全髋关节置换术治疗顽固性股骨颈骨折1例先天性疼痛不敏感伴无汗症患者。

Q1 Medicine Joints Pub Date : 2017-09-12 eCollection Date: 2017-12-01 DOI:10.1055/s-0037-1606619
Augusto Dagnino, Nicola Ursino, Carlo A M Ripamonti, Carlo E Fiorentini, Michele Scelsi, Riccardo D'Ambrosi, Nicola M Portinaro
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引用次数: 1

摘要

先天性无汗性疼痛不敏感(CIPA)是一种极其罕见的疾病,其特征是自主神经和感觉神经功能障碍,对深层和浅层疼痛刺激不敏感,不能出汗和流泪,轻度至中度智力迟钝,有自残行为。由于疼痛不能作为一种保护机制,对这些患者来说,根深蒂固的肌肉骨骼损伤的相关后果是一个主要问题。出于同样的原因,患者在术后康复过程中也存在风险,在选择骨科植入物时应考虑到这一点。据我们所知,迄今为止文献中仅报道了一例全髋关节置换术。一位21岁患CIPA的白人男性患者来就诊,主诉左髋关节功能受限。无外伤史报道。x光片显示股骨颈骨折伴股骨头严重坏死和吸收。我们决定进行全髋关节置换术,采用骨水泥假体和骨水泥双活动杯。术后过程和康复令人满意,1年Harris髋关节评分的临床结果非常好。
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Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis.

Congenital insensitivity to pain with anhidrosis (CIPA) is an extremely rare disorder characterized by autonomic and sensory nerves malfunction with insensitivity to both deep and superficial painful stimuli, inability to sweat and produce tears, and mild to moderate mental retardation with self-mutilating behavior. Related consequences of inveterate musculoskeletal injuries represent a major issue for these patients, since pain cannot act as a protection mechanism. For the same reason, the patients are at risk during postoperative rehabilitation, which should be taken into account when selecting an orthopaedic implant. To our knowledge, only one case of total hip arthroplasty has been reported in the literature to date. A 21-year-old Caucasian male patient affected with CIPA arrived at our attention complaining about a functional limitation of the left hip. No history of trauma was reported. The X-rays showed an inveterate femoral neck fracture with a severe necrosis and resorption of the femoral head. We decided to perform a total hip arthroplasty with a cemented stem and a cemented dual mobility cup. The postoperative course and rehabilitation were satisfactory, with excellent clinical results, measured with the Harris Hip Score at 1 year.

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来源期刊
Joints
Joints Medicine-Rehabilitation
CiteScore
4.30
自引率
0.00%
发文量
0
期刊介绍: Joints is the official publication of SIGASCOT (Italian Society of the Knee, Arthroscopy, Sports Traumatology, Cartilage and Orthopaedic Technology). As an Open Acccess journal, it publishes papers on clinical and basic research, review articles, technical notes, case reports, and editorials about the latest developments in knee surgery, arthroscopy, sports traumatology, cartilage, orthopaedic technology, upper limb, and related rehabilitation. Letters to the Editor and comments on the journal''s content are always welcome.
期刊最新文献
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