Patients with Ollier's Disease (OD) and Maffucci Syndrome (MS) are at risk for fractures, deformities, and limb-length discrepancies (LLDs). This study aims to determine the rate and types of surgery performed on these patients and evaluate the prevalence of developing a malignancy at a prior surgical site. A survey link was posted to a private online social media support group page specific to OD and MS patients. A total of 125 surveys were obtained from five continents. Two surveys were incomplete, and 123 surveys were evaluated with 109 OD and 14 MS patients. 89% had at least one surgery at a mean age of 9.1 years, with an overall average of 7.6 surgeries. Common surgical indications included lower-extremity LLD and deformity correction, and symptomatic enchondroma resection. 19% developed a chondrosarcoma, and 35% of these occurred at a previous surgical site. Four patients developed metastatic disease, and two had an intramedullary nail placed during a prior procedure. The majority of patients with OD and MS will undergo surgery, require multiple procedures, and may develop a chondrosarcoma at a prior surgical site. Caution should be taken in this at-risk population with special consideration for the type of surgery and implants used.
Colello et al. (Thu,) studied this question.