Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases

Background: Dysplasia epiphysealis hemimelica (DEH) is a rare developmental bone disorder with hemimelic involvement of one or more epiphysis. We report on nine new cases and discuss the clinical manifestations, the value of MRI, and the results of complete and early surgical resection of these lesi...

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محفوظ في:
التفاصيل البيبلوغرافية
المؤلف الرئيسي: Assi, C. (author)
مؤلفون آخرون: Bosch, C. (author), Louahem, D. (author), Alkar, F. (author), Mazeau, P. (author), Delfour, C. (author), Canavese, F. (author), Prodhomme, O. (author), Cottalorda, J. (author)
التنسيق: article
منشور في: 2014
الوصول للمادة أونلاين:http://hdl.handle.net/10725/5267
http://dx.doi.org/10.1016/j.otsr.2014.07.022
http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php
http://www.sciencedirect.com/science/article/pii/S1877056814002709
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author Assi, C.
author2 Bosch, C.
Louahem, D.
Alkar, F.
Mazeau, P.
Delfour, C.
Canavese, F.
Prodhomme, O.
Cottalorda, J.
author2_role author
author
author
author
author
author
author
author
author_facet Assi, C.
Bosch, C.
Louahem, D.
Alkar, F.
Mazeau, P.
Delfour, C.
Canavese, F.
Prodhomme, O.
Cottalorda, J.
author_role author
dc.creator.none.fl_str_mv Assi, C.
Bosch, C.
Louahem, D.
Alkar, F.
Mazeau, P.
Delfour, C.
Canavese, F.
Prodhomme, O.
Cottalorda, J.
dc.date.none.fl_str_mv 2014
2017-02-23T10:23:15Z
2017-02-23T10:23:15Z
2017-02-23
dc.identifier.none.fl_str_mv 1877-0568
http://hdl.handle.net/10725/5267
http://dx.doi.org/10.1016/j.otsr.2014.07.022
Bosch, C., Assi, C., Louahem, D., Alkar, F., Mazeau, P., Delfour, C., ... & Cottalorda, J. (2014). Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases. Orthopaedics & Traumatology: Surgery & Research, 100(8), 941-946.
http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php
http://www.sciencedirect.com/science/article/pii/S1877056814002709
dc.language.none.fl_str_mv en
dc.relation.none.fl_str_mv Orthopaedics & Traumatology: Surgery & Research
dc.rights.*.fl_str_mv info:eu-repo/semantics/openAccess
dc.title.none.fl_str_mv Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
dc.type.none.fl_str_mv Article
info:eu-repo/semantics/publishedVersion
info:eu-repo/semantics/article
description Background: Dysplasia epiphysealis hemimelica (DEH) is a rare developmental bone disorder with hemimelic involvement of one or more epiphysis. We report on nine new cases and discuss the clinical manifestations, the value of MRI, and the results of complete and early surgical resection of these lesions. Materials and methods: In this retrospective study, nine patients with a diagnosis of DEH were evaluated. Age at presentation ranged from 1 year to 12 years. The main complaint at diagnosis was a swelling bony mass. Angular deformities were recorded in two patients. All patients were surgically treated and followed up clinically and by imaging. Eight patients underwent excision only. Results: The average follow-up was 5.6 years (range, 2–10.5 years). All patients had a good outcome without related symptoms. No epiphysiodesis, angular deformity or recurrence was observed. One patient with femoral lesion involving the distal medial part of the epiphysis developed, four months after surgical excision, a calcification outside the area of total excision. This calcification did not increase in size at two years follow-up. Another patient with lateral involvement of the proximal tibial epiphysis presented a postoperative nervous complication. Spontaneous nervous recovery occurred three months after surgery. Discussion: MRI was useful to find a potential plane of cleavage between the epiphysis and the pathological tissue. We recommend early removing ossifications when a cleavage plane is identified. Waiting a possible complication or increasing of size does not seem logical. Of course, the treatment will be not the same if no cleavage plane is found on MRI.
eu_rights_str_mv openAccess
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identifier_str_mv 1877-0568
Bosch, C., Assi, C., Louahem, D., Alkar, F., Mazeau, P., Delfour, C., ... & Cottalorda, J. (2014). Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases. Orthopaedics & Traumatology: Surgery & Research, 100(8), 941-946.
language_invalid_str_mv en
network_acronym_str LAURepo
network_name_str Lebanese American University repository
oai_identifier_str oai:laur.lau.edu.lb:10725/5267
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spelling Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine casesAssi, C.Bosch, C.Louahem, D.Alkar, F.Mazeau, P.Delfour, C.Canavese, F.Prodhomme, O.Cottalorda, J.Background: Dysplasia epiphysealis hemimelica (DEH) is a rare developmental bone disorder with hemimelic involvement of one or more epiphysis. We report on nine new cases and discuss the clinical manifestations, the value of MRI, and the results of complete and early surgical resection of these lesions. Materials and methods: In this retrospective study, nine patients with a diagnosis of DEH were evaluated. Age at presentation ranged from 1 year to 12 years. The main complaint at diagnosis was a swelling bony mass. Angular deformities were recorded in two patients. All patients were surgically treated and followed up clinically and by imaging. Eight patients underwent excision only. Results: The average follow-up was 5.6 years (range, 2–10.5 years). All patients had a good outcome without related symptoms. No epiphysiodesis, angular deformity or recurrence was observed. One patient with femoral lesion involving the distal medial part of the epiphysis developed, four months after surgical excision, a calcification outside the area of total excision. This calcification did not increase in size at two years follow-up. Another patient with lateral involvement of the proximal tibial epiphysis presented a postoperative nervous complication. Spontaneous nervous recovery occurred three months after surgery. Discussion: MRI was useful to find a potential plane of cleavage between the epiphysis and the pathological tissue. We recommend early removing ossifications when a cleavage plane is identified. Waiting a possible complication or increasing of size does not seem logical. Of course, the treatment will be not the same if no cleavage plane is found on MRI.PublishedN/A2017-02-23T10:23:15Z2017-02-23T10:23:15Z20142017-02-23Articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article1877-0568http://hdl.handle.net/10725/5267http://dx.doi.org/10.1016/j.otsr.2014.07.022Bosch, C., Assi, C., Louahem, D., Alkar, F., Mazeau, P., Delfour, C., ... & Cottalorda, J. (2014). Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases. Orthopaedics & Traumatology: Surgery & Research, 100(8), 941-946.http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.phphttp://www.sciencedirect.com/science/article/pii/S1877056814002709enOrthopaedics & Traumatology: Surgery & Researchinfo:eu-repo/semantics/openAccessoai:laur.lau.edu.lb:10725/52672021-03-19T10:03:24Z
spellingShingle Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
Assi, C.
status_str publishedVersion
title Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
title_full Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
title_fullStr Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
title_full_unstemmed Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
title_short Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
title_sort Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases
url http://hdl.handle.net/10725/5267
http://dx.doi.org/10.1016/j.otsr.2014.07.022
http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php
http://www.sciencedirect.com/science/article/pii/S1877056814002709