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

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

J Pediatr Orthop. 2017 Dec;37(8):e459-e463

Authors: Wright J, Kazzaz S, Hill RA

Abstract
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.

PMID: 26650577 [PubMed - indexed for MEDLINE]

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