Artioli Elena, Mazzotti Antonio, De Pellegrin Maurizio, Arceri Alberto, Zielli Simone Ottavio, Langone Laura, Faldini Cesare
IRCCS Istituto Ortopedico Rizzoli, Via Giulio Cesare Pupilli 1, 40136, Bologna, Italy.
1st Orthopaedic and Traumatologic Clinic, Italy.
J Orthop. 2024 Feb 20;52:49-54. doi: 10.1016/j.jor.2024.02.036. eCollection 2024 Jun.
Dysplasia epiphysealis hemimelica is a rare non-inherited condition characterized by the unilateral predominance of osteochondromas in one or more epiphyses, with ankles and knees being the most affected joints. Treatment approaches vary based on the localization of the disease, encompassing both conservative and surgical options. Due to its rarity, there is a lack of definitive surgical guidelines or specific treatment modalities. Therefore, the objective of this systematic review was to thoroughly investigate dysplasia epiphysealis hemimelica to provide evidence-based guidance for managing this condition, specifically focusing on the foot and ankle.
A systematic search was performed on PubMed and the Cochrane Library to identify all published articles related to dysplasia epiphysealis hemimelica of the foot and ankle. Individual patient information, such as gender, age, disease type, follow-up, localization, clinical presentation, intervention, and complications, were systematically extracted from each article and analyzed.
Twenty-five eligible publications were included in the review, involving a total of 70 patients (16 females, 53 males). The mean age was 9.6 years (SD 7.3). The talus was the most prevalent location and clinical presentations included mass and pain in 54% of cases. Surgical procedures were chosen in 92% of patients, with 95% undergoing mass excision. Recurrence was the most frequent complication, observed in 9% of cases.
Raising awareness about dysplasia epiphysealis hemimelica is crucial for early diagnosis and treatment, positively impacting clinical outcomes. Vigilant monitoring is essential during observational management, as unchecked mass growth can complicate surgical intervention. Surgical treatment focuses on mass excision, feasible even at a young age but requiring precision to prevent recurrence or secondary arthritis.
IV.
骨骺半侧发育异常是一种罕见的非遗传性疾病，其特征为一个或多个骨骺处骨软骨瘤单侧占优势，踝关节和膝关节是最常受累的关节。治疗方法因疾病部位而异，包括保守治疗和手术治疗。由于其罕见性，缺乏明确的手术指南或特定的治疗方式。因此，本系统评价的目的是全面研究骨骺半侧发育异常，为该疾病的管理提供循证指导，尤其关注足踝部。
在PubMed和Cochrane图书馆进行系统检索，以识别所有已发表的与足踝部骨骺半侧发育异常相关的文章。从每篇文章中系统提取个体患者信息，如性别、年龄、疾病类型、随访、部位、临床表现、干预措施和并发症，并进行分析。
本评价纳入了25篇合格的出版物，共涉及70例患者（女性16例，男性53例）。平均年龄为9.6岁（标准差7.3）。距骨是最常见的部位，54%的病例临床表现为肿块和疼痛。92%的患者选择了手术治疗，其中95%接受了肿块切除术。复发是最常见的并发症，9%的病例出现复发。
提高对骨骺半侧发育异常的认识对于早期诊断和治疗至关重要，对临床结果有积极影响。在观察性管理期间，密切监测至关重要，因为肿块不受控制的生长会使手术干预复杂化。手术治疗侧重于肿块切除，即使在年轻时也可行，但需要精确操作以防止复发或继发性关节炎。
IV级。
