title: Limb lengthening and correction of deformity in the lower limbs of children with osteogenesis imperfecta.
abstract: We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI.
authors.affiliation: Sheffield Children's Hospital, Sheffield, England, UK.
authors.forename: K A N
authors.initials: KA
authors.lastname: Saldanha
authors.forename: M
authors.initials: M
authors.lastname: Saleh
authors.forename: M J
authors.initials: MJ
authors.lastname: Bell
authors.forename: J A
authors.initials: JA
authors.lastname: Fernandes
mesh_terms: D000293:Adolescent; D001858:Bone Nails; D002648:Child; D004867:Equipment Design; D005260:Female; D005269:Femur / Q000601:surgery*; D006801:Humans; D018889:Ilizarov Technique / Q000009:adverse effects / Q000295:instrumentation*; D007870:Leg Length Inequality / Q000601:surgery; D008297:Male; D010013:Osteogenesis Imperfecta / Q000601:surgery*; D019857:Osteogenesis, Distraction / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D013977:Tibia / Q000601:surgery*; D016896:Treatment Outcome; D014945:Wound Healing