Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases.
Orthop Traumatol Surg Res
; 100(8): 941-6, 2014 Dec.
Article
em En
| MEDLINE
| ID: mdl-25453924
ABSTRACT
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 ANDMETHODS:
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. LEVEL OF EVIDENCE IV.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Tíbia
/
Doenças do Desenvolvimento Ósseo
/
Fêmur
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
Limite:
Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
Orthop Traumatol Surg Res
Ano de publicação:
2014
Tipo de documento:
Article