Congenital Pyriform Aperture Stenosis: Not All Patients Require Open Repair

Evaluate the management options for congenital nasal pyriform aperture stenosis (CNPAS).

Case series.

Tertiary referral children's hospital.

The medical records of CNPAS patients managed from January 2015 to 2023 were reviewed for medical history, management, and outcomes.

Fifteen patients met inclusion criteria. Six patients (40.0%) had a central mega incisor and 4 (26.7%) had holoprosencephaly. Three (20%) patients were managed with medications alone. Mean PA width was similar between patients managed medically versus those managed surgically (mean width 6.2 ± 0.15 mm vs 6.1 ± 0.38 mm). Median age at first procedure was 50 (range 8-263) days. Seven (46.7%) underwent an initial balloon nasal dilation and 5 (33.3%) underwent an open sublabial repair. Operative time was lower in the balloon dilation group (mean 27 vs 104 minutes). Five patients had a single balloon dilation and 2 required revision procedures: 1 underwent a second balloon dilation, and 1 patient underwent a subsequent open sublabial procedure. Four patients with an initial open sublabial approach returned to the operating room for nasal balloon dilation or debridement due to nasal synechiae.

There has been general shift towards minimally invasive surgery. In our series, 8 (53.3%) patients were managed successfully with medical therapy or a single nasal balloon dilation procedure. Of those who underwent an open surgical repair, most (4/5) required a revision procedure due to synechiae. Given the relative success and lower operative time of balloon dilation, this option should be considered in the management algorithm for CNPAS.