Epibulbar Dermoid Cyst in Association with VACTERL or VATER Syndrome

To present a case of unilateral epibulbar dermoid cyst in a girl with VACTERL syndrome.Case Report: A 4-year-old girl was referred with a white, soft and painless mass in the inferotemporal limbus of the left eye having presented since birth. The patient was healthy with normal growth. Other ocular examinations were normal. Systematic review revealed a group of abnormalities including vertebral scoliosis, imperforate anus, rectovaginal fistula, atrial septal defect, horseshoe kidneys, bladder diverticulitis, and a neurogenic bladder. Accordingly, she had been diagnosed with the VATERL association at birth. Defects of any organ system have been reported to be associated with VACTERL. Orbital dermoid is a rare non-VACTERL type defect in patients with VACTERL spectrum. However, there is one report in the literature indicating the association between the VACTERL syndrome and epibulbar dermoid cysts. The presence of an ipsilateral epibulbar dermoid cyst should lead to thorough investigation for other syndromes like VACTERL spectrum.