HEREDITARY MULTIPLE EXOSTOSIS

 

HEREDITARY MULTIPLE EXOSTOSIS

•Develops during childhood.

•Generally asymptomatic but can cause mechanical symptoms due to impingement or lump formation.

•Usually sporadic but can be part of:
ü Hereditary multiple exostoses (Diaphyseal Aclasia)
ü Trevor disease (Dysplasia epiphysealis hemimelica

•Most common location:
ü Lower limb (DISTAL FEMUR > PROXIMAL TIBIA)
ü Upper limb (HUMERUS)

X-RAY FEATURES:

•Sessile or pedunculated.
•Arises from metaphyseal region typically projecting away from epiphysis.
•Associated broadening of the metaphysis from which it arises.
 
•Cartilage cap can be thin or thick with rings and arc calcification.
 
CT FEATURES-

•Better demonstration of medullary continuity and cartilage cap.

MRI FEATURES-

•Assessment of malignant transformation 

              - Cartilage cap thickness (>1.5 cm)

              - Neurovascular compromise