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Correspondence to Author: Marcelo Vicente de Andrade Sobrinho, Larissa Batista Pegorin, Giovanna Soares Nutels, Catherine Pancini Rezende, Marina Marques Denobi*.  

Ophthalmology Resident, PUC Campinas Hospitalm Av John Boyd Dunlop, s/n, Jardim Ipaussurama, Campinas, São Paulo, Brazil.

DOI: 10.52338/jocp.2025.4353

Abstract:

Goldenhar syndrome was first described by Maurice Goldenhar in 1952 as an oculoauricular dysplasia. Later, in 1963, Gorlin et al included some vertebral anomalies to the syndrome. Oculo-auriculo vertebral spectrum (OAVS) is a rare and congenital disorder that results from the abnormal development of the first and second branchial arches, but the etiology is unknown, comprising multifactorial mechanisms. The objective of the present study is to facilitate its recognition and assist in the management of future cases. This is a case report of a 9-year-old child who was referred to the [hospital puc campinas] due to a corneoscleral lesion in the right eye. Ophthalmological and general clinical evaluation showed signs of Oculoauriculovertebral dysplasia or Goldenhar syndrome. After complementary exams, limbal dermoid excision surgery with lamellar keratoplasty was performed. In this case, after the resection of the limbal dermoid, the patient showed an improvement in visual acuity, although partial. Therefore, we emphasize the importance of early diagnosis to prevent amblyopia.

Keywords: cornea, limbal dermoid, Goldenhar syndrome, keratoplasty.

Citation:

Dr. Marcelo Vicente de Andrade Sobrinho, Goldenhar Syndrome: A Case Report. Journal of Clinical Pediatrics 2025.