[Clinical study of Cross-Union surgery for treatment of pseudarthrosis of tibia with neurofibromatosis type 1 in children].

Publisher: Hua xi yi ke da xue Fu shu di 1 yi yuan Country of Publication: China NLM ID: 9425194 Publication Model: Print Cited Medium: Print ISSN: 1002-1892 (Print) Linking ISSN: 10021892 NLM ISO Abbreviation: Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi Subsets: MEDLINE

Publication: Chengdu Shi : Hua xi yi ke da xue Fu shu di 1 yi yuan
Original Publication: Chengdu Shi : Hua xi yi ke da xue Fu shu di 1 yi yuan,

Pseudarthrosis*/surgery ; Pseudarthrosis*/etiology ; Neurofibromatosis 1*/surgery ; Neurofibromatosis 1*/complications ; Tibia*/surgery; Humans ; Male ; Female ; Child ; Retrospective Studies ; Child, Preschool ; Adolescent ; Treatment Outcome ; Infant ; Leg Length Inequality/surgery ; Leg Length Inequality/etiology ; Fracture Fixation, Internal/methods ; Tibial Fractures/surgery

Objective: To evaluate the effectiveness of Cross-Union surgery for the treatment of pseudarthrosis of the tibia (PT) with neurofibromatosis type 1 (NF1).
Methods: 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.
Results: 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 ( P >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 ( P >0.05); the tibial coronal/sagittal angulation significantly improved when compared with that before operation ( P <0.05). According to Inan imaging evaluation criteria, 1 case was good, 6 cases were fair, and 1 case was poor.
Conclusion: 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.

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Keywords: Neurofibromatosis type 1; children; pseudarthrosis of the tibia; radiolological evaluation; re-fracture
Local Abstract: [Publisher, Chinese] 评估Cross-Union术式治疗儿童Ⅰ型神经纤维瘤病（neurofibromatosis type 1，NF1）型胫骨假关节（pseudarthrosis of the tibia，PT）的临床疗效。. [Publisher, Chinese] 回顾分析2018年1月—2023年12月收治且符合选择标准的8例NF1型PT患儿临床资料。其中男5例，女3例；手术年龄1.8～13.3岁，中位年龄3.5岁。Paley分型：2a型2例、3型2例、4a型2例、4c型2例。首次手术5例，既往手术后再骨折3例。术前6例存在下肢不等长，其中2例不等长超过2.0 cm；除1例踝关节融合外，余7例均为踝外翻。记录术前胫骨冠状面/矢状面成角情况，术后随访观察假关节愈合及再骨折发生情况，测量并记录术前及末次随访时下肢不等长、胫距角。采用Inan方法评估影像学效果。. [Publisher, Chinese] 8例患儿均获随访，随访时间12～37个月，平均23.5个月。1例术后12个月假关节未达骨愈合，再次手术后3个月达骨愈合；其余患儿假关节均达骨愈合，愈合率87.5%，愈合时间4～8个月，平均5.3个月。所有患儿随访期间均未发生再骨折。末次随访时，新增2例肢体不等长患儿，不等长分别为0.7 cm和1.3 cm；2例术前不等长超过2.0 cm者分别由4.1、12.6 cm改善为2.1、9.0 cm；8例患儿手术前后下肢不等长比较差异均无统计学意义（ P >0.05）。末次随访时，6例患儿仍存在踝外翻，手术前后胫距角差异无统计学意义（ P >0.05）；胫骨冠状面/矢状面成角较术前明显改善，差异有统计学意义（ P <0.05）。根据Inan影像学评价标准评价为良1例，一般6例，差1例。. [Publisher, Chinese] Cross-Union术式治疗儿童NF1型PT可达到良好骨愈合效果，再骨折风险低；但对于肢体不等长及踝外翻等伴随畸形效果不明显。.

Date Created: 20241021 Date Completed: 20241021 Latest Revision: 20241101

20241101

PMC11522534

10.7507/1002-1892.202406056

39433495