Abstract：
Congenital arhinia-microphthalmos syndrome or BOSMA syndrome is an exceptionally rare clinical syndrome characterized by unilateral or bilateral complete absence of the nasal cavity associated with several craniofacial, ocular, and systemic anomalies. Lacrimal drainage anomalies are secondary to absent nasolacrimal duct and usually present as dilated lacrimal sac or mucoceles. While navigation-guided dacryocystorhinostomies into the contralateral nasal cavity are described for unilateral arhinia, the way forward for the complete absence of the nose and nasal cavity is still unclear. A multidisciplinary team from the specialties of genetics, plastic surgery, ophthalmic plastics and reconstructive surgery, otorhinolaryngology, and endocrinology should get involved very early on for better continuity of care.
摘要：
先天性arhinia-microthromos综合征或BOSMA综合征是一种非常罕见的临床综合征，其特征是与多个颅面相关的单侧或双侧鼻腔完全缺失，眼,和系统异常。泪道引流异常继发于鼻泪管缺失，通常表现为扩张的泪囊或黏液囊肿。虽然描述了导航引导的泪囊鼻腔造口术进入对侧鼻腔的单侧arhinia，目前尚不清楚完全没有鼻子和鼻腔的前进方向。来自遗传学专业的多学科团队，整形手术,眼科塑料和重建手术，耳鼻咽喉科,内分泌学应尽早参与，以更好地保持护理的连续性。
