Klekamp Jörg
Asklepios Kliniken Bad Abbach, Bad Abbach, Germany.
Eur Spine J. 2025 Feb 13. doi: 10.1007/s00586-025-08728-w.
Split cord malformations represent a small group among tethered cord syndromes. This paper presents the largest series of adult patients with this disorder reported to date. Neuroradiological features, clinical symptoms, surgical management and data on short- and long-term outcomes are analyzed.
94 adults (mean age 54.7 ± 15 years) presented with split cord malformations between 1991 and 2024 and were evaluated (follow-up of 64 ± 84 months). Radiological features, intraoperative findings, and neurological examinations before and after surgery were analyzed. Long-term outcomes were evaluated with Kaplan-Meier statistics.
35 patients presented with a split cord separated in two dural tubes (type I), 59 patients demonstrated a split cord type II with both hemicords in a single dural sac. 79 patients featured a low positioned conus and 10 patients a dermal sinus. In 31 patients (33%), the malformation was combined with a hamartoma, i.e. lipoma or dermoid, epidermoid, neurenteric or neuroepithelial cyst. The commonest clinical course consisted of radicular pain and slowly progressive neurological deficits. 51 patients underwent 59 operations with untethering the split cord, transection of the filum terminale, and hamartoma removal if applicable. There was no permanent surgical morbidity except for patients requiring a revision in split cords type I combined with a hamartoma. 61% considered their postoperative condition improved. Radicular pain responded best with only marginal neurological changes after surgery. Postoperative progression-free courses for 10 years corresponded to the complexity of the malformation: 83.3% experienced 10 year progression-free outcomes after first operation on any split cord without associated hamartoma. If a hamartoma accompanied the split cord, this figure dropped to 59.3%.
The natural history of split cord malformations in adults is benign with slow neurological progression. Surgery should be reserved for symptomatic patients and provide untethering of all structures involved in the split as well as transection of the filum terminale. Associated dysraphic cysts require complete resection, while lipomas may be resected subtotally. The overall prognosis for patients requiring a revision is considerably reduced. Therefore, referral of these patients to appropriate centers is advisable.
脊髓纵裂畸形在脊髓拴系综合征中占比小。本文呈现了迄今为止报道的最大规模的成年脊髓纵裂畸形患者系列。分析了神经放射学特征、临床症状、手术治疗及短期和长期预后数据。
1991年至2024年间，94例成年患者（平均年龄54.7±15岁）出现脊髓纵裂畸形并接受评估（随访时间64±84个月）。分析了放射学特征、术中发现以及手术前后的神经学检查结果。采用Kaplan-Meier统计方法评估长期预后。
35例患者脊髓纵裂位于两个硬膜囊内（I型），59例患者表现为脊髓纵裂II型，双侧脊髓位于单个硬膜囊内。79例患者圆锥位置较低，10例患者有皮样窦。31例患者（33%）的畸形合并有错构瘤，即脂肪瘤或皮样囊肿、表皮样囊肿、神经肠囊肿或神经上皮囊肿。最常见的临床病程为神经根性疼痛和缓慢进展的神经功能缺损。51例患者接受了59次手术，包括松解脊髓纵裂、切断终丝，如有需要切除错构瘤。除I型脊髓纵裂合并错构瘤且需要翻修的患者外，无永久性手术并发症。61%的患者认为术后状况有所改善。神经根性疼痛术后改善最佳，神经功能仅有轻微改变。术后10年无进展的病程与畸形的复杂程度相关：任何无相关错构瘤的脊髓纵裂首次手术后，83.3%的患者10年无进展。如果脊髓纵裂伴有错构瘤，这一比例降至59.3%。
成年脊髓纵裂畸形的自然病程是良性的，神经功能进展缓慢。手术应仅适用于有症状的患者，需松解脊髓纵裂中所有受累结构并切断终丝。相关的神经管闭合不全囊肿需要完全切除，而脂肪瘤可部分切除。需要翻修的患者总体预后明显降低。因此，建议将这些患者转诊至合适的中心。
