de Oliveira Jéssica Pereira, Lodovichi Fernando Felipe, Gomes Miria Benincasa, Custódio Eda Marconi, Denadai Rafael, Raposo-Amaral Cesar Augusto, Ghizoni Enrico, Raposo-Amaral Cassio Eduardo
Faculty of Medicine, University of Campinas, Campinas.
Institute of Psychology, University of São Paulo, São Paulo.
J Craniofac Surg. 2018 Sep;29(6):1430-1433. doi: 10.1097/SCS.0000000000004522.
Treacher Collins syndrome has a wide variation of clinical presentation, including airway obstruction and hearing loss. The objective of this study was to assess patient-rated quality of life in Treacher Collins syndrome, who received multidisciplinary treatment at a specialized craniofacial hospital, and to compare their quality of life with a control sample.
Treacher Collins patients under treatment in a multidisciplinary setting were assessed with the World Health Organization Quality of Life (WHOQOL-100) questionnaire. The Siviero scale was used to classify quality of life as unsatisfactory (scores below 25), intermediate (scores between 25 and 75), and satisfactory (scores above 75). Quality of life comparison between Treacher Collins patients and 2342 unaffected individuals from a previous database was performed using the analysis of variance test. Differences were considered significant at P < 0.05.
Treacher Collins patients demonstrated an overall intermediate and satisfactory (score >60%) quality of life in almost all WHOQOL-100 facets and domains. The comparison of the scores between unaffected individuals and Treacher Collins patients demonstrated a significant difference (P < 0.05) in the environment domain.
The highest functioning Treacher Collins syndrome patients under multidisciplinary treatment presented intermediate and satisfactory quality of life, consistent with quality of life scores of unaffected controls. In the environment domain, Treacher Collins patients presented even higher scores of quality of life than the control group.
CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.
特雷彻·柯林斯综合征临床表现差异很大，包括气道阻塞和听力丧失。本研究的目的是评估在一家专业颅面医院接受多学科治疗的特雷彻·柯林斯综合征患者自评的生活质量，并将他们的生活质量与一个对照样本进行比较。
使用世界卫生组织生活质量问卷（WHOQOL - 100）对在多学科环境中接受治疗的特雷彻·柯林斯综合征患者进行评估。使用西维罗量表将生活质量分类为不满意（得分低于25分）、中等（得分在25至75分之间）和满意（得分高于75分）。使用方差分析测试对特雷彻·柯林斯综合征患者与先前数据库中的2342名未受影响个体之间的生活质量进行比较。P<0.05时差异被认为具有统计学意义。
在几乎所有WHOQOL - 100方面和领域中，特雷彻·柯林斯综合征患者总体生活质量为中等和满意（得分>60%）。未受影响个体与特雷彻·柯林斯综合征患者得分的比较显示，在环境领域存在显著差异（P<0.05）。
接受多学科治疗的功能最高的特雷彻·柯林斯综合征患者生活质量为中等和满意，与未受影响对照组的生活质量得分一致。在环境领域，特雷彻·柯林斯综合征患者的生活质量得分甚至高于对照组。
临床问题/证据水平：治疗性，III级。
