Dysplasia Epiphysealis Hemimelica Treatment & Management

Updated: Jun 07, 2018
  • Author: David A Forsh, MD; Chief Editor: Harris Gellman, MD  more...
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Treatment

Approach Considerations

Dysplasia epiphysealis hemimelica (DEH), or Trevor disease, should be treated if the lesion is causing deformity, pain, or interference with function.

Supportive joint care, consisting of short-term splinting of the joint, may be beneficial in management. Most cases are treated surgically, but recurrence is common. [29, 38, 39] The most common procedures are excision of the mass and corrective osteotomy. [30]  Excision may be arthroscopic or open. Corrective osteotomy and, subsequently, hemiepiphysiodesis have been described for correction of deformity. [30, 23]  Surgery is contraindicated if no medical symptoms or no mechanical block is present.

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Surgical Therapy

Most reported cases of DEH in the literature have been treated surgically. Surgical treatment has involved excising the mass and correcting any angular deformity while preserving the integrity of the affected joint to the extent possible. [40, 41]

Oberc et al reported on their experience in treating DEH in six children. [42] Of the four patients treated surgically, two experienced late complications. Of the two patients treated conservatively, one had a positive outcome and the other a negative outcome involving hip ankylosis. The authors advised that conservative treatment involving physical therapy be employed initially in the region of pain and that surgical treatment be considered if pain, joint deformation, or limited range of motion (ROM) persists.

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Complications

Recurrence of the angular deformity after the corrective osteotomy may be anticipated if the growth plate at the affected joint is open and active and the lesion itself has not been removed. [30, 31]

Other potential complications include the following:

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Long-Term Monitoring

After surgery, a short period of immobilization with a cast or splint is required for the affected limb, followed by active ROM of the joint.

Bosch et al conducted a retrospective study of nine patients with DEH who were treated surgically and were followed up clinically with imaging. [44] Magnetic resonance imaging (MRI) was found to be useful for identifying a potential plane of cleavage between the epiphysis and the pathologic tissue. The investigators recommended early removal of ossifications when a cleavage plane is identified and advised against waiting for a possible complication or for an increase in size.

Because of the risk of recurrence, patients should be followed until skeletal maturity. [24]

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