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

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.

• Authors: Kate Blackmore, David M Wynne
• Journal: International journal of pediatric otorhinolaryngology (Int J Pediatr Otorhinolaryngol) Vol. 74 Issue 8 Pg. 967-9 (Aug 2010) ISSN: 1872-8464 [Electronic] Ireland
• PMID: 20626079 (Publication Type: Case Reports, Journal Article)

Topics

• Abnormalities, Multiple (diagnosis, therapy)
• Choanal Atresia (diagnosis)
• Combined Modality Therapy
• Constriction, Pathologic (congenital, diagnosis)
• Female
• Growth Disorders (congenital, diagnosis)
• Holoprosencephaly (diagnosis)
• Humans
• Infant, Newborn
• Magnetic Resonance Imaging (methods)
• Maxilla (abnormalities)
• Nasal Obstruction (congenital, diagnosis)
• Prognosis
• Rare Diseases
• Respiratory Distress Syndrome, Newborn (diagnosis, etiology)
• Syndrome
• Tomography, X-Ray Computed (methods)