GOLDENHAR SYNDROME ASSOCIATED WITH AN EPIBULBAR DERMOID: CASE REPORT

Mamani Maquera D.J.; Bernal A.P. · Revista de la Facultad de Medicina Humana (2022) · DOI: 10.25176/rfmh.v22i2.4292

Resumen

We present the case of a 7-year-old male schoolboy with Goldenhar syndrome associated with an epibulbar choristoma. The patient presents a homogeneous tumor, round-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 limbal epibulbar dermoid. Conjunctivoplasty was performed to cover the scleral defect and use amniotic membrane to cover the corneal and scleral defect. The limbal epibulbar dermoid is the most common type of conjunctival choristoma and should be considered in the differential diagnosis of pediatric epibulbar tumors.

Instituciones: Universidad Nacional de San Agustín de Arequipa; Universidad Católica de Santa María