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The hip in hereditary multiple exostoses.

D E Porter1, M K Benson, G A Hosney

  • 1Nuffield Orthopaedic Centre, Oxford, England.

The Journal of Bone and Joint Surgery. British Volume
|October 18, 2001
PubMed
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Hereditary multiple exostoses (HME) commonly causes hip dysplasia and coxa valga due to osteochondroma load. These hip abnormalities may stem from an inherited defect or the osteochondromas themselves.

Area of Science:

  • Orthopedics
  • Radiology
  • Genetics

Background:

  • Hereditary multiple exostoses (HME) is a genetic disorder characterized by the development of multiple osteochondromas.
  • Hip abnormalities, including dysplasia and coxa valga, are frequently observed in HME patients.

Purpose of the Study:

  • To radiologically define hip dysplasia and coxa valga characteristics in HME patients.
  • To quantify the 'osteochondroma load' and its effect on hip morphology.
  • To investigate labral pathology, osteoarthritis, and malignant changes in HME hips.

Main Methods:

  • Radiological analysis of 24 hips in 12 patients diagnosed with HME.
  • Quantification of osteochondroma load around the hip.
  • Measurement of hip parameters including neck-shaft angle, centre-edge angle, and Sharp's acetabular angle.

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Main Results:

  • Coxa valga and hip dysplasia were prevalent, with median values for neck-shaft angle (156°), centre-edge angle (23°), and Sharp's acetabular angle (44°).
  • Significant femoral neck overgrowth and increased neck/shaft diameter ratio were noted in HME hips compared to controls.
  • Osteochondroma load correlated with coxa valga and influenced hip dysplasia indices.

Conclusions:

  • HME is associated with significant hip anomalies, suggesting a potential generalized inherited defect.
  • Osteochondromas may play a direct role in precipitating characteristic hip abnormalities seen in HME.
  • Further research is needed to elucidate the precise mechanisms linking HME to hip pathology.