Acute Respiratory Distress in the Newborn: Congenital Nasal Pyriform Aperture Stenosis the Underlying Pathology

Abstract

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal upper airways obstruction. It can occur as an isolated anomaly or be associated with other cranial-facial defects. Clinically, CNPAS manifests as apneic crises, episodic cyanosis, and difficulty in feeding. Here we report a newborn developing respiratory distress after birth that required oxygen support and was confirmed diagnosis of CNPAS by computed tomography (CT) scan. The newborn had successfully undergone surgical intervention via the nasolabial approach.

Navigating Congenital Nasal Obstruction: A Contemporary Surgical Paradigm

“…Although it is a very rare cause of neonatal airway obstruction, the diagnosis of CNPAS in this newborn was slightly delayed [6]. The possibility of CNPAS arose when the pediatric team was unable to wean the child off of oxygen.…”

“…It is a pear-like-shaped opening bounded superiorly by the nasal bone, inferiorly by the palatine process of the maxilla, and laterally by the nasal process of the maxilla. The etiology of the pathology is uncertain; however, it appears to be due to maxillary nasal process enlargement in the fourth month of fetal development [6].…”

“…It may occur alone or with other congenital anomalies such as holoprosencephaly, cleft palate, SMMCI, and central nervous system and endocrine problems [6]. 14-66% of CNPAS children have SMMCI [6].…”

“…It may occur alone or with other congenital anomalies such as holoprosencephaly, cleft palate, SMMCI, and central nervous system and endocrine problems [6]. 14-66% of CNPAS children have SMMCI [6]. Both posterior choanal atresia and CNPAS cause nasal airway obstruction and cyclical cyanosis that improves with crying and worsens with feeding.…”

“…There are currently no reliable criteria for determining whether CNPAS warrants surgical treatment. Lin et al propose that not all symptomatic CNPAS cases necessarily require surgery [6]. Indications for surgery include failure to thrive, dependence on supplemental oxygen, obstructive sleep apnea, and cyanosis [7].…”