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"The face often predicts the brain" [5, 110]. Parallelism of face and brain phenotypes generally observed in HPE. |
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| Group |
Morphology |
HPE degree |
|
|
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| (I) Cyclopia |
Single or double eye Proboscis |
Alobar |
| (II) Ethmocephaly |
Distinct sockets Extreme ocular hypotelorism Proboscis |
Alobar |
| (III) Cebocephaly |
Ocular hypotelorism Proboscis, with single nostril |
Alobar |
| (IVA) |
Ocular hypotelorism Midline cleft lip Flat nose |
Alobar or Semi-lobar |
| (IVB) |
Ocular hypotelorism Midline cleft lip (complete or partial) Flat nose |
Semi-lobar or lobar |
Dubourg et al. Orphanet Journal of Rare Diseases 2007 2:8 doi:10.1186/1750-1172-2-8 |
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