Menezes Arnold H
Department of Neurosurgery, University of Iowa Hospitals and Clinics 200 Hawkins Drive, 1824 JPP, Iowa City, IA 52242, USA.
Childs Nerv Syst. 2008 Oct;24(10):1109-22. doi: 10.1007/s00381-008-0600-1. Epub 2008 Apr 10.
Congenital and developmental osseous abnormalities and anomalies that affect the craniocervical junction complex can result in neural compression and vascular compromise and can manifest itself with abnormal cerebrospinal fluid dynamics. An understanding of the development of the craniocervical junction is essential to recognize the pathological abnormalities.
Atlas assimilation, segmentation failures, os odontoideum, basilar invagination, and the various syndromes that affect the craniocervical junction have been analyzed. The natural history provides an added insight into its treatment.
Proatlas segmentation abnormalities surrounded the foramen magnum and the posterior arch of C1. Hindbrain herniation was associated in 33 of the 90 children involved. Spastic quadriparesis presented in 80% and lower cranial nerve abnormalities in 33%. Vertebrobasilar dysfunction was observed in 40% and trauma presentation seen in 60% of individuals. Atlas assimilation was present in 550 individuals who were evaluated for craniovertebral junction abnormalities. Hindbrain herniation occurred in 38%. Segmentation failure of C2 and C3 vertebrae compounded the abnormal dynamics resulting in atlantoaxial instability. This was a reducible instability with formation of pannus around the odontoid process until it became irreducible at approximately 14 years of age. Unilateral atlas assimilation caused torticollis in children. Os odontoideum was investigated regarding craniocervical trauma at a young age.
The conclusion was that os odontoideum was associated with an unrecognized fracture in children below the age of 5 with a previously normal odontoid structure as observed in our series. Atlas and axis abnormalities were reviewed in this series. This large database has provided an understanding of the natural history of many entities and allowed treatment protocols to be established that have stood the test of time.
影响颅颈交界区复合体的先天性和发育性骨异常可导致神经受压和血管受损，并可表现为脑脊液动力学异常。了解颅颈交界区的发育对于识别病理异常至关重要。
分析了寰椎融合、节段性发育不全、齿状突骨、基底凹陷以及影响颅颈交界区的各种综合征。其自然病史为其治疗提供了更多见解。
原寰椎节段性异常围绕枕骨大孔和C1后弓。90例患儿中有33例伴有后脑疝。80%出现痉挛性四肢瘫，33%出现低位脑神经异常。40%观察到椎基底动脉功能障碍，60%的个体有外伤表现。在接受颅颈交界区异常评估的550例个体中存在寰椎融合。38%发生后脑疝。C2和C3椎体节段性发育不全使异常动力学加剧，导致寰枢椎不稳定。这是一种可复位的不稳定，齿状突周围形成血管翳，直到大约14岁时变得不可复位。单侧寰椎融合导致儿童斜颈。对儿童颅颈外伤时的齿状突骨进行了研究。
结论是，如我们系列研究中所观察到的，齿状突骨与5岁以下儿童未被识别的骨折有关，这些儿童之前齿状突结构正常。本系列回顾了寰椎和枢椎异常。这个大型数据库提供了对许多疾病自然病史的理解，并允许建立经得起时间考验的治疗方案。
