Division of Neurosurgery, Department of Neuroscience (DINOGMI), Ospedale Policlinico San Martino-IST, University of Genoa, Genoa, Italy; Division of Neurosurgery, IRCCS Istituto Giannina Gaslini Children's Hospital, Genoa, Italy.
Division of Neurosurgery, IRCCS Istituto Giannina Gaslini Children's Hospital, Genoa, Italy.
World Neurosurg. 2019 Aug;128:77-82. doi: 10.1016/j.wneu.2019.04.117. Epub 2019 Apr 19.
Bilateral lambdoid and posterior sagittal synostosis is a rarely encountered multisutural craniosynostosis accounting for 0.3%-0.7% of all craniosynostoses. It has been named "Mercedes-Benz Syndrome" (MBS) because of the bone ridges that resemble the Mercedes-Benz logo. The typical head shape is usually described as anterior turricephaly combined with mild brachycephaly. We describe a case of pure MBS without involvement of other sutures. Since we believe that MBS is a different pathology when other sutures are involved, we will discuss our case with a review of the literature, including all reported cases of bilateral lambdoid and posterior sagittal synostosis with no other sutures involved but sagittal and bilateral lambdoid.
A 3-month-old female baby came to our attention because of a turrycephalic appearance of the head. Findings of the neurologic examination were unremarkable. Computed tomography scan showed premature fusion of posterior third of sagittal suture and bilateral lambdoid suture. Magnetic resonance imaging did not show relevant intracranial abnormalities. She underwent sagittal decompression extended posteriorly below the lambdoid suture combined with biparietal decompression to obtain expansion of both parieto-frontal bones and posterior fossa. Post-operative course was uneventful. Follow-up at 3 months showed a good aesthetic result, and results of the neurologic examination were unremarkable.
Pure MBS is a rare condition that unlike other multisutural complex craniosynostosis is rarely associated with genetic syndromes, has a low rate of associated intracranial anomalies, and has less need for more skull-remodeling surgery. The choice of the most suitable surgical procedure must be tailored on the patient, considering preoperative head appearance and eventually associated anomalies.
双侧矢状缝和后矢状缝融合是一种罕见的多缝颅缝早闭症，占所有颅缝早闭症的 0.3%-0.7%。由于骨嵴类似于梅赛德斯-奔驰（Mercedes-Benz）的标志，因此被命名为“梅赛德斯-奔驰综合征（MBS）”。典型的头型通常表现为前尖头畸形，伴有轻度短头畸形。我们描述了一例单纯 MBS 病例，不涉及其他缝。由于我们认为 MBS 在涉及其他缝时是一种不同的病理，因此我们将结合文献复习讨论我们的病例，包括所有报道的双侧矢状缝和后矢状缝融合而不涉及其他缝但涉及矢状缝和双侧矢状缝的病例。
一名 3 个月大的女婴因头部呈尖头畸形而引起我们的注意。神经系统检查未见明显异常。计算机断层扫描显示矢状缝后三分之一和双侧矢状缝过早融合。磁共振成像未显示相关颅内异常。她接受了矢状骨切开术，向后延伸至矢状缝和双侧矢状缝下方，同时行双顶骨骨切开术，以获得双侧顶额骨和后颅窝的扩张。术后过程平稳。3 个月的随访显示美容效果良好，神经系统检查未见明显异常。
单纯 MBS 是一种罕见的疾病，与其他多缝复杂颅缝早闭症不同，它很少与遗传综合征相关，颅内异常相关率低，需要更多颅骨重塑手术的可能性较小。最合适的手术方案的选择必须根据患者的情况量身定制，考虑术前头部外观和最终相关的异常。
