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<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

and Its management<br />

성균관의대<br />

삼성서울병원<br />

심종섭


Etiology of Undergrowth<br />

• Congenital limb deficiency<br />

: congenital femoral deficiency,<br />

congenital fibular deficiency, tibial hemimelia<br />

• Asymmetrical neurological disorders<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

: hemiplegic CP, poliomyelitis, hemimyelomeningocele<br />

• Traumatic causes: malunion, growth plate arrest<br />

• Hemiatrophy: Idiopathic, Russel-Silver syndrome<br />

• Other causes: infection, tumor, post-irradiation,<br />

Blount’s disease, LCPD, unilateral clubfoot,<br />

congenital pseudarthrosis of the tibia


<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Trauma sequelae residual poliomyelitis osteochondromatosis


Etiology of Overgrowth<br />

• Post-traumatic overgrowth<br />

:femur shaft fracture, tibia shaft fracture<br />

• Soft tissue overgrowth syndrome<br />

:gigantism with neurofibromatosis, Klippel-<br />

Trenaunay syndrome, Beckwith-Wiedemann<br />

syndrome, Proteus syndrome<br />

• Idiopathic hemihypertrophy<br />

• Chronic inflammatory arthritis (ex. RA)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Idiopathic<br />

hemihypertrophy<br />

Klippel-Trenaunay<br />

syndrome<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Neurofibromatosis


<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Mechanism of compensation<br />

• The child with LLD usually compensates<br />

better than the adult.<br />

• The child can compensate for minor<br />

degrees of LLD by walking on the toes of<br />

shot leg.<br />

• The adult seldom compensate that<br />

manner- tend to heel-toe gait: vaulting gait<br />

& excessive pelvic motion and tilt.


Effect in Gait cycle<br />

The amplitude of vertical pelvic motion is increased by LLD.<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


• Pelvic obliquity<br />

Hip - Effect<br />

• Relatively uncovered hip of long leg and<br />

increased coverage of the hip of the short<br />

leg.<br />

• Increased risk of DA of the long leg<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Effect in the Hip Joint<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Decreased in CE angle on the long leg side<br />

-- decreased in the load bearing area<br />

-- causes late degenerative arthritis.


Effect in the Spine<br />

• Low back pain and late degenerative arthritis:<br />

controversial<br />

• LLD- causes increased incidence of scoliosis<br />

• Severity of the problem<br />

-- related to the severity of LLD<br />

-- uncompensated or uncorrected<br />

-- onset of age<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


<strong>Leg</strong>-<strong>length</strong><br />

Patient Assessment-clinical<br />

1)<strong>Leg</strong>-<strong>length</strong><br />

-Apparent <strong>length</strong><br />

: from the umbilicus to the medial malleolus<br />

-Real <strong>length</strong><br />

: from the ASIS to the medial malleolus<br />

-Wood block test<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Apparent <strong>length</strong>(UMD) Real <strong>length</strong>(SMD)


Wood block test<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


<strong>Leg</strong>-<strong>length</strong><br />

Patient Assessment-clinical<br />

1) <strong>Leg</strong>-<strong>length</strong><br />

2) Pelvic obliquity<br />

3) Angular deformity of the Joint<br />

4) Joint instability<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


<strong>Leg</strong>-<strong>length</strong><br />

Radiological Assessment<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

1)Teleroentgenogram<br />

- Single exposure of both leg on a long film<br />

- Reveals angular deformity<br />

- Error of magnification<br />

- Best technique for small children


<strong>Leg</strong>-<strong>length</strong><br />

Radiological Assessment<br />

2) Orthoradiograph<br />

- Exposures each joints individually<br />

- Avoiding errors of magnification<br />

- multiple exposure<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


<strong>Leg</strong>-<strong>length</strong><br />

Radiological Assessment<br />

3) Scanogram<br />

- Moving film cassette<br />

- Avoid magnification<br />

- Cannot assess whole shape of leg<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Computed<br />

Scan Digital Radiograph Scanogram<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Estimation of skeletal maturity<br />

• Atlas<br />

• Sum of scores<br />

- Bone age -<br />

• Statistical combination of scores<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Bone age<br />

-Atlas-<br />

• Greulich and Pyle atlas (1959)<br />

- Rapid interpretation<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

- Few changed in the hand during the critical time of<br />

puberty<br />

- 성장과정중 아이들마다 편차가 많다.<br />

- Interobserver error<br />

- Hand or Wrist 의 anomaly 있는 경우 측정이 어렵다.


