Abstract
Congenital nasal piriform aperture stenosis (CNPAS) is an uncommon but important cause of nasal obstruction in infants. When it is severe, nasal decongestants or temporary nasal airway devices cannot address the problem adequately. The most common surgical technique used to treat the bony piriform aperture stenosis is the sublabial approach and drill-out of the nasal aperture followed by nasal stenting. This paper describes an infant with bilateral CNPAS who underwent a single successful balloon dilatation and short-term stenting of the nasal piriform apertures without the need for additional procedures.
1
Introduction
Congenital nasal piriform aperture stenosis (CNPAS) is a rare but important cause of nasal obstruction in infants. It is secondary to bony overgrowth of the nasal process of the maxilla accompanied by an abnormal development of the palate and is usually bilateral. There is a bony ridge along the undersurface of the palate that points to an etiology of abnormal overlapping of the lateral palatal shelves during fusion of the secondary palate . Nasal obstruction caused by CNPAS is usually severe and cannot be adequately addressed with nasal decongestants or temporary nasal airway devices that would help in mucosal causes of obstruction.
Infants are obligate nasal breathers and bilateral nasal obstruction cannot be tolerated for a long period of time. Most children quickly suffer from respiratory compromise and associated problems including apneas, cyanosis, tachypnea, desaturations, poor feeding, aspiration, and failure to thrive. Most children are diagnosed at birth or shortly after. The initial suspicion is raised when there is failure to pass a nasal soft catheter. In some instances where rigid catheters are used, the persistence of effort trying to pass the catheter results in severe damage to the nasal mucosa and intranasal scarring. Properly sized nasal trumpets to provide a temporary nasal airway cannot be inserted without causing mucosal damage and subsequent scarring. The conservative management of bilateral CNPAS early in the infant’s life has to be quickly abandoned to prevent impending complications.
The most common surgical technique used to address the bony piriform aperture stenosis is the well-established sublabial approach and drill-out of the bone followed by nasal stenting of variable duration . However, the procedure carries risks including risks of surgery at a very young age, and possible future maxillofacial and dental growth disturbances. In rare cases when the CNPAS is also associated with a central mega-incisor, hypopituitarism, and/or intracranial abnormalities including holoprosencephaly; in these instances, the surgical risk becomes incalculable at the time surgery is needed.
Diagnosis is made by CT imaging and examination. On CT scans, the piriform aperture width is measured. If the combined piriform aperture width on CT is less than 11 mm in a term infant, this is diagnostic for CNPAS. Abnormal dentition such as a central mega-incisor or the presence of a midline bony ridge along the underside of the hard palate is confirmatory imaging findings .
In very young children, there is considerable maxillofacial plasticity in the bony and cartilaginous structures. Pre-surgical nasoalveolar molding (PNAM) used by one of the authors (A.A.G.) and other cleft surgeons , take advantage of this property in children with wide lip and palatal clefts. PNAM is used to mold and shape the alveolus, primary palate and nasal cartilaginous structures prior to definitive surgical repair of the lip and palate. This is done with great success. With the help of PNAM, severe deformities of palatal rotation as well as nasal deformities associated with wide clefts of the lip and palate have been successfully managed to reduce the need for surgeries such as lip adhesion prior to definitive repairs, and in some instances, alveolar ridge bone grafting. Most success with PNAM is accomplished with superior aesthetics of the subsequent lip repair (author’s subjective opinion) through elimination of repair under tension, and the reduction in the number of lifetime surgical procedures (palatoplasty combined with gingivoperiosteoplasty and lip repair combined with nasal alar and septal repair).
Maxillofacial plasticity is the property that makes success with PNAM possible and the same properties in the very young child can be exploited to treat CNPAS with balloon dilation. This paper describes the author’s experience in an infant with bilateral CNPAS who underwent a single successful balloon dilatation of the stenotic nasal piriform apertures without the need for open sublabial surgery. To date the child remains asymptomatic (9 months) and has patent nasal airways bilaterally. Although the experience in one patient is painfully insufficient to draw any firm conclusions, the relatively non-invasive nature and success of the procedure prompted this publication in anticipation of the adoption of this technique by peers to establish efficacy in a greater number of children.
2
Case report
A 2-week-old white male baby was referred from a regional hospital for evaluation and treatment of nasal obstruction. He was full term with an Apgar score of 8 and 9 and a weight of 4098 grams. He was referred to the local neonatal intensive care unit (NICU) due to respiratory distress, suspected sepsis, and abnormal initial CBC (WBC 25,000, CRP elevated, 10% band form leukocytes, Htc 56.7%). He did not need oxygen and was never intubated. Nasal catheters could not be passed. He was diagnosed with nasal edema and possible choanal stenosis/atresia. His workup was otherwise negative and he was discharged home a week later with recommendation to follow-up with a pediatric ENT for further evaluation and treatment. A nasal decongestant spray (Little Noses—oxymetazoline), proton-pump inhibitor (Prevacid—lansoprazole), and nasal saline were prescribed.
The parents stated that he did well initially after discharge. He took about 2–3 ounces of formula every 3 hours. However, they observed that he struggled with breathing during feedings and that they had to stop to help him breathe. He was very fussy and woke up crying several times per night. Nasal decongestant helped him calm down and get back to sleep. They had been using the decongestant at least twice a day. Cyanosis and apnea had not been observed. They state that he always seemed congested and that he did have retractions while breathing that seemed to have gotten worse. The father is an emergency medical technician (EMT) and states that he had noticed him using his accessory muscles to breathe.
There is no family history of congenital defects, but dad states that both he and his father had small noses as infants (however neither ever required further evaluation or surgery).
He presented at 2 weeks of age to our outpatient clinic. Exam results are as follows: BP, 84/47 mm Hg; pulse, 140/min, temperature, 97.9 °F (36.6 °C), and respiratory rate, 24/min. No weight change, fatigue, fever, chills or night sweats were reported.
Nasal examination showed small nasal vestibules and anterior rhinoscopy was not successful even with the smallest ear speculum. Nasal endoscopy after decongestion and topical anesthesia was attempted but was also not successful. A 2.5-mm pediatric flexible endoscope and a 2.7-mm rigid Hopkins rod could not be advanced through either nasal vestibule. In addition, a soft PVC #14 FR (Rusch, fixed flange) nasal trumpets could not be passed.
The patient was admitted for observation, CT scan, and airway evaluation. The non-contrast axial CT scan showed CNPAS with a combined bilateral nasal vestibular aperture width of 4.08 mm ( Fig. 1 ). Direct laryngoscopy showed a normal larynx. Bronchoscopy showed normal trachea and bronchi. Nasal endoscopy was attempted but the nasal passages were very narrow, left side narrower than the right side. A #4Fr red rubber catheter was passed through the right nose with lubrication and guidance using the 2.7-mm 0° Hopkins telescope. A soft PVC #12 FR nasal trumpet (length 60 mm) was passed using the #4 FR red rubber catheter as a guide.
