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Epidermal nevus syndrome is a rare congenital disorder affecting only a few hundred people in the world. It has ophthalmic, dermatological, and neurological manifestations, with varied presentation. Here, we report a case of two-year-old child who presented with epibulbar mass in left eye, pigmented nevi over left side of the body and alopecia over left side of parieto-temporal scalp. Imaging confirmed epibulbar mass and presence of calcification of choroid on ipsilateral side with presence of arachnoid cyst of brain with underlying pachygyria. Neurological examination was normal and dermatologist confirmed presence of verrucous nevi over skin. Excisional biopsy of epibulbar mass revealed a complex choristoma with presence of lacrimal gland tissue. Underlying ocular findings were near normal with normal posterior segment. It is a rare form of epidermal nevus syndrome with near normal ocular findings in the presence of anterior and posterior choristoma, which has not been reported. [ABSTRACT FROM AUTHOR]

A healthy 18-year-old girl presented with a superotemporal epibulbar mass that was present since birth. Computed tomography revealed a cystic mass with fat-like low-density and a central oval-shaped mass that was isodense to the bone in the superotemporal region of the globe. Histopathology of the excised tumour revealed osseous choristoma and dermolipoma. Our review of literature yielded nine reported cases of epibulbar osseous choristoma within a dermolipoma and we reviewed these cases. [ABSTRACT FROM AUTHOR]

Choroidal osteoma is a rare, benign, ossifying tumour of the choroid of unknown aetiology. In contrast to other types of intraocular ossification, choroidal osteoma is found typically in young healthy females in the second or third decades of life with no history of systemic or ocular disease. Choroidal osteoma is a deep, pale yellow lesion with distinct geographic borders at the juxtapapillary or macular region, with branching ‘spider’ vessels on the surface of the tumour. These features should help differentiate choroidal osteoma from other types of intraocular tumour and the diagnosis can be confirmed with ultrasonography and computerised tomography. Here we report an initially unilateral case of choroidal osteoma, which decalcified over 20 years but during the same period the fellow eye also developed a choroidal osteoma to become a bilateral case. Despite the benign nature of the tumour, vision may be compromised by gradual atrophy of the overlying retina, serous retinal detachment, accumulation of sub-retinal fluid and sub-retinal haemorrhage associated with choroidal neovascularisation. Frequent examinations are recommended for patients with choroidal osteoma, for early detection of a subretinal neovascular membrane and potential treatment with laser photocoagulation. [ABSTRACT FROM AUTHOR]

Epibulbar osseous choristoma occurs rarely and is commonly mistaken for a dermoid or dermolipoma. We report two cases: an 8-year-old boy and a 79-year-old lady with epibulbar osseous choristomas where computed tomography showed an extraocular lesion in the temporal quadrant and diagnosis was confirmed by excision biopsy. The osseous component was adherent to the sclera in the young patient, while it was loosely attached to the surrounding tissue in the older patient. We believe that the 79-year-old lady is the oldest reported patient with an epibulbar osseous choristoma. [ABSTRACT FROM AUTHOR]