1. A boy who had multiple problems in lower extremity
Hui Taek Kim, M.D
Department of Orthopaedic Surgery
Pusan National University Hospital

2. Brief history At birth : foot presentation (at a maternity hospital)
At 2 wks after birth : Bilt. feet → cast Tx for 4 weeks (another clinic)
At 1y 5 m : Bilt. vertical talus → open reduction
At 2 y 4 m : Bilt. hip dislocation → open reduction
At 10 y 9 m : Rt. VRO, Lt. VDRO
At 13 y 9 m : Rt. hip subluxation → Rt. Steel’s triple OT, VDRO
Genu valgum → hemiepiphysiodesis with staple
Rt. Achilles tightness → Rt. TAL
Cryptorchism → Rt. orchiopexy, testis biopsy
• At 14 y 9 m : External tibial torsion → Bilt. T-F IR OT

3. 1. Vertical talus - open reduction at 1 yr 6 mos

4. At 8 yr 9 mo
POP 7 yr 3 mo

5. At 15 yr 5 mo
POP 14 yr (Last F/U)

6. 2. Bilateral hip dislocation
At 2 yrs 3 mos
→ Bilateral OR
At 8 yrs 9 mos
POP 6 yrs 5 mos

7. Anteversion
60° °
At 10 yrs 9 mos

8. At 10yr 9 mo
Rt. VDRO, Lt. VRO

9. 3 yrs after second operation
Anteversion
45° °
At 13yrs 9 mos
3 yrs after second operation

10. Rt. triple innominate OT, Rt. VDRO (15° varus, 25° derotation)
At 13yr 9 mo
Rt. triple innominate OT,
Rt. VDRO (15° varus, 25° derotation)

11. Rt. femoral anteversion: 35°
Lt. femoral anteversion 32°

12. At 15 yr 5 mo
Last F/U

13. 3. Genu valgum

14. Preop
13 yr 6 mo
POP 4 Mo
13 yr 10 mo
POP 5 Mo
13 yr 11 mo

15. At 15 yr 4 mo

16. 4. Lateral tibial torsion

17. 전면 보행

18. FPA : 60°
→ 40° correction
FPA : 30°
→ 10° correction

19. Knee neutral position

20. 45°
35°
40°
35°

21. Knee neutral
42°
47°

22. TFA : 45°
TFA : 45°

23. Tibial torsion
63° °

24. Tibial torsion
25° °
At 14 yr 9 mo
T-F IR OT

25. At 15 yr 5 mo
(Last F/U)

26. 5. Muscle weakness of L/E • Waddling gait • Rt. Achilles tightness
• Weak muscle strength of lower limb
(3/5 in proximal region, 4/5 in distal region)
• Absent to hypoactive DTR of lower extremities

27. EMG-NCV (at 10 yrs 1 mo)
• Multiple lumbar nerve root lesion with myelopathy
and/or its complication (eg. cord tethering).
• No problem on bladder and bowel.
• Possibility of arthrogryposis multiplex congenita
could not be ruled out.

28. L-spine MRI (at 10 yrs 1 mo)
T2 sagittal
T1 sagittal

29. T12 / L1
L1 / L2
L2 / L3
L3 / L4
L4 / L5
L5 / S1

30. 6. Rt. cryptorchism At 13 yrs 9 mos: Rt. orchiopexy, testis biopsy
→ maturation arrest
scanty germ cells and mostly sertolli cells

31. Discussion Diagnosis: a syndrome ?
Progressive deformities of the lower extremity associated with muscle weakness ?