Congenital Nasal Pyriform Aperture Stenosis
Abstract
Introduction:
Congenital nasal pyriform aperture is one of the newborns' main aetiologies of respiratory distress. Immediate diagnosis and intervention are mandatory because of the condition's life-threatening characteristics.
Case presentation:
It is a case of a 26-day full-term male infant admitted to the neonatal icu for severe respiratory distress (stridor, abdominal respiration, rapid and shallow breathing, and chest retractions. The anterior rhinoscopy highlighted a narrowing of the nasal fossa. A nasal ct scan demonstrated a congenital stenosis in the pyriform aperture. The ent surgeon and pediatric physician indicated urgent surgery to correct the pyriform aperture stenosis. The child improved his nasal breathing and was discharged from the icu on the 10th day.
Discussion:
Congenital stenosis of the pyriform aperture (cspa) is one of the main aetiologies of newborn nasal obstruction, and it is due to excessive bony growth of the medial nasal process of the maxilla (2), narrowing the bony part of the nasal cavity. The diagnosis of cspa is based on nasal endoscopy (narrow nasal fossa due to bone protrusion) and a ct scan (confirms the site of
obstruction and shows normal choanae anatomy). The correctional surgical
intervention of cspa consists of pyriform aperture enlargement by endoral
sublabial approach using small burs (2-3 mm).
Conclusion:
Cspa is considered a minor
form of holoprosencephaly other than an isolated deformity as it once was
thought to be.
Keywords: congenital nasal pyriform aperture; abdominal respiration; rhinoscopy
1. Introduction
Congenital nasal pyriform
aperture is one of the newborns' main aetiologies of respiratory distress. It
is due to a bony overgrowth of the nasal lateral process of the maxilla.
Immediate diagnosis and intervention are mandatory because of the condition's
life-threatening characteristics. It is considered a minor form of
holoprosencephaly other than an isolated deformity as it once was thought to
be.
2.
Case
presentation
It is a case of a 26-day
full-term male infant admitted to the neonatal icu for acute respiratory
distress, a weight of 2900 g; at the initial assessment, he showed severe
respiratory distress (stridor, abdominal respiration, rapid and shallow
breathing, and chest retractions) a small nasal tube could not pass anteriorly
as was custom in a normal nasal examination, an anterior rhinoscopy highlighted
a narrowing of the nasal fossa. A nasal ct scan demonstrated a congenital
stenosis in the pyriform aperture (figure
1). Due to the gravity of the respiratory distress and inability to feed
the infant, both the ent surgeon and pediatric physician indicated urgent
surgery to correct the pyriform aperture stenosis. A sublabial approach was
conducted, and as the bony margins were exposed, we used small burs 2.0 and 2.5
mm to widen both pyriform apertures laterally and avoid damage to both the
nasolacrimal duct and floor of the nasal fossa. A 3.5 mm endotracheal tube
passed both pyriform apertures, confirming the procedure's efficacity. We opted
for two silastik-made conformers and silastik nasal splints to avoid adherences
formation (figure 2). The infant was
transferred back to the icu, where he was intubated for four days. We removed
both nasal splints on the 5th day, as the infant was also extubated
on the same day. The nasal conformers were drawn on the 7th day
postoperatively. The child improved his nasal breathing and was discharged from
the icu on the 10th day.
Figure 1. Axial section showing pyriform stenosis and nasal fossa obstruction.
Figure 2. Postoperative dressing and nasal conformers positioning
3. Discussion
Neonates are nasal breathers,
and any cause of nasal obstruction could have severe respiratory consequences1. Congenital stenosis of the
pyriform aperture (cspa) is one of the main aetiologies of newborn nasal
obstruction, and it is due to excessive bony growth of the medial nasal process
of the maxilla2, narrowing the bony
part of the nasal cavity. The main symptomatic presentation is repeated spells
of cyanosis relieved with a cry1,3.
The diagnosis of cspa is based on nasal endoscopy (narrow nasal fossa due to
bone protrusion) and a ct scan (confirms the site of obstruction and shows
normal choanae anatomy) 4.
The treatment of cspa depends
on the initial evaluation of the child. In mild cases, we used conservative methods
such as nasal decongestants and humidification3;
otherwise, severe cases of respiratory distress need endotracheal intubation
and surveillance in icu before an eventual surgical repair6. The correctional surgical intervention of
cspa consists of pyriform aperture enlargement by endoral sublabial approach
using small burs (2-3 mm)7. Using
nasal conformers and silastik nasal splints is usual to prevent scar-related
stenosis and to reduce recurrences and must be retained for at least 6-7 days8.
Surgical complications consist
essentially of damage to the dental germs and nasolacrimal duct, which can be
preven
Ted by drilling anteriorly to the head of the inferior turbinate and avoiding injuring the mucosa of the nasal floor5.
4. Conclusion
Cspa should be considered a potential cause of nasal obstruction in newborns when there are signs of respiratory distress and difficulty passing a small catheter in the anterior nasal valve. Ct is the gold standard examination to confirm the diagnosis, and the therapeutic intervention could vary from conservative to surgical procedure depending on the initial assessment.
References