Mercedes Benz pattern craniosynostosis also known as pure bilateral lambdoid and sagittal synostosis (BLSS) is a pattern of craniosynostosis 1,2.
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Epidemiology
The estimated incidence is at around 0.3 - 0.7 % 3.
Clinical presentation
Cognitive function is grossly normal. Dolichocephalic head shape, frontal bossing, prominent or flat occiput, with often concomitant short stature.
Pathology
The craniosynostosis in a majority of cases is isolated.
The associated anomalies of BLSS include cerebellar tonsillar herniation, hydrocephalus, venous abnormalities, enlarged bifrontal subarachnoid spaces, syringomyelia, microcephaly with bilateral deafness, and gyration anomalies.
Radiographic features
CT
Three-dimensional CT imaging shows bilateral lambdoid and posterior sagittal synostosis. The bone ridges resemble the Mercedes Benz log.
The skull vault's typical shape is flattening of the occipital bone with sublambdoid indentation, resulting in a small posterior cranial fossa (Acute Posterior Angle) compensated bossing of the frontal bone.
The head shape usually is described as anterior turricephaly combined with brachycephaly and hypertelorism.
MRI
It is useful during the follow-up period to evaluate tonsil herniation and other unusual pathologies associates.
Treatment and prognosis
Different surgical techniques are used. The surgical management may vary according to the phenotypic patterns. Also, some cases require foramen magnum decompression.4
History and etymology
It was first described in 1976 by Neuhauser as craniofacial dyssynostosis. The term "Mercedes Benz" syndrome was conceived by Moore in 1998 due to the characteristic fused sutures on three-dimensional CT imaging because of the bone ridges that resemble the Mercedes Benz log.
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