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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-mut...

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Autores principales: Dagnino, Augusto, Ursino, Nicola, Ripamonti, Carlo A. M., Fiorentini, Carlo E., Scelsi, Michele, D'Ambrosi, Riccardo, Portinaro, Nicola M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Georg Thieme Verlag KG 2017
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5738471/
https://www.ncbi.nlm.nih.gov/pubmed/29270564
http://dx.doi.org/10.1055/s-0037-1606619
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author Dagnino, Augusto
Ursino, Nicola
Ripamonti, Carlo A. M.
Fiorentini, Carlo E.
Scelsi, Michele
D'Ambrosi, Riccardo
Portinaro, Nicola M.
author_facet Dagnino, Augusto
Ursino, Nicola
Ripamonti, Carlo A. M.
Fiorentini, Carlo E.
Scelsi, Michele
D'Ambrosi, Riccardo
Portinaro, Nicola M.
author_sort Dagnino, Augusto
collection PubMed
description 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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spelling pubmed-57384712017-12-21 Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis Dagnino, Augusto Ursino, Nicola Ripamonti, Carlo A. M. Fiorentini, Carlo E. Scelsi, Michele D'Ambrosi, Riccardo Portinaro, Nicola M. Joints 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. Georg Thieme Verlag KG 2017-09-12 /pmc/articles/PMC5738471/ /pubmed/29270564 http://dx.doi.org/10.1055/s-0037-1606619 Text en https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License, which permits unrestricted reproduction and distribution, for non-commercial purposes only; and use and reproduction, but not distribution, of adapted material for non-commercial purposes only, provided the original work is properly cited.
spellingShingle Dagnino, Augusto
Ursino, Nicola
Ripamonti, Carlo A. M.
Fiorentini, Carlo E.
Scelsi, Michele
D'Ambrosi, Riccardo
Portinaro, Nicola M.
Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis
title Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis
title_full Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis
title_fullStr Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis
title_full_unstemmed Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis
title_short Total Hip Arthroplasty in an Inveterate Femoral Neck Fracture in a Patient with Congenital Insensitivity to Pain with Anhidrosis
title_sort total hip arthroplasty in an inveterate femoral neck fracture in a patient with congenital insensitivity to pain with anhidrosis
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5738471/
https://www.ncbi.nlm.nih.gov/pubmed/29270564
http://dx.doi.org/10.1055/s-0037-1606619
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