Developments in the Orthopaedic Management of Children With Stüve-Wiedemann Syndrome: Use of the Fassier-Duval Telescopic Rod to Maintain Correction of Deformity.

BACKGROUND

Stüve-Wiedemann syndrome is a rare inherited condition, which is frequently fatal in infancy. Those patients who survive into childhood demonstrate a complex progressive deformity of the long bones, with high rates of recurrence after initial successful correction. Because of the rarity of the condition there is, at present, limited evidence on the most appropriate treatment.

METHODS

We describe our experience in the management of 4 patients, who underwent correction of deformity and fixation with Fassier-Duval telescopic rods.

RESULTS

In this series we have seen good correction of deformity and maintenance of alignment, with improvement in the walking ability of children treated with this technique.

CONCLUSION

Fassier-Duval rodding has a role in the prevention of recurrence of deformity and should be considered as a means to reduce the number of operative procedures required.

LEVEL OF EVIDENCE: Level IV-therapeutic.