Other Congenital & Developmental Knee & Leg Disease. Jong Sup Shim,M.D. Department of Orthopedic Surgery Samsung Medical Center

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1 Other Congenital & Developmental Knee & Leg Disease Jong Sup Shim,M.D. Department of Orthopedic Surgery Samsung Medical Center Sungkyunkwan University School of Medicine

2 Rotational Deformity

3 Intoeing Outtoeing

4 Rotational Deformity Abnormal rotation of the tibia & femur -- torsion : > 2 SD from average Medial Femoral Torsion Medial Tibial Torsion Torsional Malalignment Syndrome

5 Rotational profile(staheli,1985)

6 Medial Femoral Torsion Intoeing after age 4 Bilateral >>> Unilateral Female >> Male W sitting position Rotational profile : Hip I/R > E/R TFA normal FPA decreased (intoeing)

7 Foot Progression Angle -- negative

8 Internal rotation -- increased External rotation -- decreased

9 Thigh Foot Angle : Normal W Position

10 Medial Tibial Torsion Intoeing under age 3 Bilateral>> Unilateral Usually combined with physiologic genu varum Rotational profile : Hip rotation - symmetric, normal TFA - decreased FPA - decreased (intoeing)

11 Genu varum Thigh Foot Angle : Negative

12 Natural History Spontaneously improved in most cases Medial femoral torsion -- knee pain (-) -- hip pain (-) -- increased risk of degenerative arthritis in hip (-) Medial tibial torsion -- no evidence of functional problems

13 Treatment Conservative treatment -- ineffective, unnecessary

14 Operative treatment Indication -- Severe torsional deformity (beyond 3 SD) + functional & cosmetic disability -- after age 8

15 Operative treatment Femur -- rotational osteotomy - level: intertrochanter, subtrochanter, midshaft, supracondylar Tibia -- rotational osteotomy - level: proximal (correction of concomitant angular deformity) distal

16 Angular Deformity

17 Knee Angle Normal growth pattern

18 1yr6mo 4yr

19 6

20 Infantile tibia vara (Blount disease) Growth disorder involving the medial portion of the proximal tibial epiphyseal plate Localized varus deformity Progressive Early walker, Black & Obese baby

21 Etiology Infantile tibia vara No clear distinction between physiologic bowing and tibia vara Common etiologic factor -- early weight bearing by a child with or without overweight increased physeal shear stress on medial aspect eventually disruption of longitudinal growth (Hueter- Volkmann s law)

22 Radiological finding infantile tibia vara Langenskiold (1952) 6th distinct radiological stage with disease progression and maturation

23 Standard Standing AP view Femur-Tibia Angle Drennan angle(metaphyseal-diaphyseal Angle) (Levine & Drennan,1982; Felden,1993) 0ver 16 o : radiolograhic sign at risk 11 o 16 o : relative radiographic sign at risk

24

25 Management infantile tibia vara Brace (Schoenecker,2001) -- Mild deformity (Langenskiold stage 1,2) -- start within age 2 yr 6mo -- KAFO with fixed knee joint : possibly all-day application(>23h/d) -- usually 1yr6mo 2yr duration : correction must be achieved less than age 4 -- best result :unilateral deformity -- important factor : compliance

26 D-angle : D-angle: 15 0 / /9 0 2yr 2mo/female 18mo/female

27

28 Management infantile tibia vara Osteotomy -- indication Langenskiold stage < 4( over age 3) Progressive deformity, bilateral -- optimal result stage 3 (before age 4) -- correction : varus angulation + medial rotational deformity (usually toward 10 valgus angulation)

29 4yr/male

30

31 Management infantile tibia vara Physeal bridge resection -- severe deformity or recurred cases -- osteotomy + concomitant medial physeal bar resection Physeal elevating osteotomy -- severe deformity (Stage >5) -- medial physeal elevation osteotomy + concomitant lateral epiphysiodesis -- usually leg length equalization required

