Very rare. Congenital narrowing of the anterior part of the bony nose, just behind the nostrils: it is due to hypertrophy of the nasal and maxillary processes, and to their medial displacement. This pear-shaped hole is the anterior bony opening of the nose while the choanae form its posterior opening.

    piriform aperture (in red)

The malformation is usually bilateral and symmetrical. It is sometimes associated with other malformations of the median line as:

- solitary median maxillary central incisor (see this term)

- holoprosencephaly (see this term)

- eye anomalies.

Signs: in case of severe narrowing, nasal obstruction and respiratory distress at birth; later respiratory discomfort or during breastfeeding in case of moderate stenosis. This respiratory discomfort subsides when crying because respiration is then through the mouth.

Diagnosis: clinical examination of the nose, often using a small fibroscope, permits to detect the narrowing of the anterior part of the nose. CTscan confirms the diagnosis, putting the bone shrinkage on evidence and allows the diagnosis of any associated abnormalities.

In case of important feeding difficulties, a surgical treatment (bony enlargement by the sublabial route) may be necessary.

Anesthetic implications:

exclude associated anomalies; risk of respiratory obstruction at induction of anesthesia (an oropharyngeal cannula is useful, as in case of choanal atresia); nasal intubation is impossible before surgical repair

References:        

-        Trigui L, Ben thabet A, Gargouri A, Hmida N, Ben Hamad N, Rekik A.
Sténose congénitale de l’orifice piriforme.
Rev Stomatol Chir Maxillofac 2010 ; 111 : 155-7.

-        Thomas E., Gibikote S., Panwar J.S., Mathew J.
ACongenital nasal pyriform nasal aperture: A rare cause of nasal airway obstruction in a neonate.
Indian J Radiology and Imaging 2010; 20(4):266-268

Updated: November 2017