Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia

Version 1 : Received: 11 May 2021 / Approved: 12 May 2021 / Online: 12 May 2021 (11:22:56 CEST)

A peer-reviewed article of this Preprint also exists.

Paley, D. Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia. Children 2021, 8, 540. Paley, D. Extensive Limb Lengthening for Achondroplasia and Hypochondroplasia. Children 2021, 8, 540.

Journal reference: Children 2021, 8, 540
DOI: 10.3390/children8070540

Abstract

Extensive limb lengthening (ELL) was completed in 75 patients: 66 achondroplasia and 9 hypochondroplasia. The average lengthening was 27cm for achondroplasia (12-40cm) and 17cm for hypochondroplasia (range 10-25cm). There were 48 females and 27 males. Lengthening was done either by 2-segment (14 patients; both tibias and/or both femurs) or by serial 4-segment lengthenings (64 patients; both femurs and tibias same time). Most patients also had bilateral humeral lengthening. Lengthenings were either juvenile-onset (31), adolescent-onset (38) or adult-onset (6). The average age at final follow-up was 26 years old (range 17-43 years). There were few permanent sequelae of complications. The most serious was one paraparesis. All patients returned to activities of normal living and only one was made worse by the surgery (paraparesis). This is the first study to show that ELL can lead to increase of height into the normal height range. Previous studies showed mean increases of height of up to 20cm, while this study consistently showed an average increase of 30 cm (range 15-40cm) for juvenile-onset and increase of 26cm (range 15-30cm) for adolescent-onset. This results in lower normal height at skeletal maturity for males and females. The adult-onset had a mean increase of 16.8 (range 12-22cm). This long-term follow-up study shows ELL can be done safely even with large lengthenings and that 4-segment lengthening may offer advantages over 2-segment lengthening. While the majority of cases were performed using external fixation, implantable limb lengthening promises to be an excellent alternative and perhaps an improvement.

Keywords

achondroplasia; hypochondroplasia; dwarfism; short-limb; short stature; FGFR3; skeletal dysplasia; genetic condition; extensive limb lengthening

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