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Background: Intramedullary nailing (IN) seems to be the best primary surgical treatment for patients with either polyostotic fibrous dysplasia or McCune-Albright syndrome (PFD/MAS) when the femur and tibia are totally affected by fibrous dysplasia (FD) and pain, fracture and deformity are likely to occur. However, other management protocols have been applied in these cases, often leading to disabling sequelae. This study sought to evaluate if IN could also have been effective as a salvage procedure to provide patients with satisfactory results, regardless of the poor results due to the improper treatment previously performed., Materials and Methods: Twenty-four retrospectively registered PFD/MAS patients with 34 femurs and 14 tibias totally affected by fibrous dysplasia had received various treatments with unsatisfactory results in other institutions. Before the IN performed in our hospital, 3 patients were wheelchair bound; 4 were fractured; 17 limped; and many used an aid for walking. Salvage IN was performed in our hospital at a mean patient age of 23.66 ± 6.06 years (range, 15-37 years). The patients were evaluated before-except for the four fractured ones-and after IN using the validated Jung scoring system, and the data were statistically analyzed., Results: The mean length of follow-up after IN was 9.12 ± 3.68 years (range, 4-17 years). The patients' mean Jung score significantly improved from 2.52 ± 1.74 points before IN to 6.78 ± 2.23 at follow-up (p < 0.05). Ambulation was improved in ambulatory patients and restored in wheelchair users. The complication rate was 21%., Conclusions: Regardless of the high rate of complications, IN may be considered a reliable surgical procedure to salvage a failed treatment in PFD/MAS, with long-lasting satisfactory results achieved in most patients. Trial registration statement: Not applicable., Level of Evidence: IV., (© 2023. The Author(s).)

Background: 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., Methods: 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., Results: 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., Conclusion: 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 of Evidence: Level IV-case series., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Polyostotic fibrous dysplasia (PFD) generally cause deformities and fractures of femur and tibia and surgery is often required. The current surgical treatment for deformities is based on single or multiple osteotomies followed by stabilization with intramedullary nails, which are commonly used also for fractures. One of the most common surgical complications of intramedullary nailing is represented by surgical site infection with possible extension to the whole skeletal segment. In the present study we evaluated the incidence of surgical site infections in 44 patients affected by PFD in which 91 femurs or tibiae underwent intramedullary nailing to treat deformities or fractures. We never observed any infection of the operated femurs or tibiae until the final follow-up. The only post surgical infection was present in a patient with monomelic involvement at the contralateral non affected limb, which was surgically treated for limb length inequality, by femur shortening osteotomy stabilized by an intramedullary nail. The most likely hypothesis to explain the complete absence of infections in these patients may be related to the high local concentration of prophylactic antibiotic in the highly vascularized fibrodysplastic bone., (Copyright 2020 Biolife Sas. www.biolifesas.org.)

Seven patients affected by McCune-Albright syndrome with polyostotic fibrous dysplasia were operated on either for fracture fixation or for correction of bone deformity. Ten femurs, three tibiae, and one humerus were stabilized by intramedullary titanium nailing with a modified unreamed femoral nail (UFN) and standard unreamed tibial (UTN) and humeral (UHN) nails. The age of the patients at operation ranged from 8 to 30 years, with an average of 17 years, and the length of follow-up ranged from 8 months to 4 years, with an average of 2 years. Before operation, each patient had suffered from a minimum of three fractures to a maximum of 12, with an average of six fractures. The femur was the most affected skeletal segment. Before surgery, two patients were wheelchair-dependent owing to their severe lower limb bone deformities. At follow-up, all the patients were painless and all were able to walk even though one had to wear a bilateral long leg brace and another had to ambulate with the aid of two crutches. No patient had had additional fractures after surgery. The main intra-operative technical problems of this demanding surgical procedure were: a) reaming of a new medullary canal through the fibrodysplastic bone; b) the amount of blood loss in femoral surgery that ranged from 800 to 2,500 ml.