CP1013403-1

1. Management for the long limb.Is there a role for amputation?Amy L. McIntosh MDPediatric Orthopedic Surgeon CP1013403-1

2. Limb Hypertrophy • Soft tissue and or bone enlargement • Extremities • Lower (nearly all) • Upper • Pelvis and Thorax

3. Evaluation • History • Examination • Imaging • Radiographs • Bone age • Scanogram CP1013403-2

4. Phyiscal Examination • Assess LLD w/ pt. standing • Add blocks (known height) until the pelvis is level

5. 1 inch

7. Radiographs (Bone Age) • A film of the left hand • Compared to a book of radiographs of children’s hands of known ages • Gives an estimate of bone maturity growth remaining

8. Telorengenogram Orthoroentgenogram Scanogram (x-ray/ CT) Radiographs (Measure Length Discrepancy)

9. Length of x-ray shadow TELEO CP1013403-4

10. CP1013403-5

11. A A A a a a b c d Length of x-ray shadow ORTHO CP1013403-6

12. CP1013403-7

13. Direction of motion SCANO Roentgen ray tube Slit diaphragm Slit-like roentgenray beam Cassette CP1013403-8

14. CP1013403-9

15. Discrepancy Management (CM) <2 No treatment or shoe lift 2-5 Growth Modulation 5-12.5 Consider bone-lengthening >12.5 Combinations of above or amputation Leg-Length DiscrepancyGuidelines for Management

16. Rule of thumb Westh and Menelaus (1981) Growth remaining Anderson and Green (1963) III. Striaght line Moseley (1978) IV. Paley Multiplier Method (2000) LIMB LENGTH DISCREPANCYPrediction Methods

20. Theres an APP for that!!

21. Discrepancy Management (CM) <2 No treatment or shoe lift 2-5 Growth Modulation 5-12.5 Consider bone-lengthening >12.5 Combinations of above or amputation Leg-Length DiscrepancyGuidelines for Management

22. Epiphysiodesis: (kill the growth plate) Eight Plates (squeeze the growth plate) Growth Modulation

23. Open Epiphysiodesis

24. Percutaneous Epiphysiodesis

25. Screw Epiphysiodesis

26. Eight Plates

27. Discrepancy Management (CM) <2 No treatment or shoe lift 2-5 Growth Modulation 5-12.5 Consider bone-lengthening >12.5 Combinations of above or amputation Leg-Length DiscrepancyGuidelines for Management

32. Discrepancy Management (CM) <2 No treatment or shoe lift 2-5 Growth Modulation 5-12.5 Consider bone-lengthening >12.5 Combinations of above or amputation Leg-Length DiscrepancyGuidelines for Management

33. Yes!!! Significant length discrepancy Poor underlying bone quality for lengthening Dysfunctional/ painful limb Is there a role for amputation??

37. CT angiogram

38. MRI femur

39. Preoperative labs: Hemoglobin 10.8 g/dL, Hematocrit 35.8%, Platelets 98 x 109 /L INR 1.4, Prothrombin time 13.0 seconds, D-Dimer >2000 ng/mL Creatinine 0.5 Kasabach-Merritt Syndrome Klippel Tenaunay Syndrome

40. Intraoperative (June 21, 2010): Anterior flap fashion with plastic surgery Vascular surgery present for hemostasis control - numerous vascular channels despite use of tourniquet Patient became unstable – procedure stopped at transfemoral amputation Fluid status Estimated blood loss: 5.7 liters Fluids: 8 units FFP, 4 units Cryo, 2 units PRBC, 11 units cell saver, 1 pack platelet, 1500 cc albumin, 5,400 cc crystalloid Klippel Tenaunay Syndrome

41. July 2, 2010

42. July 5, 2010 – increasing pain and requirement of blood products. Returned to OR with vascular surgery EBL 22.7L, Received 59.7L resuscitation: 24L pRBCs, 18L FFP, 1.8L platelets, 1.6L cryo, 3 VII Factor Klippel Tenaunay Syndrome

43. July 29, 2010

44. August 12, 2010

45. August 5, 2010

46. August 20, 2010

47. September 13, 2010

48. Present day