关键词: Bilateral total hip arthroplasty Early-onset hip osteoarthritis Spondyloepiphyseal dysplasia

来  源:   DOI:10.13107/jocr.2021.v11.i01.1986   PDF(Pubmed)

Abstract:
UNASSIGNED: Spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA) is a rare inherited dysfunction with autosomal recessive inheritance. SEDT-PA is also named as progressive pseudorheumatoid arthropathy of childhood as it is associated with multiple joint contractures and arthritis. We report a case of SEDT-PA managed with bilateral stage total hip arthroplasty.
UNASSIGNED: A 22-year-old lady presented with severe bilateral hip arthritis. Based on her clinical and radiological features described in this article, she was diagnosed as having SEDT-PA. She was managed with bilateral stage total hip arthroplasty. The pre-operative planning and technical challenges of performing this procedure have been described.
UNASSIGNED: Dysfunctions originally of genetic origin like spondyloepiphyseal dysplasia tarda mimics and is commonly misdiagnosed as juvenile chronic arthritis. These patients have disabling early-onset hip arthritis which requires surgery. Arthroplasty is challenging in these patients because of the low proximal femur offset but good results can be obtained after thorough pre-operative planning to tackle intraoperative difficulties.
摘要:
脊柱骨发育不良伴进行性关节病(SEDT-PA)是一种罕见的遗传性功能障碍,具有常染色体隐性遗传。SEDT-PA也被称为儿童进行性假性类风湿关节病,因为它与多发性关节挛缩和关节炎有关。我们报告了一例SEDT-PA在双侧全髋关节置换术中进行治疗。
一位22岁的女士出现严重的双侧髋关节炎。根据本文描述的临床和放射学特征,她被诊断患有SEDT-PA.她接受了双侧阶段全髋关节置换术。已经描述了执行该程序的术前计划和技术挑战。
最初的遗传起源的功能障碍,如脊柱骨发育不良,通常被误诊为青少年慢性关节炎。这些患者患有致残的早发性髋关节炎,需要手术。由于股骨近端偏移较低,因此在这些患者中进行关节成形术具有挑战性,但是经过彻底的术前计划以解决术中困难,可以获得良好的结果。
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