Management for the long limb.Is there a role for amputation?

Amy L. McIntosh MDPediatric Orthopedic Surgeon

Management for the long limb.Is there a role for amputation?

Amy L. McIntosh MDPediatric Orthopedic Surgeon

CP1013403-1

Limb HypertrophyLimb HypertrophyLimb HypertrophyLimb Hypertrophy

• Soft tissue and Soft tissue and or bone or bone enlargementenlargement

• ExtremitiesExtremitiesLower (nearly Lower (nearly all)all)UpperUpperPelvis and Pelvis and ThoraxThorax

EvaluationEvaluation

CP1013403-2

• History

• Examination

• Imaging

Radiographs

Bone age

Scanogram

• History

• Examination

• Imaging

Radiographs

Bone age

Scanogram

Phyiscal ExaminationPhyiscal ExaminationPhyiscal ExaminationPhyiscal Examination

• Assess LLD w/ pt. Assess LLD w/ pt. standingstanding

• Add blocks Add blocks (known height) (known height) until the pelvis is until the pelvis is levellevel

1 inch

Radiographs (Bone Age)Radiographs (Bone Age)Radiographs (Bone Age)Radiographs (Bone Age)

• A film of the left handA film of the left hand

• Compared to a book Compared to a book of radiographs of of radiographs of children’s hands of children’s hands of known agesknown ages

• Gives an estimate of Gives an estimate of bone maturitybone maturity growth remaininggrowth remaining

Radiographs (Measure Length Radiographs (Measure Length Discrepancy)Discrepancy)

Radiographs (Measure Length Radiographs (Measure Length Discrepancy)Discrepancy)

• TelorengenogramTelorengenogram

• OrthoroentgenogramOrthoroentgenogram

• Scanogram (x-ray/ CT)Scanogram (x-ray/ CT)

CP1013403-4

TELEOTELEO

Length of x-ray shadowLength of x-ray shadow

CP1013403-5

Length of x-ray shadowLength of x-ray shadow

AA AA AA

aa aa aa

bb cc dd

CP1013403-6

ORTHOORTHO

CP1013403-7

CP1013403-8

SCANOSCANO

Roentgen ray tubeRoentgen ray tube

Slit diaphragmSlit diaphragm

Slit-like roentgenray beam Slit-like roentgenray beam

CassetteCassette

Direction of motionDirection of motion

CP1013403-9

Leg-Length DiscrepancyLeg-Length DiscrepancyGuidelines for ManagementGuidelines for Management

DiscrepancyDiscrepancy Management Management(CM)(CM)<2 <2 No treatment or shoe liftNo treatment or shoe lift

2-5 2-5 Growth ModulationGrowth Modulation

5-12.55-12.5 Consider bone-lengtheningConsider bone-lengthening

>12.5>12.5 Combinations of above or Combinations of above or amputationamputation

LIMB LENGTH DISCREPANCYLIMB LENGTH DISCREPANCYPrediction MethodsPrediction Methods

I.I. Rule of thumb Westh and Menelaus (1981)Rule of thumb Westh and Menelaus (1981)

II.II. Growth remaining Anderson and Green (1963)Growth remaining Anderson and Green (1963)

III. Striaght line Moseley (1978)III. Striaght line Moseley (1978)

IV. Paley Multiplier Method (2000)IV. Paley Multiplier Method (2000)

Theres an APP for that!!Theres an APP for that!!Theres an APP for that!!Theres an APP for that!!

Leg-Length DiscrepancyLeg-Length DiscrepancyGuidelines for ManagementGuidelines for Management

DiscrepancyDiscrepancy Management Management(CM)(CM)<2 <2 No treatment or shoe liftNo treatment or shoe lift

2-5 2-5 Growth ModulationGrowth Modulation

5-12.55-12.5 Consider bone-lengtheningConsider bone-lengthening

>12.5>12.5 Combinations of above or Combinations of above or amputationamputation

Growth ModulationGrowth ModulationGrowth ModulationGrowth Modulation

•Epiphysiodesis: (kill Epiphysiodesis: (kill the growth plate)the growth plate)

•Eight Plates (squeeze Eight Plates (squeeze the growth plate)the growth plate)

Open EpiphysiodesisOpen EpiphysiodesisOpen EpiphysiodesisOpen Epiphysiodesis

Percutaneous EpiphysiodesisPercutaneous EpiphysiodesisPercutaneous EpiphysiodesisPercutaneous Epiphysiodesis

