A.
K Gupta et al
Calicut Medical Journal 2011; 9(1): e8
Case Report
Congenital nasal pyriform aperture stenosis: a rare cause of neonatal nasal
obstruction
Anil Kumar Gupta, Hari Singh, Aneesh Mathew Manalel, Nitin Kumar Jaiswal
Department of Radiodiagnosis, S. N. Medical College, Agra, India
__________________________________________________________________________________________
Abstract
Congenital nasal pyriform aperture stenosis
(CNPAS) is a recently described and an unusual
cause of airway obstruction in neonates as a result
of narrowing of the nasal pyriform aperture. CNPAS
is thought to represent a microform of
holoprosencephaly. It may be confused with
choanal atresia which is much more common,
however the diagnosis is usually clearly established
by CT. We describe a case of full-term newborn
infant with CNPAS presented with respiratory
distress after delivery.
Key words: Nasal cavity, pyriform aperture,
holoprosencephaly, mega-incisor, CNPAS.
Introduction
Congenital nasal pyriform aperture stenosis
(CNPAS) was first described by Brown et al. in
1
1989 . CNPAS is a rarer cause of nasal bony
obstruction in neonates. The diagnosis may be
suggested by history and physical examination;
however, it should be confirmed radiologically by a
CT scan of the nasal cavity. It has been suggested
that a pyriform aperture width less than 8 mm in a
term infant is diagnostic of CNPAS. This anomaly
has been reported as an isolated feature or can be
associated with craniofacial or central nervous
2
system anomalies . If a single central maxillary
mega-incisor is present, intracranial defects are
3
more likely .
Case report
A full term female baby was born by normal vaginal
delivery to a 22 year-old multipara woman. The
antenatal history was
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uncomplicated. The birth weight was 2.6 kg. During
initial assessment by the paediatrician, she was
noted at birth to have respiratory difficulty in the
form of increased respiratory rate (68/min) and
noisy breathing which was relieved on crying. There
was no cyanosis and child was maintaining oxygen
saturation (SpO2 >95%). A 6F naso-gastric tube
was passed with difficulty on right side, but could
not be passed on left side. For further evaluation
she was shifted to neonatal intensive care unit and
put on saline nebulisation. On examination the
external nose and nasal vestibular opening were
normal. There was no dysmorphic features.
Probable diagnosis of unilateral (left sided)
choanal atresia was made. CT examination of
nasal cavity and paranasal sinuses was advised to
evaluate for choanal atresia or any other structural
anomalies.
The CT scan demonstrated bony overgrowth and
medial approximation of nasal processes of
maxillary bone causing severe narrowing in bilateral
pyriform apertures, which together measured 4mm
in width at the level of inferior meatus (Fig 1a & 1b).
No occlusion is seen in choanal region on either
side. No evidence of single central maxillary mega-
incisor noted.
After 3 days of supportive treatment the patient
improved clinically; respiratory rate stabilised (40-
50/min), noisy breathing reduced, started feeding
normally and gaining weight. So, the patient was
discharged and advised review if symptoms
worsen.
A.K Gupta et al
Calicut Medical Journal 2011; 9(1): e8
a b
Fig 1a & b: Axial (a) and coronal (b) CT scan of nasal cavity and paranasal sinuses showing narrowing of
pyriform aperture due to bony overgrowth of nasal process of maxillary bone (arrows).
Discussion
The pyriform aperture is the narrowest, most
anterior portion of the nasal airway, so a slight
decrease in its cross sectional area will significantly
increase the nasal airway resistance. A bony
overgrowth of the maxillary nasal processes that
leads to narrowing of the bony part of nasal cavity is
thought to be responsible for this deformity. Initially
CNPAS was considered an isolated deformity;
subsequently it was found to have an association
with holoprosencephaly. This hypothesis is based
on the observation that solitary central upper
incisor, one of the manifestations of
holoprosencephaly, is found in many cases of
2
CNPAS . Some reports described cases associated
with endocrine affections, such as growth hormone
deficit, thyroid dysgenesis, hypothyroidism and
episodes of hypoglycemia and absence of anterior
1
hypophysis with panhypopituitarism . So, there are
two forms of CNPAS: the isolated form and the form
4 5 .
associated with other abnormalities . The presence
of associated brain and pituitary anomalies should
be excluded by the mean of MRI and endocrinologic
evaluation. In the work up of CNPAS it is
considered that the presence of central mega-
incisor is the indication for further workup with brain
MRI3. Considering the fact that there was no
evidence of central mega-incisor or other clinical
features suggestive of CNS abnormality our case
was assumed as an isolated case of CNPAS and
MRI of brain was not done.
CNPAS is the cause of neonatal nasal obstruction
ranging from mild to severe. The CNPAS presents
with respiratory distress, episodic apnoea, cyclic
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cyanosis and feeding difficulties. These signs and
symptoms occur in bilateral choanal atresia, a well-
known etiology, and therefore, it could be the
etiology assumed by the neonatologist. So,
immediate recognition of this condition and
appropriate treatment are necessary to prevent
asphyxia. CNPAS may be suspected clinically by
nasal deformity with failure to pass a nasogastric
tube more than 1cm into the nasal fossae1.
The diagnosis of pyriform aperture stenosis can be
made accurately with CT scan, by obtaining thin
(1.5-3 mm) contiguous axial sections in a plane
parallel to anterior hard palate. Direct coronal
imaging is useful but the diagnosis is readily made
on the basis of the transverse CT findings. It is
important to demonstrate the narrowing on
contiguous sections, as apparent narrowing may be
caused by oblique imaging. The normal range of
width of pyriform aperture in age group of 0-6
months is 8.8- 17.2 mm (median width-13.5 mm) .
Each pyriform aperture width less than 3 mm or
whole pyriform aperture width less than 8 mm
4
confirms the diagnosis of CNPAS . CT may also
show a hypoplastic triangular hard palate with a
marked mid-palatal vomerine ridge and a single
median maxillary incisor tooth (mega-incisor) 6.
Mild cases of CNPAS can be treated
conservatively, with saline humidification and topical
nasal decongestants, until their nasal cavities grow
and overcome the obstruction1,2 . Surgery is
indicated in cases of severe respiratory distress,
feeding difficulties, and when there is no benefit
with conservative methods. Surgical management
A.K Gupta et al
Calicut Medical Journal 2011; 9(1): e8

of CNPAS includes dilation and stenting or management is necessary to relieve respiratory

resection of inferior margin of the bone in anterior
nasal aperture through a sub labial approach to the
pre-maxilla depending upon the extent of stenosis 2.
Conclusion
CNPAS is an uncommon but an important cause of
neonatal nasal obstruction. This entity should be
kept in the differential diagnosis of any neonate or
infant with signs and symptoms of upper air way
obstruction. Early diagnosis and timely
distress and preservation of patients lives.
Diagnosis may be suggested by physical
examination. However, final conclusion is reached
only using CT scan. In all cases of CNPAS,
especially which are associated with single central
maxillary mega-incisor, a multidisciplinary approach
is required to rule out other craniofacial, brain or
endocrine abnormalities.

__________________________________________________________________________________________
Competing interest: none

Funding:none

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Corresponding author
Aneesh Mathew M
Department of Radiodiagno S. N. Medical College, Agra
Phone no. - 09759471380
E mail anish1364@gmail.com

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