Gao Fei, Chen Guo, Wang Ruoyu, Huang Pan, Wang Jing, Xu Weihua
Department of Orthopaedics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.
The First People's Hospital of Jiangxia District, Wuhan city, China.
BMC Musculoskelet Disord. 2020 Mar 14;21(1):167. doi: 10.1186/s12891-020-3146-3.
Dysplasia epiphysealis hemimelica (DEH), also known as Trevor's disease, is a rare skeletal developmental disorder affecting the epiphyses in pediatric patients. DEH is characterized by an asymmetric osteochondral overgrowth arising from either the medial or lateral portion of an epiphysis and usually occurs in the joints of lower limbs, most commonly in the knees and ankles. However, bilateral involvement in an adult is extremely rare, and total knee arthroplasty (TKA) for a patient with DEH has been reported only once before.
Here, we present a case of bilateral DEH of the knees that caused varus deformity and dysfunction of the lower limbs in a 50-year-old man. TKA was performed for treatment, and the patient had satisfactory function with no angular knee deformity and a normal range of motion after 1 year of follow-up.
The patient in this case exhibited its specific clinical and radiological features of late-term DEH and TKA was proved to be an appropriate procedure for treating the severe deformity caused by this rare disease.
骨骺发育异常半侧肥大(DEH),又称特雷弗病,是一种罕见的骨骼发育障碍,影响小儿患者的骨骺。DEH的特征是骨骺内侧或外侧出现不对称的骨软骨过度生长,通常发生在下肢关节,最常见于膝关节和踝关节。然而,成人双侧受累极为罕见,此前仅有1例报道为DEH患者行全膝关节置换术(TKA)。
在此,我们报告1例50岁男性双膝双侧DEH病例,该病例导致下肢内翻畸形和功能障碍。行TKA治疗,随访1年后,患者功能良好,无膝关节角状畸形,活动范围正常。
该病例患者表现出晚期DEH的特定临床和放射学特征,TKA被证明是治疗这种罕见疾病所致严重畸形的合适手术。