32 Idiopathic genu varum & Genu valgum Genu varum : common in Asians : familial : risk of degenerative arthritis -- uncertain

33 12yr/female 20yr/male

34 Idiopathic genu varum & Genu valgum Genu valgum : common in Caucasian, black : common in obese : intermalleolar distance > 10cm consideration of treatment

35 5yr/male 14yr/male

36 Treatment Idiopathic genu varum & genu valgum Conservative treatment: no evidence of certain effect Operative treatment : in severe cases I) Stapling (Zuege,1979; Mielke,1996) genu valgum -- at least 2 yrs of remaining growth : > yr of skeletal age : extraperiosteal position (subperiosteal- risk of permanent epiphysiodesis) : staple removal : within 2 yrs

37 12yr/female 1yr 2yr

38 Treatment Idiopathic genu varum & Genu valgum Operative treatment 2) Hemiepiphysiodesis (Bowen,1985,1992) -- lateral or medial partial growth arrest on growing child 3) Corrective osteotomy -- adolescent or mature patient

39 Lateral Hemiepiphysiodesis R= 5 cm Bone age = 12 yr Growth remaining in proximal tibia = 1.0 cm (by Green & Anderson growth remaining chart) (by R.Bowen,1985) 2 π x 5(cm) : = 1.0(cm) : y Varus angulation : 12 0 y = y = 360 /31.4=

40 Female/ 12 yr Lateral hemiepiphysiodesis (with fibula) Preop Postop 1yr

41 18yr/male Corrective Osteotomy with Ilizarov

42 Congenital Pseudarthrosis of the Tibia (CPT)

43 Incidence CPT 1/190,000 (Jacobson,1983) 1/140,000 (Andersen,1976) 1/250,000 (Paterson,1989)

44 Clinical Feature CPT Affected tibia sclerosis, cyst formation, tapering and anterolateral bowing of the distal tibia in early childhood Usually progresses to develop pathologic fracture Nonunion

45 Female/ 6m 12m 2yr

46 Male > Female Unilateral >>> Bilateral Anterolateral bowing : usually 0-2 years of age Fracture usually occurs by 1-3 years of age with no or minimal trauma * ( over 5 years : late onset CPT -- Good prognosis )

47 Associated condition 1) Neurofibromatosis (40-90% of CPT ) -- Evidence of relation with prognosis? Morrisy (1981) -- not affect the result Crossett (1988),Crawford(1999) -- poor prognosis

48 Differential Diagnosis Focal fibrocartilaginous dysplasia Posteromedial bowing of the tibia Physiologic bowing Benign Anterolateral bowing of the tibia (with duplication of the great toe)

49 Natural History CPT No tendency for the pseudarthrosis lesion to heal spontaneously Response to treatment and the tendency toward recurrent deformity seem to improve near puberty

50 Prognostic factor CPT Type of pseudarthrosis Age of patient Time of union Level of nonunion Behaviour of the hamartomatous tissue Technical skill of surgeon

51 CPT Treatment

52 Prevention of Fracture Long leg brace : controversial -- Few reports have shown this to be effective Prophylactic bypass graft (McFarland,1939) : Controversial

53 Progressive angular deformity before fracture : Corrective osteotomy should not be performed -- it is better to let nature take its course!!

54 CPT Treatment of Established Fracture

55 Principle Extensive & meticulous excision of the surrounding hamartomatous tissue Rigid fixation Osteo-induction by autogenous bone graft Prevention of the refracture by prolonged immobilization

56 Method of Treatment Dual onlay graft Electrical stimulation Intramedullary nailing Vascularized fibular graft Ilizarov treatment Amputaton

57 Method of Treatment Dual onlay graft Electrical stimulation Intramedullary nailing Vascularized fibular graft Ilizarov treatment Amputaton

58 Congenital Pseudarthrosis of the Fibula

59 Clinical Feature May occur in the absence of tibial pseudarthrosis Progressive ankle valgus Neurofibromatosis : more frequently associated than tibial pseudarthrosis

60 Clinical Feature Tibia : cortical thickening and only moderate bow, no constriction -- Boyd type IV or Type V Significantly overlap with the various categories of tibial pseudarthrosis

61 Treatment Anterolateral bowing with mild valgus : ankle-foot-orthosis Moderate to severe valgus : 1) Fibula IM nailing with Autogenous BG, 2) distal T-F joint synostosis Varus osteotomy of the intact distal tibia : should not be carried out until puberty -- risk of pseudarthrosis!!

