1. Deformity correction and lengthening in fibular hemimelia HR Song, MD Department of Orthopedic Surgery, Guro Hospital Korea University College of Medicine, Seoul, Korea Prof. Hae Ryong Song Dr.Ji Yeol Yoon Dr. Jae Woo Cho Department of orthopaedic surgery, Institute of Rare Disease, Guro Hospital, Seoul, Korea

2. Fibular hemimelia Associated anomalies Ankle instability– ball and socket joint Ankle instability– ball and socket joint Valgus foot with the absence of one or more lateral rays Valgus 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 patella Hypoplasia of the lateral femoral condyle or patella Knee instability – ACL insufficiency Knee instability – ACL insufficiency

3. Fibular hemimelia Achterman–Kalamchi classification Achterman–Kalamchi classification Type IA Type IB Type II

4. Fibular hemimelia Until recently the accepted treatment of choice for severe fibular hemimelia has been Syme’s or Boyd’s amputation. Until recently the accepted treatment of choice for severe fibular hemimelia has been Syme’s or Boyd’s amputation. The alternative of distraction lengthening using the Ilizarov technique is now available. The alternative of distraction lengthening using the Ilizarov technique is now available.

5. Material and methods Period from 2004 to 2008 Period from 2004 to 2008 5 cases(5 patients) of fibular hemimelia 5 cases(5 patients) of fibular hemimelia (II : 4 cases, IA : 1 case) (II : 4 cases, IA : 1 case) M:F= 4:1 M:F= 4:1 Average age = 10.8 yrs Average age = 10.8 yrs Average LLD= 7.8 cm Average LLD= 7.8 cm

6. Brief patient data casetype age at operation (yrs) LLD Estimated LLD Femoral Hypoplasia Foot conditionInitial treatment 1II37cm 7.3 * 1.859 =13 (-) ankle valgus deformity hind foot equinovarus Rocker-bottom deformity Absence of 4th & 5th ray Tibial lengthening 2II49cm 9.0 * 1.731 = 15 (+) wedged shaped distal tibia talocalcaneal coalition ankle valgus hind foot equinovarus Absence of 5th ray Simultaneous femoral & tibial lengthening 3II206cm6(+)hind foot valgus Simultaneous femoral & tibial lengthening 4Ib146cm 6 * 1.135 = 7 (+) pesplanus foot Ball & Socket ankle joint Absence of 4th & 5th ray Simultaneous femoral & tibial lengthening 5II1311cm 11*1.106 = 12 (+) pesplanus foot hind foot valgus Absence of 4th & 5th ray Simultaneous femoral & tibial lengthening

7. Case 1: Male/3Y 6M Initial LLD (R<L) - 7cm Final estimated LLD (R<L) - 13cm 2004-7-4

8. Combined deformity Cartilage anlage

9. Tibial lengthening of 10cm / 4months Healing index- 12 day/cm First surgery for lengthening Immediate Post OP

10. 2005-01-27 Complication developed - Fracture at lengthening site - Anterior bowing : 41° 2005-03-03 During follow up

11. Secondary surgery for correction Immediate Post OP 4 months later Anterior angulation- 19 ° Tibial lengthening of 1cm / 4months Healing index- 120 day/cm

12. 2006-2-32006-2-24 3 rd surgery- 3.5 cm lengthened Healing index- 125day/3.5cm - 35day/cm Follow up

13. 2009-2-18(Final)2007-7-19 Final LLD 9mm (604/595) 2004-7-4 Follow up Final lengthening: 14.5cm Final LLD: 9mm (604/595)

14. Case 2: Male/4Y 4M Initial LLD (R<L) - 9cm Final estimated LLD (R<L) - 14cm 2004-7-5

15. 2004-07-222004-11-24 Simultaneous femoral and tibial lengthening Immediate Post OP Tibial lengthening of 8cm / 4months Femoral lengthening of 5cm / 4months Healing index (Tibia)- 15 day/cm Healing index (Femur)- 24 day/cm

16. 2005-1-132005-2-11 2005-5-06 2009-01-07 Refracture at the femoral lengthening site Complications Progressive valgus deformity Residual LLD 7 cm Initial

17. Results CaseLLD Estimated LLD Lengthening achieved Healing index (day/Cm) Complication during lenthening Final complication 171314.5T:12Fracture at lengthening site Progressive Knee valgus deformtiy 291513T: 15/ F:24Fracture at lengthening site Progressive Knee valgus deformtiy Residual LLD 3665.2T:37.5/F:125Equinus deformityPeroneus nerve dysfunction 46710T:30/ F:30 Equinus deformity Knee flexion contracture Progressive Knee valgus deformtiy 5111211T:34/F:60Fracture at lengthening siteKnee flexion contracture

18. Amputation vs lengthening 30 limb in 25 patient 30 limb in 25 patient 15-amputation/ 10-lengthening 15-amputation/ 10-lengthening Amputation is better than lengthening Amputation is better than lengthening McCarthy 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 lengthening Amputation is better than lengthening Tomas-Gil et al; Acta Orthop Belg;2002

19. Amputation 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 hemimelia Ilizarov E/F is good Treatment option for fibula hemimelia Basbozkurt 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 with axis correction should be considered as an alternative to amputation Preserving the limb with fibular hemimelia by lengthening with axis correction should be considered as an alternative to amputation Barbara Jasiewicz et al; J Pediatr Orthop; 2006

20. How to manage the foot problem? Progressive Ankle Valgus :-(Jack C. Y. Cheng et al,JBJS [Br] 1998) Progressive Ankle Valgus :-(Jack C. Y. Cheng et al,JBJS [Br] 1998) During lengthening - foot should be included in frame After completion of lengthening Mild- AFO Mild- AFO Severe- soft tissue release &/or supramalleolar Severe- soft tissue release &/or supramalleolar corrective osteotomy corrective osteotomy Unstable ankle valgus- ankle arthrodesis Unstable ankle valgus- ankle arthrodesis (Deborah F. Stanitski et al,JPO,2003) (Deborah F. Stanitski et al,JPO,2003)

21. Thank you !!!