Congenital nasal pyriform aperture stenosis in association with solitary median maxillary central incisor: unique radiologic features

Abstract

Solitary median maxillary central incisor (SMMCI) coexists in 34%-65% of patients initially diagnosed with congenital nasal pyriform aperture stenosis. SMMCI, a genetic syndrome, warrants consideration for further screening because of its high prevalence of other diagnostic possibilities—specifically central defects, like nasal obstruction and hypothalamo-pituitary axis abnormalities. We report on a presentation of SMMCI with congenital nasal pyriform aperture stenosis which highlights the unique radiologic features and notes the relationship between these two central associated findings in the literature.

Solitary Median Maxillary Central Incisor due to Nasal Pyriform Aperture Stenosis in Fetus: The First Prenatal Ultrasound Case Report

“… 4 As mentioned in the introduction part, the single median incisor is a rare dental abnormality that could be isolated or could be part of many different syndromes or syndromic association such as the SMMCI syndrome, CHARGE, VACTERL, or velocardiofacial syndrome. 1 – 3 Solitary median maxillary central incisor syndrome is a rare complex syndrome that consists of multiple developmental defects mainly affecting the midline, including the cranial bones, the maxillary and its dentition (specifically the roots of the central incisors), the nasal airways (choanal atresia, midnasal stenosis, or congenital pyriform aperture stenosis), 5 – 8 and sometimes the brain (holoprosencephaly), associated with other midline structures of the body (cleft lip or palate, esophageal atresia, abnormalities of the external genital organs). 2 These children are also usually small in size, often born preterm, hypotrophic, or born from a diabetic mother.…”

A Rare Cause of Neonatal Respiratory Distress: Congenital Stenosis of Piriform Aperture

“…The majority of these obstructions result from choanalatresia, which affects 1 in 8000 live births [1]. CNPAS is a rare and unsusal cause of nasal obstruction that occurs around 1 in 25,000 births [3].…”

“…The exact etiology remains unclear. Chromosomal abnormalities with HPE genes have been reported in some cases [3]. Pyriform aperture stenosis occurs due to embryological malformations of the primary palate, which are associated with a hard triangular-likepalate and the bone over growth of the nasal process of the maxilla [4].…”

Congenital nasal pyriform aperture stenosis: a rare cause of upper airway obstruction in newborn

“…Although commonly described as arising from overgrowth of the nasal process of the maxilla, some authors defend that it results from medialisation of normally shaped maxilla 1. It is associated with other conditions and midline defects like solitary median maxillary central incisor (SMMCI) and holoprosencephaly 1 2…”

“…The diagnosis is suggested by physical examination due to failure to pass a nasogastric tube or slim nasendoscope within the first 1 cm of the nostrils and confirmed by CT findings of pyriform aperture width <11 mm (measured on an axial level of the inferior meatus) in a full-term neonate 1 2. The main differential diagnose is choanal atresia that is a congenital narrowing of the posterior nasal airway by a bony or membranous septum 1 2. First-line treatment is based on airway measures such as nasal saline solutions, decongestants or intranasal steroids, tried at least for 15 days 1.…”