BACKGROUND Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups. CONCLUSION Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.

中文翻译：

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Chiari-1 畸形和脊髓空洞症手术治疗中的枕颈融合和腹侧减压：来自 Park-Reeves 脊髓空洞症研究联盟的数据分析

背景 枕颈融合术 (OCF) 和腹侧减压术 (VD) 可用于治疗伴有脊髓空洞症 (SM) 的小儿 Chiari-1 畸形 (CM-1)，作为后颅窝减压术 (PFD) 的辅助手段，可治疗复杂的颅椎交界处病理. 目的：在一个多中心队列的儿童 CM-1 和 SM 受试者接受 PFD 治疗时，检查影响 OCF 和 OCF/VD 使用的因素。方法 Park-Reeves Syringomyelia Research Consortium 登记处用于检查 637 名小脑扁桃体异位 ≥ 5 mm、空洞直径 ≥ 3 mm 且在其指数 PFD 后至少随访 1 年的受试者。对单独接受 PFD 的受试者和接受 PFD + OCF 或 PFD + OCF/VD 的受试者进行了比较。结果 所有 637 名患者均接受了 PFD，其中 505 名（79.2%）进行了硬膜成形术，132 名（20.8%）未进行了硬膜成形术。共有 12 名受试者在其管理的某个时间点继续患有 OCF（PFD + OCF），而 4 名受试者患有 OCF 和 VD（PFD + OCF/VD）。在数据完整的患者中，扁桃体病史 (3/10, P = .011)、Klippel-Feil (2/10, P = .015) 和基底节内陷 (3/12, P < .001) 增加在 OCF 组内，而在 OCF/VD 组中仅基底内陷（1/4，P < .001）增加。与单纯 PFD 组 (145.3 ± 12.7°）。pB-C2 在各组之间没有差异。结论 尽管单独 PFD 足以治疗绝大多数 CM-1/SM 患者，但偶尔可能会使用 OCF 或 OCF/VD。颅底和脊柱病理学和 CXA 可以深入了解 OCF 和/或 OCF/VD 的需求。