Limb lengthening and deformity correction in patients with severe fibular hemimelia: experience of the children's university hospital in Belgrade

Abstract

Background Fibular hemimelia (FH) is a congenital disease manifested by valgus deformity and instability of the knee joint, shortening and deformity of the tibia, hypoplasia and deformities of the foot and ankle.The aim of this paper was to determine the efficacy of the strategy of separate reconstruction of the foot along with correction of tibia deformity, and then tibial lengthening in patients with FH of types 3 and 4 according to Paley.Methods This retrospective study is based on an analysis of the treatment of 12 children with severe FH. The patients, aged no older than 24 months, were treated for foot reconstruction along with correction of tibial deformity followed by a separate stage of tibial lengthening. Tibial lengthening was performed in the age 4.6 ± 1.2 years. The long-term result of treatment was evaluated at least 1 year after the end of tibial lengthening. Evaluation criteria after tibia lengthening were external osteosynthesis index, amount of lengthening, assessment of outcomes according to Lascombes. Walking ability was assessed using Gillette questionnaire.Results The approach we used gave excellent and good results in 83 % of cases after the first reconstructive stage. Complications and recurrences of deformities encountered during the first stage were eliminated during subsequent planned limb lengthening. The average magnitude of lengthening was 6.4 ± 2.4 cm (37.2 ± 12.4 % of the initial segment length). The index of external osteosynthesis was 22.9 ± 12.2 days/cm. Monofocal distraction osteosynthesis was used in 9 cases and bifocal osteosynthesis in three cases. The results of lengthening were classified by Lascombes as IA in 7 cases, IB in four cases, 2B in one case.Discussion In severe FH, the question of reconstruction or early amputation remains open. There are two opinions on the staging of reconstructive orthopedic surgery and tibial lengthening in young children with severe FH.Conclusion The strategy of reconstruction of the foot and ankle joint at an early age (16-24 months) in children with severe FH followed by lengthening of the lower leg (at the age of 4-6 years) proved to be effective and can be used when it is chosen by the patient's parents. In 3C type cases, the use of external fixation to correct the deformity and simultaneously lengthen the tibia at the first stage is an alternative reasonable strategy option.