Background: Osteofibrous dysplasia (OFD) is a rare non neoplastic, self-limited intracortical fibro-osseous lesion that most commonly affects the diaphysis of the tibia and fibula of children, the best treatment is still debated. Therefore we performed a retrospective study in children mostly under 10 years old with OFD aiming to determine whether early surgery is necessary and which is the best treatment. Hypothesis: Symptomatic OFD lesions should be treated proactively, and appropriate treatment can achieve favorable outcome. Methods: We retrospectively reviewed 23 patients with OFD of the tibia (n = 22) and fibula. Management varied according to the severity of symptoms (deformity, pain or pathological fracture) and the extent of the lesion. Cases were divided into four groups (Gr.): Gr. 1: observation (n = 4); Gr. 2: curettage and allograft (n = 6); Gr. 3: curettage, allograft and elastic stable intramedullary nailing (ESIN) fixation (n = 9); Gr. 4: extra-periosteal resection and bone transport (n = 5). One patient received two different treatments successively (23 patients and 24 cases). All patients had regular clinical and radiographic follow-up to assess bone consolidation and complications. Results: In Gr. 1, four asymptomatic patients underwent observation after open biopsy. Gr. 4 had the lowest local recurrence rate: 0 cases (0%) vs. 4 (66.7%; Gr. 2) vs. 1 (11.1%; Gr. 3) (p = 0.002), incidence of deformity: 0 case (0%) vs. 0 (0%; Gr. 2) vs. 3 (50%; Gr. 3) (p = 0.023), and pathological fracture: 0 case (0%) vs. 0 (0%; Gr. 2) vs. 1 (16.7%; Gr. 3) (p = 0.006), although the consolidation time was longer: 6.8 vs. 2 (Gr. 2) vs. 2.7 months (Gr. 3) (p = 0.017) and the rate of complication was higher than those of Gr. 2 and Gr. 3 (p < 0.05). Gr. 3 had a lower local recurrence rate: 1 case (11.1%) vs. 4 cases (66.7%) (p = 0.002), incidence of deformity: 0 case (0%) vs. 3 cases (50%) (p = 0.023), and pathological fracture: 0 case vs. 1 case (16.7%) (p = 0.006) than Gr. 2. Discussion: Open biopsy is an important step in the management of patients with imaging suggestive of OFD in order to rule out Adamantinoma and other bone tumors or infection. Observation should be reserved for asymptomatic patients, while surgical treatment is indicated in patients with persistent pain, pathological fracture or significant deformity of the tibia. ESIN is a valid option to preserve the anatomical axis of the tibia in symptomatic patients with lesions of limited size; large circumferential lesions or recurrence can be managed by extra-periosteal resection and bone transport, although the complication rate can be relatively high. Level of evidence: III; case control study.
Does the management of osteofibrous dysplasia of the tibia and fibula in children should be tailored to the extent and location of the lesion? A case control study investigating different surgical options
Canavese, Federico;
2023-01-01
Abstract
Background: Osteofibrous dysplasia (OFD) is a rare non neoplastic, self-limited intracortical fibro-osseous lesion that most commonly affects the diaphysis of the tibia and fibula of children, the best treatment is still debated. Therefore we performed a retrospective study in children mostly under 10 years old with OFD aiming to determine whether early surgery is necessary and which is the best treatment. Hypothesis: Symptomatic OFD lesions should be treated proactively, and appropriate treatment can achieve favorable outcome. Methods: We retrospectively reviewed 23 patients with OFD of the tibia (n = 22) and fibula. Management varied according to the severity of symptoms (deformity, pain or pathological fracture) and the extent of the lesion. Cases were divided into four groups (Gr.): Gr. 1: observation (n = 4); Gr. 2: curettage and allograft (n = 6); Gr. 3: curettage, allograft and elastic stable intramedullary nailing (ESIN) fixation (n = 9); Gr. 4: extra-periosteal resection and bone transport (n = 5). One patient received two different treatments successively (23 patients and 24 cases). All patients had regular clinical and radiographic follow-up to assess bone consolidation and complications. Results: In Gr. 1, four asymptomatic patients underwent observation after open biopsy. Gr. 4 had the lowest local recurrence rate: 0 cases (0%) vs. 4 (66.7%; Gr. 2) vs. 1 (11.1%; Gr. 3) (p = 0.002), incidence of deformity: 0 case (0%) vs. 0 (0%; Gr. 2) vs. 3 (50%; Gr. 3) (p = 0.023), and pathological fracture: 0 case (0%) vs. 0 (0%; Gr. 2) vs. 1 (16.7%; Gr. 3) (p = 0.006), although the consolidation time was longer: 6.8 vs. 2 (Gr. 2) vs. 2.7 months (Gr. 3) (p = 0.017) and the rate of complication was higher than those of Gr. 2 and Gr. 3 (p < 0.05). Gr. 3 had a lower local recurrence rate: 1 case (11.1%) vs. 4 cases (66.7%) (p = 0.002), incidence of deformity: 0 case (0%) vs. 3 cases (50%) (p = 0.023), and pathological fracture: 0 case vs. 1 case (16.7%) (p = 0.006) than Gr. 2. Discussion: Open biopsy is an important step in the management of patients with imaging suggestive of OFD in order to rule out Adamantinoma and other bone tumors or infection. Observation should be reserved for asymptomatic patients, while surgical treatment is indicated in patients with persistent pain, pathological fracture or significant deformity of the tibia. ESIN is a valid option to preserve the anatomical axis of the tibia in symptomatic patients with lesions of limited size; large circumferential lesions or recurrence can be managed by extra-periosteal resection and bone transport, although the complication rate can be relatively high. Level of evidence: III; case control study.
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