Primary Canalicular MALT Lymphoma Masquerading as Chronic Canaliculitis.

A 67-year-old female presented with chronic canaliculitis and underwent canalicular marsupialization. During the procedure, a fleshy mass was found in the canaliculus, which was excised completely and sent to pathology. Histology confirmed the diagnosis of extranodal marginal zone mucosa-associated lymphoid tissue lymphoma. The patient underwent staging with positron emission tomography/CT scan, which did not show any hypermetabolic foci elsewhere in the body, so the patient elected to undergo close observation without further treatment. At 12 months of follow-up, the patient has remained disease-free.

Orbital Dermoid Cysts in Monozygotic Twins.

Two healthy 12-year-old monozygotic twin sisters presented with strikingly similar, painless orbital masses along their frontozygomatic suture line that had been slowly enlarging since birth. The masses were clinically consistent with orbital dermoid cysts and the patients underwent excision of their lesions, with the diagnosis confirmed by histological analysis. There are prior case reports of both nasal and ovarian dermoid cysts in twins, however, no prior case of orbital dermoid cysts in twins have been described. These dermoid cysts are generally thought to be a sporadic disorder of embryogenesis, yet the authors' case suggests genetics may play a role in the underlying etiology of dermoid cysts.

Rheumatoid Arthritis-Associated Orbital Vasculitis.

A patient with a history of rheumatoid arthritis presented with bilateral orbital lesions while on abatacept. Biopsy of the lesions yielded pathologic assessment showing vasculitis with a mixed inflammatory infiltrate notable for numerous eosinophils. Despite initial improvement on cyclophosphamide therapy, she developed progression of the orbital lesions and lesions elsewhere on her body. All serologic studies were normal except for increased levels of rheumatoid factor and eosinophilia. The patient underwent a second biopsy, which was consistent with her initial pathology. The patient was started on high-dose mycophenolate mofetil treatment with a good response. After 1 year of treatment, the patient's lesions have nearly completely resolved. This patient illustrates the diagnostic and therapeutic challenges encountered when treating orbital vasculitis. Furthermore, this case shows the importance of not categorizing vasculitis with unclear etiology under the umbrella diagnosis of atypical granulomatosis with polyangiitis. This patient did not exhibit the serologic or pathologic characteristics of granulomatosis with polyangiitis, and she did not respond to treatment as would be expected for granulomatosis with polyangiitis. Maintaining an open approach to diagnosis and treatment was crucial in obtaining a favorable outcome for this patient.

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.

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 patient's 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.

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.

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.

Pulmonary and ophthalmic involvement with Erdheim-Chester disease: a case report and review of the literature.

Erdheim-Chester disease is a rare nonfamilial histiocytic disorder of unknown etiology with characteristic long bone findings. The 3-year survival rate for patients with Erdheim-Chester disease is 50%. Approximately 50% of patients have disease involvement in other tissues, including skin, retro-orbital and periorbital tissues, pituitary-hypothalamic axis, heart, kidney, retroperitoneum, breast, skeletal muscle, and sinonasal mucosa; about 20% of patients have lung involvement. Prognosis generally depends on the extent of the extraosseous disease. For patients with lung involvement, gender distribution is equal, but men typically present at an older age than do women. Approximately 80% of patients present with dyspnea, and most patients have diffuse interstitial infiltrates and pleural and/or interlobar septal thickening on chest radiology. Characteristic lung histopathology includes the accumulation of histiocytes with variable amounts of fibrosis and a variable lymphoplasmacytic infiltrate in a lymphangitic distribution. Immunostains are diagnostically useful, showing immunopositivity for CD68 and factor XIIIa and immunonegativity for CD1a. Birbeck granules are uniformly absent ultrastructurally.

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.