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Children's Orthopaedics

CONGENITAL ABSENCE OF THE FIBULA: OUTCOME OF AMPUTATION OR EXTENSION PROSTHESIS IN THE MANAGEMENT OF SEVERE LOWER LIMB DEFORMITY

The British Society for Children' s Orthopaedic Surgery (BSCOS) and the Swedish Paediatric Orthopaedic Society (SBOF), Combined Meeting, March 2017



Abstract

Purpose

This study compares outcomes in patients with complete congenital fibula absence, associated with severe lower limb deformity, treated with an amputation protocol to those using an extension prosthesis.

Method

32 patients were identified. 9 patients (2M: 7F, median age at presentation of 22 yrs) utilized an extension prosthesis. 23 patients (16M: 7F, median age at presentation of 10 months) underwent 25 amputations during childhood: only two underwent tibial kyphus correction to facilitate prosthetic wear.

Mobility was assessed using the SIGAM and K scores. Quality of life was assessed using the PedsQL inventory questionnaire; pain by a verbal severity score. Patients undergoing amputation were further subdivided by age, below and above 2 yrs at the time of surgery.

Results

19 Syme and one Boyd amputation in 19 patients were performed early (mean age 15 months). 4 Syme and one trans-tibial amputation in 4 patients took place in older children (mean age 6.6 years).

K Scores were significantly higher (mean 4 versus 2) and pain scores lower in the amputation group allowing high impact activity compared to community ambulation with an extension prosthesis. The SIGAM and PedsQL scores were all better in the amputation group, but not significantly so. There was no significant difference in the scores based on the time of amputation.

Conclusion

Complete fibula absence can present with significant lower limb deformity. Parental counselling regarding management is paramount in achieving the optimum functional outcome. Childhood amputation for severe limb length inequality and foot deformity in congenital fibula absence offers excellent short term functional outcome with prosthetic support.

The tibial kyphus deformity does not need routine correction and facilitates prosthetic suspension. Accommodative extension prosthesis does offer reasonable long term function but outcome scores are lower.