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 Torsional Deformity (Rotational Deformity)
3 Torsional Deformity Abnormal rotation of the tibia & femur -- torsion : > 2 SD from average Normal rotation of the tibia & femur -- version : within 2 SD
4 Rotational profile(staheli,1985) Hip Rotation Foot Progression Angle Thigh Foot Angle Foot Deformity
5 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)
6 6yr/female- intoeing gait Foot Progression Angle -- negative
7 Internal rotation -- increased External rotation -- decreased
8 Thigh Foot Angle : Normal W Position
9 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)
10 2yr/male- intoeing gait Genu varum Thigh Foot Angle : Negative
11 Torsional Malalignment Syndrome Excessive Medial Femoral Rotation + Lateral Tibial Rotation (compensation?) Patello-Femoral Instability or Pain Treatment : IF Severe, Femoral + Tibial osteotomy
12 14 yr-old female/ Genu varum
13 Natural History Torsional deformity 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
14 Treatment Torsional deformity Conservative treatment -- ineffective, unnecessary
15 Operative treatment Torsional deformity Indication -- Severe torsional deformity (beyond 3 SD) + functional & cosmetic disability -- after age 8
16 Operative treatment Torsional deformity Femur -- rotational osteotomy - level: intertrochanter, subtrochanter, midshaft, supracondylar Tibia -- rotational osteotomy - level: proximal (correction of concomitant angular deformity) distal
17 Angular Deformity
18 Knee Angle Normal growth pattern
19 1yr6mo 4yr
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 : radiolographic sign at risk 11 o 16 o : relative radiographic sign at risk
24 Metaphsyeal-Diaphyseal Angle
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 (Park et al,2005) genu varum -- under age of 10
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 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 1 yr
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 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 )
46 Associated condition Neurofibromatosis (40-90% of CPT ) -- Evidence of relation with prognosis? Morrisy (1981) -- not affect the result Crossett (1988),Crawford(1999) -- poor prognosis
47 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
48 Prognostic factor CPT Type of pseudarthrosis Age of patient Time of union Level of nonunion Behaviour of the hamartomatous tissue Technical skill of surgeon
49 CPT Treatment
50 Prevention of Fracture Long leg brace : controversial -- Few reports have shown this to be effective Prophylactic bypass graft (McFarland,1939) : Controversial
51 Progressive angular deformity before fracture : Corrective osteotomy should not be performed -- it is better to let nature take its course!!
52 CPT Treatment of Established Fracture
53 Principle Extensive & meticulous excision of the surrounding hamartomatous tissue Rigid fixation Osteo-induction by autogenous bone graft Prevention of the refracture by prolonged immobilization
54 Method of Treatment Dual onlay graft Electrical stimulation Intramedullary nailing Vascularized fibular graft Ilizarov treatment Amputaton
55 Method of Treatment Dual onlay graft Electrical stimulation Intramedullary nailing Vascularized fibular graft Ilizarov treatment Amputaton
56 Intramedullary nailing Williams rod Bailey-Dubow rod Sofield rod Steinmann pin IM nailing across the ankle & subtalar joint for fixation of the distal small segment
57 Intramedullary nailing Anderson et al (1992) :by William's rod -- 9/10 patients : union Baker et al (1992) :by William's rod -- 13/18 patients : union Kim et al(2002) :the most important factor - location - presence of pseudarthrosis of the fibula
58 Intramedullary nailing problem (a) stiff ankle & hindfoot (b) refracture (c) valgus of ankle joint (d) the potential risk of lesions of the growth plate of the distal tibia
59 1mo/male
60 IM nailing with brace
61 1yr6mo IM nail change with B.G Postop Postop 2 yr
62 Vascularized fibular graft ( Goldberg,1988; Maroylovic,1991; Weiland, 1991; Jupiter,1993; Coleman,1994 ) Contralateral or ipsilateral fibular graft Union rate : % Indication Bone defect more than 3 cm Recurrence after other treatment
63
64 Vascularized fibular graft Disadvantage (a) Morbidity of the donor site (contralateral fibula) (b) Nonunion or angular deformity in junction site (c) Surgical difficulty (d) Long operation time
65 Ilizarov treatment Method (Boero et al,1997) (1) Direct compression of nonunion site (2) Resection and compression (3) Resection and bone transport
66 Ilizarov treatment Advantage (a) provide High union rate : % (Fabry,1988; Plawecki,1990; Paley, 1992; Gahnem,1997) (b) Ability to correct the all type of angulation (c) Correction of shortening
67 Ilizarov treatment Disadvantage (a) Time consuming (b) Poor tolerance (indication: 3 yrs or older) (b) Risk of infection of the wire track (c) Risk of the refracture through the wire hole
68 Female /4yr6mo
69 Postop 8mo Bone transport: 6 cm
70 Postop 12mo
71 Congenital Pseudarthrosis of the Fibula
72 Clinical Feature May occur in the absence of tibial pseudarthrosis Progressive ankle valgus Neurofibromatosis : more frequently associated than tibial pseudarthrosis
73 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!!
74 4yr/female Preop Postop
75 Congenital Posteromedial Angulation of the Tibia
76 Congenital Kyphoscoliotic Tibia
77 Clinical Feature Etiology : unknown (developmental failure during embryonic period) Posteromedial bowing at the junction of the middle & distal thirds : deg. posteromedial angulation
78 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
79
80
81
82 Clinical Feature Proximal tibia & fibula : normal No increased susceptibility to fracture No pseudarthrosis By the age of 2 : 50% of the angulation corrected
83 Treatment Stretching exercise after birth In severe case : splint or above knee cast -- controversial Limb-length equalization -- frequently required
84 Congenital Dislocation and Subluxation of the knee
85 Incidence 1% of DDH (Jacobson,1985) Female > Male Bilateral > Unilateral
86 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
87 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
88
89 Treatment Congenital Dislocation and Subluxation of the knee
90 Physiologic group Excellent prognosis : Passive manipulation and serial casting until deg. flexion --- Pavlik harness for a few months
91
92
93 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.
94 Treatment Hip & Knee dislocation : knee deformity should be corrected before hip reduction!
95 Congenital Dislocation of the Patella
96 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
97 Clinical Presentation vastus lateralis: absent or contracted patella: small or misshapen gradual developing genu valgum & external rotation of the tibia
98 Classification (Eilert, 2001) Persistent Dislocation Obligatory Dislocation
99 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
100 Persistent dislocation Female/ 3yr Nail-patellar syndrome Obligatory dislocation Male/ 8 yr
101 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
102 Treatment Operative technique -- extensive lateral release -- medial plication -- vastus medialis advancement -- gracilis or semitendinosus tenodesis -- transfer of lateral half or entire patella tendon
103 3 yr/ female, bilateral Nail-patellar syndrome Lateral release Medial plication V. medialis advancement Semitendinosus tenodesis Postop 3 yr
104 4 YR/ Male, right Lateral release Medial plication V.medialis advancement Semitendinosus tenodesis Postop 5 yr
105
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