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Abstract：
BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is an uncommon malformation and a rare cause of respiratory distress in neonates that may require early surgical intervention. The aim of this study is to report our experience in patients operated for CNPAS and present characteristics of this population.
METHODS: A retrospective, analytical cohort study was conducted of patients who underwent surgical treatment for CNPAS in the Hospital de Pediatría Garrahan from May 2008 to May 2018.
RESULTS: Nineteen patients underwent surgical treatment; 47.36% had some associated genetic syndrome. The surgical approach was sublabial. Follow-up was more than 45 days in 14 patients, 9 did not require additional treatment, and the remaining 5 required further surgical and/or medical treatment. Short-term post-surgical outcome was good in 85.7%.
CONCLUSIONS: Better post-surgical results were obtained in patients in whom endonasal stenting was not used and who did not present associated craniofacial malformations.
METHODS: A retrospective, analytical cohort study was conducted of patients who underwent surgical treatment for CNPAS in the Hospital de Pediatría Garrahan from May 2008 to May 2018.
RESULTS: Nineteen patients underwent surgical treatment; 47.36% had some associated genetic syndrome. The surgical approach was sublabial. Follow-up was more than 45 days in 14 patients, 9 did not require additional treatment, and the remaining 5 required further surgical and/or medical treatment. Short-term post-surgical outcome was good in 85.7%.
CONCLUSIONS: Better post-surgical results were obtained in patients in whom endonasal stenting was not used and who did not present associated craniofacial malformations.
摘要：
背景：先天性鼻梨状孔狭窄（CNPAS）是一种罕见的畸形，是新生儿呼吸窘迫的罕见原因，可能需要早期手术干预。这项研究的目的是报告我们在接受CNPAS手术的患者中的经验以及该人群的特征。
方法：回顾性研究，我们对2008年5月至2018年5月在Garrahan医院接受CNPAS手术治疗的患者进行了分析性队列研究.
结果：19例患者接受了手术治疗；47.36%有一些相关的遗传综合征。手术方法为阴唇下。14例患者随访时间超过45天,9不需要额外的治疗，其余5人需要进一步的手术和/或药物治疗。术后短期预后良好，占85.7%。
结论：在未使用鼻内支架置入术和未出现相关颅面畸形的患者中获得了更好的术后效果。
方法：回顾性研究，我们对2008年5月至2018年5月在Garrahan医院接受CNPAS手术治疗的患者进行了分析性队列研究.
结果：19例患者接受了手术治疗；47.36%有一些相关的遗传综合征。手术方法为阴唇下。14例患者随访时间超过45天,9不需要额外的治疗，其余5人需要进一步的手术和/或药物治疗。术后短期预后良好，占85.7%。
结论：在未使用鼻内支架置入术和未出现相关颅面畸形的患者中获得了更好的术后效果。
