Medical Therapy
The goals of medical management of multiple epiphyseal dysplasia (MED) are as follows:
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To alleviate pain
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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.
Kim et al reported favorable midterm outcomes with conservative management in 40 patients with hip joints affected by MED. [33]
Surgical Therapy
The goals of surgical therapy for MED are as follows:
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Pain relief
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Correction of angular deformities
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Correction of joint contractures
Treatment options include realignment procedures and arthroplasty to manage advanced osteoarthritis.
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 the following:
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Malpositioning of the tibial tubercle
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Hypoplasia of the femoral condyle
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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:
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A large femoral head, which is deficient in acetabular coverage
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A short femoral neck
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Coxa vara
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A high greater trochanter
Hip
Hip arthroscopy has been used for the treatment of acute or semiacute changes in hip pain in patients with MED. [31] 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. [34]
Medial displacement osteotomy (Chiari osteotomy) has been used to treat symptomatic patients with painful hips. In a study by Andrzejewski et al, 14 patients were reported to have favourable outcomes. [35] The Chiari osteotomy was found to reduce the pain, improve the hip function, and delay the need for total hip 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. [36] Patients with MED or other musculoskeletal dysplasias who undergo total joint arthroplasty may be at increased risk for surgical-site infection and perioperative hemorrhage, possibly because of the anatomic complexity encountered. [37]
Angular deformities
Angular deformities can be corrected by means of realignment osteotomy performed near the time of skeletal maturity or by means of hemiepiphyseal stapling. [38] 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.
Knee
Treatment options for the knee include the following:
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Corrective osteotomy for the femur, the tibia, or both to correct angular deformity
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Removal of loose bodies
Treatment options for a double-layered patella in symptomatic patients include the following [39] :
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Excision of one fragment
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Fusion of two fragments
For the treatment of osteoarthritis, total knee replacement is necessary.
Complications
Complications that may arise after surgical treatment of MED include the following:
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Recurrence of deformities
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Aseptic loosening of prosthetic components
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Intraoperative or postoperative periprosthetic fractures
Long-Term Monitoring
In addition to standard postoperative care, a comprehensive multidisciplinary approach is important and essential for rehabilitation.
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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Anteroposterior (AP) radiograph of the pelvis shows bilateral hip changes.
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Anteroposterior (AP) radiographs of the knee shows characteristic changes of multiple epiphyseal dysplasia (MED).
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Anteroposterior (AP) radiographs of the feet.
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Lateral radiographs of the right and left feet.
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Radiograph shows alignment of the lower extremities.
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Anteroposterior (AP) radiograph of the spine.
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Lateral radiograph of the spine.
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Lateral radiographs of the elbows.
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Anteroposterior (AP) radiograph of the hand.
