Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in newborns. This paper reports a case of severe CNPAS that required endotracheal intubation immediately after birth, and eventually, surgical intervention. At birth, the width of the pyriform aperture was only 4 mm, and the patient was completely unable to breathe through his nose. We performed tracheostomy at 23 days of age and waited for the patient to grow, but at 56 days of age, the width of the pyriform aperture was not sufficient (6 mm) for the patient to breathe through his nose. Therefore, surgical dilation of the pyriform aperture by a sublabial approach was performed on day 79 after birth, and the width was increased to 14 mm. Postoperative stent placement was performed for two weeks. After the removal of the stents, the patient could finally breathe through his nose, and the postoperative course was uneventful, with no restenosis after four months. CNPAS is a rare cause of nasal obstruction, but it can cause respiratory distress in infants because they are dependent on nasal breathing. Conservative treatments are initially recommended for CNPAS; however, in severe cases where conservative treatments are ineffective, surgical treatment is recommended.

UNASSIGNED: The single median incisor is a rare dental abnormality that could be isolated or could be part of many different syndromes or syndromic association with poor prognosis.
UNASSIGNED: We report the first prenatal ultrasound description of a 31-year-old patient, gravida 1, para 0, whose male fetus was diagnosed at 25 weeks\' gestation with a single median incisor suggestive of nasal pyriform aperture stenosis in Montpellier University Hospital (France). A fetal magnetic resonance imaging (MRI) performed at 30 weeks\' gestation retrieved no intracranial midline cerebral anomalies and confirm nasal pyriform aperture stenosis suspicion. Amniocentesis, performed at 31 weeks, found a normal fetal karyotype (46XY) and a normal comparative genomic hybridization (CGH) array. After term vaginal delivery, clinical and radiological examination confirmed the diagnosis of an isolated single median maxillary central incisor linked to nasal pyriform aperture stenosis.
UNASSIGNED: Prenatal diagnosis of a single median incisor due to nasal pyriform aperture stenosis is feasible and enables close postnatal follow-up.
UNASSIGNED: Fuchs F, Chadelle M, Captier G, et al. Solitary Median Maxillary Central Incisor due to Nasal Pyriform Aperture Stenosis in Fetus: The First Prenatal Ultrasound Case Report. Int J Clin Pediatr Dent 2020;13(3):295-298.

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Chromosomal analysis showed heterozygous SIX3 gene deletion at 2p21 region resulting in a more severe form of holoprosencephaly.
El síndrome del incisivo central único de la línea media del maxilar es un trastorno raro que implica anomalías de la línea media, como holoprosencefalia, anomalías de las fosas nasales, fisura palatina, labio leporino, hipotelorismo, microcefalia y panhipopituitarismo. La estenosis congénita del orificio nasal anterior es una causa mortal de dificultad respiratoria neonatal debido al estrechamiento del orificio nasal anterior, y podría confundirse con la atresia de coanas. En este informe, presentamos el caso de un recién nacido con síndrome del incisivo central único de la línea media del maxilar acompañado de otras anomalías, tales como holoprosencefalia, estenosis del orificio nasal anterior, microcefalia y panhipopituitarismo. El cariotipado mostró una deleción heterocigota en el gen SIX3 en la región 2p21, que produjo una forma más grave de holoprosencefalia.

OBJECTIVE: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of upper airway obstruction in the newborn. CNPAS is diagnosed clinically and confirmed with CT scanning. Early diagnosis and management is essential for this potentially life-threatening condition. Patients can be managed conservatively or surgically. Surgical treatment is usually reserved for those patients that fail conservative treatment. Our objective was to provide a radiologically-measured pyriform aperture (PA) width that predicts the need for surgical intervention.
METHODS: This study was a retrospective chart review of patients treated in a tertiary paediatric hospital as well as a review of the literature. Outcome measures were defined as surgical or conservative intervention for the management of congenital pyriform aperture stenosis.
RESULTS: Data from 26 individual patients (7 patients from our own case series and 19 patients from previously published reports) was analysed to calculate those patients requiring surgical intervention.
CONCLUSIONS: A PA width of less than 5.7 mm in a neonate is 88% sensitive and specific in predicting that a patient will require surgical intervention.

Congenital nasal pyriform aperture stenosis (CNPAS) is a recently defined clinical entity that causes airway obstruction in the neonate as a result of narrowing of the nasal pyriform aperture. The pyriform aperture is the narrowest, most anterior portion of the nasal airway, and a slight decrease in its cross sectional area will significantly increase the nasal airway resistance. This entity should be kept in the differential diagnosis of any neonate or infant with signs and symptoms of upper air way obstruction. The CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclic cynosis.