Cargando…

Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva

INTRODUCTION: Fibrodysplasia ossificans progressiva (FOP) is the severest disease of ossification in the human. It forms an exoskeleton gradually. This process is started for a nodule that ossifies as days goes by. The initial pathway cannot be stopped unless early immunosuppress management is start...

Descripción completa

Detalles Bibliográficos
Autores principales: Colmenares-Bonilla, Douglas, Gonzalez-Segoviano, Alejandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Indian Orthopaedic Research Group 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5974675/
https://www.ncbi.nlm.nih.gov/pubmed/29854691
http://dx.doi.org/10.13107/jocr.2250-0685.990
_version_ 1783326862866382848
author Colmenares-Bonilla, Douglas
Gonzalez-Segoviano, Alejandra
author_facet Colmenares-Bonilla, Douglas
Gonzalez-Segoviano, Alejandra
author_sort Colmenares-Bonilla, Douglas
collection PubMed
description INTRODUCTION: Fibrodysplasia ossificans progressiva (FOP) is the severest disease of ossification in the human. It forms an exoskeleton gradually. This process is started for a nodule that ossifies as days goes by. The initial pathway cannot be stopped unless early immunosuppress management is started very early after the aggression has been received. These aggressions may vary from contusions, viral illnesses, surgery, and other minor trauma. Because of this process, the FOP patient gradually worsens and decreases its autonomy as described from ability to walk, dress, or even to eat and breathe. CASE REPORT: An 11-year-old female comes to evaluation for multiple ossifications, which worsen her autonomy and the ability to walk. The last ossification keeps the knee bent at 90° by a bony bridge from the femur to the tibia. To try to walk, it is necessary to flex hip, and ankle, with tilting of the pelvis, with pain and loss of autonomy. Due to the characteristic ossification pattern, FOP is diagnosed and the possibility of surgery to prolong autonomic gait is suggested. The procedure is performed under immunosuppression, and the knee is extended to a position that allows the patient to walk again freely. The steroid management is continued the convenient time to avoid exacerbation of the disease at a distance, and suspend it definitively afterward. CONCLUSIONS: The FOP is adding an exoskeleton and its episodes of exacerbation end in new ossifications that deteriorate the quality of life. Once the ossifications have appeared, it is not possible to stop or reverse the process. Performing an osteotomy to prolong gait is a risky procedure. However, the present study shows that final ossification is expected at the point of resection of the extracted bone and suggests that immunosuppressing the patient could prevent distant exacerbations. The risk-benefit ratio of the procedure may be favorable if it is performed at a quiescent stage of the disease.
format Online
Article
Text
id pubmed-5974675
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher Indian Orthopaedic Research Group
record_format MEDLINE/PubMed
spelling pubmed-59746752018-05-31 Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva Colmenares-Bonilla, Douglas Gonzalez-Segoviano, Alejandra J Orthop Case Rep Case Report INTRODUCTION: Fibrodysplasia ossificans progressiva (FOP) is the severest disease of ossification in the human. It forms an exoskeleton gradually. This process is started for a nodule that ossifies as days goes by. The initial pathway cannot be stopped unless early immunosuppress management is started very early after the aggression has been received. These aggressions may vary from contusions, viral illnesses, surgery, and other minor trauma. Because of this process, the FOP patient gradually worsens and decreases its autonomy as described from ability to walk, dress, or even to eat and breathe. CASE REPORT: An 11-year-old female comes to evaluation for multiple ossifications, which worsen her autonomy and the ability to walk. The last ossification keeps the knee bent at 90° by a bony bridge from the femur to the tibia. To try to walk, it is necessary to flex hip, and ankle, with tilting of the pelvis, with pain and loss of autonomy. Due to the characteristic ossification pattern, FOP is diagnosed and the possibility of surgery to prolong autonomic gait is suggested. The procedure is performed under immunosuppression, and the knee is extended to a position that allows the patient to walk again freely. The steroid management is continued the convenient time to avoid exacerbation of the disease at a distance, and suspend it definitively afterward. CONCLUSIONS: The FOP is adding an exoskeleton and its episodes of exacerbation end in new ossifications that deteriorate the quality of life. Once the ossifications have appeared, it is not possible to stop or reverse the process. Performing an osteotomy to prolong gait is a risky procedure. However, the present study shows that final ossification is expected at the point of resection of the extracted bone and suggests that immunosuppressing the patient could prevent distant exacerbations. The risk-benefit ratio of the procedure may be favorable if it is performed at a quiescent stage of the disease. Indian Orthopaedic Research Group 2018 /pmc/articles/PMC5974675/ /pubmed/29854691 http://dx.doi.org/10.13107/jocr.2250-0685.990 Text en Copyright: © Indian Orthopaedic Research Group http://creativecommons.org/licenses/by-nc/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Colmenares-Bonilla, Douglas
Gonzalez-Segoviano, Alejandra
Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva
title Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva
title_full Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva
title_fullStr Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva
title_full_unstemmed Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva
title_short Bone Resection Osteotomy in Fibrodysplasia Ossificans Progressiva
title_sort bone resection osteotomy in fibrodysplasia ossificans progressiva
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5974675/
https://www.ncbi.nlm.nih.gov/pubmed/29854691
http://dx.doi.org/10.13107/jocr.2250-0685.990
work_keys_str_mv AT colmenaresbonilladouglas boneresectionosteotomyinfibrodysplasiaossificansprogressiva
AT gonzalezsegovianoalejandra boneresectionosteotomyinfibrodysplasiaossificansprogressiva