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伴有脊髓空洞症和脊柱侧弯的Chiari I型畸形

Chiari I malformation associated with syringomyelia and scoliosis.

作者信息

Ghanem I B, Londono C, Delalande O, Dubousset J F

机构信息

Département de Chirurgie Orthopédique, Hôpital Hotel-Dieu de France, Paris, France.

出版信息

Spine (Phila Pa 1976). 1997 Jun 15;22(12):1313-7; discussion 1318. doi: 10.1097/00007632-199706150-00006.

DOI:10.1097/00007632-199706150-00006
PMID:9201833
Abstract

STUDY DESIGN

A retrospective review of a series of 12 children who underwent suboccipital foraminotomy and duroplasty for Chiari I malformation.

OBJECTIVE

To assess the effects of this surgery on associated syringomyelia and scoliosis.

SUMMARY OF BACKGROUND DATA

Suboccipital foraminotomy for the treatment of syringomyelia associated with Chiari I malformation was greatly stimulated by Gardner's hydrodynamic theory, and its results proved to be encouraging. However, several authors reported improvement or stabilization of associated scoliosis after this surgery.

METHODS

A retrospective review was conducted on 12 patients who underwent suboccipital foraminotomy for Chiari I malformation associated with syringomyelia. Neurologic Impairment, extent of syringomyelia, and severity of associated spinal deformity were assessed preoperatively and at a 4.5-year average follow-up (range, 2.1-12 years). Anomaly of superficial abdominal reflexes was found in all cases, and para or tetraparesis in three cases. Syringomyelia was of variable localization and extent. Scoliosis was present in 7 cases (greater than 40 degrees in 5 cases).

RESULTS

Diminution or complete disappearance of syringomyelia was observed in 11 cases, 3 months to 1 year after surgery. Superficial abdominal reflexes anomaly improved in four cases. Minimal neurologic deficit persisted in one case. Scoliosis improved in one case, remained unchanged in one case, and progressed in the five cases with preoperative severe deformity, requiring instrumentation and fusion.

CONCLUSIONS

Improvement of syringomyelia and neurologic deficit, observed with suboccipital foraminotomy, supports the theory that abnormal hydrodynamics of the cerebral spinal fluid is most likely to cause these deficits.

摘要

研究设计

对12例因 Chiari I 型畸形接受枕下开颅减压术和硬脑膜成形术的儿童进行回顾性研究。

目的

评估该手术对相关脊髓空洞症和脊柱侧弯的影响。

背景资料总结

Gardner 的流体动力学理论极大地推动了枕下开颅减压术治疗与 Chiari I 型畸形相关的脊髓空洞症,其结果令人鼓舞。然而,几位作者报道了该手术后相关脊柱侧弯有所改善或稳定。

方法

对12例因 Chiari I 型畸形合并脊髓空洞症接受枕下开颅减压术的患者进行回顾性研究。术前及平均4.5年随访(范围2.1 - 12年)时评估神经功能损害、脊髓空洞症程度及相关脊柱畸形严重程度。所有病例均发现浅腹壁反射异常,3例出现肢体轻瘫或四肢瘫。脊髓空洞症的定位和范围各不相同。7例存在脊柱侧弯(5例大于40度)。

结果

术后3个月至1年,11例脊髓空洞症减轻或完全消失。4例浅腹壁反射异常有所改善。1例仍有轻微神经功能缺损。1例脊柱侧弯改善,1例无变化,5例术前严重畸形者脊柱侧弯进展,需行器械固定和融合术。

结论

枕下开颅减压术可改善脊髓空洞症和神经功能缺损,支持脑脊液流体动力学异常最有可能导致这些缺损的理论。

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