Management of idiopathic pediatric syringohydromyelia
Management of idiopathic pediatric syringohydromyelia
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DOI：
10.3171/2015.3.peds14433
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发表时间：
2015-10-01
影响因子：
1.9
通讯作者：
Couture, Daniel E.
中科院分区：
文献类型：
--
作者：
Rodriguez, Analiz;Kuhn, Elizabeth N.;Couture, Daniel E.
梗阻性脊髓空洞症常在脊髓影像学检查中发现。文献几乎没有提供关于是否需要随访或治疗的决策指导。本研究的目的是回顾作者的经验，在管理小儿脊髓积水不明caused.METHODS一个单一的机构回顾性审查的所有情况下，涉及儿童患者进行脊柱MRI从2002年至2012年进行。特发性脊髓空洞症（IS）患者被确定并分为2个亚组：无并发症的特发性脊髓空洞症和与脊柱侧凸相关的IS。结果98例患者（女性50例，男性48例）符合纳入标准。诊断空洞的中位年龄为11.9岁。中位最大空洞尺寸为2 mm（范围0.5-17 mm），跨越5个椎体节段（范围1-20个椎体节段）。37例患者患有脊柱侧凸。最常见的主诉是背痛（26%）。78例患者（80%）接受了临床随访，中位随访时间为20.5个月（范围1-143个月）。36%的患者在就诊时存在神经功能缺损; 64%的病例在末次随访时稳定或改善。38例患者（39%）进行了放射学随访，中位持续时间为13个月（范围2-83个月）。76%的患者的空洞大小没有变化，而16%的患者空洞大小减小，8%的患者空洞大小增加。36例患者接受了临床和放射学随访。14例患者（39%）的临床和影像学病程一致，11例患者（31%）无变化，3例患者（8%）临床和影像学改善。没有患者在临床和放射学过程中同时恶化。一例脊柱侧凸和肌营养不良患者接受了直接手术治疗的空洞，随后有恶化的临床过程和减少空洞size.Conclusions仍然是一个缺乏的数据有关的管理儿科IS。与脊柱侧凸相关的IS可使神经外科决策复杂化。在该队列中，影像学上的空洞大小增加与临床恶化之间不一致，表明手术决策应反映临床过程而不是影像学过程。
OBJECT Syringohydromyelia is frequently identified on spinal imaging. The literature provides little guidance to decision making regarding the need for follow-up or treatment. The purpose of this study was to review the authors' experience in managing pediatric syringohydromyelia of unknown cause.METHODS A single-institution retrospective review of all cases involving pediatric patients who underwent spinal MRI from 2002 to 2012 was conducted. Patients with idiopathic syringohydromyelia (IS) were identified and categorized into 2 subgroups: uncomplicated idiopathic syrinx and IS associated with scoliosis. Clinical and radiological course were analyzed.RESULTS Ninety-eight patients (50 female, 48 male) met the inclusion criteria. Median age at diagnosis of syrinx was 11.9 years. Median maximum syrinx size was 2 mm (range 0.5-17 mm) and spanned 5 vertebral levels (range 1-20 vertebral levels). Thirty-seven patients had scoliosis. The most common presenting complaint was back pain (26%). Clinical follow-up was available for 78 patients (80%), with a median follow-up of 20.5 months (range 1-143 months). A neurological deficit existed at presentation in 36% of the patients; this was either stable or improved at last follow-up in 64% of cases. Radiological follow-up was available for 38 patients (39%), with a median duration of 13 months (range 2-83 months). There was no change in syrinx size in 76% of patients, while 16% had a decrease and 8% had an increase in syrinx size. Thirty-six patients had both clinical and radiological follow-up. There was concordance between clinical and radiological course in 14 patients (39%), with 11 patients (31%) showing no change and 3 patients (8%) showing clinical and radiological improvement. No patients had concurrent deterioration in clinical and radiological course. One patient with scoliosis and muscular dystrophy underwent direct surgical treatment of the syrinx and subsequently had a deteriorated clinical course and decreased syrinx size.CONCLUSIONS There remains a paucity of data regarding the management of pediatric IS. IS in association with scoliosis can complicate neurosurgical decision making. There was no concordance between radiological syrinx size increase and clinical deterioration in this cohort, indicating that surgical decision making should reflect clinical course as opposed to radiological course.