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Fibrous histiocytoma of the conjunctiva.

PURPOSE: To review the clinical features and course of six patients with fibrous histiocytoma (FH) of the conjunctiva. DESIGN: Retrospective, observational clinical case series. METHODS: Chart review of six consecutive patients with unilateral cases of conjunctival FH was conducted. Clinical presentation, treatment, histopathologic condition, and follow-up information were recorded. RESULTS: The mean patient age was 37 years (median, 38 years; range, 12 to 72 years). There were five white patients, one black patient, five male patients, and one female patient. The tumor was present for a mean of three months (median, five months; range, one to 12 months) and was unilateral (one right eye, five left eyes). In all cases, the tumor was a tan, dome-shaped limbal mass in the conjunctival stroma with visible intrinsic vessels. The mean tumor basal dimension was 7 mm (median, 5 mm; range, 4 to 11 mm). Surgical resection was performed in all cases, and histopathologic study demonstrated benign FH in four cases and malignant FH in two cases. Those cases with benign FH showed no recurrence over nine months (median, eight months; range, three to 18 months). One patient with malignant FH showed recurrence and required repeat excision. The other patient with malignant FH was treated with plaque radiotherapy to maintain control. There was no evidence of orbital invasion or remote metastasis in any case over the mean follow-up period of 21 months (median, 10 months; range, three to 80 months). CONCLUSION: FH is a rare conjunctival tumor that can show benign or malignant features. Complete resection is advised. Malignant FH can demonstrate recurrence that necessitates wide resection and radiotherapy.

Adolescent↗

Rituximab in primary conjunctiva lymphoma.

Our experience with a patient with a primary conjunctiva lymphoma who was treated with conventional dose of rituximab obtaining a complete response without acute and late local toxicity for the eye.

Adult↗

Osseous choristoma of the conjunctiva simulating extraocular extension of retinoblastoma.

PURPOSE: To report the clinicopathologic features of an epibulbar osseous choristoma simulating extraocular extension of retinoblastoma. DESIGN: Interventional case report. METHODS: A 7-month-old male presented with a unilateral intraocular mass localized to the temporal retina and a white pedunculated lesion extending from the conjunctiva, anterior to the insertion of the lateral rectus muscle. Clinical examination and computed tomography scan findings led to the diagnosis of retinoblastoma with extraocular extension. The child underwent excision of the epibulbar lesion and enucleation of the globe. RESULTS: The histopathologic examination of the epibulbar lesion revealed an osseous choristoma. The intraocular contents displayed features typical of retinoblastoma without extraocular extension. CONCLUSION: An epibulbar osseous choristoma can simulate extraocular extension of retinoblastoma in an eye harboring the intraocular malignancy.

Bone and Bones↗

Bacillary angiomatosis of the anterior orbit, eyelid, and conjunctiva.

PURPOSE: To report a case of bacillary angiomatosis of the lower eyelid, conjunctiva, and anterior orbit. DESIGN: Interventional case report. METHODS: A 76-year-old immunocompromised male patient developed a firm vascularized nodule in his left lower eyelid and anterior orbit. RESULTS: An excisional biopsy was performed. Histopathologic examination revealed an abnormal vascular proliferation and a mixed inflammatory infiltrate. A Warthin-Starry stain showed numerous bacilli. These findings are characteristic of bacillary angiomatosis. A serologic specimen was positive for antibodies to Bartonella Quintana. CONCLUSION: The lower lid and anterior orbit are rare locations for bacillary angiomatosis. Our case brings to attention the increasing importance of Bartonella infection as a causative agent of ophthalmic diseases.

Aged↗

HMB-45 antibody reactivity in pigmented lesions of the conjunctiva.

