Department of Plastic Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center.
Department of Plastic Surgery, University of Pittsburgh School of Medicine.
J Craniofac Surg. 2021 Sep 1;32(6):2059-2063. doi: 10.1097/SCS.0000000000007646.
Bilateral lambdoid and sagittal synostosis, or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in frontal bossing, a tapered posterior fossa, and an anteriorly displaced cranial vertex. Its ideal surgical correction must result in posterior expansion, skull elongation, and caudal repositioning of the vertex. We present a craniometric analysis of skull changes with posterior-superior distraction and introduce a novel craniometric measure: vertex position. In this study, a retrospective review was performed to analyze outcomes of posterior cranial vault distraction osteogenesis (PVDO) using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0). Four patients underwent PVDO at mean age 10.61 ± 3.16 months utilizing a posterior-superior distraction vector. Linear distraction distance averaged 30.30 ± 0.90 mm with a mean consolidation period of 3.98 ± 0.72 months. Mean corrected change in intra-cranial volume was 236.30 ± 3.71 mL, at an average rate of 7.81 ± 2.00 mL/mm of distraction. Increases in anterior cranial height (7.83 ± 2.51 mm), middle cranial height (8.43 ± 4.21 mm), posterior cranial height (13.15 ± 7.45 mm), and posterior cranial fossa height (21.99 ± 8.55 mm) were observed. Cranial vertex demonstrated a mean posterior movement of 0.18 ± 0.13. PVDO utilizing a posterior-superior distraction vector for management of nonsyndromic bilateral lambdoid and sagittal synostosis effectively increases intracranial volume and height and provides an esthetic outcome with posterior movement of the cranial vertex.
双侧矢状和人字缝早闭，又称梅赛德斯-奔驰综合征，是一种罕见的颅缝早闭综合征，表现为额部突出、颅后窝变窄、颅顶前部移位。理想的手术矫正必须使颅后扩展、颅骨延长和颅顶后部复位。我们对后路超上方牵张颅骨成形术（posterior-superior distraction osteogenesis，PVDO）后的颅骨变化进行了颅测量分析，并引入了一种新的颅测量指标：颅顶位置。在这项研究中，我们对 2016 年至 2019 年采用后路超上方牵张矢量进行的颅后穹窿骨切开术（posterior cranial vault distraction osteogenesis，PVDO）的结果进行了回顾性分析。颅顶位置以颅顶矢状径的比例（0-1.0）从额部到尾部进行测量。4 例患者在平均 10.61±3.16 月龄时因双侧矢状和人字缝早闭接受了后路超上方牵张术。线性牵开距离平均为 30.30±0.90mm，平均固定期为 3.98±0.72 个月。颅内容积平均校正变化为 236.30±3.71ml，平均牵开率为 7.81±2.00ml/mm。前颅高度（7.83±2.51mm）、中颅高度（8.43±4.21mm）、后颅高度（13.15±7.45mm）和后颅窝高度（21.99±8.55mm）均增加。颅顶的平均后移为 0.18±0.13。对于非综合征性双侧矢状和人字缝早闭的治疗，采用后路超上方牵张矢量的 PVDO 可有效增加颅内容积和高度，并通过颅顶后部的移动提供美观的效果。
