Congenital nasal pyriform aperture stenosis in association with solitary median maxillary central incisor: unique radiologic features
نویسندگان
چکیده
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.
منابع مشابه
Anatomical consideration of the congenital nasal pyriform aperture stenosis: localized dysostosis without interorbital hypoplasia.
Congenital nasal pyriform aperture stenosis may be isolated or associated with other midline anomalies. The aim of the study was to describe the measurements and features of the interorbital structure and midface in congenital nasal pyriform aperture stenosis. The computed tomography scans of eight patients (two girls and six boys) were retrospectively reviewed. Several distances were obtained ...
متن کاملClinical course and implications of congenital nasal pyriform stenosis and solitary median maxillary central incisor in a newborn: a case report
INTRODUCTION Congenital nasal pyriform aperture stenosis and solitary median maxillary central incisor are uncommon anomalies and are associated with further malformations. Solitary median maxillary central incisor itself has initially no impact on a child's health, but congenital nasal pyriform aperture stenosis is a potentially life-threatening condition. CASE PRESENTATION A Caucasian baby ...
متن کاملSolitary median maxillary central incisor (SMMCI) syndrome
Solitary median maxillary central incisor syndrome (SMMCI) is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35th-38th day(s) from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI. The ...
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