Conjunctival pigmented lesions, including ten compound nevi, three subepithelial nevi, two acquired melanoses, and six melanomas, were examined histologically and immunohistochemically to determine the specificity of mouse monoclonal HMB-45 antibody for these lesions. Eleven of 13 nevi, two of two acquired melanoses, and six of six melanomas stained with this antibody. Conjunctival melanomas showed intense and diffuse cytoplasmic staining; compound nevi and subepithelial nevi showed less intense but diffuse reaction. There was strong staining in melanocytic cells at the junction of the epithelium and substantia propria in compound nevi and acquired melanoses. Unlike skin nevi, conjunctival nevi show HMB-45 reactivity in their stromal components. Immunoreactivity to HMB-45 does not distinguish benign from atypical or malignant melanocytic lesions of the conjunctiva.

Adolescent↗

Bacillary angiomatosis of the conjunctiva.

A 70-year-old man had unilateral congestion of the right upper eyelid, which contained a nodular mass. A biopsy was performed, and histologic, immunocytochemical, and ultrastructural studies disclosed a pseudoneoplastic proliferation of endothelial cells and pericytes in a region containing clumps of bacteria. This combination of histologic features is characteristic of bacillary angiomatosis, which has been described in the skin, particularly in association with immunodeficient states, especially acquired immunodeficiency syndrome, but not in the conjunctiva. A second biopsy contained a diffuse polyclonal lymphocytic infiltrate in which large lymphocytes with irregular nuclei and mitotic figures were prominent. Systemic examination disclosed mild splenomegaly and a benign paraproteinemia. Treatment with topical gentamicin and systemic erythromycin brought about a complete resolution of the symptoms and signs within eight weeks, and there has been no sign of recurrence for the past two years.

Aged↗

Fractionated epibulbar I-125 plaque radiotherapy for recurrent mucoepidermoid carcinoma of the bulbar conjunctiva.

PURPOSE/METHODS: A patient with recurrent conjunctival mucoepidermoid carcinoma was treated successfully with surgical excision of the tumor and fractionated epibulbar I-125 plaque radiotherapy. RESULTS/CONCLUSIONS: Local excision with fractionated epibulbar plaque radiotherapy is an effective means of eradication in select cases of recurrent mucoepidermoid carcinomas of the conjunctiva. This treatment may avoid more aggressive surgical treatment and preserve vision.

Aged↗

Intraocular invasion by papillary squamous cell carcinoma of the conjunctiva.

Two patients with incompletely excised invasive papillary squamous cell carcinoma of the corneoscleral limbal conjunctiva had subtle clinical signs suggesting incomplete transmural extension or localized intraocular tumor invasion. We attempted a wide en bloc resection procedure in each case. Histopathologic examination disclosed that the surgical margins were involved with tumor cells and the eyes were subsequently enucleated. The enucleated eyes disclosed more extensive microscopic intraocular involvement than had been anticipated.

Adult↗

Mitomycin C trabeculectomy in eyes with cicatricial conjunctiva.

PURPOSE: To demonstrate favorable outcome of mitomycin C-augmented trabeculectomy in eyes with broad cicatricial conjunctiva created by previous surgeries. METHODS: Forty-six eyes (40 patients) with extensive conjunctival scarring that had undergone mitomycin C trabeculectomy were reviewed retrospectively. RESULTS: After a mean follow-up +/- SD of 13.7 +/- 7.8 months (range, 6 to 36 months), intraocular pressure was well controlled, below or equal to 16 mm Hg and 21 mm Hg, respectively, in 33 (72%) and 44 (96%) of the 46 eyes. In all eyes, a functional filtering bleb was present during the follow-up periods. CONCLUSION: Mitomycin C trabeculectomy after dissection of conjunctival scar tissue may be useful for treating refractory glaucoma.

Adult↗

Phenotypic characterization of T cells infiltrating the conjunctiva in chronic allergic eye disease.

