Abstract

MAQUERA, Diego José Mamani  and  BERNAL, Adriana Palomino. Goldenhar syndrome associated with an epibulbar dermoid: Case report. Rev. Fac. Med. Hum. [online]. 2022, vol.22, n.2, pp.422-426.  Epub Mar 16, 2022. ISSN 1814-5469.  http://dx.doi.org/10.25176/rfmh.v22i2.4393.

We present the case of a 7-year-old male child with Goldenhar syndrome associated with an epibulbar choristoma. The patient presents a homogeneous tumor, rounded in shape, with well-defined borders, of a soft consistency, vascularized, approximately 9x10 mm, yellowish-white in color, adhered to the sclera and cornea, with hairy formations on its surface, located at the level of the sclerocorneal limbus of the temporal-lower quadrant of his right eye. The histopathology study showed the presence of hair follicles and sebaceous glands, immersed in a stroma of fibrous connective tissue with a superficial squamous-type lining, which defined the diagnosis of limbar epibulbar dermoid. Conjunctivaplasty was performed to cover the scleral defect and use amniotic membrane to cover the corneal and scleral defect. The limbar epibulbar dermoid is the most common type of conjunctival choristoma and should be considered in the differential diagnosis of pediatric epibulbar tumors.

Keywords : Goldenhar syndrome; epibulbar choristoma; epibulbar dermoid. (Source : MeSH - NLM)..

        · abstract in Spanish     · text in English | Spanish     · English ( pdf ) | Spanish ( pdf )