Debra J. Shetlar

Combined hamartoma of sensory retina and retinal pigment epithelium.

We report two cases of combined hamartoma of the sensory retina and retinal pigment epithelium (CHR-RPE) in which apparent growth of the lesion was observed. In case 1, the eye was enucleated with a presumed diagnosis of juxtapapillary malignant melanoma. Histopathologically, the enucleated globe showed an elevated peripapillary mass containing disorganized retinal tissue intermixed with vascular and glial elements as well as tubules of proliferating retinal pigment epithelium. We have summarized the clinical features of 53 patients with CHR-RPE reported between 1952 and 1988 excluding the cases compiled by the Macular Society Collaborative Study. While the latter study found an equal sex predilection among their cases, we found a 70% male preponderance among the 53 patients. Of the 54 lesions observed in 53 patients, 76% were juxtapapillary, 17% were macular, and 7% were peripheral. Furthermore, periodic follow-up examination disclosed apparent enlargement of the mass in six patients, five of whom underwent enucleation of the globe for suspected melanoma.

Bovine pericardium versus homologous sclera as a wrapping for hydroxyapatite orbital implants.

Purpose To report our experience with bovine pericardium as a wrapping material for hydroxyapatite orbital implants after enucleation and to compare the exposure rates of the implants wrapped with bovine pericardium versus donor sclera. Methods We retrospectively reviewed the records of all patients who received bovine pericardium–wrapped or donor sclera–wrapped hydroxyapatite implant after primary enucleation between March 1995 and December 2001. Results Of the 26 patients who received donor sclera–wrapped implants after enucleation, 1 (3.8%) had implant exposure. Of the 26 patients who received bovine pericardium–wrapped implants after enucleation, 6 (23%) had implant exposure. The incidence of implant exposure with the use bovine pericardium wrapping material was found to be significantly higher than with sclera (P =0.05). Six of the 7 implant exposures were noted in the first 6 months after placement of the orbital implant. Five of the 6 exposed bovine pericardium–wrapped hydroxyapatite implants were associated with socket infection. The case of exposure of the sclera-wrapped implant was treated conservatively by observation. Six patients who had exposure of bovine pericardium–wrapped implants required multiple repairs because of recurrent exposures. Four of these patients eventually required removal of the implant. Conclusions Despite the advantages of using bovine pericardium as a wrapping material for hydroxyapatite orbital implants, we observed a significantly higher incidence of exposure with bovine pericardium compared with donor sclera in the early postoperative period. Use of bovine pericardium as a wrapping material for orbital implants should be avoided unless some future modifications of the technique can be developed to prevent such complications.

A clinicopathologic study of three carcinoid tumors metastatic to the orbit : immunohistochemical, ultrastructural, and DNA flow cytometric studies

Three patients with histopathologically proven orbital metastatic lesions from a carcinoid tumor are reported. Computed tomography (CT) disclosed a well-circumscribed orbital mass in two of three cases. Each tumor was studied by both light and electron microscopy as well as immunohistochemistry and DNA flow cytometry. A review of the literature discloses the relatively rare occurrence of carcinoid tumors metastasizing to the eye and orbit. With the exception of one case, the reported metastatic carcinoid tumors to the uvea all developed from primary bronchial carcinoids. In contrast, the vast majority of the reported orbital metastases arose from ileal carcinoids. Immunohistochemical markers such as chromogranin A, synaptophysin, and serotonin have proven to be useful diagnostic tools in evaluating carcinoid tumors. Furthermore, DNA flow cytometry may be of prognostic value in patients with metastatic carcinoid tumor.

Corneoscleral necrosis after episcleral Au-198 brachytherapy of uveal melanoma.

Purpose: To describe the risk factors for the development of corneoscleral necrosis (CSN) and its management in patients with primary choroidal or ciliochoroidal melanoma who underwent episcleral Au-198 brachytherapy. Methods: Clinical records of patients with symptoms of dry eye, foreign body sensation, pain, and evidence of CSN after Au-198 brachytherapy for uveal melanoma treated over a 22-year period were reviewed retrospectively. Risk factors for the development of CSN were identified and various methods of management were evaluated. The data were analyzed using multivariant analysis. A P < 0.05 was taken as a level of statistical significance. Results: Of the 202 eyes of 202 patients treated with Au-198 radioactive plaque, 15 (7.4%) patients with symptomatic complaints of dry eye and pain showed evidence of CSN. First signs were noted as early as 1 month to as late as 5 years (median time 5 months) after the treatment. Risk factors for the development of CSN included tumor thickness greater than 6 mm and ciliary body involvement (P < 0.05). Associated risk factor included intraocular pressure greater than 21 mmHg. Four patients required conservative management, 11 patients required scleral patch and/or conjunctival flaps, and 6 eyes eventually required enucleation. Eyes which developed CSN were more likely to undergo enucleation compared with eyes having no evidence of CSN (P < 0.05). None of the eyes with CSN, which required enucleation because of the failed treatment, had histopathologic evidence of recurrent tumor or tumor invading sclera. Conclusion: Corneoscleral necrosis may occur soon or several years after Au-198 brachytherapy for uveal melanoma. Risk factors for CSN include tumor thickness greater than 6 mm, ciliary body involvement, and intraocular pressure >21 mmHg. Closer follow-up, early recognition, and timely intervention may avert serious consequences.

A unique case of foreign-body associated orbital myositis.

A 50-year-old woman presented with a 2-week history of diplopia and right-sided orbital pain with eye movement. Examination revealed an edematous, ptotic right upper eyelid with conjunctival hyperemia, proptosis, and significant limitation to upward and downward ductions on the right. MRI was significant for a homogeneously enhancing lesion within the superior rectus muscle. A laboratory evaluation seeking an infectious, inflammatory, or autoimmune process was nonrevealing. A diagnosis of orbital myositis was made, and the patient experienced significant improvement with oral corticosteroids. The patients symptoms, however, recurred after attempts at a slow taper of the corticosteroids. An orbital biopsy of the lesion revealed fibroadipose tissue containing irregularly shaped yellow-white deposits birefringent under polarized light, suggestive of silica crystals. The patient denied any history of trauma or prior surgery. An intraorbital triamcinolone injection to the superior orbit allowed resolution of symptoms and a successful taper off systemic corticosteroids.

Primary Neuroendocrine Tumor of the Orbit Presenting With Acute Proptosis

Primary neuroendocrine tumors of the orbit are exceedingly rare and typically present with gradual, progressive exophthalmos. In this report, an otherwise healthy 64-year-old woman undergoes resection of a well-differentiated neuroendocrine tumor after presenting with acute proptosis. An extensive clinical and radiographic evaluation reveals no other evidence of disease, establishing the diagnosis of a primary neuroendocrine tumor. The case presentation is followed by a brief review of the classification, presentation, and evaluation of orbital neuroendocrine tumors.

Small cell neuroendocrine carcinoma of the eyelid.

This is a case presentation of a rare primary small cell neuroendocrine carcinoma of the eyelid. This tumor was diagnosed and differentiated from Merkel cell carcinoma using histopathology, immunohistochemistry, and electron microscopy.