Pyeritz R E, Sack G H, Udvarhelyi G B
Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Am J Med Genet. 1987 Oct;28(2):433-44. doi: 10.1002/ajmg.1320280221.
Neurologic problems caused by vertebral stenosis in the thoracolumbosacral (TLS) region are common in achondroplasia. Surgical decompression by means of laminectomy is recommended often, but its long-term results have not been assessed. We reviewed the clinical history of 22 achondroplastic patients who had at least one TLS laminectomy performed before 1981. Symptoms predated the first TLS laminectomy by a mean of 2.3 years (range 0.1-17 years). Preoperatively, 91% of patients had motor function impairment, 86% had sensory dysfunction, 86% had neurogenic claudication, 77% had radicular pain, 59% had symptomatic bladder dysfunction, and 32% had fecal incontinence. Only upper motor neurons were affected in 45%, only lower motor neurons in 27%, and both in 27%. Follow-up after the first TLS laminectomy averaged 8 years. Of the 20 patients who initially improved neurologically, 12 had functional improvement for more than 5 years. However, 11 of these 12 subsequently regressed and 10 had additional laminectomies. Long-term neurologic and functional improvement was associated with both a short duration of symptoms preoperatively and absence of cervical stenosis. Because of hypertrophic scarring, 9 patients developed compression at the site of the initial TLS laminectomy and required re-operation 6.4 years (range 1-11 years) later. We conclude that TLS laminectomy is an effective treatment for spinal stenosis if performed early in the course of the neurologic syndrome. However, some patients have, or later develop, compression adjacent to the myelographic site of stenosis, and some develop hypertrophic scarring at the site of initial decompression. We therefore suggest that the first TLS laminectomy extend (1) 3 levels cephalad to the myelographic block, (2) at least to S2, and (3) laterally at least to the facets.
在软骨发育不全患者中，胸腰段（TLS）椎管狭窄引起的神经问题很常见。通常建议通过椎板切除术进行手术减压，但其长期效果尚未得到评估。我们回顾了1981年前至少接受过一次TLS椎板切除术的22例软骨发育不全患者的临床病史。症状出现时间比首次TLS椎板切除术平均早2.3年（范围0.1 - 17年）。术前，91%的患者存在运动功能障碍，86%有感觉功能障碍，86%有神经源性间歇性跛行，77%有神经根性疼痛，59%有症状性膀胱功能障碍，32%有大便失禁。仅上运动神经元受累的占45%，仅下运动神经元受累的占27%，两者均受累的占27%。首次TLS椎板切除术后的随访平均为8年。最初神经功能有所改善的20例患者中，12例功能改善超过5年。然而，这12例中的11例随后病情恶化，其中10例接受了再次椎板切除术。长期神经功能和功能改善与术前症状持续时间短以及无颈椎管狭窄有关。由于肥厚性瘢痕形成，9例患者在最初TLS椎板切除部位出现压迫，需要在6.4年（范围1 - 11年）后再次手术。我们得出结论，如果在神经综合征病程早期进行TLS椎板切除术，是治疗椎管狭窄的有效方法。然而，一些患者在脊髓造影显示的狭窄部位附近已经存在或后来出现压迫，一些患者在初始减压部位出现肥厚性瘢痕形成。因此，我们建议首次TLS椎板切除术应（1）向头侧延伸至脊髓造影阻塞部位上方3个节段，（2）至少延伸至S2，（3）向外侧至少延伸至关节突。
