Abstract
Background: Multiple osteochondromas is a rare skeletal disorder characterized by the presence of osteocartilaginous protrusions causing bony deformities, especially around the knee. Guided growth by temporary hemiepiphyseal stapling is the treatment of choice to correct the deformity by modulating the residual physeal growth of the lower limbs. Although this procedure is increasingly practiced, inconclusive evidence exists regarding its effectiveness in children with multiple osteochondromas. The study aims to compare the outcomes of temporary hemiepiphyseal stapling for correcting genu valgum in children with multiple osteochondromas vs. idiopathic cases. Methods: In this retrospective cohort study, we included patients admitted at a single institution from 2008 to 2018. A total of 97 children (77 idiopathic, 20 multiple osteochondromas) were enclosed, accounting for 184 limbs treated by temporary hemiepiphyseal stapling. We investigated if children with multiple osteochondromas had a similar successful rate of correction, rate of complications, and correction velocity compared to children with idiopathic genu valgum. Results: Overall, 151 limbs (82%) achieved complete correction or overcorrection, with idiopathic cases having a significantly higher rate of success compared to pathologic cases (88% vs. 55%; p < 0.001). In addition, multiple osteochondromas children sustained a higher rate of major complications (p = 0.021) and showed significantly lower correction velocity (p = 0.029). Conclusion: Temporary hemiepiphyseal stapling is effective in both idiopathic and multiple osteochondromas children, although the latter often achieved incomplete correction, had a higher risk of complications, and required a longer time of stapling. We suggest to anticipate the timing of intervention; otherwise, children with multiple osteochondromas and severe valgus deformity, approaching skeletal maturity, could undergo combined femoral and tibial stapling.
Highlights
Multiple osteochondromas (MO; OMIM #133700, #133701), is a rare condition caused by mutations on the exostosin-1 (EXT1) or exostosin-2 (EXT2) gene in 90% of patients [1]
We asked if MO children have a similar rate of correction, rate of complications, and correction velocity compared with children with idiopathic genu valgum and analyzed which factors could predict the rate and velocity of correction
According to a method proposed by several authors [18,19], we identified four zones on both the medial and lateral side of the knee, on the basis of the mechanical axis deviation (MAD) location at staple removal (−2 or less = uncorrected; −1 = partially corrected; +1 = corrected; +2 or more = overcorrected)
Summary
Multiple osteochondromas (MO; OMIM #133700, #133701), is a rare condition caused by mutations on the exostosin-1 (EXT1) or exostosin-2 (EXT2) gene in 90% of patients [1]. Guided growth by temporary hemiepiphyseal stapling (HeS) has become the treatment of choice to correct the knee deformities by modulating the residual growth of the physes [12,13,14,15,16,17]. Guided growth by temporary hemiepiphyseal stapling is the treatment of choice to correct the deformity by modulating the residual physeal growth of the lower limbs. This procedure is increasingly practiced, inconclusive evidence exists regarding its effectiveness in children with multiple osteochondromas. The study aims to compare the outcomes of temporary hemiepiphyseal stapling for correcting genu valgum in children with multiple osteochondromas vs idiopathic cases. We suggest to anticipate the timing of intervention; otherwise, children with multiple osteochondromas and severe valgus deformity, approaching skeletal maturity, could undergo combined femoral and tibial stapling
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