Departments of Otolaryngology (T.M.W., J.D.P.)
Children's Hospital Colorado (T.M.W., J.D.P., D.M.M.), Aurora, Colorado.
AJNR Am J Neuroradiol. 2021 Jun;42(6):1163-1166. doi: 10.3174/ajnr.A7056. Epub 2021 Mar 25.
Various etiologies have been theorized for the development of congenital nasal pyriform aperture stenosis (CNPAS). Imaging possibly implicates abnormal fusion of the midline palatal suture and deficient lateral growth of the midface in affected neonates.
A single-center, retrospective study was performed at a tertiary care pediatric hospital involving neonates and infants between 0 and 90 days of life. Maxillofacial CT scans of patients were reviewed. Abnormality of the palatal suture and midface transverse dimensions were measured and analyzed in patients with and without CNPAS.
A total of 109 patients between 0 and 90 days of life had maxillofacial CT scans. Thirteen patients were classified as having CNPAS, 27 patients had normal scans (control group), and 69 patients were excluded because of the presence of other craniofacial anomalies. All patients with CNPAS had evidence of abnormal fusion of the midline palatal suture. Zero patients without CNPAS had a midline palatal suture abnormality. The mean widths of the pyriform aperture were 5.7 mm (SD, 1.7) in the CNPAS group and 13.1 mm (SD, 2.7) in the control group ( < .0001). The mean distance between the superior portions of the nasolacrimal ducts was 9.1 mm (SD, 2.1) in the CNPAS group, and the mean of the control group was 13.4 mm (SD, 2.2) ( < .0001).
Patients with CNPAS have abnormal fusion of the midline palatal suture and exhibit lateral growth restriction of the midface. This may implicate synostosis of the midline palatal suture and abnormal midface growth.
先天性鼻前梨状孔狭窄（CNPAS）的发病机制有多种学说。影像学可能提示中线腭缝融合异常和中面部横向生长不足。
本研究为单中心回顾性研究，在一家三级儿科医院进行，纳入年龄 0 至 90 天的新生儿和婴儿。回顾分析患者的颌面 CT 扫描结果。测量并分析有无 CNPAS 患者的腭缝异常和中面部横向尺寸。
共有 109 名 0 至 90 天的患者行颌面 CT 扫描。13 名患者被归类为 CNPAS，27 名患者为正常扫描（对照组），69 名患者因存在其他颅面异常而被排除。所有 CNPAS 患者均存在中线腭缝融合异常。无一例无 CNPAS 的患者存在中线腭缝异常。CNPAS 组梨状孔的平均宽度为 5.7mm（SD，1.7），对照组为 13.1mm（SD，2.7）（<0.0001）。CNPAS 组鼻泪管上缘之间的平均距离为 9.1mm（SD，2.1），对照组为 13.4mm（SD，2.2）（<0.0001）。
CNPAS 患者存在中线腭缝融合异常，表现为中面部横向生长受限。这可能提示中线腭缝融合和异常的中面部生长。
