Ensing Amy E, Islam Aseeyah, Kallogjeri Dorina, Lieu Judith E C
Washington University School of Medicine - Department of Otolaryngology, Head and Neck Surgery, St. Louis, MO, United States.
Washington University School of Medicine - Department of Otolaryngology, Head and Neck Surgery, St. Louis, MO, United States.
Int J Pediatr Otorhinolaryngol. 2025 Jun;193:112359. doi: 10.1016/j.ijporl.2025.112359. Epub 2025 Apr 26.
Congenital nasal pyriform aperture stenosis (CNPAS) is a rare and potentially life-threatening source of neonatal upper airway obstruction. Treatment can be medical or surgical. Our objective was to describe CNPAS presentation and management.
Pooled data analysis of records of children identified with CNPAS from relevant publications and between 2014 and 2024 at our institution was performed. Published studies identified by a medical librarian were assessed independently by two reviewers.
From 210 abstracts, 170 were identified as relevant, and 106 studies were included. Data from 10 children with CNPAS from our institution and 310 from the medical literature were analyzed. The majority 74.2 % (n = 230) of our sample received a surgical intervention for CNPAS. Pyriform aperture diameter of children receiving only medical management (mean = 5.3 mm; SD = 1.6) was not different from that of children who received or were recommended surgery (mean = 5.1 mm; SD = 1.5) with a mean difference of 0.2 mm (95 % CI -0.4 to 0.7). Presence of apnea (adjusted odds ratio [aOR] = 9.1; 95 % CI 2.4 to 35) and stridor/noisy breathing (aOR = 3.9; 95 % CI 1.4 to 10.8) were associated with higher odds of having surgery performed or recommended.
Management decisions in CNPAS have been driven by individual patient presentation rather than pyriform aperture diameter. Clinicians should use pyriform aperture diameter as a diagnostic rather than prognostic tool, as a narrow pyriform aperture diameter may not necessitate surgery in the absence of severe symptoms.
先天性鼻梨状孔狭窄（CNPAS）是新生儿上呼吸道梗阻的一种罕见且可能危及生命的病因。治疗方法可以是药物治疗或手术治疗。我们的目的是描述CNPAS的临床表现和治疗方法。
对2014年至2024年期间在我们机构以及相关出版物中确诊为CNPAS的儿童记录进行汇总数据分析。医学图书馆员检索到的已发表研究由两名审阅者独立评估。
从210篇摘要中，确定了170篇相关摘要，纳入了106项研究。分析了来自我们机构的10例CNPAS患儿的数据以及来自医学文献的310例患儿的数据。我们样本中的大多数（74.2%，n = 230）接受了CNPAS的手术干预。仅接受药物治疗的儿童梨状孔直径（平均值 = 5.3毫米；标准差 = 1.6）与接受或被建议手术的儿童（平均值 = 5.1毫米；标准差 = 1.5）没有差异，平均差异为0.2毫米（95%可信区间 -0.4至0.7）。呼吸暂停（调整后的优势比[aOR] = 9.1；95%可信区间2.4至35）和喘鸣/呼吸嘈杂（aOR = 3.9；95%可信区间1.4至10.8）与进行手术或被建议手术的较高几率相关。
CNPAS的治疗决策是由个体患者的临床表现而非梨状孔直径驱动的。临床医生应将梨状孔直径用作诊断工具而非预后工具，因为在没有严重症状的情况下，梨状孔直径狭窄可能不需要手术。
