MULTIPLE EPIPHYSEAL DYSPLASIA

Peter J. Curcione, D.O., Resident, Orthopaedic Surgery

Robert P. Stanton, M.D., Attending, Pediatric Orthopaedic Surgery

August 17, 1995

CLINICAL CASE PRESENTATION

ORTHOPAEDIC DEPARTMENT

THE ALFRED I. DUPONT INSTITUTE

WILMINGTON, DELAWARE

Etiology/History:
Clinical and Radiographic:
Treatment:

Progressive disorder usually leading to osteoarthritis in weight-bearing joints by the second or third generation of life.

Patients should refrain from contact sports, high impact activities, (ie. jogging, running).

Weight control should be emphasized early on.

Patients tend to tire easily... they must rest more frequently.

Osteotomies about the knee can be done in order to correct alignment.

Varus osteotomy of the femur should be avoided, as these people tend to develop a varus deformity and incongruity as a result of this dysplasia.

If AVN is superimposed on M.E.D.. then stretching programs should be started . No data is yet available on results of surgical containment or bracing of hips.

Patients will most likely require joint replacement surgery in adulthood.

Bibliography:

1. Treble,N.J.,Jensen,F.O.,Bankier,A.,Rogers,J.G.,Cole,W,G. Development of the hip in Multiple Epiphyseal Dysplasia. J.B.J.S. 1990; 72-B: 1061-1064.

2. Mackenzie, W.G., Basset, G.S., Mandell, G.A., Scott, C.I. Avascular necrosis of the hip in Multiple Epiphyseal Dysplasia. J.P.O. 1990; 9: 666-671.

3. Schlesinger, A.E., Poznanski, A.K., Pudlowski, R.M., Millar, E.A. Distal femoral epiphysis: Normal standards for thickness and application to bone dysplasias. Radiology. 1986; 159: 515-519.

4. Poznanski, A.K., Hernandez, R.J., Guire, K.E., Bereza, U.L., Garn, S.M. Carpal length in children- A useful measurement in the diagnosis of rheumatoid arthritis and some congenital malformation syndromes. Radiology 1978; 129: 661-668.

5. Ingram, R.R. Early diagnosis of Multiple Epiphyseal Dysplasia. J.P.O. 1992; 12: 241-244.

6. Crossan,J.F. Bilateral failure of the capital femoral epiphysis: Bilateral Perthes' disease, Multiple Epiphyseal Dysplasia, Psuedoachondroplasia and Spondyloepiphyseal Dysplasia Congenita and Tarda. J.P.O. 1983; 3:297-301.

7. Stanescu, R., Stanescu, V., Muriel, M., Maroteaux, P. Multiple Epiphyseal Dysplasia, Fairbank Type: Morphologic and biochemical study of cartilage. Am. J. Med. Gen. 1993; 45: 501-507.

8. Deere,M., Blanton, S.H., Scott, C.I., Langer, L.O., Pauli, R.M., Hecht, J.T. Genetic heterogeneity in M.E.D. Am. J, Hum. Genet. 1995; 56:698-704.