Andrade Eduardo C, Júnior Vanier S, Didoni Ana L S, Freitas Priscila Z, Carneiro Araken F, Yoshimoto Fabiana R
Hospital de Reabilitação de Anomalias Crânio Faciais, Universidade de São Paulo, Bauru.
Braz J Otorhinolaryngol. 2005 Jan-Feb;71(1):107-10. doi: 10.1016/s1808-8694(15)31296-9. Epub 2006 Jan 2.
Treacher Collins Syndrome--or mandibulofacial dysostosis--is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. Incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins Syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins Syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.
特雷彻·柯林斯综合征——又称下颌面骨发育不全——是一种罕见的疾病,表现出不同程度的多种颅面畸形。这是一种涉及第一和第二鳃弓的先天性畸形。据估计,活产婴儿中该病的发病率在1/40000至1/70000之间。该疾病的特征包括耳廓异常、面骨发育不全、具有下睑缺损的反蒙古样睑裂倾斜以及腭裂。特雷彻·柯林斯综合征很少与后鼻孔闭锁相关。一个多学科团队,包括颅面外科医生、眼科医生、言语治疗师、牙科医生和耳鼻喉科医生,是管理这些患者的最合适组合。本研究报告了一例罕见的伴有后鼻孔闭锁的特雷彻·柯林斯综合征病例,并进行了文献综述和多学科干预。