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.

Osteogenezis İmperfektalı Hastada İntramedüller Çivi ile Kırık ve Deformite Tedavisi

“…Saldanha ve ark. 9 OI'lı hastaya deformite ve boy eşitsizliğini gidermek için ilizarov ve unilateral eksternal fiksasyonla düzeltici osteotomi ve uzatma işlemi uygulamışlardır (10). Uzatma ve boy eşitsizliğini gidermek için başarılı bir tedavi seçeneği olarak sunmuşlardır.…”

Distal Femoral Osteotomy: Is Internal Fixation Better than External?

“…The literature suggests that progressive osteoarthritis is associated with malalignment of the joints [8,9,25,35,38]. The benefits of early femoral osteotomy to correct the deformity are twofold: biomechanical studies and studies containing short-term data suggest that realignment often reduces symptoms [8,9] and may prevent joint degeneration before arthritic changes [8,9,38], and restoration of normal alignment in the patients with arthritis often can decrease pain, improve function, and slow further degeneration [9,38].…”

Kinematic adjustability of unilateral external fixators for fracture reduction and alignment of axial dynamization

“…It is worth to note that the alignment of axial dynamization shares the same kinematic constraints on external fixators as the alignment of bone lengthening (Gugenheim, 2004;Saldanha et al, 2004). The constraint equations and the criteria established for the alignment of axial dynamization apply to external fixator for the alignment of bone lengthening.…”