International Journal of Pediatric Otorhinolaryngology
Case reportA case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia
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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Cited by (21)
Orthodontic space closure in a young female patient with solitary median maxillary central incisor syndrome
2021, American Journal of Orthodontics and Dentofacial OrthopedicsCitation Excerpt :However, a prosthodontic replacement for a maxillary central incisor with either a resin-bonded bridge or an implant-borne crown after orthodontic space opening is a more aggressive procedure than the only minimally invasive restoration of the anterior dentition after orthodontic space closure or spatial distribution. Although many clinical papers about the SMMCI syndrome have been published, to our knowledge, up to date, no single case report with the extraction of the SMMCI and orthodontic space closure is available in the peer-reviewed literature.12-14,22-27 Space closure in association with minimally invasive restorative treatment bears several advantages over the space opening alternative, especially that the major part of the entire treatment is already finished during the patient's teenage years, and that no removable or fixed interim substitution of the missing tooth with the inherent risk of root approximation during the preimplant retention time, as reported by Olsen and Kokich,28 is necessary.
Congenital nasal pyriform aperture stenosis repair: A case series and discussion of postoperative care
2021, Otolaryngology Case ReportsCitation Excerpt :Nasal cannulas and masks require fixation to the neonate and therefore cannot be used effectively following CNPAS repair [26] [Fig. 2]. Length of stenting is highly debated in literature, some publications recommending up to 28 days [2,8,10,22,27–29]. With use of this rigorous postoperative care regimen, we recommend stent removal as soon as POD 5 with an additional 48 hours of inpatient observation before discharge to ensure that restenosis and mucosal dehiscence do not occur.
Congenital nasal obstruction due to pyriform aperture stenosis. A case series
2015, Anales de PediatriaCongenital piriform aperture stenosis and odontogenic disorders
2013, Revue de Stomatologie, de Chirurgie Maxillo-faciale et de Chirurgie OraleCongenital nasal pyriform aperture stenosis: A report of 10 cases and literature review
2012, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Once adequately widened, stents are placed to avoid restenosis. In our unit, we have successfully used size 3.5 portex endotracheal tubes fashioned as nasal stents with a common anterior cuff [4]. Nasal stents were cited further back if a choanal atresia repair was also performed.
Congenital choanal atresia and pyriform aperture stenosis
2011, International Journal of Pediatric Otorhinolaryngology ExtraCitation Excerpt :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.