Clinique Maussins-Nollet, COS Ramsay Santé, Paris, France.
AP-HP, Raymond Poincaré Hospital, Reference Center for Ehlers-Danlos Syndromes, Garches, France.
Orphanet J Rare Dis. 2024 Sep 23;19(1):351. doi: 10.1186/s13023-024-03261-3.
Ehlers-Danlos syndrome (EDS) is a hereditary disease characterised by joint hypermobility, skin hyperextensibility and tissue fragility. Hypermobile EDS (hEDS is the more frequent subtype. Joint surgery may benefit certain patients after failure of medical treatments, but there is no consensus on the optimal surgical management of patients with hEDS. The aims of this retrospective study were to chart the surgical management of patients with hEDS, to determine the role of arthroscopy and to evaluate the functional results of joint surgery, including the reintervention rates.
A total of 69 patients with non-vascular EDS were evaluated (60 female; 87%). Mean (SD) age at first surgery was 25.6 ± 11.1 years. Among the 69 patients, first surgeries were carried out on the knee (n = 50; 39.4%), ankle (n = 28; 22.0%), shoulder (n = 22; 17.3%), wrist (n = 18; 14.2%) and elbow (n = 9; 7.1%). One-fifth of all first operations (20.8%) were carried out by arthroscopy, most often on the knee (36% of knee surgery cases). At the time of primary surgery, the surgeon was alerted to the diagnosis or suspicion of hEDS in only 33.9% of patients. The rate of reoperations (2 to ≥ 5) was 35.7% (10/28) for the ankle, 40.9% (9/22) for the shoulder, 44.4% (4/9) for the elbow, 50% (9/18) for the wrist and 60% (30/50) for the knee. Local or regional anaesthesia was badly tolerated or ineffective in 27.8%, 36.4% and 66.6% of operations on the wrist, shoulder and elbow, respectively. Overall, the majority of patients (> 70%) were satisfied or very satisfied with their surgery, particularly on the non-dominant side. The lowest satisfaction rate was for shoulder surgery on the dominant side (58.3% dissatisfied).
Surgery for joint instability has a greater chance of success when it is carried out in patients with a known diagnosis of EDS before surgery. The majority of patients were satisfied with their surgery and, with the exception of the knee, there was a low rate of reoperations (≤ 50%). Arthroscopic procedures have an important role to play in these patients, particularly when surgery is performed on the knee.
埃勒斯-当洛斯综合征（EDS）是一种遗传性疾病，其特征为关节过度活动、皮肤过度伸展和组织脆弱。可动性 EDS（hEDS 是更为常见的亚型。关节手术后可能对某些药物治疗失败的患者有益，但对于 hEDS 患者的最佳手术管理尚无共识。本回顾性研究的目的是描述 hEDS 患者的手术管理情况，确定关节镜的作用，并评估关节手术的功能结果，包括再次手术率。
共评估了 69 例非血管性 EDS 患者（60 例女性；87%）。首次手术的平均（SD）年龄为 25.6±11.1 岁。在 69 例患者中，首次手术分别在膝关节（n=50；39.4%）、踝关节（n=28；22.0%）、肩关节（n=22；17.3%）、腕关节（n=18；14.2%）和肘关节（n=9；7.1%）进行。所有初次手术的五分之一（20.8%）为关节镜手术，其中膝关节（36%的膝关节手术）最常见。在初次手术时，只有 33.9%的患者的外科医生注意到或怀疑 hEDS。踝关节的再手术率（2 次至≥5 次）为 35.7%（28 例中的 10 例），肩关节为 40.9%（22 例中的 9 例），肘关节为 44.4%（9 例中的 4 例），腕关节为 50%（18 例中的 9 例），膝关节为 60%（50 例中的 30 例）。在腕关节、肩关节和肘关节手术中，分别有 27.8%、36.4%和 66.6%的患者对局部或区域麻醉耐受性差或无效。总体而言，大多数患者（>70%）对手术感到满意或非常满意，尤其是在非优势侧。对优势侧肩关节手术的满意度最低（58.3%不满意）。
在手术前已知 EDS 诊断的情况下，对关节不稳定进行手术，其成功率更高。大多数患者对手术感到满意，除膝关节外，再次手术率（≤50%）较低。关节镜手术在这些患者中具有重要作用，尤其是在膝关节手术中。
