Isolated Unilateral Frontosphenoidal Craniosynostosis

Isolated unilateral frontosphenoidal synostosis is a rare and often misdiagnosed cause of synostotic frontal plagiocephaly. It is important to distinguish the various synostotic causes of frontal plagiocephaly from deformational frontal plagiocephaly because operative intervention is required in synostotic plagiocephaly to avoid progressive deformity, whereas deformational plagiocephaly typically improves without surgery. This study reports a patient with a unique case of anterior plagiocephaly caused by left-sided frontosphenoidal craniosynostosis. The workup should include a thorough history and clinical examination followed by computed tomography scan with three-dimensional reconstruction. Frontosphenoidal synostosis should be suspected in the absence of a coronal synostosis in a child with anterior unilateral plagiocephaly and treated surgically. Good aesthetic and functional results occur with prompt diagnosis and early surgical correction.