Langerhans cell histiocytosis of the spine in children - Long-term follow-up
Langerhans cell histiocytosis of the spine in children - Long-term follow-up
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DOI：
10.2106/00004623-200408000-00019
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发表时间：
2004-08-01
影响因子：
5.3
通讯作者：
Dormans, JP
中科院分区：
文献类型：
--
作者：
Garg, S;Mehta, S;Dormans, JP
背景：朗格汉斯细胞组织细胞增多症导致儿童脊柱的破坏性病变。很少有大型、长期的研究评估了儿童本病诊断后的临床和影像学表现、自然史、现代治疗方法的结果以及正常脊柱生长和稳定性的维持。方法：1970年至2003年间，本院对26例经活检证实的朗格汉斯细胞组织细胞增生累及脊柱的患儿进行治疗。他们总共有44个受累椎（20个颈椎，14个胸椎和10个腰椎）。通过x线片测量椎体塌陷，分为1级（0% - 50%塌陷）或11级（51% - 100%塌陷），再分为A级（对称塌陷）或B级（不对称塌陷）。脊柱后部的病变被分类为III级。23名儿童随访两年或更长时间（平均9.4年），并对该组患者的治疗和长期结果进行分析。结果：病变以颈椎为主（p:!）0.02)。26名儿童中有16名（62%）被发现患有多灶性骨骼疾病。颈椎和腰椎病变更常与多节段脊柱疾病相关。x线片显示的初始塌陷程度为IA级20例，113级3例，IIA级10例，1113级9例，III级2例。与ii级病变相比，i级病变更可能伴有对称塌陷。4名儿童出现脊柱畸形，其中2名后来需要脊柱融合术。未观察到初始塌陷的程度与随后脊柱畸形的发展之间的关系。尽管采用了不同的治疗方法，但在最近的随访中，所有患者都活得很好，所有体征和症状都得到了缓解，没有活动性疾病的证据。结论：我们发现颈椎病变的患病率特别高，多发性骨骼病变的患病率也很高。与椎平面的经典发现相反，我们发现更严重的病变往往导致不对称塌陷；然而，未发现不对称塌陷与随后脊柱畸形的发展有关。在没有全身性疾病或脊柱畸形的情况下，这些脊柱病变的自然历史是这样的，通常不需要积极的手术治疗；只需要随访以监测恢复和脊柱平衡。证据级别：治疗性研究，I级（病例系列[无，或历史对照组]）。有关证据水平的完整描述，请参见作者说明。
Background: Langerhans cell histiocytosis causes destructive lesions in a child's spine. Few large, long-term studies have evaluated the clinical and radiographic presentation, natural history, outcomes of modern treatment approaches, and maintenance of normal spinal growth and stability after the diagnosis of this disease in children.Methods: Twenty-six children with biopsy-proven Langerhans cell histiocytosis involving the spine were treated at our institution between 1970 and 2003. They had a total of forty-four involved vertebrae (twenty cervical, fourteen thoracic, and ten lumbar). Vertebral body collapse was measured on radiographs and classified as grade 1 (0% to 50% collapse) or grade 11 (51% to 100% collapse) and subclassified as A (symmetric collapse) or B (asymmetric collapse). Lesions of the posterior elements of the spine were classified as grade III. Twenty-three children were followed for two years or more (mean, 9.4 years), and the analyses of treatment and long-term outcomes were performed in that group of patients.Results: There was a predominance of lesions in the cervical spine (p :! 0.02). Sixteen (62%) of the twenty-six children were found to have multifocal skeletal disease. Cervical and lumbar lesions were more commonly associated with multilevel spinal disease. The extent of the initial collapse seen radiographically was grade IA for twenty vertebrae, 113 for three, IIA for ten, 1113 for nine, and III for two. Grade-I lesions were more likely to be associated with symmetric collapse than were grade-II lesions. Spinal deformity developed in four children, and two later required spinal fusion. No relationship was observed between the grade of the initial collapse and the subsequent development of spinal deformity. Despite heterogeneous treatment, all patients were alive and well with resolution of all presenting signs and symptoms and no evidence of active disease at the time of the most recent follow-up.Conclusions: We found a particularly high prevalence of lesions in the cervical spine and a high prevalence of multiple skeletal lesions. In contrast to the classic finding of vertebra plana, we found that more severe lesions often led to asymmetric collapse; yet, asymmetric collapse was not found to be associated with the development of subsequent spinal deformity. The natural history of these lesions in the spine in the absence of systemic disease or spinal deformity is such that aggressive surgical management is usually not indicated; only follow-up is necessary to monitor recovery and spinal balance.Level of Evidence: Therapeutic study, Level I (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.