Massimi L, Caldarelli M, Paternoster G, Novegno F, Tamburrini G, Di Rocco C
Pediatric Neurosurgery, Catholic University Medical School; Rome, Italy -
Neuroradiol J. 2008 Feb 18;21(1):65-70. doi: 10.1177/197140090802100108.
Surgical treatment of Chiari I malformation and associated syringomyelia includes several different techniques with various degrees of invasiveness. Most extensive procedures may provide good long-term outcome in a good proportion of cases but are burdened by a quite high risk of postoperative complications. Thirty children operated on by simple bone decompression are retrospectively reviewed to assess the effectiveness of a less invasive technique. The present series comprises 30 children (18 females, 12 males; mean age: 68 months) treated from 1993 to 2005. All patients underwent foramen magnum decompression by means of suboccipital craniectomy and resection of the fibrous band at the level of foramen itself. Twenty-one children also required C1 laminectomy while a dural delamination was performed in 11 cases. The mean current follow-up is 4.3 years (1-12.6 years). Head and/or neck pain was the most frequent preoperative finding (56.7%), followed by upper and lower extremity weakness (20.0%), ataxia (20.0%) and vertigo (27.7%). Syringomyelia was present in 12 patients. A significant improvement of preoperative clinical symptoms and signs was observed in 28 patients (93.3%). Two children required adjunctive surgery. Neuroimaging revealed minor postoperative modifications in most cases regardless of tonsils location, while syringomyelia was reduced in size in 50% of the cases. Complication rate and length of hospital stay were significantly reduced compared with the literature data and our own experience using more invasive techniques. These data, compared with the literature, allow us to conclude that suboccipital craniectomy and Cl laminectomy (possibly integrated by dural delamination) is an effective and safe treatment for symptomatic children with Chiari I malformation and syringomyelia.
Chiari I型畸形及相关脊髓空洞症的外科治疗包括几种不同的技术，其侵袭程度各异。大多数广泛的手术在相当一部分病例中可能会带来良好的长期效果，但术后并发症风险相当高。对30例接受简单骨减压手术的儿童进行回顾性研究，以评估一种侵入性较小的技术的有效性。本系列包括1993年至2005年期间治疗的30名儿童（18名女性，12名男性；平均年龄：68个月）。所有患者均通过枕下颅骨切除术进行枕骨大孔减压，并在枕骨大孔水平切除纤维带。21名儿童还需要进行C1椎板切除术，11例进行了硬脑膜分层。目前的平均随访时间为4.3年（1 - 12.6年）。头和/或颈部疼痛是最常见的术前表现（56.7%），其次是上肢和下肢无力（20.0%）、共济失调（20.0%）和眩晕（27.7%）。12例患者存在脊髓空洞症。28例患者（93.3%）术前临床症状和体征有显著改善。2名儿童需要辅助手术。神经影像学显示，大多数情况下术后改变较小，与扁桃体位置无关，而50%的病例中脊髓空洞症体积缩小。与文献数据以及我们自己使用更具侵入性技术的经验相比，并发症发生率和住院时间显著降低。与文献相比，这些数据使我们得出结论，枕下颅骨切除术和C1椎板切除术（可能结合硬脑膜分层）是治疗有症状的Chiari I型畸形和脊髓空洞症儿童的一种有效且安全的治疗方法。
