摘要
目的 描述儿童骨骼局灶性纤维软骨发育不良（FFCD）的临床与X线特征,探讨其自然病程与治疗方法的选择.方法 2004年至2009年期间收治的4例儿童FFCD,男3例,女1例,发病年龄8个月～1岁9个月,就诊年龄为1岁～2岁1个月;病变位于胫骨近端2例、股骨远端1例、尺骨远端1例.4例均手术治疗.结果 2例（股骨与尺骨病变）经手术病灶切除、截骨矫形或骨痂牵伸延长,经过18个月和66个月随访观察,发现成角畸形或短缩畸形得到完全矫正;另2例胫骨近端病变因成角畸形＞20°,患儿年龄≤2岁,采取单纯病灶切除,术后6个月和12个月的X线片,显示其成角畸形呈现进行性减少,但仍需继续观察.4例病理结果均为FFCD.结论 儿童胫骨FFCD具有自然矫正的潜力,特别是2岁以下者允许临床观察,但股骨、尺骨和肱骨FFCD因自然矫正潜力很小,常需要手术治疗.
Objective To review the clinic presentation, radiographic and MRI appearance of Focal Fibrocartilaginous Dysplasia（FFCD）in children. Methods We retrospectively assessed the radiological and MRI characteristics of FFCD in 4 children. The operative indications and methods were evaluated. Results We reported 4 children with FFCD which have been confirmed by pathology, of which distal femur was involved in one case and distal ulna in one child and proximal tibia in the remaining two infants. The cases involved in distal ulna and distal femur were treated with excision of the lesion, correction osteotomy or femur lengthening with Ilizarov techenique. The two infants with proximal tibia involvement underwent simple excision of the lesion in order to accelerate the correction and to confirm the diagnosis histologically, The. results of the two patients involved distal ulna and distal femur were satisfactory. The angular deformity were completely corrected and normal length was restored at follow-up 66 months and 18 months post-operatively. The proximal tibia vara of FFCD in two infant children improved progressively after 6 and 12 months operatively. Conclusions Focal fibrocartilaginous dysplasia （FFCD） is a rare benign condition inducing bowing deformity of the long bones and the lesions often involved proximal tibia,distal femur and distal ulna. Radiologically, the lesions is characterized by a lucent defect with marginal sclerosis in the medial metepysis of the long bone. The entity represents a bony anchor preventing natural sliding of the periosteum during growth. Treatment indications result from this concept,of which the lesions with a metaphyseal-diaphyseal angle less than 20 degrees,observation is needed for 6 to 12 months; if the deformity improves, the tether likely broke spontaneously, and no treatment is required; if the deformity worsens,, curettage or corrective osteotomy will be needed.
出处
《中华小儿外科杂志》
CSCD
北大核心
2011年第1期44-48,共5页
Chinese Journal of Pediatric Surgery
