Harlequin eye deformity

Last revised by Daniel J Bell on 4 Jan 2022

The harlequin eye deformity is characterized by elevation of the superolateral corner of the orbit. It may be seen in unilateral (plagiocephaly) or bilateral (brachycephaly) coronal suture synostosis.

History and etymology

The term harlequin eye derives from the appearance of the eyes on a harlequin mask with their exaggerated superior orbital margins. This was supposed to suggest a quizzical expression.

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Cases and figures

  • Case 1: on right side
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  • Case 2
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  • Case 3
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  • Case 4: right-sided
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  • Case 5: with plagiocephaly
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