Limb lengthening in achondroplasia

Abstract

Background:Stature lengthening in skeletal dysplasia is a contentious issue. Specific guidelines regarding the age and sequence of surgery, methods and extent of lengthening at each stage are not uniform around the world. Despite the need for multiple surgeries, with their attendant complications, parents demanding stature lengthening are not rare, due to the social bias and psychological effects experienced by these patients. This study describes the outcome and complications of extensive stature lengthening performed at our center.Materials and Methods:Eight achondroplasic and one hypochondroplasic patient underwent bilateral transverse lengthening for tibiae, humeri and femora. Tibia lengthening was carried out using a ring fixator and bifocal corticotomy, while a monolateral pediatric limb reconstruction system with unifocal corticotomy was used for the femur and humerus. Lengthening of each bone segment, height gain, healing index and complications were assessed. Subgroup analysis was carried out to assess the effect of age and bone segment on the healing index.Results:Nine patients aged five to 25 years (mean age 10.2 years) underwent limb lengthening procedures for 18 tibiae, 10 femora and 8 humeri. Four patients underwent bilateral lengthening of all three segments. The mean length gain for the tibia, femur and humerus was 15.4 cm (100.7%), 9.9 cm (52.8%) and 9.6 cm (77.9%), respectively. Healing index was 25.7, 25.6 and 20.6 days/cm, respectively, for the tibia, femur and humerus. An average of 33.3% height gain was attained. Lengthening of both tibia and femur added to projected height achieved as the 3rd percentile of standard height in three out of four patients. In all, 33 complications were encountered (0.9 complications per segment). Healing index was not affected by age or bone segment.Conclusion:Extensive limb lengthening (more than 50% over initial length) carries significant risk and should be undertaken only after due consideration.

FGFR3 in Periosteal Cells Drives Cartilage-to-Bone Transformation in Bone Repair

“…In addition, abnormal chondrocyte hypertrophy and transition to bone is associated with fibrocartilage and fibrous tissue accumulation that also interferes with bone bridging, revealing a central role of FGFR3 signaling in controlling the balance between ossification and fibrosis. These findings have major impacts on the management of patients affected with achondroplasia that may require surgery for limb lengthening as managing the level of stabilization and endochondral ossification may be crucial to allow successful healing ( Chilbule et al., 2016 ; Kitoh et al., 2014 ). Beyond this specific clinical setting, the results bring fundamental insights on the mechanisms of bone repair defects in pseudarthrosis and the role of PCs and FGFR3 in the essential step of cartilage-to-bone transformation during bone repair.…”

Surgical Results of Limb Lengthening at the Femur, Tibia, and Humerus in Patients with Achondroplasia

“…All the humeral lengthening satisfied the patients and allowed independent perineal hygiene. Surgical results of limb lengthening in achondroplastic patients were previously reported in many studies;3) however, the studies that compared the surgical results among the three long bones were rare 12). Certainly, the relatively small number of subjects was a limitation of our study.…”

A review of skeletal dysplasia research in India

“…They further emphasized that limb lengthening of more than 50% of the initial length carries significant risk. [ 65 ] A retrospective study on intramedullary rodding of long bones in 16 children with OI showed that the frequency of fractures and the related complications were dramatically reduced after careful and precise implantation of either Sheffield rods or non-elongating rods of appropriate size. [ 66 ] An interesting report by Kaur et al .…”