CONGENITAL PSEUDOARTHROSIS OF THE TIBIA ASSOCIATED WITH NEUROFIBROMATOSIS-1 - TREATMENT WITH ILIZAROVS DEVICE

We reexamined 21 patients with congenital pseudarthrosis of the leg (c ongenital pseudoarthrosis of the tibia; CPT) associated with neurofibr omatosis-1 (NF-1), greater than or equal to 2 years after the terminat ion of treatment, for a statistical study of the results obtained by u sing Ilizarov's external fixator. Of the 21 tibias operated on, 17 con solidated after the first treatment, whereas four did not. Of the 17 c onsolidated tibias, four refractured and were retreated by using a var iety of methods. Only one healed. At follow-up, which occurred greater than or equal to 2 years after the removal of the fixator, the result s were nine consolidations without deformities or with shortening <2 c m, five consolidations with axial deviation, and seven nonconsolidatio ns. The statistically significant results were that (a) patients who w ere aged 5 years or older at operation had better results, and (b) the assembly Il (resection of CPT stumps and their short-term compression possibly associated with corticotomy or epiphyseal distraction to cor rect limb discrepancy) gave better final results compared with the oth er device assemblies. We conclude that treatment with Ilizarov's fixat or allows (a) a good percentage of healing over time (66.7%), especial ly in cases of normotrophic and cystic CPT; (b) further operations wit h or without the fixator to correct secondary or residual axial deviat ion; and (c) correction of limb discrepancy. This treatment avoids ris king injury to the healthy contralateral leg. Additionally, for treatm ents that do not achieve satisfactory results, other treatment methods are not excluded. The CPT still remains a difficult problem for the o rthopedic surgeon to solve.