小儿蜡泪样骨病一例手术治疗长期随访报告及文献复习

A long-term follow up of surgical treatment for a child with melorheostosis: a case report and literature review

蜡泪样骨病是一种非常罕见的病变。小儿蜡泪样骨病的临床表现与成人截然不同,表现为骨内膜型骨质增生,在X线片上显现致密的条纹骨偏居髓腔一侧,患肢常出现严重的软组织挛缩和关节畸形。本文报告1例小儿蜡泪样骨病导致膝关节严重外翻畸形和髌骨顽固性脱位患者,应用外侧软组织松解、内侧强化软组织稳定和股内侧肌外移(动力性稳定)的手术方案。术后成功获得复位和矫形,随访19年下肢发育正常,功能良好,能参加体育运动。2014年Kitta等报告了1例相同的病例,经手术治疗获得复位和矫形,并指出手术治疗应在骨骺闭合前尽早实施。

Melorheostosis is a rare disorder of osteopathia.The clinical characteristics of melorheostosis in children is totally different from that in adults.The radiographic features of melorheostosis include the hyperostosis in endosteal on the one side of the cavum medullare and formed streakiness.The soft tissue contracture of the limb and joint deformities are the symptoms of melorheostosis.Here,the authors reported a melorheostosis case of 6 years old girl who suffered from severe valgus deformity of the right knee with permanent patellar dislocation.A surgical stabilization was applied with lateral soft tissue release,medial soft tissue stabilization and transferred the vastus medialis laterally(kinetic stability).After 19 years follow up postoperatively,the limb developed well in satisfied alignment with good function of knee joints,even participated in some sports activities.Other authors reported a similar case of melorheostosis with surgical treatment and achieved good outcomes in limb realignment and reduction.The further suggested that the surgical treatment should be produced before epiphyseal closure.