Cases reported "Xanthogranuloma, Juvenile"

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1/8. Juvenile xanthogranuloma masquerading as pediatric chronic uveitis: a clinicopathologic study.

    Juvenile xanthogranuloma (JXG) is a rare, pediatric histiocytic skin disorder that may affect the eye. It can present with protean ocular manifestations, including masquerade uveitis, heterochromia, hyphema, or glaucoma. It very rarely involves the retina and posterior segment; indeed, posterior involvement has been documented histopathologically in only one case. We present the case of a 2-year-old child with ocular JXG presenting as chronic, refractive uveitis, without skin or systemic findings. The blind, painful eye was enucleated and found to harbor a diffuse histiocytic process that involved both the anterior and posterior segments, including the retina and subretinal space. Histological, immunohistochemical, and electron microscopic studies confirmed the diagnosis of JXG. The pathologic classification and differential diagnosis of systemic histiocytic disorders are discussed. Since JXG can present as masquerade pediatric uveitis, this entity should be considered in children with atypical uveitis. In rare instances, JXG may involve the posterior segment and the retina, leading to retinal detachment and blindness.
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keywords = hyphema
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2/8. Juvenile xanthogranuloma: concurrent involvement of skin and eye.

    PURPOSE: To report a novel presentation of ocular involvement in juvenile xanthogranuloma (JXG). CASE REPORT: A 1-year-old boy with the concurrent skin and eye involvement of JXG is presented. The ocular problems included spontaneous hyphema, secondary glaucoma, and 360-degree involvement of the limbus, iris, and angle. RESULTS: To reduce the intraocular pressure (IOP), treatment was started with drops of timolol, betamethasone, and atropine and acetazolamide suspension. After 6 months of medical treatment, the skin lesions did not change, but the iris lesion enlarged without hyphema. The IOP in the right eye was controlled by the medications. CONCLUSION: Although JXG is an uncommon disease, it is one of the most important causes of spontaneous hyphema in children. iris involvement, spontaneous hyphema, and secondary glaucoma are the most common ocular problems. Corneal involvement often is characterized by a yellowish protrusion of the limbus along with blood staining of recurrent hyphema and elevated IOP. In this report, we describe an unusual case of JXG with 360-degree limbus, iris, and angle involvement. To our knowledge, this is the first report in the medical literature of 360-degree limbus involvement in JXG.
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keywords = hyphema
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3/8. Juvenile xanthogranuloma of the corneoscleral limbus: case report and review of the literature.

    Juvenile xanthogranuloma is a benign histiocytic cutaneous disorder mainly occurring in infants which may affect the eye. Ocular manifestations usually present in the form of iris lesions with secondary hyphema and glaucoma, but juvenile xanthogranuloma may also present as a corneoscleral limbal mass. We present the case of an 18-month-old female infant with ocular juvenile xanthogranuloma occurring as a corneoscleral limbal mass without associated cutaneous or systemic findings. The limbal mass lesion was excised by lamellar dissection and histopathological studies revealed histiocytes admixed with lymphocytes, plasma cells, and eosinophils; foreign body and Touton giant cells present throughout the specimen confirmed the diagnosis of juvenile xanthogranuloma. At 14-month follow-up, there was no recurrence of the limbal mass. The natural history of this uncommon ocular condition is discussed and the relevant literature reviewed. Juvenile xanthogranuloma should be considered in the differential diagnosis of any corneoscleral limbal mass lesion, particularly in children.
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keywords = hyphema
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4/8. Bilateral spontaneous hyphema in juvenile xanthogranuloma.

    This report describes a rare occurrence of bilateral, spontaneous, nontraumatic hyphema in a 6 weeks old infant, associated with a small, multiple skin lesions. The diagnosis of juvenile xanthogranuloma was confirmed by histopathological examination of the cutaneous lesions. The hyphaema cleared gradually in 2 weeks time with conservative management.
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keywords = hyphema
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5/8. Subconjunctival steroid in the management of uveal juvenile xanthogranuloma: a case report.

    Uveal juvenile xanthogranuloma (JXG) is a rare intraocular tumor which usually occurs in very young children. Most reported cases of successfully treated uveal juvenile xanthogranuloma have received systemic steroids, irradiation or excision. Some cases have responded to topical steroids alone. We report a case of JXG with recurrent hyphemas and elevated intraocular pressures despite the use of topical steroid. This patient responded to the addition of subconjunctival steroid injection. This is the first report to our knowledge demonstrating a response to periocular steroid supplementation for uveal JXG unresponsive to topical steroids.
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keywords = hyphema
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6/8. diagnosis and management of iris juvenile xanthogranuloma.

    BACKGROUND: Juvenile xanthogranuloma is a benign, self-limiting cutaneous disorder most commonly encountered during infancy. Approximately 10% of cases may develop ocular or adnexal involvement, most commonly in the iris. methods: We review clinical and morphological features of four cases of iris juvenile xanthogranuloma that reflect the diagnostic and therapeutic spectrum. RESULTS: Tissue diagnosis was confirmed in all cases; in one case, the disease was diagnosed with a skin biopsy and treated with local and systemic steroids, and its persistence in the iris was confirmed with a second tissue specimen obtained five months after systemic steroid treatment. CONCLUSION: The diagnosis and treatment of juvenile xanthogranuloma may be straightforward, particularly in cases when the ocular lesion receives early attention and responds well to topical steroids, and when there is no hyphema. However, in other instances, this entity may be difficult to manage and may necessitate iris biopsy for diagnosis and radiation therapy for treatment.
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7/8. 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.
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8/8. Juvenile xanthogranuloma of the iris in an adult.

    Juvenile xanthogranuloma is a self-limited skin disorder of young children that uncommonly affects the eye. Juvenile xanthogranuloma has been described in adults, but reported intraocular involvement is extremely rare. We report a case of juvenile xanthogranuloma diagnosed in a 25-year-old man who was seen with nontraumatic hyphema and iridocyclitis. diagnosis was made from a biopsy specimen obtained from a suspicious skin lesion. Topical and systemic steroids, radiation therapy, and finally immunosupression were required to eliminate the iris tumor clinically and resolve the patient's recurrent symptoms.
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