Deformity correction and Deformity correction and lengthening in fibular lengthening in fibular hemimelia hemimelia HR Song, MD Department of Orthopedic Surgery, Guro Hospital Korea University College of Medicine, Seoul, Korea Prof. Hae Ryong Song Prof. Hae Ryong Song Dr .Ji Yeol Yoon Dr .Ji Yeol Yoon Dr. Jae Woo Cho Dr. Jae Woo Cho Department of orthopaedic surgery, Department of orthopaedic surgery, Institute of Rare Disease, Guro Institute of Rare Disease, Guro Hospital, Seoul, Korea Hospital, Seoul, Korea
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Deformity correction and lengthening in fibular hemimelia HR Song, MD Department of Orthopedic Surgery, Guro Hospital Korea University College of Medicine,
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Deformity correction and lengthening in Deformity correction and lengthening in fibular hemimeliafibular hemimelia
HR Song, MDDepartment of Orthopedic Surgery, Guro Hospital
Korea University College of Medicine, Seoul, Korea
Prof. Hae Ryong SongProf. Hae Ryong SongDr .Ji Yeol YoonDr .Ji Yeol YoonDr. Jae Woo ChoDr. Jae Woo Cho
Department of orthopaedic surgery, Department of orthopaedic surgery, Institute of Rare Disease, Guro Hospital, Seoul, KoreaInstitute of Rare Disease, Guro Hospital, Seoul, Korea
Fibular hemimeliaFibular hemimelia
Associated anomaliesAssociated anomalies Ankle instability– ball and socket jointAnkle instability– ball and socket joint Valgus foot with the absence of one or more lateral raysValgus foot with the absence of one or more lateral rays Tarsal coalition Tarsal coalition Congenital short femur Congenital short femur Hypoplasia of the lateral femoral condyle or patellaHypoplasia of the lateral femoral condyle or patella Knee instability – ACL insufficiencyKnee instability – ACL insufficiency
Until recently the accepted treatment of choice Until recently the accepted treatment of choice for severe fibular hemimelia has been Syme’s or for severe fibular hemimelia has been Syme’s or Boyd’s amputation. Boyd’s amputation.
The alternative of distraction lengthening using The alternative of distraction lengthening using the Ilizarov technique is now available.the Ilizarov technique is now available.
Material and methodsMaterial and methods
Period from 2004 to 2008Period from 2004 to 2008 5 cases(5 patients) of fibular hemimelia5 cases(5 patients) of fibular hemimelia
(II : 4 cases, IA : 1 case)(II : 4 cases, IA : 1 case) M:F= 4:1M:F= 4:1 Average age = 10.8 yrsAverage age = 10.8 yrs Average LLD= 7.8 cm Average LLD= 7.8 cm
Brief patient dataBrief patient datacase type
age at operation
(yrs)LLD
Estimated LLD
Femoral Hypoplasia
Foot condition Initial treatment
1 II 3 7cm7.3 * 1.859
=13(-)
ankle valgus deformityhind foot equinovarus
Rocker-bottom deformityAbsence of 4th & 5th ray
Tibial lengthening
2 II 4 9cm9.0 * 1.731
= 15(+)
wedged shaped distal tibiatalocalcaneal coalition
ankle valgus hind foot equinovarusAbsence of 5th ray
Simultaneous femoral & tibial lengthening
3 II 20 6cm 6 (+) hind foot valgusSimultaneous femoral
& tibial lengthening
4 Ib 14 6cm6 * 1.135
= 7(+)
pesplanus footBall & Socket ankle jointAbsence of 4th & 5th ray
5 11 12 11 T:34/F:60 Fracture at lengthening site Knee flexion contracture
Amputation vs lengtheningAmputation vs lengthening
30 limb in 25 patient30 limb in 25 patient 15-amputation/ 10-lengthening15-amputation/ 10-lengthening Amputation is better than lengtheningAmputation is better than lengthening
McCarthy et al; J Bone Joint Surg(Am);2000McCarthy et al; J Bone Joint Surg(Am);2000
4 complete fibula hemimelia(15 yr F/U)4 complete fibula hemimelia(15 yr F/U) Amputation is better than lengtheningAmputation is better than lengthening
Tomas-Gil et al; Acta Orthop Belg;2002Tomas-Gil et al; Acta Orthop Belg;2002
Amputation vs lengtheningAmputation vs lengthening
4 fibula hemimelia using Ilizarov E/F (Mean LLD:8.7 cm)4 fibula hemimelia using Ilizarov E/F (Mean LLD:8.7 cm) Ilizarov E/F is good Treatment option for fibula hemimeliaIlizarov E/F is good Treatment option for fibula hemimelia
Basbozkurt et al; Acta orthop Traumatol Turc; 2005Basbozkurt et al; Acta orthop Traumatol Turc; 2005
10 patients with fibular hemimelia type II (Mean LLD: 5.8 cm)
Preserving the limb with fibular hemimelia by lengthening Preserving the limb with fibular hemimelia by lengthening with axis correction should be considered as an alternative to with axis correction should be considered as an alternative to amputationamputation
Barbara Jasiewicz et al; J Pediatr Orthop; 2006Barbara Jasiewicz et al; J Pediatr Orthop; 2006
How to manage the foot How to manage the foot problem?problem?
Progressive Ankle ValgusProgressive Ankle Valgus:-(Jack C. Y. Cheng et al,:-(Jack C. Y. Cheng et al,JBJS [Br] 1998)JBJS [Br] 1998)
During lengtheningDuring lengthening - - foot should be included in framefoot should be included in frame
After completion of lengtheningAfter completion of lengthening MildMild- AFO- AFO SevereSevere- soft tissue release &/or supramalleolar - soft tissue release &/or supramalleolar corrective osteotomycorrective osteotomy