Case report
A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia

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Abstract

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed.

Introduction

Solitary median maxillary central incisor syndrome is a rare developmental abnormality characterised by the presence of a central megaincisor and other associated midline abnormalities. Nasal cavity defects are well recognised in this syndrome and choanal atresia or pyriform aperture stenosis is usually present. Neonates are obligate nasal breathers and therefore nasal obstruction can be life threatening.

The presence of midline pathologies and neonatal respiratory distress should alert the physician of the possibility of SMMCI syndrome.

We present a case of both choanal atresia and pyriform aperture stenosis co-existing in a neonate with SMMCI syndrome.

Section snippets

Case report

A 2.6 kg term neonate was born by spontaneous vaginal delivery following an uncomplicated pregnancy in a regional hospital. Following delivery she developed tachyopnoea, tachycardia and was diagnosed with an anorectal malformation. Nasal CPAP, antibiotics and intravenous fluids were commenced. The severity of the respiratory distress increased which required intubation, ventilation and transfer to a tertiary centre.

She underwent a divided descending colostomy for an imperforate anus with

Discussion

SMMCI syndrome is a rare developmental disorder characterised by the unique form and position of the upper central incisor. It may occur in isolation but is often associated with multiple midline defects. First described by Scott [1] in 1958 it has an incidence of 1:50,000 live births [2]. Its aetiology is unknown.

Associated abnormalities include midline nasal cavity defects, holoprosencephaly, microcephaly, intellectual disability, congenital heart disease, cleft lip and palate,

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    CT will demonstrate the level of nasal airway obstruction, showing the characteristic narrowing of the anterior bony pyriform aperture, overgrowth and medial displacement of the nasal processes of maxilla, and the nasolacrimal duct may be encased in the abnormally narrowed anterior bony valve area [5,9,13]. The diagnostic criteria is a pyrifrom aperture width of <8–11 mm (measured on axial CT at the level of the inferior meatus, measurements should be performed at the level of the maximum transverse diameter of the pyriform aperture and posterior choanae), or the maximum transverse diameter of each aperture is ≤3 mm in a term neonate [5,8,10,12]. The management of both conditions is dictated by the degree of stenosis and the severity of symptoms.

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