Leg-length discrepancy and Its management

Transcription

1 and Its management 성균관의대 삼성서울병원 심종섭

2 Etiology of Undergrowth Congenital limb deficiency i : Congenital femoral deficiency, congenital fibular deficiency, tibial hemimelia Asymmetrical neurological disorders : Hemiplegic CP, poliomyelitis, hemimyelomeningocele Traumatic causes: malunion, growth plate arrest Hemiatrophy: Idiopathic, Russel-Silver syndrome Other causes: infection, tumor, post-irradiation, Blount s disease, LCPD, unilateral clubfoot, congenital pseudarthrosis of the tibia

3 Trauma sequelae residual poliomyelitis osteochondromatosis

4 Etiology of Overgrowth Post-traumatic overgrowth :femur shaft fracture, tibia shaft fracture Soft tissue overgrowth syndrome :Gigantism with neurofibromatosis, i Klippel-l Treunaunay syndrome, Beckwith-Wiedemann syndrome, Proteus syndrome Idiopathic hemihypertrophy p y Chronic inflammatory arthritis (ex. RA)

5 Idiopathic Klippel-Trenaunay Neurofibromatosis syndrome

6 Mechanism of compensation The child with LLD usually compensates better than the adult. The child can compensate for minor degrees of LLD by walking on the toes of shot leg. The adult seldom compensate that manner- tend to heel-toe gait: vaulting gait & excessive pelvic motion and tilt.

7 Effect in Gait cycle The amplitude of vertical pelvic motion is increased by LLD.

8 Hip - Effect Pelvic obliquity Relatively uncovered hip of long leg and increased coverage of the hip of the short leg. Increased risk of DA of the long leg

9 Effect in the Hip Joint Decreased in CE angle on the long leg side -- decreased in the load bearing area -- causes late degenerative arthritis.

10 Effect in the Spine Low back pain and late degenerative arthritis: controversial LLD- causes increased incidence of scoliosis Severity of the problem -- related to the severity of LLD -- uncompensated or uncorrected -- onset of age

11 LLD causes scoliosis LLD is not a cause of scoliosis

12 Leg-length Patient Assessment-clinical 1)Leg-length -Apparent length : from the umbilicus to the medial malleolus -Real length : from the ASIS to the medial malleolus -Wood block test

13 Apparent length Real length

14 Wood block test

15 Leg-length Patient Assessment-clinical 2) Pelvic obliquity - Measured by comparing the relative heights of the two iliac crests with patient standing with the legs together and the knee fully extended. - This assessment takes into account differences in the heights of the feet. 3) Angular deformity of the Joint - Fixed deformity considered to be corrected. 4) Joint instability

16 Leg-length Radiological Assessment 1)Teleroentgenogram - Single exposure of both leg on a long film - Reveals angular deformity - Error of magnification - Best technique for small children

17 Leg-length Radiological Assessment 2) Orthoradiograph - Exposures each joints individually id - Avoiding errors of magnification - multiple exposure

18 Leg-length Radiological Assessment 3) Scanogram ) g - Moving film cassette - Avoid magnification - Cannot assess whole shape of leg

19 Scan Digital Radiograph Scanogram

20 Estimation of skeletal l maturity - Bone age - Atlas Sum of scores Statistical combination of scores

21 Bone age -Atlas- Greulich and Pyle atlas (1959) - Children born between Few changed in the hand during the critical time of puberty - 성장과정중아이들마다편차가많다. - Interobserver error - Hand or Wrist 의 anomaly 있는경우측정이어렵다.

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23 Bone age -Scoring system- Tanner-Whitehouse method (1975) - similar to Greulich and Pyle atlas : using hand and wrist X-ray : using computerized mathematical method (using 20 landmarks in the hand and wrist scoring system) - more cumbersome, time consuming.

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25 명의 16 세까지어린이대상 - TW2 방법

26 Growth Calculation The Arithmetic Method The Growth Remaining Method The Straight line Graph Method The Multiplier method

27 The Arithmetic Method -growth calculation- Simple Chronological Age Inaccurate

28 The Growth Remaining Method -growth calculation- Skeletal age More accurate Green and Anderson growth remaining graph (Green & Anderson,1963)

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30 The Straight line Graph Method -growth calculation- Skeletal age Accurate At least 3 check points needed

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33 The Multiplier method (Paley et al, 2000) -growth calculation- Based on Green and Anderson s growth Graph 한두번의측정으로도비교적정확하게향후예상

34 Lowerlimb multiplier for boys and girls

35 Multiplier method Congenital Limb-Length Discrepancy m = x M ( : Current Limb-length discrepancy m: Limb discrepancy at skeletal maturity) Example) 현재 4cm 차이있는 Congenital hemihypertrophy 10 p ) g yp p y 세남아의최종길이차이는? 4 x = 5.24 cm

36 Developmental LLD m = + (IXG) I=1-(S-S)/(L-L) S )/(L L ) G=L(M-1) G= amount of growth remaining I=amount of growth inhibition L= current length of long limb L =length of long limb as measured on previous radiographs Lm=length of femur or tibia at skeletal maturity M=multiplier S= current length of short limb S =length of short limb as measured on previous radiographs = current limb-length discrepancy m=limb length discrepancy at skeletal maturity

37 Right femur epiphyseal infection 을앓은병력이잇는 10 세남아로 LLD 를주소로내원하였다. 최종예상 LLD 는? 8 세 10 세 Femur length(cm) right left I =1-(26-24/29-26) = 1-2/3 = 0.33 G= 29( ) 1) = 29 x = 8.99 m = 3 + (0.33 x 8.99) = = 5.97 cm