<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Bone age<br />

-Scoring system-<br />

• Tanner-Whitehouse method (1975)<br />

- similar to Greulich and Pyle atlas<br />

: using hand and wrist X-ray<br />

: using computerized mathematical method<br />

(using 20 landmarks in the hand and wrist<br />

– scoring system)<br />

- more cumbersome, time consuming.<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


• 1999<br />

- 5400명의 16세까지 어린이대상<br />

- TW2 방법<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Growth Calculation<br />

• The Arithmetic Method<br />

• The Growth Remaining Method<br />

• The Straight line Graph Method<br />

• The Multiplier method<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


The Arithmetic Method<br />

• Simple<br />

-growth calculation-<br />

• Chronological Age<br />

• Inaccurate<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


The Growth Remaining Method<br />

• Skeletal age<br />

• More accurate<br />

-growth calculation-<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Green and Anderson growth remaining graph<br />

(Green & Anderson,1963)


The Straight line Graph Method<br />

• Skeletal age<br />

• Accurate<br />

-growth calculation-<br />

• At least 3 check points needed<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


The Multiplier method<br />

(Paley et al, 2000)<br />

-growth calculation-<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

• Based on Green and Anderson’s growth<br />

Graph<br />

• 한두번의 측정으로도 비교적 정확하게 향<br />

후 예상


Lowerlimb multiplier for boys and girls<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Multiplier method<br />

• Congenital Limb-Length Discrepancy<br />

∆m =∆ x M<br />

(∆: Current Limb-<strong>length</strong> <strong>discrepancy</strong><br />

∆m: Limb <strong>discrepancy</strong> at skeletal maturity)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Example) 현재 4cm 차이 있는 Congenital hemihypertrophy<br />

10세 남아의 최종 길이차이는 ?<br />

4 x 1.310 = 5.24 cm


• Developmental LLD<br />

∆m = ∆ + (IXG)<br />

I=1-(S-S’)/(L-L’)<br />

G=L(M-1)<br />

G= amount of growth remaining<br />

I=amount of growth inhibition<br />

L= current <strong>length</strong> of long limb<br />

L’=<strong>length</strong> of long limb as measured on previous radiographs<br />

Lm=<strong>length</strong> of femur or tibia at skeletal maturity<br />

M=multiplier<br />

S= current <strong>length</strong> of short limb<br />

S’=<strong>length</strong> of short limb as measured on previous radiographs<br />

∆ = current limb-<strong>length</strong> <strong>discrepancy</strong><br />

∆m=limb <strong>length</strong> <strong>discrepancy</strong> at skeletal maturity<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


• Right femur epiphyseal infection 을 앓은<br />

병력이 있는 10세 남아로 LLD를 주소로 내<br />

원하였다. 최종 예상 LLD는 ?<br />

8세 10세<br />

Femur <strong>length</strong>(cm)<br />

right 24 26<br />

left 26 29<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

I =1-(26-24/29-26) = 1-2/3 = 0.33<br />

G= 29(1.310-1) = 29 x 0.310 = 8.99<br />

∆m = 3 + (0.33 x 8.99) = 3 + 2.97 = 5.97 cm


Time of Epiphysiodesis<br />

• Lm = L x M<br />

• Lε = Lm – Gε<br />

• Mε=Lm/Lε<br />

L= current <strong>length</strong> of long limb<br />

M=multiplier<br />

Lm= <strong>length</strong> of femur or tibia at skeletal maturity<br />

ε = desired correction following epiphysiodesis<br />

Gε=amount of femoral or tibial growth remaining at age of<br />

epiphysiodesis(Gε= ε/0.71 for femur and ε/0.57 for tibia)<br />

Lε=desired <strong>length</strong> of bone to undergo epiphysiodesis at time of<br />

epiphysiodesis<br />

Mε=multiplier at age of epiphysiodesis<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


• Right distal femur epiphyseal infection 을 앓은 병력이 있는<br />

10세 남아로 LLD를 주소로 내원하였다. 가장 적절한<br />

epiphysiodesis 시기는 ?<br />

8세 10세<br />

Femur <strong>length</strong>(cm)<br />

right 24 26<br />

left 26 29<br />

Lm = L(29) X M(1.31) = 37.99<br />

Lε = Lm(37.99 ) – Gε(3/0.71) =37.99-4.22 = 33.77<br />

Mε=Lm(37.99)/Lε(33.77) =1.125<br />

multiplier chart 보면<br />

1.13 13세<br />

1.10 13세6개월<br />

따라서 가장 적절한 시기는 13세 1개월에 해당함.<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Treatment<br />

• General Principles<br />

0-2cm: No treatment<br />

2-6cm: Shoe lift,epiphyiodesis,shortening<br />

4-20cm: Lengthening<br />

>20cm: Prosthetic fitting<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Shoe lift<br />

• Patient who do not wish or are not<br />

appropriate for surgery.<br />

• Lift higher than 5 cm poorly tolerated.<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Prosthetic fitting<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

• Significant discrepancies, deformed<br />

functionally useless feet<br />

• Discrepancies greater than 15-20cm and<br />

femoral <strong>length</strong> less than 50%<br />

• Fibular hemimelia with unstable ankle<br />

• PFFD: A/K prosthesis or BK prosthesis<br />

with Van Nes rotationplasty<br />

• Optimal age: syme amputation- end of 1yr<br />

Rotationplasty: 3 yr


Epiphysiodesis<br />

• Very low morbidity and complication rate.<br />

• Slowing growth rate of long leg and allowing<br />

short leg to catch up.<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

• Suitable for sufficient data to enable a confident<br />

prediction of <strong>discrepancy</strong> at maturity.<br />

• Tibial epiphysiodesis should be accompanied by<br />

arrest of proximal fibular physis if tibial<br />

shortening is greater than 2.5cm.


