Albiñana J, Cuervo M, Certucha J A, Gonzalez-Mediero I, Abril J C
Pediatric Orthopaedic Division, Hospital Niño Jesús, Madrid, Spain.
J Pediatr Orthop B. 1997 Jan;6(1):52-5. doi: 10.1097/01202412-199701000-00011.
Focal fibrocartilaginous dysplasia has previously been described as an etiology of genu varum deformity in children at about walking age. We have found 20 cases in the literature, and all of them were located in the medial proximal tibial metaphysis. We report five additional cases of this entity. The patients had a genu varum deformity, and the angulation was at the site of the bone lesion. Four cases were in the tibia, and one case was in the distal medial femoral metaphysis. Age at diagnosis ranged from 13 to 24 months. A cortical defect with surrounding sclerosis was observed on radiographs. The angulation usually progressed initially, and spontaneous resolution was observed in two cases at last follow-up. Open biopsy and tibial valgus osteotomy were performed in two cases. The unusual femoral case was also treated with open biopsy and medial hemicircumferential periosteal release. Spontaneous remodeling of varus angulation and resolution of bony defect may be expected in most cases, and osteotomy can be avoided.
局灶性纤维软骨发育异常此前已被描述为儿童约学步期膝内翻畸形的一个病因。我们在文献中发现了20例，所有病例均位于胫骨近端内侧干骺端。我们报告另外5例该疾病。患者存在膝内翻畸形，成角位于骨病变部位。4例在胫骨，1例在股骨远端内侧干骺端。诊断时的年龄为13至24个月。X线片显示皮质缺损伴周围硬化。成角通常最初会进展，在最后一次随访时2例观察到自发矫正。2例行开放活检及胫骨外翻截骨术。这例不常见的股骨病例也接受了开放活检及内侧半环形骨膜松解术。大多数病例可预期膝内翻成角的自发重塑及骨缺损的消退，可避免进行截骨术。
