Klekamp Jörg
Department of Neurosurgery, Christliches Krankenhaus, Danziger Str. 2, 49610, Quakenbrück, Germany,
Eur Spine J. 2014 Aug;23(8):1656-65. doi: 10.1007/s00586-014-3423-7. Epub 2014 Jun 18.
Basilar invagination is a rare craniocervical malformation which may lead to neurological deficits related to compression of brainstem and upper cervical cord as well as instability of the craniocervical junction. This study presents results of a treatment algorithm developed over a 20-year period focussing on anatomical findings, short-term and long-term outcomes.
69 patients with basilar invagination (mean age 41 ± 18 years, history 64 ± 85 months) were encountered. The clinical courses were documented with a score system for individual neurological symptoms for short-term results after 3 and 12 months. Long-term outcomes were analyzed with Kaplan-Meier statistics.
Patients with (n = 31) or without (n = 38) ventral compression were distinguished. 25 patients declined an operation, while 44 patients underwent 48 operations. Surgical management depended on the presence of ventral compression and segmentation anomalies between occiput and C3, signs of instability and presence of caudal cranial nerve dysfunctions. 16 patients without ventral compression underwent foramen magnum decompressions without fusion. 19 patients with ventral compression and abnormalities of segmentation or evidence of instability underwent a foramen magnum decompression with craniocervical (n = 18) or C1/2 (n = 1) stabilization. In nine patients with severe ventral compression and caudal cranial nerve deficits, a transoral resection of the odontoid was combined with a posterior decompression and fusion. Within the first postoperative year neurological scores improved for all symptoms in each patient group. In the long-term, postoperative deteriorations were related exclusively to instabilities either becoming manifest after a foramen magnum decompression in three or as a result of hardware failures in two patients.
The great majority of patients with basilar invagination report postoperative improvements with this management algorithm. Most patients without ventral compression can be managed by foramen magnum decompression alone. The majority of patients with ventral compression can be treated by posterior decompression, realignment and stabilization alone, reserving anterior decompressions for patients with profound, symptomatic brainstem compression.
基底凹陷症是一种罕见的颅颈交界区畸形，可导致与脑干和上颈髓受压相关的神经功能缺损以及颅颈交界区不稳定。本研究展示了一项历时20年制定的治疗方案的结果，该方案侧重于解剖学发现、短期和长期疗效。
共纳入69例基底凹陷症患者（平均年龄41±18岁，病程64±85个月）。采用个体神经症状评分系统记录临床病程，以评估3个月和12个月后的短期疗效。采用Kaplan-Meier统计分析长期疗效。
区分了有（n = 31）或无（n = 38）腹侧受压的患者。25例患者拒绝手术，44例患者接受了48次手术。手术治疗取决于腹侧受压情况、枕骨与C3之间的节段异常、不稳定体征以及尾侧颅神经功能障碍的存在。16例无腹侧受压的患者接受了枕骨大孔减压术，未进行融合。19例有腹侧受压且节段异常或有不稳定证据的患者接受了枕骨大孔减压术，并进行了颅颈（n = 18）或C1/2（n = 1）固定。9例有严重腹侧受压和尾侧颅神经缺损的患者，经口切除齿状突并联合后路减压和融合。术后第一年，各患者组的所有症状神经评分均有所改善。从长期来看，术后病情恶化仅与不稳定有关，其中3例是在枕骨大孔减压术后出现，2例是由于内固定失败所致。
绝大多数基底凹陷症患者采用该治疗方案术后病情得到改善。大多数无腹侧受压的患者仅通过枕骨大孔减压即可治疗。大多数有腹侧受压的患者可仅通过后路减压、复位和固定进行治疗，对于有严重症状性脑干受压的患者则采用前路减压。
