A Case of Goldenhar Syndrome with Postoperative Velopharyngeal Incompetence Improved by Palatal Lift Prosthesis

Abstract

Goldenhar syndrome has a multifoc al malformation involving ocular, auricular and vertebral anomalies, it is reported that about 2 % of the patients with this syndrome have cleft palate.
Acase of Goldenhar syndrome with a velopharyngeal incompetence after palatoplasty was improved by palatal lift prosthesis.
A 7-year-old male was referred to our hospital because of a speech disorder. He was born with epibulber tumor, microtia, deafness, cleft palate, scoliosis and mental retardation. He underwent palatoplasty by Furlow's method at another hospital at the age of 1 year 6months, but hypernasality remained after surgery. It was recommended to treat him with a speech appliance at our clinic.
By the cephalometric r adiography with contrast media and nasoendoscopy, it was found that the soft palate was as long as that of normal children, but the movement of soft palate was hardly recognized. Palatal lift prosthesis was applied to him, and his hypernasality disappeared a half year after the application of the prosthesis. lt was suggested that the speech appliance is effective for treating patients with postoperative velopharyngeal incompetence because of poor movement of the soft palate.