Lais A, Kasabian N G, Dyro F M, Scott R M, Kelly M D, Bauer S B
Division of Urology, Children's Hospital, Boston, Massachusetts.
J Urol. 1993 Dec;150(6):1879-83. doi: 10.1016/s0022-5347(17)35922-0.
Between 1979 and 1990, 148 newborns with myelodysplasia were followed with serial urodynamic studies and neurological assessment. Of the patients 59 (40%) exhibited changes in neurological status by age 5 years, of whom 28 (19%) showed signs of deterioration. Most changes occurred before age 2 years. A total of 22 children underwent repeat neurosurgical exploration because of a change in urethral sphincter innervation (17), deterioration of function of the lower extremities (3), or changes on computerized tomography or magnetic resonance imaging (2). Postoperative urodynamic evaluation demonstrated improvement in 11 children, stabilization in 9 and further deterioration in 2. The earlier that a change was detected and secondary surgery was performed, the better the outcome. These findings indicate that the neurological lesion in myelodysplasia is a dynamic disease process requiring continuous neurological, orthopedic and urodynamic surveillance. Early identification and prompt neurosurgical reexploration seem to arrest and even reverse the neurological deterioration that takes place in a substantial number of these children.
在1979年至1990年期间,对148例患有脊髓发育异常的新生儿进行了系列尿动力学研究和神经学评估随访。在这些患者中,59例(40%)在5岁时出现神经状态改变,其中28例(19%)出现恶化迹象。大多数改变发生在2岁之前。共有22名儿童因尿道括约肌神经支配改变(17例)、下肢功能恶化(3例)或计算机断层扫描或磁共振成像显示改变(2例)而接受了再次神经外科探查。术后尿动力学评估显示,11名儿童病情改善,9名稳定,2名进一步恶化。改变发现得越早且进行二次手术,结果越好。这些发现表明,脊髓发育异常中的神经病变是一个动态的疾病过程,需要持续的神经学、矫形学和尿动力学监测。早期识别并及时进行神经外科再次探查似乎可以阻止甚至逆转这些儿童中相当一部分人发生的神经恶化。