Deformity Correction with External fixator in
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Deformity Correction with External fixator in Pseudoachondroplasia Hae-Ryong Song, Seok-Hyun Lee*, Seung-Woo Suh, Sameer Desai, Gautam Shetty Department of Orthopedic Surgery,Korea University Guro Hospital, Seoul, * Dongguk University International Hospital, Ilsan, Korea Introduction Pseudoachondroplasia Rhizomelic dwarfism Both epiphysis and metaphysis involvement Early onset arthritis Ligament instability Windswept deformity Os odontoideum with C1-C2 instability Cartilage oliogmeric matrix protein (COMP) Ligament laxity Materials & Methods Total 7 patients (4 males, 3 females) 26 segments (14 tibiae & 12 femora) Mean age 24 yrs (7~44) Mean follow up 2.5 yrs (2 ~ 5) Materials & Methods All patients had genu varum deformity MAD avr 8.1cm (0.5 ~ 11.8) Distal femur, proximal tibia : varus & procavatum deformity Distal tibia : valgus, varus or neutral with procavatum deformity Tibia rotation : 26° int. rotation ~ 43° ext. rotation Femoral neck version : 29° anteversion ~ 10° retroversion Operative procedures Femur hybrid fixator with two rings and a proximal arch for gradual correction a lateral monofixator (Medixalign, Seoul, Korea) for acute correction and augmentation with intramedullary nails in some cases Tibia : Ilizarov’s ring fixator (U & I, Seoul, Korea) • Postoperative : • Gradual distraction started after 7 days • 1 mm/day (0.25 mm 6 hourly) : distal femoral and proximal tibial osteotomy site • 0.5 mm/day ( 0.25 mm 12 hourly) : distal tibial osteotomy site. Results Radiologic Angular correction mean 26°(AP) and 16° (Lat.) MAD correction mean 7.3 cm (4.3~11.3) Rotational correction mean 16° (10~46°) External fixation time mean 3.5 months (1.5~7.5) (EFT) Amount of lengthening mean 2.3 cm (1~4.2) External fixation index mean 55 days/cm (20~120) Results Complications 9 major & 12 minor in 24/26 segments Recurrent deformity, delayed consolidation, refracture, pin site infection, knee stiffness, residual ligament laxity, compartment syndrome Functional results Preoperatively 3 pts <50m pain-free walking 2 pts 100m 1 pt 500 m 1 pt (knee brace) 1 km Postoperatively 2 pts 0.1km pain-free walking 3 pts 1~3 km 1 pt (knee brace) 3 km 1 pt unchanged Results Radiologic Clinical results results Good 5 4 Fair 12 5 Poor 9 5 Conclusions Recurrent deformity in children and delayed consolidation in adult contributed to radiographic poor results. Recurrent varus deformity of the knee in children was inevitable because there was asymmetric epiphyseal growth. Therefore, patients need to be well informed about the possibility of recurrent or residual deformity during growth before primary surgery.