Unit of Otolaryngology, Bambino Gesù Children's Hospital, 00165, Rome, Italy.
Unit of Dentistry, Bambino Gesù Children's Hospital, 00165, Rome, Italy.
Ital J Pediatr. 2021 Sep 16;47(1):189. doi: 10.1186/s13052-021-01124-2.
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.
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.
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.
先天性鼻前孔狭窄（CNPAS）是一种罕见的疾病，可能单独发生，也可能作为多种形态综合征的一部分。目前，这种疾病的治疗仍然存在困难，尚无特定的治疗方案。传统手术已经过时，需要一种非侵入性或微创的治疗方法。然而，由于疾病的罕见性和婴儿期的特殊性，很难进行随机临床试验。
我们报告了一例 1 个月大的白人男孩患有 CNPAS 的病例。他因鼻塞、呼吸杂音、喂养困难和疑似睡眠呼吸暂停而到 Bambino Gesù 儿童医院的急诊部就诊。在住院期间，他接受了过夜脉搏血氧饱和度监测、气道内窥镜检查和颌面计算机断层扫描（CT）；最终诊断为 CNPAS 合并中度阻塞性睡眠呼吸暂停综合征。我们采用了一种创新的策略，成功地治疗了这名患者，该策略涉及耳鼻喉科医生和正畸医生的合作。
微创球囊手术联合腭部装置的使用可能成功治疗 CNPAS；它也可能治疗其他类型的鼻骨狭窄。未来的研究可能会制定出治疗共识策略。
