Multiple Epiphyseal Dysplasia Treatment & Management
- Author: Ashish S Ranade, MBBS, MRCS, MS; Chief Editor: Dennis P Grogan, MD more...
The goals of medical management of multiple epiphyseal dysplasia (MED) are to alleviate pain and to halt joint destruction and the development of early osteoarthritis. Pain management can be challenging in MED; therefore, a physical therapy program with adequate analgesia is important. Patients benefit from a referral to a rheumatologist or a pain management specialist.
Weight management is also important. When clinicians prescribe physical therapy, they should avoid recommending specific exercises that exert repetitive stresses on the patient's affected joints.
The goals of surgical therapy for MED are pain relief, correction of angular deformities, and correction of joint contractures. Treatment options include realignment procedures and arthroplasty to manage advanced osteoarthritis.
Hip arthroscopy has been used for the treatment of acute or semiacute changes in hip pain in patients with MED. A wide spectrum of intra-articular pathologies (eg, labral tears, chondral flaps, and loose bodies) can be addressed by means of arthroscopy. However, this treatment is palliative, and these patients develop early osteoarthritis.
A painful or subluxed skeletally immature hip is treated with surgical intervention. An acetabular shelf procedure can be done for coverage. Preexisting coxa vara often precludes femoral varus-producing osteotomy. In one report, good outcomes in terms of deformity correction were achieved by using intertrochanteric extension osteotomy and trochanteric arthroplasty.
Patients with MED tend to develop early-onset osteoarthritis. They become symptomatic in the second or third decade of life. Total joint arthroplasty is the last resort for the management of advanced osteoarthritis.
Angular deformities can be corrected by means of realignment osteotomy performed near the time of skeletal maturity or by means of hemiepiphyseal stapling. In a study that used stapling for angular deformity correction, it was observed that physeal behavior after staple removal was unpredictable, and overcorrection should be avoided.
Treatment options for the knee include (1) corrective osteotomy for the femur, the tibia, or both to correct angular deformity and (2) removal of loose bodies. Treatment options for a double-layered patella in symptomatic patients include (1) excision of one fragment and (2) fusion of two fragments. For the treatment of osteoarthritis, total knee replacement is necessary.
Preoperative planning plays an important role in the surgical treatment of patients with MED. Factors to consider in the planning of total knee replacement include malpositioning of the tibial tubercle, hypoplasia of the femoral condyle, and subluxation of the patella.
In surgical treatment of the hip, it is important to account for the altered anatomy of the proximal femur and the patient's young age. In particular, it is vital to address the following altered anatomic features:
A large femoral head, which is deficient in acetabular coverage
A short femoral neck
A high greater trochanter
In addition to standard postoperative care, a comprehensive multidisciplinary approach is important and essential for rehabilitation.
Complications that may arise after surgical treatment of MED include the following:
Recurrence of deformities
Aseptic loosening of prosthetic components
Intraoperative or postoperative periprosthetic fractures
Regular follow-up is important after any surgical intervention. Such follow-up is necessary to monitor the patient for progression to osteoarthritis after he or she undergoes any realignment procedure. Also, continuing care enables the clinician to detect signs of loosening and periprosthetic fractures after total joint arthroplasty; the incidence of these complications is high.
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