flow cytometry.

An unusual large tan iris mass in a 19-month-old child was removed by iridocyclectomy and studied by light microscopy, immunohistochemistry, and flow cytometry. The excised mass consisted of granulomatous inflammation with numerous osteoclast-like giant cells and scattered atypical Touton giant cells. Immunohistochemistry studies showed that the cells were most consistent with mononuclear histiocytes. Flow cytometry showed that 90% of the cells sampled were T-lymphocytes, with a predominance of T-suppresser cytotoxic cells. Juvenile xanthogranuloma (XG) of the iris can occur as a large solitary mass, without signs of intraocular inflammation or hyphema.

Biomarkers↗

Orbital dermoid cysts: clinicopathologic correlations, classification, and management. The 1997 Josephine E. Schueler Lecture.

The dermoid cyst is one of the most common space-occupying orbital lesions, but there has been no large series that details the clinical, pathologic, and therapeutic aspects of this condition. Furthermore, despite the various presentations and types of dermoid cysts, there is no clinically applicable classification of these lesions. We performed a retrospective histopathologic study of 197 consecutive orbital and periorbital dermoid cysts excised at Wills Eye Hospital during a 32-year period, correlated the histopathologic findings with the recorded clinical and radiographic observations, and devised a clinically applicable classification of orbital dermoid cysts. The mean age at excision was 17 years (range, 5 months to 85 years), and the most frequent clinical finding was a painless subcutaneous mass, present in 89% of cases. In 72% of patients, the mass was located in the superotemporal orbital region and produced no abnormalities of vision or intraocular pressure. Computed tomography and magnetic resonance imaging, performed mainly in the more recent cases, disclosed a characteristic round to ovoid cystic lesion with a well-defined lining. Histopathologically, 165 of the cysts (84%) were lined by keratinizing, stratified squamous epithelium. Ten (5%) were lined by nonkeratinizing epithelium resembling conjunctival epithelium. Hair shafts were present in the wall or lumen of the cyst in 195 (99%) of specimens, sebaceous glands in 148 (75%), and sweat glands in 39 (20%); inflammation was present in 75 cases (38%). Based on these personal observations and a review of the literature, a classification of orbital dermoid cysts is proposed that we believe will provide guidance for the ophthalmologist in the management of these lesions.

Adolescent↗

Malignant melanoma of the optic nerve.

A 67-year-old man was diagnosed as having a melanocytoma of the optic disc in the left eye. Observation during a 5-year period showed no change in the lesion. At age 72 years, he had abrupt visual loss to no light perception in the affected left eye. Clinical examination disclosed little enlargement of the papillary tumor but ultrasonographic evidence of optic nerve infiltration. Precontrast magnetic resonance imaging studies disclosed a hyperintense infiltrative lesion in the enlarged left optic nerve. Enhancement features of the lesion excluded a hemorrhagic process. The eye was removed with a 22.5-mm segment of optic nerve. Histopathologic examination showed a large, necrotic, mixed-cell malignant melanoma confined to the optic nerve. No choroidal involvement or viable melanocytoma cells were documented. This case stresses that it may be difficult to differentiate a melanocytoma from a primary malignant melanoma of the optic nerve.

Aged↗

Ophthalmic features of the organoid nevus syndrome.

BACKGROUND/PURPOSE: The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. On the basis of ophthalmoscopic and computed tomographic studies, a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some investigators and a choroidal osteoma by others. This study was undertaken to review our personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. METHODS: We reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the investigators. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. RESULTS: We identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area, and 2 had seizures and arachnoid cysts. All 5 patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the 4 patients with clear ocular media, was a flat yellow discoloration of the posterior fundus, of variable size and shape, which appeared to correlate with a dense plaque noted on ultrasonography and computed tomography. In 1 case, histopathologic examination showed that this posterior lesion contained intrascleral cartilage. CONCLUSIONS: Our observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal yellow discoloration in the fundus, probably related to intrascleral cartilage.

Adolescent↗

Branch retinal artery occlusion as the initial sign of giant cell arteritis.

PURPOSE: To describe a patient whose initial sign of giant cell arteritis was a branch retinal artery occlusion. METHODS: We examined a 77-year-old woman who developed arteritic anterior ischemic optic neuropathy three weeks after an isolated non-embolic branch retinal artery occlusion occurred in the same eye. RESULTS: The diagnosis of giant cell arteritis was confirmed by temporal artery biopsy after the patient was treated with intravenous corticosteroids. CONCLUSIONS: Although a rare cause of branch retinal vascular occlusion, giant cell arteritis should be considered in the differential diagnosis of a non-embolic branch retinal artery occlusion in elderly patients.

Aged↗

Acquired neoplasms of the nonpigmented ciliary epithelium (adenoma and adenocarcinoma).