BACKGROUND: Chronic allergic conjunctivitis comprises a spectrum of diseases including atopic keratoconjunctivitis, atopic blepharoconjunctivitis, vernal keratoconjunctivitis, and contact lens-associated giant pupillary conjunctivitis. Each condition is characterized by a complex immunopathology with a mixed cellular infiltrate. Treatment with conventional mast cell stabilizers is often unsatisfactory, and therapy depends heavily on topical corticosteroids. OBJECTIVE: Recent evidence suggests that T lymphocytes play an important role in mediating the immunopathology seen in the chronic allergic response in the skin and the lungs. METHODS: We have therefore investigated the prevalence of T cells and their subsets in the different chronic allergic eye conditions by means of immunohistochemistry. RESULTS: We found significantly increased numbers of CD4+, CD45RO+, and HLA-DR+ T cells in the conjunctiva of patients with atopic and vernal keratoconjunctivitis and giant papillary conjunctivitis, with a corresponding upregulation of markers present on antigen presenting cells. CONCLUSIONS: These findings suggest that like allergic conditions in the skin and lungs, CD4+ memory T cells are involved in the regulation of the immunopathology of chronic allergic eye responses. Further characterization of these T cells will provide the information necessary for future immunotherapeutic interventions.

Adolescent↗

Malignant melanoma of the eyelid and palpebral conjunctiva treated with iodine-125 brachytherapy.

OBJECTIVE: To evaluate the results on malignant melanomas of the eyelid and palpebral conjunctiva treated with iodine-125 (125I) brachytherapy with shielding of the eye. DESIGN: Noncomparative case series. PARTICIPANTS: Fourteen patients treated with 125I brachytherapy at an ocular oncology referral center from 1974 through 1996, all of whom had had previous debulking, incomplete resection, or recurrence after surgery. INTERVENTION: A stainless steel pericorneal ring eyeshield was attached to the extraocular muscles, over which a lid was fitted to protect the cornea without touching it. Iodine-125 seeds in polythene tubes were inserted into the eyelid and attached to the lid margin for a single plane implant. In five cases additional seeds were glued on to the shield as well for a volume implant. A median dose of 37 Gy (range, 17.3-67.6 Gy) was given over the course of 113 hours (range, 47-190 hours) to the outer surface of target volume. MAIN OUTCOME MEASURES: Local control is regarded as freedom from recurrence in those without measurable disease and complete clinical regression in those with measurable disease. Morbidity is assessed in terms of function and cosmesis. Survival is given from the time of the implant. RESULTS: There was local control in 13 patients maintained from 11 to 227 months (median, 39 months). There were two recurrences at 8 and 13 months in the first patient who received 17.3 Gy. Late complications consisted of mild eyelid telangiectasia, mild eyelid atrophy, and loss of eyelashes in most patients. Five patients, four of whom had upper eyelid tumors, experienced a dry eye, which was managed with tear supplements, and one of these patients developed a cataract. A corneal ulcer developed in one eye, which later perforated after treatment of a subsequent bulbar melanoma and was exenterated. Another eye was enucleated after treatment for a subsequent melanoma in the fornix. Cosmesis was acceptable to patient and doctor in the other 12 patients. Vision was maintained in seven patients, reduced in two, and not recorded in the remaining three patients. Three patients died of hematogenous metastases at 44, 62, and 79 months after implant, one of bronchial carcinoma at 46 months and one of an astrocytoma at 39 months. All patients were clear of local disease. The remainder survived for a median of 45 months (range, 18-227 months). CONCLUSIONS: Iodine-125 brachytherapy can be used as an alternative to wide excision or exenteration of these tumors. There was good local control, reasonable maintenance of vision, and good cosmesis.

Adult↗

An outbreak of trematode-induced granulomas of the conjunctiva.

