Diagnosis and surgical treatment of dysplasia epiphysealis hemimelica. A report of nine cases - 06/11/14

Doi : 10.1016/j.otsr.2014.07.022

C. Bosch ^a, C. Assi ^d, D. Louahem ^a, F. Alkar ^a, P. Mazeau ^a, C. Delfour ^b, F. Canavese ^e, O. Prodhomme ^c, J. Cottalorda ^a,^⁎
^a Department of Pediatric Orthopaedics Surgery, University hospital of Montpellier, 34295 Montpellier, France
^b Department of Histopathology, University hospital of Montpellier, 34295 Montpellier, France
^c Department of Pediatric Radiology, University hospital of Montpellier, 34295 Montpellier, France
^d Department of Pediatric Orthopedics Surgery, University hospital of Beirut, Beirut, Lebanon
^e Department of Pediatric Surgery, University hospital of Clermont-Ferrand, 69003 Clermont-Ferrand, France

^⁎Corresponding author. Service d’orthopédie infantile, hôpital Lapeyronie, CHU de Montpellier, 37, avenue du Doyen-Gaston-Giraud, 34295 Montpellier cedex 5, France. Tel.: +33 4 67 33 87 61; fax: +33 4 67 33 05 00.

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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 and methods

In this retrospective study, nine patients with a diagnosis of DEH were evaluated. Age at presentation ranged from 1year to 12years. 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 5.6years (range, 2–10.5years). 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.