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Multiple Epiphyseal Dysplasia Treatment & Management

  • Author: Ashish S Ranade, MBBS, MRCS, MS; Chief Editor: Dennis P Grogan, MD  more...
 
Updated: Aug 26, 2015
 

Medical Therapy

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.

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Surgical Therapy

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

Hip arthroscopy has been used for the treatment of acute or semiacute changes in hip pain in patients with MED.[28] 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.[30]

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.[31]

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.[32] 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 (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.[33]  For the treatment of osteoarthritis, total knee replacement is necessary.

Perioperative care

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
  • Coxa vara
  • A high greater trochanter

In addition to standard postoperative care, a comprehensive multidisciplinary approach is important and essential for rehabilitation.

Complications

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
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Long-Term Monitoring

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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Contributor Information and Disclosures
Author

Ashish S Ranade, MBBS, MRCS, MS Fellow in Pediatric Orthopedics, Shriners Hospitals for Children of Philadelphia

Ashish S Ranade, MBBS, MRCS, MS is a member of the following medical societies: Royal College of Surgeons in Ireland

Disclosure: Nothing to disclose.

Coauthor(s)

James J McCarthy, MD, FAAOS, FAAP Director, Division of Orthopedic Surgery, Cincinnati Children's Hospital; Professor, Department of Orthopedic Surgery, University of Cincinnati College of Medicine

James J McCarthy, MD, FAAOS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Orthopaedic Association, Pennsylvania Medical Society, Philadelphia County Medical Society, Pennsylvania Orthopaedic Society, Pediatric Orthopaedic Society of North America, Orthopaedics Overseas, Limb Lengthening and Reconstruction Society, Alpha Omega Alpha, American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Orthopaedic Surgeons

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Orthopediatrics, Phillips Healthcare, POSNA<br/>Serve(d) as a speaker or a member of a speakers bureau for: Synthes<br/>Received research grant from: University of Cincinnati<br/>Received royalty from Lippincott Williams and WIcins for editing textbook; Received none from POSNA for board membership; Received none from LLRS for board membership; Received consulting fee from Synthes for none.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Dennis P Grogan, MD Clinical Professor (Retired), Department of Orthopedic Surgery, University of South Florida College of Medicine; Orthopedic Surgeon, Department of Orthopedic Surgery, Shriners Hospital for Children of Tampa

Dennis P Grogan, MD is a member of the following medical societies: American Medical Association, American Orthopaedic Association, Scoliosis Research Society, Irish American Orthopaedic Society, Pediatric Orthopaedic Society of North America, American Academy of Orthopaedic Surgeons, American Orthopaedic Foot and Ankle Society, Eastern Orthopaedic Association

Disclosure: Nothing to disclose.

Additional Contributors

Mininder S Kocher, MD, MPH Associate Professor of Orthopedic Surgery, Harvard Medical School/Harvard School of Public Health; Associate Director, Division of Sports Medicine, Department of Orthopedic Surgery, Children's Hospital Boston

Mininder S Kocher, MD, MPH is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Sports Medicine, Pediatric Orthopaedic Society of North America, American Association for the History of Medicine, American Orthopaedic Society for Sports Medicine, Massachusetts Medical Society

Disclosure: Received consulting fee from Smith & Nephew Endoscopy for consulting; Received consulting fee from EBI Biomet for consulting; Received consulting fee from OrthoPediatrics for consulting; Received stock from Pivot Medical for consulting; Received consulting fee from pediped for consulting; Received royalty from WB Saunders for none; Received stock from Fixes-4-Kids for consulting.

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Anteroposterior (AP) radiograph of the pelvis shows bilateral hip changes.
Anteroposterior (AP) radiographs of the knee shows characteristic changes of multiple epiphyseal dysplasia (MED).
Anteroposterior (AP) radiographs of the feet.
Lateral radiographs of the right and left feet.
Radiograph shows alignment of the lower extremities.
Anteroposterior (AP) radiograph of the spine.
Lateral radiograph of the spine.
Lateral radiographs of the elbows.
Anteroposterior (AP) radiograph of the hand.
 
 
 
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