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 !!!