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Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case
Ellis-van Creveld (EVC) syndrome is a rare inherited condition with inheritance, which is autosomal recessive in nature and is also described as skeletal dysplasia (chondroectodermal). The patients present with a grave genu valgum deformity which is a major challenge in orthopedics. The current case...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9933149/ https://www.ncbi.nlm.nih.gov/pubmed/36819414 http://dx.doi.org/10.7759/cureus.33863 |
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author | Harkare, Vismay V Mankar, Sushil Faizan, Mohammed Nemade, Amit |
author_facet | Harkare, Vismay V Mankar, Sushil Faizan, Mohammed Nemade, Amit |
author_sort | Harkare, Vismay V |
collection | PubMed |
description | Ellis-van Creveld (EVC) syndrome is a rare inherited condition with inheritance, which is autosomal recessive in nature and is also described as skeletal dysplasia (chondroectodermal). The patients present with a grave genu valgum deformity which is a major challenge in orthopedics. The current case report presents a young girl of the juvenile age group who came with deformity over the bilateral lower limb with difficulty in walking and bilateral upper limb polydactyly. The patient underwent relevant investigations and examinations which were suggestive of bilateral genu valgum deformity. Since the deformity was significant, a corrective osteotomy with soft tissue release was planned followed by rehabilitative physiotherapy. Considering the extensive nature of the surgery, a staged procedure was planned. The patient on follow-up presented with a good range of motion and improved gait. Various treatment modalities have been described for the correction of the deformity but few of them are done in patients with EVC syndrome and they state varying results and high incidences of relapse. The present study focuses on corrective osteotomy with soft tissue release as a treatment modality and describes the outcome associated with the modality. Thus, stating that with proper planning and postoperative rehabilitation the patient can achieve a good functional outcome. |
format | Online Article Text |
id | pubmed-9933149 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-99331492023-02-17 Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case Harkare, Vismay V Mankar, Sushil Faizan, Mohammed Nemade, Amit Cureus Orthopedics Ellis-van Creveld (EVC) syndrome is a rare inherited condition with inheritance, which is autosomal recessive in nature and is also described as skeletal dysplasia (chondroectodermal). The patients present with a grave genu valgum deformity which is a major challenge in orthopedics. The current case report presents a young girl of the juvenile age group who came with deformity over the bilateral lower limb with difficulty in walking and bilateral upper limb polydactyly. The patient underwent relevant investigations and examinations which were suggestive of bilateral genu valgum deformity. Since the deformity was significant, a corrective osteotomy with soft tissue release was planned followed by rehabilitative physiotherapy. Considering the extensive nature of the surgery, a staged procedure was planned. The patient on follow-up presented with a good range of motion and improved gait. Various treatment modalities have been described for the correction of the deformity but few of them are done in patients with EVC syndrome and they state varying results and high incidences of relapse. The present study focuses on corrective osteotomy with soft tissue release as a treatment modality and describes the outcome associated with the modality. Thus, stating that with proper planning and postoperative rehabilitation the patient can achieve a good functional outcome. Cureus 2023-01-17 /pmc/articles/PMC9933149/ /pubmed/36819414 http://dx.doi.org/10.7759/cureus.33863 Text en Copyright © 2023, Harkare et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Orthopedics Harkare, Vismay V Mankar, Sushil Faizan, Mohammed Nemade, Amit Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case |
title | Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case |
title_full | Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case |
title_fullStr | Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case |
title_full_unstemmed | Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case |
title_short | Surgical Outcome of Complex Knee Deformity Correction in a Girl With Ellis-van Creveld Syndrome: A Report of a Rare Case |
title_sort | surgical outcome of complex knee deformity correction in a girl with ellis-van creveld syndrome: a report of a rare case |
topic | Orthopedics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9933149/ https://www.ncbi.nlm.nih.gov/pubmed/36819414 http://dx.doi.org/10.7759/cureus.33863 |
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