Bilateral lambdoid and posterior sagittal craniosynostosis—management, evolution, and outcome

Excerpt

Bilateral lambdoid and posterior sagittal synostosis (BLSS) is a rare disorder characterized by invagination of the occipital squame resulting in a step-like deformity of the occiput, and a typical head shape described as anterior turricephaly with mild brachycephaly. Neuhauser et al. were the first to report about this rare multisutural synostosis which they termed “craniofacial dyssynostosis” and proposed an autosomal recessive mode of inheritance to explain their findings [8]. Four of the seven patients they described were of Spanish/Mexican/Puerto Rican ancestry and had several features in common including an abnormal head shape, variable developmental delay, and short stature. Following this initial report, a few other authors have also reported their experience [1, 5, 7]. Moore et al. coined the term “Mercedes Benz pattern” in a report of three children who had presented with sagittal and bilateral lambdoid synostosis [6]. …