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Mehrdad Malihi, Roger Turbin, Neena Mirani, Paul D Langer; Giant Orbital Hydrocystoma in Children: Report of Three Cases. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5429.
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Hydrocystoma (also known as sudoriferous cyst) is a benign cystic proliferation of a sweat gland found commonly on the eyelid skin of adults; we report three cases of giant orbital hydrocystoma in children, expanding the clinical and pathological spectrum of this entity.
Interventional case series.
Case 1: An 8 year-old-boy presented with a 1 year history of painless progressive right proptosis. Computed tomographic and magnetic resonance imaging (MRI) revealed a well-defined, intraorbital, extraconal cystic lesion in the lateral orbit posterior to the globe causing bony erosion (Fig 1). Pathologic examination following total resection via lateral orbitotomy reveals a cystic lesion with a clear cavity lined by a smooth surface of a double layer of cuboidal cells, consistent with eccrine hydrocystoma. Case 2: A 13-year-old girl who had suffered blunt orbital trauma one year earlier developed a soft, mobile, non-tender subconjunctival mass in the temporal part of the right upper eyelid. MRI revealed a large well-defined cystic lesion in the right anterior orbit, which was later dissected from beneath the conjunctiva. Pathologic examination reveals a cystic cavity with papillary projections lined by two layers of cuboidal epithelial cells, with the innermost cells displaying eosinophilic cytoplasm and apical “snouting” (decapitation), characteristic of an apocrine hydrocystoma (Fig 2). Case 3: A two-month-old infant was noted to have a non-tender subconjunctival orbital growth visible in the medial left palpebral aperture. Surgical excision revealed a cystic lesion with a smooth internal surface. Microscopic evaluation revealed characteristics similar to case 1 and consistent with eccrine hydrocystoma.
Periocular hydrocystoma, which typically presents on the eyelid skin in adults, only rarely occur beneath the skin or conjunctiva: only 8 such lesions have previously been reported, 4 of them in the pediatric population. Presumably, the deeper location of these common skin lesions results either from embryonic rests or from traumatic implantation of glandular epithelium. All 4 previously reported pediatric cases were congenital and of apocrine subtype.Two of the cases in our series were of eccrine subtype, and one was traumatic in origin, expanding the clinical and pathological spectrum of this entity. Clinicians should be aware that an orbital cystic lesion in a child may represent a giant hydrocystoma.
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