Luo Rongjin, Song Yu, Liao Zhiwei, Yin Huipeng, Zhan Shengfeng, Yang Cao
Department of Orthopaedics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Orthopaedics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
World Neurosurg. 2019 May;125:129-135. doi: 10.1016/j.wneu.2019.01.178. Epub 2019 Feb 8.
Severe kyphoscoliosis associated with multiple giant spinal epidural arachnoid cysts (SEACs) is an extremely rare condition and remains a challenge in clinical practice. This study aimed to present a case of severe spinal deformity associated with multiple giant SEACs and to discuss strategies for the preoperative diagnosis and treatment.
A 22-year-old man with severe thoracolumbar kyphoscoliosis associated with multiple giant SEACs presented with progressive scoliosis, spastic paralysis, numbness, and abnormal gait. X-ray and magnetic resonance imaging revealed severe rigid kyphoscoliosis, extensive diffuse cystic space-occupying lesions, and diffuse spinal cord compression. After multidisciplinary consultation and discussion, the patient underwent a cyst-peritoneal shunting surgery followed by posterior vertebral column resection (PVCR) correction. The postoperative course was uneventful. Both kyphosis and scoliosis were significantly corrected, and muscle weakness of the lower extremities and sensory disturbance partially improved. At the 2-year follow-up visit, the patient could freely walk without the aid of crutches, but there were some residual neurologic deficits in both legs. A plain radiograph showed that bony fusion was achieved, and the correction was well maintained.
Cyst-peritoneal shunting surgery followed by PVCR, as in our case, could be an alternative surgical strategy for multiple giant SEACs associated with severe rigid kyphoscoliosis.
伴有多个巨大脊髓硬膜外蛛网膜囊肿（SEACs）的重度脊柱后凸侧弯是一种极为罕见的病症，在临床实践中仍然是一项挑战。本研究旨在介绍一例伴有多个巨大SEACs的严重脊柱畸形病例，并探讨术前诊断和治疗策略。
一名22岁男性，患有伴有多个巨大SEACs的重度胸腰椎脊柱后凸侧弯，表现为进行性脊柱侧弯、痉挛性瘫痪、麻木和异常步态。X线和磁共振成像显示严重的僵硬性脊柱后凸侧弯、广泛的弥漫性囊性占位性病变以及弥漫性脊髓受压。经过多学科会诊和讨论后，患者接受了囊肿 - 腹腔分流术，随后进行了后路脊柱全椎体切除术（PVCR）矫正。术后过程顺利。脊柱后凸和侧弯均得到显著矫正，下肢肌肉无力和感觉障碍部分改善。在2年的随访中，患者无需借助拐杖即可自由行走，但双腿仍存在一些残留神经功能缺损。一张平片显示实现了骨性融合，且矫正效果维持良好。
如我们病例中所示，先进行囊肿 - 腹腔分流术，然后进行PVCR，可能是伴有严重僵硬性脊柱后凸侧弯的多个巨大SEACs的一种替代手术策略。
