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A long surviving case of holoprosencephaly agnathia series
T Kamiji1, T Takagi, T Akizuki
1Department of Plastic and Reconstructive Surgery, Tokyo Metropolitan Police Hospital, Japan.
British Journal of Plastic Surgery
|July 1, 1991
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.