Goldenhar Syndrome: Quality-of-Life Analysis of 43 Consecutive Patients.

Abstract

Goldenhar syndrome is a rare congenital disease, characterized by the malformation of craniofacial features, spine, and other organs. Its pathogenesis and impact on patients' quality of life (QoL) have not been fully elucidated as yet. Therefore, data of all patients with Goldenhar syndrome, who were treated at Hannover Medical School between 1997 and 2020, were analyzed. Forty-three patients were identified. Their medical records were carefully reviewed for data regarding their age, sex, demographics, Pruzansky classification, surgical history, and affected side. Goldenhar syndrome-associated medical conditions, therapeutic procedures, and the impact of disease on QoL were assessed. Their QoL was assessed using the Oral Health Impact Profile (OHIP)-14 questionnaire. The χ 2 test and Pearson correlation were performed for statistical evaluation. Female sex was positively correlated with OHIP outcomes, whereas male sex had a negative correlation with OHIP outcomes. Therefore, females had significantly worse QoL than males. No significant correlation was observed between the Pruzansky classification and QoL or between surgical history and QoL. Goldenhar syndrome is a rare disease that presents with varying severity. Hence, prospective studies are required to further investigate the impact of Goldenhar syndrome on the QoL.