Boero S, Catagni M, Donzelli O, Facchini R, Frediani P V
Istituto Scientifico Giannina Gaslini, Genova, Italy.
J Pediatr Orthop. 1997 Sep-Oct;17(5):675-84. doi: 10.1097/00004694-199709000-00019.
We reexamined 21 patients with congenital pseudarthrosis of the leg (congenital pseudoarthrosis of the tibia; CPT) associated with neurofibromatosis-1 (NF-1), > or =2 years after the termination of treatment, for a statistical study of the results obtained by using Ilizarov's external fixator. Of the 21 tibias operated on, 17 consolidated after the first treatment, whereas four did not. Of the 17 consolidated tibias, four refractured and were retreated by using a variety of methods. Only one healed. At follow-up, which occurred > or =2 years after the removal of the fixator, the results were nine consolidations without deformities or with shortening <2 cm, five consolidations with axial deviation, and seven nonconsolidations. The statistically significant results were that (a) patients who were aged 5 years or older at operation had better results, and (b) the assembly II (resection of CPT stumps and their short-term compression possibly associated with corticotomy or epiphyseal distraction to correct limb discrepancy) gave better final results compared with the other device assemblies. We conclude that treatment with Ilizarov's fixator allows (a) a good percentage of healing over time (66.7%), especially in cases of normotrophic and cystic CPT; (b) further operations with or without the fixator to correct secondary or residual axial deviation; and (c) correction of limb discrepancy. This treatment avoids risking injury to the healthy contralateral leg. Additionally, for treatments that do not achieve satisfactory results, other treatment methods are not excluded. The CPT still remains a difficult problem for the orthopedic surgeon to solve.
我们对21例患有与1型神经纤维瘤病（NF-1）相关的小腿先天性假关节（胫骨先天性假关节；CPT）的患者进行了重新检查，这些患者在治疗结束后≥2年，以对使用伊里扎洛夫外固定器所获得的结果进行统计学研究。在接受手术的21根胫骨中，17根在首次治疗后愈合，而4根未愈合。在17根愈合的胫骨中，4根再次骨折并采用了多种方法进行再次治疗。只有1根愈合。在去除固定器后≥2年进行的随访中，结果为9根愈合且无畸形或缩短<2 cm，5根愈合伴有轴向偏差，7根未愈合。具有统计学意义的结果是：（a）手术时年龄≥5岁的患者效果更好；（b）与其他装置组合相比，组合II（切除CPT残端并对其进行短期加压，可能与骨皮质切开术或骨骺牵张术联合以纠正肢体不等长）的最终效果更好。我们得出结论，使用伊里扎洛夫固定器进行治疗能够：（a）随着时间推移有较高比例的愈合（66.7%），尤其是在营养正常和囊性CPT的病例中；（b）使用或不使用固定器进行进一步手术以纠正继发性或残余轴向偏差；（c）纠正肢体不等长。这种治疗避免了对健康对侧腿造成损伤的风险。此外，对于未取得满意效果的治疗，不排除采用其他治疗方法。CPT对于骨科医生来说仍然是一个难以解决的问题。
