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 protectionmechanism. 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 performa 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.

Total hip arthroplasty in an inveterate femoral neck fracture in a patient with congenital insensitivity to pain with anhidrosis / A. Dagnino, N. Ursino, C.A.M. Ripamonti, C.E. Fiorentini, M. Scelsi, R. D'Ambrosi, N.M. Portinaro. - In: JOINTS. - ISSN 2282-4324. - 5:4(2017 Dec), pp. 249-252. [10.1055/s-0037-1606619]

Total hip arthroplasty in an inveterate femoral neck fracture in a patient with congenital insensitivity to pain with anhidrosis

C.A.M. Ripamonti;M. Scelsi;R. D'Ambrosi;N.M. Portinaro
2017

Abstract

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 protectionmechanism. 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 performa 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.
Congenital insensitivity to pain with anhidrosis; Dual mobility cup; Femoral neck fracture; Hereditary sensory autonomic neuropathy; Total hip arthroplasty
Settore MED/33 - Malattie Apparato Locomotore
dic-2017
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/660256
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