Frontofacial Features of Unilateral Lambdoid Craniosynostosis: A Multicenter Assessment

Unilateral lambdoid craniosynostosis is differentiated from deformational plagiocephaly primarily by assessing the cranium from posterior and bird's-eye views. Findings include posterior displacement of the ipsilateral ear, ipsilateral occipitomastoid bossing, ipsilateral occipitoparietal flattening, contralateral parietal bossing, and contralateral frontal bossing. Diagnosis based off facial morphology may be an easier approach because the face is less obstructed by hair and head-coverings, and can easily be assessed when supine. However, frontofacial characteristics of unilateral lambdoid craniosynostosis are not well described.

A retrospective cohort review of patients with isolated, unilateral lambdoid craniosynostosis from the Children's Hospital of Pittsburgh and the Children's Hospital of Philadelphia was performed. Preoperative frontal and profile photographs were reviewed for salient characteristics.

Nineteen patients met inclusion criteria. Eleven patients had left lambdoid craniosynostosis, and eight had right lambdoid craniosynostosis. All patients were nonsyndromic. Patients demonstrated contralateral parietal bossing and greater visibility of the ipsilateral ear. Contralateral frontal bossing was mild. The orbits were tall and turricephaly was present in varying severity. Facial scoliosis as a C-shaped deformity was present in varying severity. The nasal root and chin pointed to the contralateral side.

The combination of greater visibility of the ipsilateral ear, contralateral parietal bossing, and C-shaped convex ipsilateral facial scoliosis are hallmark frontofacial features of unilateral lambdoid craniosynostosis. Although the ipsilateral ear is more posterior, the greater visibility may be attributed to lateral displacement from the mastoid bulge. Evaluation of long-term postoperative results is needed to assess if this pathognomonic facial morphology is corrected following posterior vault reconstruction.