Mo Yueqiang, Wu Chunxing, Ning Bo, Wang Dahui
Department of Pediatric Orthopedics, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, 201102, P. R. China.
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2024 Oct 15;38(10):1214-1219. doi: 10.7507/1002-1892.202406056.
To evaluate the effectiveness of Cross-Union surgery for the treatment of pseudarthrosis of the tibia (PT) with neurofibromatosis type 1 (NF1).
The clinical data of 8 children of PT with NF1 who met the selection criteria between January 2018 and December 2023 was retrospectively analyzed. There were 5 boys and 3 girls, and the operative age ranged from 1.8 to 13.3 years with a median age of 3.5 years. According to Paley classification, there were 2 cases of type 2a, 2 cases of type 3, 2 cases of type 4a, and 2 cases of type 4c. There were 5 cases of first operation and 3 cases of re-fracture after previous operation. Six cases had leg length discrepancy before operation, and 2 of them had shortening over 2.0 cm. Except for 1 case of ankle fusion, the other 7 cases had ankle valgus. Preoperative coronal/sagittal angulation was recorded. Postoperative pseudarthrosis healing and refracture were observed. Leg length discrepancy and tibiotalar angle were measured and recorded before operation and at last follow-up. Inan imaging evaluation criteria was used to evaluate the imaging effect.
All patients were followed up 12-37 months (mean, 23.5 months). One pseudarthrosis failed to heal at 12 months after operation and healed at 3 months after reoperation, while the other pseudarthrosis healed with a healing rate of 87.5% and a healing time of 4-8 months (mean, 5.3 months). No refracture occurred during the follow-up. At last follow-up, there were 2 new cases with leg length discrepancy, which were 0.7 cm and 1.3 cm, respectively. In 2 cases with the leg length discrepancy more than 2.0 cm before operation, the improvement was from 4.1 cm and 12.6 cm to 2.1 cm and 9.0 cm, respectively. There was no significant difference in leg length discrepancy between pre- and post-operation in 8 cases ( >0.05). At last follow-up, 6 patients still had ankle valgus, and there was no significant difference in the tibiotalar angle between pre- and post-operation ( >0.05); the tibial coronal/sagittal angulation significantly improved when compared with that before operation ( <0.05). According to Inan imaging evaluation criteria, 1 case was good, 6 cases were fair, and 1 case was poor.
Cross-Union surgery is an effective method for the treatment of PT with NF1 in children, can achieve good bone healing results with a low risk of re-fracture. The surgery may not have significant effects on leg length discrepancy and ankle valgus, and further treatment may be required.
评估交叉联合手术治疗1型神经纤维瘤病（NF1）合并胫骨假关节（PT）的有效性。
回顾性分析2018年1月至2023年12月期间符合入选标准的8例NF1合并PT患儿的临床资料。其中男5例，女3例，手术年龄为1.8至13.3岁，中位年龄为3.5岁。根据帕利分类，2a型2例，3型2例，4a型2例，4c型2例。初次手术5例，既往手术后再次骨折3例。术前6例存在下肢长度差异，其中2例短缩超过2.0 cm。除1例踝关节融合外，其余7例均有踝关节外翻。记录术前冠状面/矢状面成角情况。观察术后假关节愈合及再骨折情况。测量并记录术前及末次随访时的下肢长度差异和胫距角。采用伊南影像学评估标准评估影像学效果。
所有患者均获随访12至37个月（平均23.5个月）。1例假关节术后12个月未愈合，再次手术后3个月愈合，另一例假关节愈合，愈合率为87.5%，愈合时间为4至8个月（平均5.3个月）。随访期间无再骨折发生。末次随访时，新增2例下肢长度差异病例，分别为0.7 cm和1.3 cm。术前下肢长度差异超过2.0 cm的2例患者，改善情况分别从4.1 cm和12.6 cm降至2.1 cm和9.0 cm。8例患者术前与术后下肢长度差异无显著差异（P>0.05）。末次随访时，6例患者仍有踝关节外翻，术前与术后胫距角无显著差异（P>0.05）；与术前相比，胫骨冠状面/矢状面成角显著改善（P<0.05）。根据伊南影像学评估标准，优1例，良6例，差1例。
交叉联合手术是治疗儿童NF1合并PT的有效方法，能取得良好的骨愈合效果，再骨折风险低。该手术对下肢长度差异和踝关节外翻可能无显著影响，可能需要进一步治疗。
