The natural history of multiple osteochondromas in a large Italian cohort of pediatric patients

• DOI: 10.1016/j.bone.2020.115499
• Publication Date: 2020-06-24T16:13:10Z
• Authors: Marina Mordenti; Fei Shih; Manila Boarini; Elena Pedrini; Maria Gnoli; Diego Antonioli; Morena Tremosini; Luca Sangiorgi
• Keywords: Demographics; Natural history study

Multiple osteochondromas is a rare hereditary skeletal disorder, characterized by bony protrusions arising from growth plates on long bones during development. The disorder frequently leads to diminished stature, deformities and functional limitations. Understanding of the natural history multiple its evolution in children adolescents limited.To provide valuable information osteochondromas, inform recommendations for treatment prevent impairments caused osteochondromas.This retrospective cohort study with includes longitudinal data collected first last follow-up visit patient demographics, over 36 months disease evolution.Data were Registry Osteochondromas, which circa 1200 patients treated 2003 2017 at IRCCS Istituto Ortopedico Rizzoli Bologna.Patients ≤18 years who provided written informed consent had ≥1 12-month visit.Demographics, clinical features, incidence surgeries, (progression or regression) assessed. Results summarized using descriptive statistics, annual rates new features Kaplan-Meier estimates. Patient height was evaluated following Italian charts.158 included these analyses. Throughout follow-up, 80.4% developed 57.6% deformities, 23.4% limitation(s). New osteochondroma(s) 28.5% Month 12, 39.9% 24, 50% 36. Most detected younger population; aged 0-4 underwent significantly higher number lesions within 24 follow-up. overall deformity 12 17.7%, incidences decreasing increasing age (p = .023). In addition, analyses highlight that 13 cut off slow stature < .0005). At visit, 46.2% progression, while regression (spontaneous surgical) occurred 7.6% .007).This reports main set clinically relevant development, providing insight management development therapeutic interventions.