PURPOSE: We identified four patients with congenital nasal pyriform aperture stenosis (CNPAS) with feeding and respiratory difficulties. While feeding difficulties have been described in infants with other causes of nasal obstruction (e.g., choanal atresia), little attention has been focused on feeding in infants with CNPAS. FINDINGS: All four patients were treated with nasal steroids and nasal saline and two underwent surgical repair of the CNPAS at 2 months of age. Except for gastroesophageal reflux in one patient, upper gastrointestinal tract (UGI) contrast studies were normal in all patients. Three patients underwent bedside swallow evaluations, two of whom participated in videoflouroscopic swallow studies (VFSS) because of limited oral intake or respiratory problems during feeding. These three patients presented with intact oral-motor coordination at rest and during non-nutritive sucking, and nasal whistling, nasal congestion, or breathing difficulties/desaturations with oral feeding. On VFSS, one infant had trace aspiration with thin and thick liquids. Three patients required supplemental feeding during the first year of life. All three patients with normal brain MRIs were successful oral feeders by 16 months of age, the fourth is undergoing feeding therapy at 24 months of age. SUMMARY: Feeding problems are common in infants with CNPAS and supplemental feedings may be needed even after management of airway difficulties. Infants with CNPAS should undergo comprehensive feeding/swallowing evaluations which may include bedside swallow evaluations and VFSSs. The prognosis for transitioning to total oral feedings is good and related to the extent of the airway obstruction and presence of other comorbidities.
PURPOSE: We identified four patients with congenital nasal pyriform aperture stenosis (CNPAS) with feeding and respiratory difficulties. While feeding difficulties have been described in infants with other causes of nasal obstruction (e.g., choanal atresia), little attention has been focused on feeding in infants with CNPAS. FINDINGS: All four patients were treated with nasal steroids and nasal saline and two underwent surgical repair of the CNPAS at 2 months of age. Except for gastroesophageal reflux in one patient, upper gastrointestinal tract (UGI) contrast studies were normal in all patients. Three patients underwent bedside swallow evaluations, two of whom participated in videoflouroscopic swallow studies (VFSS) because of limited oral intake or respiratory problems during feeding. These three patients presented with intact oral-motor coordination at rest and during non-nutritive sucking, and nasal whistling, nasal congestion, or breathing difficulties/desaturations with oral feeding. On VFSS, one infant had trace aspiration with thin and thick liquids. Three patients required supplemental feeding during the first year of life. All three patients with normal brain MRIs were successful oral feeders by 16 months of age, the fourth is undergoing feeding therapy at 24 months of age. SUMMARY: Feeding problems are common in infants with CNPAS and supplemental feedings may be needed even after management of airway difficulties. Infants with CNPAS should undergo comprehensive feeding/swallowing evaluations which may include bedside swallow evaluations and VFSSs. The prognosis for transitioning to total oral feedings is good and related to the extent of the airway obstruction and presence of other comorbidities.