Achondroplasia (ACH) is a type of congenital skeletal dysplasia, showing short stature and disproportionately short limbs. The disorder has an autosomal dominant mode of inheritance and is caused by mutations in the FGFR3 gene. ACH is frequently associated with medical complications and patients living with this disorder are more likely to experience short stature-related unpleasant events. One treatment option for short stature in ACH is limb lengthening surgery. Although this surgery can potentially result in up to 20 cm of height gain, it may be associated with a range of possible complications.
Kaneko et al. in their recent manuscript published in the December 2020 issue of Bone Reports, evaluated the effects of periosteal resection procedure on acceleration of bone growth using a mouse model of achondroplasia (Fgfr3ach), as an alternative treatment to limb lengthening surgery.
Two circumferential resections were performed on the periosteum of the proximal tibia of wild-type (WT) and Fgfr3ach mice, one week apart (at 4 and 5 weeks of age). Mice were sacrificed at 6 weeks of age, when bones were excised and assessed by micro-CT and histological analysis.
Rather unexpectedly, the authors reported that ‘PR promoted longitudinal bone growth in wild-type mice, but it exhibited only a marginal effect on bone growth in Fgfr3ach mice’. They did point out that the Fgfr3ach mice used in this study have limited growth potential to begin with. This could perhaps explain the lack of substantial bone growth in Fgfr3ach mice. Using alternative mouse models of ACH which may provide different bone and cartilage metabolism, coupled with longer follow-up periods and more-than-one time points could shine a light on the efficacy of this procedure for bone growth.
Authors concluded that periosteal procedure could provide a ‘more favourable acceleration’ of longitudinal bone growth in patients with vigorous growth capacity of the physes.