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In children, intramedullary nailing (IN) has been proposed as the best treatment when the femur and tibia are totally affected by fibrous dysplasia (FD). However, in younger children IN must be repeated to maintain stabilization of the affected skeletal segment during growth. We report the long-term results in a cohort of patients in whom more than two-thirds of cases had IN repeated during growth.Twenty-nine femurs and 14 tibias totally affected by FD were treated by IN in 21 patients with polyostotic FD and McCune-Albright syndrome. Thirteen patients with 35 femoral and tibial deformities had a painful limp whereas 8 presented fractures. The patients had their first IN at a mean age of 9.26±2.68 years (range: 4 to 14 y). IN was repeated during growth in the younger patients, and all the patients underwent a mean of 2.13 femoral and 1.50 tibial IN per limb. The last IN was performed at a mean age of 16.42±1.95 years (range: 11 to 19 y). Titanium elastic nails and adult humeral nails were used in younger children, whereas adult femoral cervicodiaphyseal and interlocking tibial nails were used in older children and adolescents. At the latest follow-up, the patients were evaluated with a clinicoradiographic scale. All the data were statistically analyzed.The mean length of follow-up from the last IN was 6.47±3.10 years (range: 3 to 14 y), and the mean age of the patients at follow-up was 22.85±3.53 years (range: 14 to 29 y) when lower limbs were fully grown in all but 1 patient. Satisfactory long-term results were obtained in about 81% of our patients, while complications occurred in 32.5% of the 43 cases.Lower limb IN-that was repeated in younger children during growth-provided satisfactory long-term results in most of our patients, with fracture and deformity prevention and pain control, regardless of the high rate of complications that mainly affected the femoral cases. Missing scheduled follow-ups was the main predictor of a poor result.Level IV-case series.

We investigated the correlation between the musculoskeletal features and the cardiovascular anomalies in pediatric patients affected by Marfan syndrome, in order to identify possible orthopedic deformities that could be a warning sign for severe aortic dilatation. Moreover, we analyzed the role of the orthopedic aspects in the early diagnosis of the disease in a pediatric population. Seventy-two patients from 3 to 14 years of age, underwent interdisciplinary evaluation that included an orthopedic and cardiological examination. At the orthopedic examination, we analyzed the musculoskeletal features included in the systemic score of the revised Ghent criteria. Cardiological evaluation included a transthoracic echocardiography with definition of the cardiac Z-score, which is an index that evaluates aortic diameter. A statistical analysis was performed. We identified a statistically significant correlation between the presence of pectus excavatum and cardiac Z-score ≥3 (P = 0.022). Clinically, this data means that pectus excavatum is frequently observed in patients with larger aortic root diameter. On the contrary, no statistically significant correlation was found between the other investigated musculoskeletal features and a pathological Z-score. In the pediatric population, the diagnosis of Marfan syndrome remains difficult because many clinical manifestations are age-dependent and the Ghent criteria, usually used for adults, are not reliable in children. Our results show that the presence of pectus excavatum could help in the early identification of patients at greater risk of developing possibly fatal aortic disease. However, it is always indicated to screen all patients with Marfan syndrome for cardiac abnormalities, even in absence of pectus excavatum.