Trigui L, Ben Thabet A, Gargouri A, Hmida N, Ben Hamad A, Rekik A
Service de néonatologie, hôpital Hédi Chaker, Sfax, Tunisie.
Rev Stomatol Chir Maxillofac. 2010 Jun;111(3):155-7. doi: 10.1016/j.stomax.2009.04.004. Epub 2010 Feb 18.
Congenital nasal pyriform aperture stenosis (CNPAS) is a rare and an unusual cause of airway obstruction in newborns.
We report the case of a female neonate delivered by C-section at 39 weeks of amenorrhea for hydramnios and macrosomia. She presented with mandibular retrognathia, nasal saddling, submucous cleft palate, and loud mouth respiration. She presented with cyanosis every time feeding was attempted. CT revealed permeable choans with a single central incisor and nasal pyriform aperture stenosis. Nasal respiration returned to normal progressively after 56 days of hospitalization. The status was unchanged at 5 months.
CNPAS is a rare cause of congenital nasal obstruction. It is sometimes associated to a median incisor syndrome. The diagnosis should be made as early as possible for an optimal management. Cyanosis and swallowing disorders may be lethal consequences. Associated abnormalities should be screened for with TDM or MRI. The treatment depends on the severity and may be surgical for a severe stenosis. The prognosis is good if no severe malformation or mental retardation is associated.
先天性鼻梨状孔狭窄（CNPAS）是新生儿气道梗阻的一种罕见且不常见的病因。
我们报告一例因羊水过多和巨大儿于停经39周行剖宫产分娩的女婴。她表现为下颌后缩、鼻根部凹陷、黏膜下腭裂及经口呼吸声大。每次尝试喂奶时都会出现青紫。CT显示后鼻孔通畅，有一颗正中切牙且鼻梨状孔狭窄。住院56天后鼻呼吸逐渐恢复正常。5个月时情况未变。
CNPAS是先天性鼻梗阻的罕见病因。它有时与正中切牙综合征相关。为了进行最佳管理，应尽早做出诊断。青紫和吞咽障碍可能是致命后果。应通过TDM或MRI筛查相关异常。治疗取决于严重程度，对于严重狭窄可能需要手术治疗。如果没有合并严重畸形或智力发育迟缓，预后良好。
