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
Fibular hemimeliaFibular hemimelia Achterman–Kalamchi classificationAchterman–Kalamchi classification
Type IA Type IB Type II
Fibular hemimeliaFibular hemimelia
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
Simultaneous femoral & tibial lengthening
5 II 13 11cm11*1.106
= 12(+)
pesplanus foothind foot valgus
Absence of 4th & 5th ray
Simultaneous femoral & tibial lengthening
Case 1: Male/3Y 6MCase 1: Male/3Y 6M
Initial LLD (R<L) - 7cm Final estimated LLD (R<L) - 13cm
2004-7-4
Combined deformityCombined deformity
Cartilage anlageCartilage anlage
Tibial lengthening of 10cm / 4monthsHealing index- 12 day/cm
First surgery for lengtheningFirst surgery for lengthening
Immediate Post OP
2005-01-27 Complication developed
- Fracture at lengthening site
- Anterior bowing : 41°
2005-03-03
During follow upDuring follow up
Secondary surgery for correctionSecondary surgery for correction
Immediate Post OP
4 months later
Anterior angulation- 19 °Tibial lengthening of 1cm / 4monthsHealing index- 120 day/cm
2006-2-3 2006-2-24
3rd surgery- 3.5 cm lengthened
Healing index- 125day/3.5cm - 35day/cm
Follow upFollow up
2009-2-18(Final)2007-7-19
Final LLD 9mm (604/595)
2004-7-4
Follow upFollow up
Final lengthening: 14.5cm Final LLD: 9mm (604/595)
Case 2: Male/4Y 4MCase 2: Male/4Y 4M
Initial LLD (R<L) - 9cm Final estimated LLD (R<L) - 14cm
2004-7-5
2004-07-22 2004-11-24
Simultaneous femoral and tibial lengtheningSimultaneous femoral and tibial lengthening
Immediate Post OP
Tibial lengthening of 8cm / 4monthsFemoral lengthening of 5cm / 4monthsHealing index (Tibia)- 15 day/cmHealing index (Femur)- 24 day/cm
2005-1-13 2005-2-11
2005-5-06
2009-01-07
Refracture at the femoral lengthening site
ComplicationsComplications
Progressive valgus deformityResidual LLD 7 cm
Initial
ResultsResults
Case LLDEstimated
LLDLengthening
achievedHealing index
(day/Cm)Complication
during lentheningFinal complication
1 7 13 14.5 T:12 Fracture at lengthening siteProgressive Knee valgus deformtiy
2 9 15 13 T: 15/ F:24 Fracture at lengthening siteProgressive Knee valgus deformtiyResidual LLD
3 6 6 5.2 T:37.5/F:125 Equinus deformity Peroneus nerve dysfunction
4 6 7 10 T:30/ F:30Equinus deformityKnee flexion contracture
Progressive Knee valgus deformtiy
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
Unstable ankle valgus- ankle arthrodesisUnstable ankle valgus- ankle arthrodesis
(Deborah F. Stanitski et al,JPO,2003)(Deborah F. Stanitski et al,JPO,2003)
Thank you !!!Thank you !!!