The variable position of the ear in lambdoid synostosis.

Isolated unilateral lambdoid craniosynostosis is often confused with posterior positional plagiocephaly because of a general overlap in their clinical presentations; however, distinction between these 2 entities is important because of the differences in appropriate management. Historical literature teaches that ear position is posterior in lambdoid synostosis, whereas it is anterior in positional plagiocephaly. Recently, several cases of anterior ear position in isolated unilateral lambdoid synostosis presented to the Texas Children's Hospital. A review of the cases and literature revealed that there are now 37 cases of unilateral lambdoid synostosis in the literature in which ipsilateral ear position is reported. Twelve cases (32%) had anteriorly displaced ears, 6 cases (16%) were nondisplaced, 7 cases (19%) were displaced posteriorly, 4 cases (11%) anteroinferiorly, 1 case (3%) inferiorly, and 7 cases (19%) posteroinferiorly. Based on this review, it seems that the diagnostic significance of the external ear position is unclear at this point.