Kentucky College of Osteopathic Medicine, Pikeville, Kentucky, USA.
Desert Regional Medical Center, Palm Springs, California, USA.
Pediatr Neurosurg. 2022;57(1):1-16. doi: 10.1159/000519175. Epub 2021 Dec 3.
Craniosynostosis is a condition characterized by the premature fusion of 2 or more skull bones. Craniosynostosis of the lambdoid suture is one of the rarest forms, accounting for 1-4% of all craniosynostoses. Documented cases are separated into simple (single suture), complex (bilateral), and associated with adjacent synostoses ("Mercedes Benz" Pattern) or syndromes (i.e., Crouzon, Sathre-Chotzen, Antley-Bixler). This condition can manifest phenotypic deformities and neurological sequelae that can lead to impaired cognitive function if improperly treated or left undiagnosed. Preferred surgical techniques have varied over time but all maintain the common goals of establishing proper head shape and preventing of complications that could contribute to aforementioned sequelae.
This comprehensive review highlights demographic distributions, embryological development, pathogenesis, clinical presentation, neurological sequelae, radiologic findings, surgical techniques, surgical outcomes, and postoperative considerations of patients with lambdoid craniosynostosis presentation. In addition, a systematic review was conducted to explore the operative management of lambdoid craniosynostosis using PubMed, Embase, and Scopus databases, with 38 articles included after screening. Key Messages: Due to a low volume of published cases, diagnosis and treatment can vary. Large overlap in presentation can occur in patients that display lambdoid craniosynostosis and posterior plagiocephaly, furthering the need for comprehensive analysis. Possessing the knowledge and tools to properly assess patients with lambdoid craniosynostosis will allow for more precise care and improved outcomes.
颅缝早闭是一种颅骨 2 块或多块提前融合的病症。矢状缝颅缝早闭是一种罕见的形式，占所有颅缝早闭的 1-4%。已记录的病例分为单纯型（单一缝）、复杂型（双侧）、并与相邻颅缝融合（“梅赛德斯-奔驰”模式）或综合征（即，Crouzon、Sathre-Chotzen、Antley-Bixler）相关。这种情况可能表现出表型畸形和神经后遗症，如果处理不当或未确诊，可能导致认知功能受损。首选的手术技术随时间而变化，但都保持着建立适当头部形状和预防可能导致上述后遗症的并发症的共同目标。
本综述重点介绍了矢状缝颅缝早闭患者的人口统计学分布、胚胎发育、发病机制、临床表现、神经后遗症、影像学发现、手术技术、手术结果和术后注意事项。此外，还通过 PubMed、Embase 和 Scopus 数据库对矢状缝颅缝早闭的手术管理进行了系统评价，筛选后纳入了 38 篇文章。关键信息：由于发表病例数量较少，诊断和治疗可能存在差异。表现出矢状缝颅缝早闭和后部斜头畸形的患者可能存在较大的重叠表现，因此需要进行全面分析。拥有正确评估矢状缝颅缝早闭患者的知识和工具将能够提供更精确的护理和改善预后。
