Arthrogryposis Multiplex Congenita is a rare congenital disorder associated with multiple musculoskeletal contractures which causes substantial morbidity. Knee involvement is commonly seen among children with arthrogryposis, with flexion contracture being the most frequent. The purpose of this study was to assess the effectiveness of orthopaedic procedures, namely distal femoral supracondylar extension osteotomy and/or Ilizarov external fixator, on the ambulation status of children with knee flexion contracture and whether any functional gains are maintained at the latest follow-up. Fifteen patients were identified and their medical records reviewed. The mean age at their first surgery was 7.6 years (range, 2-16 years). The etiology for all patients was amyoplasia. The mean length of follow-up was 58 months (range, 12-117 months). Contractures were treated with femoral extension osteotomy (n=8), Ilizarov external fixator (n=2), or both (n=5).Background
Methods
The new Fassier-Duval Telescopic IM System (FD-rod) has the advantage of a single entry point over the traditional telescopic rods such as the Bailey-Dubow or Sheffield rods. Although encouraging early results were presented by François Fassier, there is no formal publication in the literature as yet. The first 24 consecutive cases (age 1.5-12.5 years) with a minimum of 1 year follow-up (1-2.4 years) after femoral and/or tibial FD-rods were reviewed to assess complications involving migration, non-telescoping, joint intrusion, infections and re-operation rates in patients with Osteogenesis imperfecta (OI, 15 cases), congenital tibial pseudarthrosis (CPT) in Neurofibromatosis Type1 (NF1, 2 cases), and Epidermal Naevus syndrome (1 case). In 6 cases of patients with Hypophosphataemic Rickets FD-rods were combined with an Ilizarov frame.Background
Methods
