背景:先天性鼻梨状孔狭窄(CNPAS)是一种罕见的疾病,可以单独发生或作为多形成综合征的一部分发生。管理仍然很困难。没有具体的治疗方案。传统手术将是不合时宜的;需要非侵入性或微创治疗选择。然而,该疾病的罕见性和婴儿背景使随机临床试验变得困难。
方法:我们介绍了一个1个月大的白人男孩患有CNPAS的病例。他因鼻塞而向班比诺·格斯图儿科医院急诊科就诊,嘈杂的呼吸,喂养困难,怀疑是睡眠呼吸暂停.住院期间,他接受了夜间脉搏血氧饱和度检查,气道内窥镜检查,和颌面部计算机断层扫描(CT);最终诊断为CNPAS伴中度阻塞性睡眠呼吸暂停综合征。我们使用创新策略成功治疗了患者,该策略涉及耳鼻喉外科医师和正畸医师之间的合作。
结论:微创球囊手术和放置腭装置的组合可以成功治疗CNPAS;它也可以治疗其他类型的鼻骨狭窄。未来的研究可能允许实践共识治疗策略的发展。
BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that may occur alone or as part of a multi-formative syndrome. Management remains difficult. There is no specific treatment protocol. Traditional surgery would be anachronistic; a non-invasive or minimally invasive therapeutic option is required. However, the rarity of the disease and the infantile context render randomised clinical trials difficult.
METHODS: We present the case of a one-month-old Caucasian boy with CNPAS. He presented to the Emergency Department of the Bambino Gesù Pediatric Hospital with nasal obstruction, noisy breathing, feeding difficulties, and suspected sleep apnoea. During hospitalisation, he underwent overnight pulse oximetry, airway endoscopy, and maxillofacial computed tomography (CT); the final diagnosis was CNPAS with moderate obstructive sleep apnoea syndrome. We successfully treated the patient using an innovative strategy that involved collaboration between ear-nose-and-throat surgeons and orthodontists.
CONCLUSIONS: A combination of minimally invasive balloon surgery and placement of a palatal device may successfully treat CNPAS; it may also treat other types of nasal bone stenosis. Future studies may allow the development of practice consensus treatment strategies.