Rate and Type of Surgery and Prevalence of Malignancy in Patients With Ollier's Disease and Maffucci Syndrome

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.