62 28yr/male

63 Congenital Posteromedial Angulation of the Tibia

64 Congenital Kyphoscoliotic Tibia

65 Clinical Feature Etiology : unknown (developmental failure during embryonic period) Posteromedial bowing at the junction of the middle & distal thirds : deg. posteromedial angulation

66 Clinical Feature Foot (a) Hyperdorsiflexed; calcaneovalgus posture (b) Limited plantar flexion (c) No bony anomaly on foot & ankle

67 Clinical Feature Unilateral Shortening (5-27%) : inhibition of growth & tardy development of the secondary ossification center of the distal tibial epiphysis Severe angulation : greater discrepancy

68 Clinical Feature Proximal tibia & fibula : normal No increased susceptibility to fracture No pseudarthrosis By the age of 2 : 50% of the angulation corrected

69 Treatment Stretching exercise after birth In severe case : splint or above knee cast -- controversial Limb-length equalization -- frequently required

70 2 yr 1 yr 3 yr

71 1 yr 2 yr 3 yr

72

73 Congenital Dislocation and Subluxation of the knee

74 Incidence 1% of DDH (Jacobson,1985) Female > Male Bilateral > Unilateral

75 Clinical Feature Recurvatum deformity of the knee Result of the intrauterine position or true subluxation or dislocation at the knee level Breech fetal position predisposes the child to this condition

76 Clinical Feature In more severe cases, hip dislocation or clubfoot deformities are also present May be associated with an underlying condition ; arthrogryposis or Larsen syndrome

77

78 Treatment Congenital Dislocation and Subluxation of the knee

79 Physiologic group Excellent prognosis : Passive manipulation and serial casting until deg. flexion --- Pavlik harness for a few months

80

81

82 Pathologic group Initial effort : serial manipulation and casting Surgery Indication : if 60 deg. of knee flexion and adequate reduction of the tibiofemoral joint cannot be accomplished before the patient reaches 4 months of age.

83 Treatment Hip & Knee dislocation : knee deformity should be corrected before hip reduction!

84 Congenital Dislocation of the Patella

85 Clinical Presentation patella dislocation at birth often familial and bilateral occasionally accompanied by other abnormalities - Arthrogryposis multiplex congenita - Down syndrome - Larsen s syndrome - Nail patella syndrome

86 Clinical Presentation vastus lateralis: absent or contracted patella: small or misshapen gradual developing genu valgum & external rotation of the tibia

87 Classification (Eilert, 2001) Persistent dislocation Obligatory dislocation

88 Persistent dislocation - Persistent - Often obvious in infancy - Frequently with generalized syndrome - Knee flexion contracture (+) - Functional disability - Early surgical correction Obligatory dislocation - Reducible - Present at age 5-10 years - Isolated anomaly - ROM:normal - Little functional disability - Surgical correction: can be delayed until symptomatic

89 Persistent dislocation Female/ 3yr Nail-patellar syndrome Obligatory dislocation Male/ 8 yr

90 Diagnosis Usually difficult to make diagnosis before 3 4 yr old. Plain x-ray: difficult to define: lack of ossification of the patella MRI Ultrasonography

91 Treatment Operative technique -- extensive lateral release -- medial plication -- vastus medialis advancement -- gracilis or semitendinosus tenodesis -- transfer of lateral half or entire patella tendon

92 4 YR/ Male, right Lateral release Medial plication V.medialis advancement Semitendinosus tenodesis Postop 5 yr

93

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