Chatrath Ajay, Marino Alexandria, Taylor Davis, Elsarrag Mazin, Soldozy Sauson, Jane John A
Department of Neurological Surgery, University of Virginia Health System, P.O. Box 800212, Charlottesville, VA, 22908-0711, USA.
Childs Nerv Syst. 2019 Oct;35(10):1793-1799. doi: 10.1007/s00381-019-04310-0. Epub 2019 Jul 30.
To review the natural history of asymptomatic and symptomatic pediatric Chiari I malformations with and without syringomyelia.
We reviewed the literature for case reports and case series describing the natural history of asymptomatic and symptomatic children with Chiari I malformations with and without syringomyelia. Our review included approximately 700 asymptomatic children without syringomyelia, 100 symptomatic children without syringomyelia, 22 asymptomatic children with syringomyelia, and 11 symptomatic children with syringomyelia. Symptomatic and imaging outcomes at the point of last reported follow-up were noted to describe the natural history of Chiari I malformations in children.
Our review of about 700 asymptomatic children with CM-I without syrinx revealed that most children do not exhibit new-onset symptoms (5-6%) or syrinx (2-3%). The nearly 100 published cases of symptomatic CM-I without syrinx suggest that about half of children report symptomatic improvement (48%) and few report symptomatic worsening (7%). New-onset syrinx is rarely observed (2%). Few cases have been published about asymptomatic and symptomatic CM-I with syrinx as syringomyelia are generally regarded to be an indication for surgical intervention. Nevertheless, all 22 children with asymptomatic CM-I with syringomyelia included in this study were asymptomatic at follow-up, with syrinx resolution observed in 18 children and tonsillar herniation improvement observed in 16 children. Overall, our review of asymptomatic pediatric CM-I with or without syringomyelia suggests that its natural history is much more favorable than previously acknowledged and that the literature generally favors conservative management of these cases.
Our review of asymptomatic pediatric CM-I with or without syringomyelia suggests that its natural history is much more favorable than previously acknowledged and that the literature generally favors conservative management of these cases. Further study of symptomatic pediatric CM-I is necessary to better understand its natural history.
回顾有无脊髓空洞症的无症状和有症状小儿Chiari I型畸形的自然病史。
我们检索了文献中的病例报告和病例系列，这些报告和系列描述了有无脊髓空洞症的无症状和有症状的Chiari I型畸形患儿的自然病史。我们的综述纳入了约700例无脊髓空洞症的无症状患儿、100例无脊髓空洞症的有症状患儿、22例有脊髓空洞症的无症状患儿和11例有脊髓空洞症的有症状患儿。记录最后一次报告随访时的症状和影像学结果，以描述小儿Chiari I型畸形的自然病史。
我们对约700例无脊髓空洞的CM-I无症状患儿的综述显示，大多数患儿未出现新发症状（5 - 6%）或脊髓空洞（2 - 3%）。近100例已发表的无脊髓空洞的有症状CM-I病例表明，约一半患儿报告症状改善（48%），很少有患儿报告症状恶化（7%）。很少观察到新发脊髓空洞（2%）。关于有无脊髓空洞的无症状和有症状CM-I的病例报道很少，因为脊髓空洞症通常被视为手术干预的指征。然而，本研究纳入的所有22例有脊髓空洞症的无症状CM-I患儿在随访时均无症状，18例患儿的脊髓空洞消失，16例患儿的扁桃体疝改善。总体而言，我们对有无脊髓空洞症的无症状小儿CM-I的综述表明，其自然病史比以前认识的要好得多，并且文献普遍支持对这些病例进行保守治疗。
我们对有无脊髓空洞症的无症状小儿CM-I的综述表明，其自然病史比以前认识的要好得多，并且文献普遍支持对这些病例进行保守治疗。有必要对有症状的小儿CM-I进行进一步研究，以更好地了解其自然病史。