PURPOSE: To describe the epidemiologic, clinical, and histopathologic features of trematode granulomas of the conjunctiva, eyelid, and anterior chamber in pediatric patients. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Forty-one children from a southern Indian village with conjunctival granulomas. METHODS: The village of Sellananthal was selected for a field visit after analysis of earlier hospital-based allergic conjunctival granuloma cases. Children with ocular diseases were examined, and histories of exposure to assumed risk factors and clinical findings were evaluated. Selected patients were brought to the base hospital for excisional biopsy. Serial sections obtained from the excised nodules were examined for the presence of a parasite. MAIN OUTCOME MEASURES: Histopathologic examination of excised conjunctival lesions or response of lesions to local medical therapy. RESULTS: In this year-long prospective study, 41 children (16 years or younger; 38 boys and 3 girls) with clinical features of allergic conjunctival granulomas were examined. Thirty-four patients were from a single village located in the southern Indian state of Tamil Nadu; the remaining 7 were from various parts of the same state. All children swam in their village's freshwater pond. Twenty patients with nodules less than 5 mm in diameter received medical treatment; 13 with larger nodules underwent surgical excision of the lesions. Nine of these 13 cases revealed a zonal granulomatous inflammation admixed with eosinophilic leukocytes; 4 of these 9 displayed fragments of the tegument and internal structures of a trematode and Splendore-Hoeppli phenomenon. The remaining 4 of the 13 cases revealed nongranulomatous inflammation made up of lymphocytes, histiocytes, and eosinophils. Eight patients refused surgical treatment. CONCLUSIONS: In southern India, one cause of allergic conjunctival granulomas in children seems to be trematode infection. The clustering of cases in a single village and exposure to a village freshwater pond indicate the need for an epidemiologic investigation and study of the parasite's life cycle. Sporadic cases from other parts of the state with similar histories of exposure to their local pond or river water suggest a widespread distribution of the etiologic agent.

Adolescent↗

Sebaceous gland adenoma of the tarsal conjunctiva in a patient with Muir-Torre syndrome.

PURPOSE: To highlight the recognition and diagnosis of Muir-Torre syndrome (MTS) in patients with sebaceous tumors of the eyelid/conjunctiva and to determine the role of immunohistochemical markers as a screening test in patients with Muir-Torre syndrome. DESIGN: Single interventional case report. METHODS: The clinical and family history was reviewed from the records of a 41-year-old man who had a sebaceous gland adenoma of the tarsal conjunctiva of the left upper eyelid. The lesion was completely excised and submitted for histopathologic examination. Immunohistochemical stains using an antibody to MSH2 were performed on the paraffin-embedded sections of the tumor. RESULTS: Histopathologic examination of the tumor showed a lobular pattern with basaloid cells at the periphery of the lobules with central areas of sebaceous differentiation. Immunohistochemical stains showed a lack of MSH2 expression in the tumor, which is highly consistent with MTS. CONCLUSIONS: Muir-Torre syndrome should be considered in patients initially seen with sebaceous tumors of the eyelid. Immunohistochemistry for MSH2 is a practical initial approach to screen for MTS in patients with sebaceous tumors.

Adenoma↗

Management of inflammation in the cornea and conjunctiva.

The ability of the cornea to react to inflammatory stimuli is limited, and in most instances the polymorphonuclear leukocyte is primarily responsible for corneal damage. Of currently available drugs, corticosteroids are the most effective for the non-specific control of these inflammatory cells. Principles for obtaining maximal therapeutic effect from minimal amounts of the drug are presented. The use of corticosteroids in the treatment of a keratitis caused by various types of replicating microbial agents is discussed. The response to therapy of inflammatory processes in the conjunctiva seemingly differs from the response in the cornea. Suggestions are offered for corticosteroid use in conjunctivitis.

Administration, Topical↗

Multicentric Kaposi's sarcoma of the conjunctiva in a male homosexual with the acquired immunodeficiency syndrome.

