Kirby David J, Dietz Harry C, Sponseller Paul D
Department of Orthopaedic Surgery, The Johns Hopkins University School of Medicine.
Departments of Pediatrics.
J Pediatr Orthop. 2018 Sep;38(8):e455-e461. doi: 10.1097/BPO.0000000000001203.
We studied the prevalence and treatment outcomes of spondylolisthesis in patients with Loeys-Dietz syndrome (LDS).
Clinical data and lumbosacral imaging of 138 patients with LDS were reviewed. Spondylolisthesis (L4-L5 or L5-S1) and spondylolysis were characterized by multimodal imaging and correlated with clinical data. Treatments and outcomes were characterized for patients with spondylolisthesis. Associations were determined using the Fisher exact, Mann-Whitney, and Student t tests (α=0.05).
Twenty-four patients (17%) had spondylolysis and 23 (17%) had spondylolisthesis. Median age at spondylolisthesis diagnosis was 11 (interquartile range, 9.5) years. In patients in whom measurement was possible (n=20), mean (±SD) slip was 48% (±35%). Nineteen patients had L5-S1 slip and 4 had L4-L5 slip. Of the patients with spondylolisthesis, 5 had no evidence of spondylolysis; of those with spondylolysis, all but 6 had spondylolisthesis. Eleven patients with spondylolisthesis underwent posterior spinal fusion (PSF) to treat slip progression, pain, and/or neurological deficit. Spondylolisthesis recurred in 1 patient who underwent PSF with bone graft arthrodesis alone (no instrumentation). The other 10 patients underwent PSF with instrumentation and fusion. Three patients additionally underwent Bohlman interbody fusion. Two patients developed implant failure. S2 fixation was performed at revision to achieve fusion in these patients. Mean Meyerding grade improved in patients who underwent arthrodesis, from 3.9 (±1.2) to 1.9 (±1.3) (P=0.002). Complications were 2 cerebrospinal fluid leaks, 2 transient postoperative paresthesias, and 1 case each of pseudarthrosis at S1-S2, wound dehiscence, and transient urinary incontinence. No significant associations between LDS type and lumbosacral abnormalities were found.
High-grade spondylolisthesis is common in LDS and usually associated with spondylolysis. Patients requiring surgery for spondylolisthesis present during childhood and do well after instrumented PSF. Interbody fusion and stabilization of S1 and S2 can prevent physeal deformation. LDS should be considered in patients with high-grade spondylolisthesis. Patients with LDS should be monitored for spondylolisthesis and spondylolysis starting when they are young.
Level IV-retrospective study.
我们研究了洛伊迪茨综合征（LDS）患者腰椎滑脱的患病率及治疗结果。
回顾了138例LDS患者的临床资料和腰骶部影像学检查结果。通过多模态影像学对腰椎滑脱（L4-L5或L5-S1）和椎弓根峡部裂进行特征分析，并与临床资料进行关联分析。对腰椎滑脱患者的治疗方法及结果进行特征分析。采用Fisher精确检验、Mann-Whitney检验和Student t检验确定相关性（α=0.05）。
24例（17%）患者有椎弓根峡部裂，23例（17%）有腰椎滑脱。腰椎滑脱诊断时的中位年龄为11岁（四分位数间距为9.5岁）。在可测量的患者中（n=20），平均（±标准差）滑脱率为48%（±35%）。19例患者为L5-S1滑脱，4例为L4-L5滑脱。在腰椎滑脱患者中，5例无椎弓根峡部裂证据；在有椎弓根峡部裂的患者中，除6例外均有腰椎滑脱。11例腰椎滑脱患者接受了后路脊柱融合术（PSF）以治疗滑脱进展、疼痛和/或神经功能缺损。1例仅接受植骨融合（无内固定）的PSF患者出现腰椎滑脱复发。其他10例患者接受了带内固定和融合的PSF手术。3例患者还接受了Bohlman椎间融合术。2例患者出现内固定失败。翻修时进行S2固定以实现这些患者的融合。接受融合术的患者平均Meyerding分级从3.9（±1.2）改善至1.9（±1.3）（P=0.002）。并发症包括2例脑脊液漏、2例术后短暂感觉异常，以及S1-S2处假关节形成、伤口裂开和短暂性尿失禁各1例。未发现LDS类型与腰骶部异常之间存在显著相关性。
高度腰椎滑脱在LDS中很常见且通常与椎弓根峡部裂相关。因腰椎滑脱需要手术治疗的患者在儿童期发病，接受带内固定装置的PSF术后效果良好。椎间融合及S1和S2的稳定可预防骨骺变形。高度腰椎滑脱患者应考虑LDS。LDS患者应从年轻时就开始监测腰椎滑脱和椎弓根峡部裂情况
IV级——回顾性研究。
