Pyriform Aperture Growth Curve and CNPAS in Kids

Can a more comprehensive treatment plan be based on a growth curve of the pyriform aperture for patients with congenital nasal pyriform aperture stenosis (CNPAS)?

Bottom Line
The growth curve of the pyriform aperture in children with CNPAS can aid in treatment planning and predict clinical outcome, but the severity of clinical symptoms rather than the absolute distance between bilateral nasal processes of the maxilla is the most important factor for determining treatment.

Background: Because CNPAS can be life threatening for obligate nasal breathers, early diagnosis is imperative. Computed tomography (CT) and nasal endoscopy are the imaging modalities of choice for establishing a CNPAS diagnosis, but there are no definitive criteria for diagnosing the anomaly. It is difficult to decide on the most appropriate treatment due to a lack of data on the natural course of pyriform aperture development.

Study design: Single-institution retrospective review of CNPAS patients from November 1997 to December 2014.

Setting: Mackay Memorial Hospital, Hsinchu, Taiwan.

Synopsis: Patients were divided into five different age groups: Group 1 (neonates <1 month of age); Group 2 (infants between 1 and 4 months); Group 3 (infants between 4 and 12 months; Group 4 (children between 12 and 36 months); and Group 5 (children >36 months). Fifty-four measurements were collected (Group 1=16, mean interprocess distance (IPD) 3.57 mm; Group 2=15, mean IPD 4.08 mm; Group 3=8, mean IPD 5.19 mm; Group 4=10, mean IPD 6.61 mm; Group 5=5, mean IPD 9.20 mm). There were no significant IPD differences between Groups 1 and 2, but there was a marginally significant difference between Groups 1 and 3, indicating that pyriform aperture widening begins at around 4 months of age. The most commonly reported anomaly presenting with CNPAS was holoprosencephaly (HPE). Other associated abnormalities included midline nasal cavity defects, microcephaly, intellectual disability, congenital heart disease, cleft lip and palate, hypopituitarism, hypotelorism, esophageal/duodenal atresia, ambiguous genitalia, and short stature. Surgery indications include the presence of sleep apnea, extubation failure, inability to insert a feeding tube, frequent feeding difficulties with cyanosis, and nonresponsiveness to conservative management.

Citation: Lin KL, Lee KS, Yang CC, et al. Natural course of congenital nasal pyriform aperture stenosis. Laryngoscope. 2016;126:2399–2402.