UNASSIGNED: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that results in neonatal respiratory difficulty. The purpose of this systematic review was to compare surgical outcomes of drilling versus dilation techniques in the treatment of CNPAS.
UNASSIGNED: Pubmed, Embase, and Cochrane Clinical Trials databases were searched for terms \"congenital nasal pyriform aperture stenosis\" or \"pyriform aperture stenosis\" from 2010 to 2021. Twenty-five studies were included that evaluated pediatric patients treated surgically for CNPAS with available outcomes data including complications, revisions, and length of stay.
UNASSIGNED: A total of 51 patients with CNPAS were pooled from included studies. The median age was 29 days, 56.9% were female, and 54.9% were born full-term. The median pyriform aperture width before surgery was 5.00 mm (IQR = 4.10, 6.45). Forty (78.4%) patients underwent sublabial drilling, while 6 had a dilation procedure performed with hegar cervical dilators, 2 had a balloon dilation, and 3 were dilated with either an acrylic device, endotracheal tube, or bougie. There were no post-operative complications for 76.5% of patients, while a second surgery was required in 9 (17.6%) patients. The median length of stay was 11 days (IQR = 4, 26). No statistically significant difference was observed between sublabial drilling and surgical dilation techniques with respect to complications, need for revision surgery, or length of stay.
UNASSIGNED: Current literature is insufficient to determine if drilling or dilation is more effective in the treatment of CNPAS.

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal respiratory distress. We report a case series of four infants with similar radiological dimensions but while two needed surgery, two could be managed conservatively. The clinical presentation of the child and the response to conservative treatment, rather than the radiological dimensions were the main predictors for surgical intervention.

Congenital bony nasal cavity stenosis is caused by alterations of the normal embryological development of the nasal cavity. Depending on the site of the obstruction, the most important types of stenosis are: choanal atresia and stenosis, congenital nasal pyriform aperture stenosis, congenital midnasal stenosis, arhinia and nasal septum deviation. Although they are uncommon, they could be potentially life-threatening conditions that require early diagnosis and proper treatment. In case of neonatal nasal obstruction, appropriate differential diagnosis with other causes, such as rhinitis and sinonasal masses, are performed by nasal endoscopy and radiological exams. Treatment strategy consisting of medical nasal therapies and endoscopic or open nasal surgery should be tailored according to the types and the degree of the stenosis. When indicated, endoscopic endonasal approach is considered the most effective technique in neonates warranting minimal surgical invasiveness and maximum effect. In order to promote the management of these rare yet clinically relevant neonatal nasal breath disorders, we review the current trends in diagnosis and treatment of congenital bony nasal cavity stenosis.

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.

Patients with congenital nasal pyriform aperture stenosis (CNPAS) may become less symptomatic with age. Therefore, we aimed to develop a growth curve of the pyriform aperture so that a more comprehensive plan can be designed for CNPAS patients who show little response to conservative treatment.
A single-institution study, retrospective review of CNPAS patients during the period November 1997 to December 2014.
We measured the distances between the bilateral nasal processes of the maxilla (interprocess distance [IPD]) on three-dimensional computed tomography images and then divided the patients into five different age groups. A growth curve of the pyriform aperture was then constructed based on the distance-age relationship.
Fifty-four IPD measurements were included. The mean IPD was 3.57 mm in neonates < 1 month old, 4.08 mm in infants aged 1 to 3 months, 5.19 mm in the 4-month to 11-month age group, 6.61 mm in the 12-month to 36-month age group, and 9.20 mm in children > 36 months of age. We found that the cubic curve was the most appropriate growth curve, and that growth tended to be slower from 3.5 years to 6 years of age.
The growth curve of the pyriform aperture in children with CNPAS developed in this study can aid in treatment planning and predict clinical outcome of CNPAS patients. Although CNPAS patients may become less symptomatic with age, when the observed IPD falls progressively farther from the curve, more aggressive intervention should be considered, such as changing the management strategy from observation to conservative treatment or from conservative treatment to surgery.
4 Laryngoscope, 126:2399-2402, 2016.

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 rare congenital malformation caused by overgrowth of the maxillary bone. We report on two patients, brothers born 3 and 1½ years apart, both presented at birth with radiographically diagnosed CNPAS. Both siblings also were born with ocular albinism, which is known to have X-linked inheritance. Subsequent genetic testing demonstrated a 97 kb deletion in the p arm of the X chromosome in both siblings and their mother. This deletion encompasses a gene known to cause ocular albinism (GPR143), as well as partial deletion of two other genes, TBL1X and SHROOM2. This is the first reported case of CNPAS in siblings, both males, sharing a maternally inherited Xp22.2 deletion.