BACKGROUND/PURPOSE: Acquired neoplasms of the nonpigmented ciliary body epithelium (NPCE) are rare, and most information about them has come from single case reports. This study was undertaken to review the authors' experience with a series of patients with acquired neoplasms of the NPCE, to delineate the clinical and histopathologic features of these tumors, and show how they differ from ciliary body melanoma. METHODS: A clinicopathologic review was conducted on acquired tumors of the NPCE that were evaluated by the authors and a review of the English language literature was done. The data from the authors' cases were compared with previously reported cases. RESULTS: The authors had personal experience with nine patients with acquired tumors of the NPCE and found 18 other patients with these tumors in the literature. Of the authors' patients, all tumors were predominantly nonpigmented and were white to light-tan in color. Associated clinical findings included signs of intraocular inflammation in all patients, secondary cataract in eight (89%), and subluxation of the lens in six (67%). Eight of the tumors were managed successfully by local resection and one by enucleation. Histopathologically, the tumors showed considerable variation from patient to patient. Seven tumors were classified as benign adenoma and two as low-grade adenocarcinoma. There was no local recurrence or systemic metastases. Although tumors of the NPCE historically have been misdiagnosed clinically as ciliary body melanoma, our study suggests that they have some characteristic features that severe to differentiate them from melanoma and other ciliary body lesions. In contrast to melanoma, acquired neoplasms of the NPCE are amelanotic and are more likely to have an irregular surface, associated inflammatory signs, to transmit light well during transillumination, and show high internal reflectivity with ultrasonography. CONCLUSION: Acquired neoplasms of the NPCE have characteristic clinical and histopathologic features that should suggest the diagnosis. Due to their anterior location in the ciliary body, local resection (rather than enucleation) is usually the treatment of choice. The visual prognosis is fair, and the systemic prognosis is excellent.

Adenocarcinoma↗

Congenital neoplasms of the nonpigmented ciliary epithelium (medulloepithelioma).

BACKGROUND: Most reports on intraocular medulloepithelioma have been single case descriptions. The authors reviewed ten patients with intraocular medulloepithelioma, discuss some little known clinical features of this entity, and make diagnostic and therapeutic recommendations. PATIENTS AND METHODS: The records of the authors' patients with histopathologically confirmed medulloepithelioma were reviewed, and the clinical features, diagnostic problems, management, histopathology, and prognosis were assessed. RESULTS: Of the ten patients, nine were children, ranging in age from 2 months to 10 years, and one patient was 58 years of age at the time of clinical presentation. In seven patients, there was a delay in diagnosis, ranging from 3 to 28 months, and four patients underwent surgery for cataract, glaucoma, or other secondary conditions while the tumor was unsuspected. All patients had a nonpigmented ciliary body mass and a notched or subluxated lens, and six had neovascular glaucoma and evident cysts in the mass. The authors' initial management was enucleation in four patients and local resection in six. Of the six patients managed by local resection, five eventually required enucleation, four because of local tumor recurrence and one because of ocular inflammation and discomfort. Pathologically, nine tumors were classified as malignant (5 teratoid, 4 nonteratoid) and one as benign. In one patient (the adult), metastasis to the parotid gland developed. CONCLUSION: Intraocular medulloepithelioma generally occurs in the first decade of life as a nonpigmented ciliary body mass. It has a tendency to cause secondary neovascular glaucoma, a characteristic lens notch and subluxation, and a neoplastic cyclitic membrane. Enucleation is the best treatment for advanced cases. For smaller circumscribed lesions, local resection may be attempted, but such treatment is followed frequently by local recurrence. Although most medulloepitheliomas are cytologically malignant, distant metastasis is uncommon.

Cataract Extraction↗

Complete ring melanoma of the choroid.

BACKGROUND: Ring melanoma of the uvea is a term generally applied to elongated, circumferentially oriented malignant melanomas of the iris and ciliary body; a complete ring melanoma involving almost exclusively the choroid is extremely uncommon. METHODS: The authors present a case report and clinicopathologic correlation of complete ring melanoma of choroid managed by enucleation. RESULTS: A 74-year-old woman was found to have a diffuse, melanotic thickening of the midzonal to peripheral choroid circumferentially for 360 degrees and an associated subtotal serous retinal detachment with shifting subretinal fluid in her left eye. The thickest portion of the mass measured only 2.3 mm by ultrasound biometry. The eye was enucleated, and histopathologic analysis confirmed a diffuse malignant melanoma composed predominantly of spindle B cells. CONCLUSIONS: A complete ring melanoma of the choroid is a rare and unusual variant of diffuse uveal malignant melanoma. The pertinent differential diagnosis of choroidal ring melanoma includes diffuse choroidal melanocytoma, choroidal melanocytosis associated with ocular melanocytosis, bilateral diffuse uveal melanocytic proliferation associated with systemic carcinoma, and diffuse uveal lymphocytic infiltration.

Aged↗

Recurrent keratopathy after penetrating keratoplasty for aniridia.

Corneal lesions in aniridia include peripheral pannus and epithelial abnormalities that may advance centrally, resulting in the need for penetrating keratoplasty. Three patients with aniridia who underwent repeated corneal surgical procedures (one keratectomy and six penetrating keratoplasties) are described herein. A clinicopathological correlation was performed. In all three patients there was evidence of recurrent pannus and epitheliopathy on histopathological examination, which correlates with the clinical findings. These findings suggest that the pathophysiology of recurrent aniridia keratopathy may be related to a primary abnormality in the stem cells of the recipient corneal limbus. Aniridic pannus and epitheliopathy recur in grafts after penetrating keratoplasty and threaten the transplanted cornea. The risk of recurrent keratopathy should be considered when recommending a surgical procedure to patients with aniridia.

Adolescent↗