screening

AKA

Diaphyseal Aclasis / Multiple Osteochondromas

Definition

Heritable skeletal dysplasia

Epidemiology

AD with variable penetrance (96%)

Malignant Transformation

Incidence of malignant transformation much higher 

- 10% overall

- 1 % / year

Chondrosarcoma (CS) > Osteosarcoma (OS)

Pathogenesis

Virchow's Triad

1. Venous stasis

2. Hypercoagulability

3. Endothelial damage

Starts as platelet nidus at valves

- thrombogenic materials elaborated by platelets

- leads to development of fibrin thrombus

- thrombus grows

Thrombus may 

- detach as embolus

- be completely dissolved / recanalise

- organise with valve incompetence