SciELO - Scientific Electronic Library Online

vol.116 issue1Biodegradable airway stent for the treatment of bronchial obstruction in the child: Case reportPaüister-KiUian syndrome in a Mexican mestizo patient: Case report author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand




  • Have no cited articlesCited by SciELO

Related links

  • Have no similar articlesSimilars in SciELO


Archivos argentinos de pediatría

Print version ISSN 0325-0075On-line version ISSN 1668-3501


ILHAN, Ozkan et al. Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: case report. Arch. argent. pediatr. [online]. 2018, vol.116, n.1, pp.e130-e134. ISSN 0325-0075.

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Chromosomal analysis showed heterozygous SIX3 gene deletion at 2p21 region resulting in a more severe form of holoprosencephaly.

Keywords : Congenital nasal pyriform aperture stenosis; Solitary median maxillary central incisor; Holoprosencephaly; Hypopituitarism; Respiratory distress.

        · abstract in Spanish     · text in English | Spanish     · English ( pdf ) | Spanish ( pdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License