Screw EpiphysiodesisScrew EpiphysiodesisScrew EpiphysiodesisScrew Epiphysiodesis

Eight PlatesEight PlatesEight PlatesEight Plates

Leg-Length DiscrepancyLeg-Length DiscrepancyGuidelines for ManagementGuidelines for Management

DiscrepancyDiscrepancy Management Management(CM)(CM)<2 <2 No treatment or shoe liftNo treatment or shoe lift

2-5 2-5 Growth ModulationGrowth Modulation

5-12.55-12.5 Consider bone-lengtheningConsider bone-lengthening

>12.5>12.5 Combinations of above or Combinations of above or amputationamputation

Leg-Length DiscrepancyLeg-Length DiscrepancyGuidelines for ManagementGuidelines for Management

DiscrepancyDiscrepancy Management Management(CM)(CM)<2 <2 No treatment or shoe liftNo treatment or shoe lift

2-5 2-5 Growth ModulationGrowth Modulation

5-12.55-12.5 Consider bone-lengtheningConsider bone-lengthening

>12.5>12.5 Combinations of above or Combinations of above or amputationamputation

Is there a role for amputation??Is there a role for amputation??Is there a role for amputation??Is there a role for amputation??

• Yes!!!Yes!!!

• Significant length discrepancySignificant length discrepancy

• Poor underlying bone quality for Poor underlying bone quality for lengtheninglengthening

• Dysfunctional/ painful limbDysfunctional/ painful limb

CT angiogramCT angiogramCT angiogramCT angiogram

MRIMRIMRIMRI

femur

Klippel Tenaunay SyndromeKlippel Tenaunay SyndromeKlippel Tenaunay SyndromeKlippel Tenaunay Syndrome

• Preoperative labs:Preoperative labs:• Hemoglobin 10.8 g/dL, Hematocrit 35.8%, Hemoglobin 10.8 g/dL, Hematocrit 35.8%,

Platelets 98 x 10Platelets 98 x 1099 /L /L• INR 1.4, Prothrombin time 13.0 seconds, INR 1.4, Prothrombin time 13.0 seconds,

D-Dimer >2000 ng/mL D-Dimer >2000 ng/mL• Creatinine 0.5Creatinine 0.5

• Kasabach-Merritt SyndromeKasabach-Merritt Syndrome

Klippel Tenaunay SyndromeKlippel Tenaunay SyndromeKlippel Tenaunay SyndromeKlippel Tenaunay Syndrome

• Intraoperative (June 21, 2010):Intraoperative (June 21, 2010):Anterior flap fashion with plastic surgeryAnterior flap fashion with plastic surgery• Vascular surgery present for hemostasis control - Vascular surgery present for hemostasis control -

numerous vascular channels despite use of numerous vascular channels despite use of tourniquettourniquet

Patient became unstable – procedure stopped at Patient became unstable – procedure stopped at transfemoral amputationtransfemoral amputation

Fluid statusFluid status

Estimated blood loss: 5.7 litersEstimated blood loss: 5.7 litersFluids: 8 units FFP, 4 units Cryo, 2 Fluids: 8 units FFP, 4 units Cryo, 2

units PRBC, 11 units cell saver, 1 units PRBC, 11 units cell saver, 1 pack platelet, 1500 cc albumin, pack platelet, 1500 cc albumin, 5,400 cc crystalloid5,400 cc crystalloid

July 2, 2010July 2, 2010July 2, 2010July 2, 2010

Klippel Tenaunay SyndromeKlippel Tenaunay SyndromeKlippel Tenaunay SyndromeKlippel Tenaunay Syndrome

– July 5, 2010 – increasing pain and requirement of blood July 5, 2010 – increasing pain and requirement of blood products. Returned to OR with vascular surgeryproducts. Returned to OR with vascular surgery

• EBL 22.7L, Received 59.7L resuscitation: 24L EBL 22.7L, Received 59.7L resuscitation: 24L pRBCs, 18L FFP, 1.8L platelets, 1.6L cryo, 3 VII pRBCs, 18L FFP, 1.8L platelets, 1.6L cryo, 3 VII FactorFactor

July 29, 2010July 29, 2010July 29, 2010July 29, 2010

August 12, 2010August 12, 2010August 12, 2010August 12, 2010

August 5, 2010August 5, 2010August 5, 2010August 5, 2010

August 20, 2010August 20, 2010August 20, 2010August 20, 2010

September 13, 2010September 13, 2010September 13, 2010September 13, 2010

Present dayPresent dayPresent dayPresent day