A long surviving case of holoprosencephaly agnathia series.
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.
Kamiji
T
T
Department of Plastic and Reconstructive Surgery, Tokyo Metropolitan Police Hospital, Japan.
Takagi
T
T
Akizuki
T
T
Kurukata
M
M
Ohmori
K
K
D001827:Body Height; D001835:Body Weight; D002675:Child, Preschool; D005774:Gastrostomy; D016142:Holoprosencephaly / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D008297:Male; D008334:Mandible / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014139:Tracheostomy