Arlis H, Ward R F
Department of Otolaryngology, Manhattan Eye, Ear, and Throat Hospital, New York, NY.
Arch Otolaryngol Head Neck Surg. 1992 Sep;118(9):989-91. doi: 10.1001/archotol.1992.01880090105027.
Congenital nasal pyriform aperture stenosis has recently been described as a cause of nasal airway obstruction in the newborn. As some investigators have observed, the nasal pyriform aperture is narrowed owing to bony overgrowth of the nasal process of the maxilla. This overgrowth is confirmed by computed tomography. In six previously reported cases, follow-up as long as 16 months revealed normal facial growth. We describe six patients with congenital nasal pyriform aperture stenosis. On eruption of deciduous dentition, four of the six patients were found to have a single prominent central maxillary incisor, ie, "megaincisor." Computed tomographic scans have confirmed this dental anomaly as well as maxillary bony overgrowth. A single maxillary incisor has been described as an autosomal dominant microform of holoprosencephaly, a developmental field defect. We propose that in some patients congenital nasal pyriform aperture stenosis may represent more than an isolated congenital abnormality of the airway and may, in fact, be a midfacial dysostosis with associated endocrine and central nervous system abnormalities.
先天性鼻梨状孔狭窄最近被描述为新生儿鼻气道阻塞的一个原因。正如一些研究者所观察到的，鼻梨状孔因上颌骨鼻突的骨质过度生长而变窄。这一过度生长通过计算机断层扫描得以证实。在先前报道的6例病例中，长达16个月的随访显示面部生长正常。我们描述了6例先天性鼻梨状孔狭窄患者。在乳牙萌出时，6例患者中有4例被发现有一颗突出的上颌中切牙，即“巨切牙”。计算机断层扫描证实了这种牙齿异常以及上颌骨骨质过度生长。单一的上颌中切牙已被描述为全前脑畸形的一种常染色体显性微形态，这是一种发育领域缺陷。我们提出，在一些患者中，先天性鼻梨状孔狭窄可能不仅仅是一种孤立的气道先天性异常，实际上可能是一种伴有相关内分泌和中枢神经系统异常的面中部发育不全。
