A long surviving case of holoprosencephaly agnathia series.

Abstract

The rare syndrome of the agnathia with microstomia, aglossia, synotia (the external ears approaching one another in the midline) and brain malformation (agnathia-holoprosencephaly) was reported by Pauli et al. (1983) as a developmental field defect. This syndrome has two subgroups. One is more severe with brain malformation (holoprosencephaly), and the other is less severe without brain malformation. This report presents a long surviving case of this syndrome without brain malformation.

• Authors: T Kamiji, T Takagi, T Akizuki, M Kurukata, K Ohmori
• Journal: British journal of plastic surgery (Br J Plast Surg) Vol. 44 Issue 5 Pg. 386-9 (Jul 1991) ISSN: 0007-1226 [Print] England
• PMID: 1873621 (Publication Type: Case Reports, Journal Article)

Topics

• Body Height
• Body Weight
• Child, Preschool
• Gastrostomy
• Holoprosencephaly (diagnostic imaging, surgery)
• Humans
• Male
• Mandible (abnormalities, diagnostic imaging, surgery)
• Syndrome
• Tomography, X-Ray Computed
• Tracheostomy