Sciubba Daniel M, Noggle Joseph C, Marupudi Neena I, Bagley Carlos A, Bookland Markus J, Carson Benjamin S, Ain Michael C, Jallo George I
Department of Neurosurgery, Johns Hopkins University, Baltimore, Maryland 21287, USA.
J Neurosurg. 2007 May;106(5 Suppl):372-8. doi: 10.3171/ped.2007.106.5.372.
Achondroplasia is a hereditary form of dwarfism caused by a defect in endochondral bone formation, resulting in skeletal abnormalities including short stature, shortened limb bones, macrocephaly, and small vertebral bodies. In the pediatric population, symptomatic spinal stenosis occurs at all spinal levels due to the abnormally narrow bone canal. In this study, clinical outcomes were assessed in children with achondroplasia after spinal canal decompression.
A retrospective review was conducted involving pediatric patients with heterozygous achondroplasia and symptomatic stenosis after decompressive procedures at the authors' institution within a 9-year period. Measured outcomes included resolution of symptoms, need for repeated surgery, presence of fusion, development of deformity, and complications. Forty-four pediatric patients underwent a total of 60 decompressive procedures. The average patient age at surgery was 12.7 years (range 5-21 years). Forty-nine operations were performed for initial treatment of stenosis, and 11 were performed as revision surgeries on previously operated levels. A large proportion of patients (> 60%) required additional cervicomedullary decompressions, most often preceding the symptoms of spinal stenosis. Of the initial procedures, decompression locations included 32 thoracolumbar (65%), 10 lumbar (20%), four cervical (8%), two cervicothoracic (4%), and one thoracic (2%). Forty-three of the decompressive procedures (72%) included spinal fusion procedures. Of the 11 revisions, five were fusion procedures for progressive deformity at levels previously decompressed but not fused (all thoracolumbar), five were for decompressions of symptomatic junctional stenosis with extension of fusion, and one was for repeated decompression at the same level due to recurrence of symptomatic stenosis.
Decompression of the spinal canal in pediatric patients with achondroplasia can be accomplished safely with significant clinical benefit. Patients with a history of cervicomedullary compression may be at an increased risk of developing symptomatic stenosis prior to adolescence. Fusion procedures are recommended in patients with a large decompression overlying a thoracolumbar kyphosis to avoid progressive postoperative deformity.
软骨发育不全是一种遗传性侏儒症，由软骨内骨形成缺陷引起，导致骨骼异常，包括身材矮小、四肢骨骼缩短、巨头畸形和椎体较小。在儿科人群中，由于骨管异常狭窄，所有脊柱节段都会出现症状性椎管狭窄。在本研究中，对软骨发育不全患儿行椎管减压术后的临床结果进行了评估。
对作者所在机构9年内接受减压手术的杂合性软骨发育不全和有症状性狭窄的儿科患者进行回顾性研究。测量的结果包括症状的缓解、再次手术的必要性、融合的存在、畸形的发展和并发症。44例儿科患者共接受了60次减压手术。手术时患者的平均年龄为12.7岁（范围5 - 21岁）。49例手术用于初次治疗狭窄，11例作为对先前手术节段的翻修手术。很大一部分患者（>60%）需要额外的颈髓减压，最常在出现椎管狭窄症状之前进行。初次手术中，减压部位包括胸腰段32例（65%）、腰段10例（20%）、颈段4例（8%）、颈胸段2例（4%）和胸段1例（2%）。43例减压手术（72%）包括脊柱融合手术。在11例翻修手术中，5例是针对先前减压但未融合节段（均为胸腰段）的进行性畸形的融合手术，5例是针对有症状性交界性狭窄并延长融合范围的减压手术，1例是因症状性狭窄复发在同一节段进行的再次减压手术。
对软骨发育不全的儿科患者进行椎管减压可安全实施，并具有显著的临床益处。有颈髓受压病史的患者在青春期前出现症状性狭窄的风险可能增加。对于胸腰段后凸畸形上方进行大范围减压的患者，建议行融合手术以避免术后出现进行性畸形。