38 Time of Epiphysiodesis Lm = L x M Lε = Lm Gε Mε=Lm/Lε L= current length of long limb M=multiplier Lm= length of femur or tibia at skeletal maturity ε = desired d correction following epiphysiodesis i i Gε=amount of femoral or tibial growth remaining at age of epiphysiodesis(gε= ε/0.71 for femur and ε/0.57 for tibia) Lε=desired d length of bone to undergo epiphysiodesis i i at time of epiphysiodesis Mε=multiplier at age of epiphysiodesis

39 Right distal femur epiphyseal infection 을앓은병력이있는 10 세남아로 LLD 를주소로내원하였다. 가장적절한데 epiphysiodesis 시기는? 8세 10세 Femur length(cm) right left Lm = L(29) X M(1.31) = Lε = Lm(37.99 ) Gε(3/0.71) = = Mε=Lm(37.99)/Lε(33.77) 77) = multiplier chart 보면 세 세6개월따라서가장적절한시기는 13세 1개월에해당함.

40 Treatment General Principles 0-2cm: No treatment 2-6cm: Shoe lift,epiphyiodesis,shortening shortening 6-20cm: Lengthening >20cm: Prosthetic fitting

41 Shoe lift Patient who do not wish or are not appropriate for surgery. Lift higher than 5 cm poorly tolerated.

42 Prosthetic fitting Significant discrepancies, deformed functionally useless feet Discrepancies greater than 15-20cm and femoral length less than 50% Fibular hemimelia with unstable ankle PFFD: A/K prosthesis or BK prosthesis with Van Nes rotationplasty Optimal age: syme amputation- end of 1yr Rotationplasty: 3 yr

43 Epiphysiodesis Very low morbidity and complication rate. Slowing ggrowth rate of long leg and allowing short leg to catch up. Suitable for sufficient data to enable a confident prediction of discrepancy at maturity. Tibial epiphysiodesis should be accompanied by arrest of proximal fibular physis if tibial shortening is greater than 2.5cm.

44 Epiphysiodesis Phemister technique (JBJS,1933)

45 Epiphysiodesis Blount (staple) technique (CORR,1949) 12yr/female 1yr 2yr

46 Epiphysiodesis Percutaneous technique (Bowen R, et al,corr,1984)

47 Male/14yr (Sk.age), LLD: 2.5.cm Idiopathic hemihypertrophy

48 Male/14yr Proximal tibia & Distal femur epiphysiodesis, Lt

49 Postop 3 yr

50 Male/14yr Polyostotic fibrous dysplasia Tibial overgrowth, 2cm, right

51 Proximal Tibia & Fibula Epiphysiodesis, Rt Postop 3 yr

52 Epiphysiodesis PETS(Percutaneous Epiphysiodesis using Transepiphyseal pp Screws) (Metaizeau JP, et al, JPO,1998)

53 Problems of Epiphysiodesis Undercorrection -- growth or angulation Overcorrection -- growth or angulation Rebound phenomenon (staples or screws) Failure of growth restoration Staple breakage or bending

54 Shortening operation Mature patient Tibia< 4cm, Femur< 5cm Neurovascular complication is higher in tibia, fasciotomy is advisable.

55 Neurofibromatosis, F/16 yr LLD, 4cm Genu valgum,lt

56 Femur Corrective osteotomy 1cm shortening Tibia 3cm shortening

57 Preop Postop 2yr

58 Growth stimulation Circumferential release of periosteum Foreign material implanted next to growth plate Sympathectomy Surgical constructed AV fistula Periosteal stripping -- none has been successful enough to be clinically useful.

59 Limb lengthening operation Codvilla(1905) first described limb lengthening Compere & Sofield (1936) Anderson (1952) Wagner (1978) De Bastiani (1986) Ilizarov (1989)

60 Transiliac One stage lengthening g - Shortening < 3cm - Acetabular dysplasia Femoral and Tibial Complication -Nerve injury -Artery occlusion -Reflex sympathetic dystrophy -Intraop. fracture -Joint stiffness or subluxation

61 Distraction Epiphysiolysis Chonodrodiastasis (Gelbke,1951, De Bastiani,1986) - Separation of the epiphyseal plate - Immature patient - Risk of septic arthritis - Painful stiffness of the joint - Premature closure of the physis

62 Gradual lengthening - Distraction Osteogenesis- Ilizarov technique 1) Corticotomy: preserve endosteal & periosteal blood supply 2) Ilizarov Ring fixator: permit micro-axial i motion 3) Latency period: 7-14 days 4) Proper rate & Rhythm: 0.25mm x4 / day 5) Encourage Joint motion

63 Gradual lengthening Device for gradual lengthening - Unilateral fixator - Circular ring fixator (Ilizarov, Taylor spatial frame ) - Combined internal and external fixation (Lengthening over IM Nailing) - Totally implantable lengthening device Albizzia nail ISKD Fitbone

64 Ring fixator Correction of complex deformity (as well as lengthening) Ilizarov Taylor spatial frame

65 Combined Internal and external fixation (LON) Shorten the period of external fixation Shorten the period of external fixation Reducing the rate of regenerate bone fracture

66 Gradual lengthening - totally implantable lengthening device- ISKD Fitbone

67 Gradual lengthening Complication of gradual lengthening : 14%-134% Untoward Events - Paley D,CORR, Problems - not requiring operative intervention to resolve - Obstacles - requiring operative intervention but without permanent sequelae - Complications - intraoperative injury or anything resulting in permanent sequelae

68 Gradual lengthening-complication Nerve or vessel injury during application of external fixator Incomplete osteotomy Premature consolidation Poor regenerate bone formation Joint subluxation Pin site infection Neuropraxia Deep infection Regenerate bone fracture Subsequent growth disturbance of the lengthened limb Psychological l stress Muscle weakness Joint contracture Loss of joint motion

69 Thank you for your attention!