Objective: To summarize the preliminary effectiveness of surgical treatment of Erdheim-Chester disease (ECD), so as to improve the understanding of the disease by orthopedic surgeons.
Methods: The clinical data of 9 patients with ECD between December 2012 and October 2017 were retrospectively analysed. There were 6 males and 3 females with an median age of 42 years (range, 8-61 years). The disease duration was 4-59 months (mean, 39 months). There were 2 cases of multiple lesions, including 1 case involving soft tissue of the buttocks and bilateral tibia, 1 case involving the sinus, skull base, and proximal right tibia; 7 cases with single lesion, including 3 cases of right femoral neck, 1 case of proximal right tibia, 1 case of right humerus, and 2 cases of ribs. Nine patients were diagnosed according to clinical manifestations, imaging examination, and pathological diagnosis. Four patients underwent needle biopsy before operation and 5 patients were diagnosed by postoperative pathology examination. Five cases underwent lesional scraping and internal fixation, 1 case underwent bone scraping and bone grafting, and 3 cases underwent lesion resection. One of the multiple lesions was treated with interferon and hormone.
Results: Nine patients underwent the surgery safely. There was no fever, wound exudation, infection, etc., and the incisions healed by first intention. All the patients were followed up 4-59 months with an average of 31.4 months. One patient with bilateral tibia and hip soft tissue involvement continued to receive medical treatment, and the tumor was controlled without significant increasing. The remaining 8 patients were examined for X-ray films at 3, 6, and 12 months after surgery, the bone has been fused and the steel plate and intramedullary nail were firmly fixed, and no tumor recurrence was observed. At 1 year after surgery, the pain symptoms of the patients improved and returned to normal life; 3 of them who involving the right femoral neck walked freely, and the quality of life improved significantly.
Conclusion: ECD patients can achieve the purpose of eliminating lesions and relieving pain after surgical treatment, and the surgical treatment has the advantages of quick relief of pain, improved quality of life, small side effects, and low economic cost when compared with medical treatment.
目的: 总结 Erdheim-Chester 病（Erdheim-Chester disease，ECD）手术治疗的近期疗效，提高骨科医师对该病的认识。.
方法: 回顾性分析 2012 年 12 月－2017 年 10 月收治的 9 例 ECD 患者，男 6 例，女 3 例；年龄 8～61 岁，中位年龄 42 岁。病程 4～59 个月，平均 39 个月。多发病变 2 例，其中 1 例累及臀部软组织、双侧胫骨，1 例累及鼻窦、颅底、右肱骨近端；单发病变 7 例，其中累及右股骨颈 3 例，右胫骨近端 1 例，右肱骨干 1 例，肋骨 2 例。9 例均依据临床表现、影像学检查及病理学诊断确诊，其中 4 例术前行穿刺活检术，5 例术后病理确诊。5 例行病变刮除植骨内固定术，1 例行病变刮除植骨术，3 例行病变切除术；其中 1 例多发病变术后采用干扰素、激素等综合治疗。.
结果: 9 例患者均安全完成手术，术后均无发热、伤口渗液、感染等发生，切口均Ⅰ期愈合。患者均获随访，随访时间 4～59 个月，平均 31.4 个月。1 例双侧胫骨及臀部软组织受累及的患者仍继续接受内科治疗，肿瘤得到控制，未见明显增大；余 8 例患者术后 3、6、12 个月复查病变部位 X 线片示，植骨已融合，钢板、髓内钉均牢固固定、未见松动，肿瘤无复发。术后 1 年患者疼痛症状均改善，恢复正常生活；其中 3 例右股骨颈累及患者行走自如，生活质量明显提升。.
结论: ECD 患者经过手术治疗均可消除病变、解除疼痛，术后近期未见复发；且手术治疗相较于内科治疗有快速解除疼痛、提高生活质量、副作用小等优点。.
Keywords: Erdheim-Chester disease; Langerhans cell histiocytosis; lipid granulomatosis; surgical treatment.