Mehrotra A, Nair A P, Das K, Chunnilal J S, Srivastava A K, Sahu R, Kumar Raj
Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow 226014, India.
Childs Nerv Syst. 2012 Nov;28(11):1943-50. doi: 10.1007/s00381-012-1801-1. Epub 2012 May 30.
Congenital atlantoaxial dislocation (AAD) is the most common bony abnormality affecting the craniovertebral junction. The paediatric population has specific problems like preoperative diagnostic difficulties, precise neurological examination, radiological diagnosis, surgical problems including physical fitness to tolerate surgery (including problems of anaesthesia), technical difficulties in surgery and problems related to immobilization.
A total of 229 consecutive paediatric (≤18 years) patients of AAD visited our centre from the period of January 1997 to August 2011. Twenty-nine cases were excluded from the study as these cases were diagnosed as CVJ tuberculosis, 31 cases were excluded as they were traumatic and the remaining 169 cases were retrospectively analysed. These patients were operated by a single experienced surgeon (the senior author) at the Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow. The preoperative and the postoperative clinical evaluation of the patient was done by the Kumar and Kalra myelopathic scoring system. The score was recorded in the preoperative period, at the time of discharge, at 3 months follow-up and at 6 months follow-up.
Ninety-four were males and 75 were females. The age range was 4 to 18 years with mean age 9.96±3.78 years. The follow-up ranged from 3 to 120 months with mean follow-up being 39.03±13.38 months. One hundred five cases were of fixed/irreducible AAD, and 64 were cases of mobile/reducible AAD. Majority of these cases presented with features of pyramidal tract involvement, and 108 cases had compromised pulmonary function test. One hundred thirty-seven cases had improved outcome, and 18 cases were in the same grade in the postoperative period with only 14 cases either deteriorated or died.
Congenital paediatric AAD are a different subset of abnormalities and have a satisfactory outcome. Preoperative evaluation must also include identification of various syndromes associated with paediatric AAD and respiratory reserve. Cormack-Lehane grade can be helpful in selecting borderline cases for postoperative need of tracheostomy. Majority of the cases have a good outcome, and therefore, surgery should be offered even in severe grade.
先天性寰枢椎脱位（AAD）是影响颅颈交界区最常见的骨骼异常。儿科患者存在一些特殊问题，如术前诊断困难、精确的神经学检查、放射学诊断、手术问题，包括耐受手术的身体状况（包括麻醉问题）、手术技术困难以及与固定相关的问题。
1997年1月至2011年8月期间，共有229例连续的儿科（≤18岁）AAD患者就诊于我们中心。29例因被诊断为颅颈交界区结核而被排除在研究之外，31例因创伤性原因被排除，其余169例进行回顾性分析。这些患者均由勒克瑙市桑贾伊·甘地医学科学研究生学院的一位经验丰富的外科医生（资深作者）进行手术。患者的术前和术后临床评估采用库马尔和卡尔拉脊髓病评分系统。评分记录于术前、出院时、3个月随访时和6个月随访时。
男性94例，女性75例。年龄范围为4至18岁，平均年龄9.96±3.78岁。随访时间为3至120个月，平均随访时间为39.03±13.38个月。105例为固定/不可复位AAD，64例为活动/可复位AAD。这些病例大多数表现为锥体束受累特征，108例肺功能测试受损。137例预后改善，18例术后处于相同等级，仅14例病情恶化或死亡。
先天性儿科AAD是异常情况的一个不同子集，预后良好。术前评估还必须包括识别与儿科AAD相关的各种综合征以及呼吸储备情况。科马克-莱汉分级有助于选择术后需要气管切开术的临界病例。大多数病例预后良好，因此，即使在严重分级的情况下也应进行手术。
