Epibulbar Dermoid Cyst in a Patient with VACTERL Association

Abstract

The VACTERL association is an association of multiple congenital malformations Such as vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities in which the rate and number of the characteristic anomalies varies according to the population. We present a case of VACTERL association who was referred with a nodular dermoid cyst in her left eye since birth. Dermoid cysts as a part of Goldenhar’s syndrome and the VACTERL association have been reported very rarely; however there have been a few reports of children having the VACTERL association and orbital dermoid. This shows the possibility of finding orbital dermoid cysts in the context of VACTERL association and demonstrates that such cysts could be a surrogate for feasible diagnosis of VACTERL association. Nonetheless, additional findings of an ipsilateral orbital dermoid cyst should stimulate serious discussion concerning the pathophysiology and etiology of this complex disorder.