Surgery for Nonsyndromic Single-Suture Craniosynostosis Clinical Presentation

Updated: Aug 11, 2023
  • Author: Jonathan S Black, MD, FACS, FAAP; Chief Editor: Brian H Kopell, MD  more...
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History and Physical Examination

The abnormal head shape is often noticeable at birth and leads to a referral to a specialist in cranial deformity. Typically, a pediatric neurosurgeon or a craniofacial plastic surgeon is able to determine the diagnosis on physical examination before a confirmatory imaging study is performed.

Many infants referred by pediatricians to neurosurgeons and craniofacial plastic surgeons for abnormalities in head shapes do not have craniosynostosis and instead have positional (or deformational) plagiocephaly. Positional plagiocephaly most often masquerades as either lambdoid or unilateral coronal craniosynostosis.

The most common type of positional plagiocephaly is characterized by unilateral flattening in the occipital region (mimicking lambdoid craniosynostosis). The severity of the flattening can vary but often leads to a parallelogram shape of the skull as viewed from above, with anterior displacement of the ipsilateral ear, forehead, and cheek. This forehead asymmetry mimics unilateral coronal craniosynostosis.

Careful clinical evaluation usually suffices to distinguish craniosynostosis from positional plagiocephaly. Lambdoid craniosynostosis causes a parallelogram-shaped skull as viewed from behind, in which the ear ipsilateral to the flattening is inferiorly deflected and the contralateral parietal bone experiences compensatory superior growth. Lambdoid synostosis consistently presents with a mastoid bulge or bossing on the side of the fusion which is not present in children with deformational plagiocephaly.

Although both positional plagiocephaly and unilateral coronal craniosynostosis are associated with forehead asymmetry, the nature of the asymmetry is quite different. Whereas in positional plagiocephaly the forehead and orbital rim contralateral to the flattened occiput are normal, the forehead and orbital rim in unilateral coronal craniosynostosis are recessed and the contralateral frontal bone is overgrown.