Nath Pratap Chandra, Mishra Sudhansu Sekhar, Deo Rama Chandra, Mahanta Itibrata
Department of Neurosurgery, SCB Medical College, Cuttack, Odisha, India.
Department of Neurosurgery, SCB Medical College, Cuttack, Odisha, India.
World Neurosurg. 2016 Apr;88:689.e9-689.e12. doi: 10.1016/j.wneu.2015.11.082. Epub 2015 Dec 24.
Congenital anomalies are commonly encountered in the craniovertebral junction because of its unique embryologic development. The craniovertebral junction usually comprises the occiput, atlas, and axis. However, malrotation of the atlas (C1) in between the occiput (C0) and axis (C2) with Chiari I malformation as well as unilateral hypertrophy of the atlanto-occipital joint and a lateral mass manifesting features of high cervical myelopathy is a rarely reported anomaly.
A 22-year-old woman presented to us with high cervical compressive myelopathy. Imaging revealed rotation of the C1 vertebra approximately 20° toward the left side with right atlanto-occipital joint dislocation and hypertrophy. Imaging also revealed hypertrophy of a right lateral mass of C1, Chiari I malformation, and right atlantoaxial dislocation exposing the right C2 superior facet. She underwent a right extreme far lateral approach comprising a right C1 posterior arch excision and medialization of the right vertebral artery with excision of the hypertrophied atlanto-occipital joint extradurally and posteromedial excision of the superior articular facet of C1. The patient's compressive myelopathy features improved postoperatively.
We report the successful management of this rare congenital anomaly and review the literature.
由于颅颈交界区独特的胚胎发育过程，先天性畸形在该区域较为常见。颅颈交界区通常由枕骨、寰椎和枢椎组成。然而，寰椎（C1）在枕骨（C0）和枢椎（C2）之间发生旋转并伴有Chiari I畸形，以及寰枕关节单侧肥大和侧块出现高位颈髓病特征的情况则鲜有报道。
一名22岁女性因高位颈髓压迫性脊髓病前来就诊。影像学检查显示C1椎体向左旋转约20°，同时伴有右侧寰枕关节脱位和肥大。影像学检查还显示C1右侧侧块肥大、Chiari I畸形以及右侧寰枢椎脱位，暴露了右侧C2上关节面。她接受了右侧极外侧入路手术，包括切除右侧C1后弓、将右侧椎动脉内移，硬膜外切除肥大的寰枕关节以及C1上关节面的后内侧切除。患者术后压迫性脊髓病症状有所改善。
我们报告了对这种罕见先天性畸形的成功治疗，并对相关文献进行了回顾。
