guided growth

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)

Goals

1. Equal leg lengths

- goal is mild to moderate isolated discrepancy

2. Unequal leg lengths

- goal with paralysis / equinus foot

- aim 1-2 cm short for clearance

3. Level Pelvis

- should use blocks to estimate functional correction

4. Vertical LS Spine

- vertical spine more important than level pelvis