A 38-year-old male homosexual with the new Acquired Immunodeficiency Syndrome (AIDS) had biopsy proven Kaposi's sarcoma of the right palpebral conjunctiva, extraocular mucocutaneous areas, and lymph nodes. Histologically, the palpebral tumor was characterized by atypical spindle cell proliferation and multiple slit-like vessels. Staining for Factor VIII-related antigen was positive in the cytoplasm of some tumor cells. Electron microscopy disclosed Weibel-Palade bodies in cells lining scattered slit-like vascular channels. The palpebral tumor mass was relatively nonadherent to its surrounding tissues and thus its simple excision was noteworthy; despite the prominent vascularity of the tumor, minimal bleeding was associated with its surgical resection. Any patient with AIDS should have all mucosal surfaces routinely examined for the presence of Kaposi's sarcoma lesions.

Acquired Immunodeficiency Syndrome↗

Inverted mucoepidermoid papillomas of the epibulbar conjunctiva.

Juxtalimbal epibulbar inverted papillomas that were locally excised without recurrence developed in two women, aged 42 and 51 at the time of initial presentation. There is only one previous report of this unusual conjunctival neoplasm, which documented involvement of the plical, caruncular, or tarsal conjunctiva. The tumor grew by acanthosis of the surface nonkeratinizing squamous epithelium, with invagination into the underlying connective tissue in the absence of inflammation or a tendency toward keratinization. The initial invaginations may be cystic in character, but solid lobules secondarily originated from their walls to push into the connective tissue. Mucus-producing goblet cells were scattered throughout the lesions; highly distinctive eosinophilic globoid bodies, probably reflecting inspissated mucoid material, were found in the walls of the cysts and entrapped within their secretions. Conjunctival inverted papilloma does not exhibit locally aggressive behavior, does not involve extensive segments of the conjunctival epithelium, and does not display diffuse spread or multicentricity. The authors therefore believe that it should be clearly separated from inverted squamous (Schneiderian) papillomas of the nasal cavity and sinuses.

Adult↗

Metastatic melanoma within and to the conjunctiva.

Two patients with epibulbar juxtalimbal primary conjunctival melanomas experienced local intralymphatic metastases to the inferior cul-de-sac, and a hematogenous metastasis to the conjunctiva developed in five other patients with cutaneous melanomas. Whether reflective of a local or distant metastasis, all of the lesions histopathologically were located in the substantia propria, and were separated from the overlying epithelium by a thin mantle of collagen. There was no evidence of atypical intraepithelial melanocytic proliferation, as would be expected in association with a primary conjunctival melanoma. Two of the cutaneous metastases exhibited a binodular or multinodular appearance that correlated histopathologically with variably confluent micronodules suggestive of the origin of the clinical lesion from a shower of tumor cell emboli. Patients with local intralymphatic spread from a primary conjunctival melanoma may experience additional lesions in the conjunctival sac or eyelid skin and are at risk for regional or distant metastases. They should be examined closely several times a year. The patients with the distant metastases all had their previously diagnosed primary cutaneous tumors on the truncal skin (a similar tendency emerges from a review of previous ocular cases), typically had myriad other cutaneous lesions, and two of them had a neoplastic iridocyclitis and vitreitis. These patients tended to die of the disseminated tumors within 1 year after conjunctival metastases developed.

Adult↗

Mast cells in conjunctiva affected by cicatricial pemphigoid.

Ocular cicatricial pemphigoid (OCP) is characterized by progressive conjunctival subepithelial fibrosis often leading ultimately to corneal blindness. Mast cells have been shown to play a role in several fibrotic disorders, but the role of mast cells in OCP is unknown. The authors compared the mast cell population in conjunctival biopsy specimens from 14 OCP patients and from six controls by using specific histochemical stains for mast cell subsets. The total mast cell number and the ratio of connective tissue mast cells to mucosal mast cells (MMCs) were significantly higher in OCP than in normal conjunctiva (P less than 0.05). This report is the first analysis of mast cell subsets in human ocular tissue. The results suggest that connective tissue mast cells (CTMCs) may play an important role in OCP and that therapy directed toward mast cells and their mediators may be an appropriate avenue for further exploration.

Adult↗