Epiphysiodesis<br />

• Phemister technique<br />

(JBJS,1933)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Epiphysiodesis<br />

• Blount (staple) technique (CORR,1949)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

12yr/female 1yr 2yr


Epiphysiodesis<br />

• Percutaneous technique<br />

(Bowen R, et al,CORR,1984)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Male/14yr (Sk.age), LLD: 2.5.cm<br />

Idiopathic hemihypertrophy<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Male/14yr<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Proximal tibia & Distal femur epiphysiodesis, Lt


Postop 3 yr<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Male/14yr<br />

Polyostotic fibrous dysplasia<br />

Tibial overgrowth, 2cm, right<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Postop 3 yr


Epiphysiodesis<br />

• PETS(Percutaneous Epiphysiodesis using<br />

Transepiphyseal Screws)<br />

(Metaizeau JP, et al, JPO,1998)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


8-plated growth plate<br />

Epiphysiodesis<br />

Stevens P, et al. JPO, 2006, 2008<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Problems of Epiphysiodesis<br />

• Undercorrection<br />

-- growth or angulation<br />

• Overcorrection<br />

-- growth or angulation<br />

• Rebound phenomenon (staples or screws)<br />

• Failure of growth restoration<br />

• Staple breakage or bending<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Shortening operation<br />

• Mature patient<br />

• Tibia< 4cm, Femur< 5cm<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

• Neurovascular complication is higher in<br />

tibia, fasciotomy is advisable.


Neurofibromatosis, F/16 yr<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

LLD, 4cm<br />

Genu valgum,Lt


Femur<br />

Corrective osteotomy<br />

1cm shortening<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Tibia<br />

3cm shortening


Preop Postop 2yr<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Growth stimulation<br />

• Circumferential release of periosteum<br />

• Foreign material implanted next to growth plate<br />

• Sympathectomy<br />

• Surgical constructed AV fistula<br />

• Periosteal stripping<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

-- none has been successful enough to be<br />

clinically useful.


Limb <strong>length</strong>ening operation<br />

• Codvilla(1905) – first described limb <strong>length</strong>ening<br />

• Compere & Sofield (1936)<br />

• Anderson (1952)<br />

• Wagner (1978)<br />

• De Bastiani (1986)<br />

• Ilizarov (1989)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


• Transiliac<br />

One stage <strong>length</strong>ening<br />

- Shortening < 3cm<br />

- Acetabular dysplasia<br />

• Femoral and Tibial<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Complication<br />

-Nerve injury<br />

-Artery occlusion<br />

-Reflex sympathetic dystrophy<br />

-Intraop. fracture<br />

-Joint stiffness or subluxation


Distraction Epiphysiolysis<br />

• Chonodrodiastasis (Gelbke,1951, De Bastiani,1986)<br />

- Separation of the epiphyseal plate<br />

- Immature patient<br />

- Risk of septic arthritis<br />

- Painful stiffness of the joint<br />

- Premature closure of the physis<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Gradual <strong>length</strong>ening<br />

- Distraction Osteogenesis-<br />

• Ilizarov technique<br />

1) Corticotomy: preserve endosteal & periosteal<br />

blood supply<br />

2) Ilizarov Ring fixator: permit micro-axial motion<br />

3) Latency period: 7-14 days<br />

4) Proper rate & rhythm: 0.25mm x4 / day<br />

5) Encourage joint motion<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Gradual <strong>length</strong>ening<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

• Device for gradual <strong>length</strong>ening<br />

- Unilateral fixator<br />

- Circular ring fixator (Ilizarov, Taylor spatial frame )<br />

- Combined internal and external fixation<br />

(Lengthening over IM Nailing)<br />

(Lengthening with submscular plate)<br />

- Totally implantable <strong>length</strong>ening device<br />

Albizzia nail<br />

ISKD<br />

Fitbone


Ring fixator<br />

Correction of complex deformity<br />

(as well as <strong>length</strong>ening)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Ilizarov Taylor spatial frame


Combined Internal and external fixation<br />

(Lengthening Over Nail)<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

• Shorten the period of external fixation<br />

• Reducing the rate of regenerate bone fracture


<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

Combined Internal and external fixation<br />

(submuscular plating after distraction osteogenesis)<br />

Oh, CW et al. JBJS Br 2009 :91: 1394-9<br />

Oh, CW et al. JPO B 2008 17: 265-9


Gradual <strong>length</strong>ening<br />

- totally implantable <strong>length</strong>ening device-<br />

ISKD Fitbone<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong>


Gradual <strong>length</strong>ening<br />

• Complication of gradual <strong>length</strong>ening<br />

: 14%-134%<br />

Untoward Events - Paley D,CORR,1990<br />

<strong>Leg</strong>-<strong>length</strong> <strong>discrepancy</strong><br />

- Problems - not requiring operative intervention to resolve<br />

- Obstacles - requiring operative intervention but without<br />

permanent sequelae<br />

- Complications - intraoperative injury or anything resulting<br />

in permanent sequelae


Thank you<br />

for your attention !


Thank you<br />

for your attention !

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