Developmental Dysplasia of the Hip-1.pptx

Developmental Dysplasia of the Hip

1. Membantu Rekan Sejawat Dokter Meraih Cita-Cita Menjadi Dokter Spesialis. DDH

2. DDH DEVELOPMENTAL DYSPLASIA OF THE HIP

3. DEFINITION A spectrum of abnormalities in the development of hip joint, that ranges from mild acetabular dysplasia to irreducible dislocation

4. CLASSIFICATION Reduced and stable, but dysplastic Reduced but dislocatable > Barlow Dislocated but reducible > Ortolani Dislocated and irreducible

5. DEVELOPMENTAL DYSPLASIA OF THE HIP About DDH Is one of the most important and challenging congenital abnormality of musc . System The more accepted term: DDH  because it: Realistically indicates a dynamic disorder, potentially capable, as the baby develops, of getting better or worse Fact that small % of hips that by all criteria seem normal at birth may actually become dislocated/ subluxated as late as 6-10 mo when the infant’s hips extend to stand erect  here, it is not truly congenital, and has important medicolegal implication DDH= displacement ( Klisic 1989, Salter)/ dysplasia (North America) / dislocation (North America) *traditional term: CDH (congenital dislocation of the hip)

6. DEVELOPMENTAL DYSPLASIA OF THE HIP When we say DDH, it includes: Dislocation & subluxation of hip + Related secondary dysplasia (failure of adequate bony development of the acetabulum and t he proximal femur) Almost as common as clubfoot, yet is not so obvious at birth Demand a specific method of examination for its detection in the newborn, yet regrettably, is still not being recognized sufficiently early  may even escape detection until after a child starts to walk Unless treated early and well, it will inevitably lead to painful crippling degenerative arthritis of the hip in adult life 1/3 all arthritis of hips in adult life = caused by sequelae of DDH Making early diagnosis is so rewarding! Failure to make this is tragic!  always examine the hips joint in every infant entrusted to your care

7. DDH is considered as process or chains of events  that, in the beginning at least, can be arrested and even reversed Terms Dislocation (luxation) of the hip = femoral head being completely outside the socket (acetabulum), but still within the stretched and elongated capsule ( intracapsular ) Subluxation of the hip = femoral head riding laterally and proximally, but still in contact with at least part of acetabulum  is usually reduced and stable when the hip is flexed & abducted, but is subluxated when the hip is extended & adducted

8. *when the hip remains dislocated or subluxated  the bony development of the acetabulum and proximal femur (which was normal at birth) becomes progressively abnormal = Acetabular dysplasia Femoral dysplasia **thus, dysplasia is secondary to the displacement  therefore, it is developmental rather than congenital *DDH can be in: Otherwise normal children (this chp )  the common, typical type Prenatal ( teratologic ) type of truly congenital dislocation associated with: spinal bifida, arthrogryposis Incidence Common (1.5:1000 live births) >50% cases: bilateral  can be Dislocations of both hips Subluxation of both hips One of each

9. EPIDEMIOLOGY Girls:boys = 8:1 More common when there is positive family history or breech presentation of the infant Higher incidence in the custom of maintaining the hips of newborns in harmful position of extension & adduction by various means of swaddling, e.g. Cradleboards in North American Indians Tightly wrapped blankets in all cultures Higher in infants with: Congenital muscular torticollis Metatarsus adductus

10. RISK FACTORS

11. DJD of both hips in an adult, secondary to inadequate treatment of DDH during childhood

12. Etiology & Pathology As end result of combined genetic + environmental factors Hips joint develop well in utero, where it is constantly maintained in acute (complete) flexion At birth, 1 in 80 child exhibit an undue degree of congenital hip joint laxity  this is probably genetically determined If, at moment of birth, or even within the first few weeks, the previously flexed hips are passively EXTENDED in the presence of such marked joint laxity  the femoral head may dislocate  subsequently can reduce (relocate) / remain dislocated = that is why the old-age practice of a suspending newborn infant by the ankle is no longer acceptable *Thus, at birth, the abnormal hip can be dislocated but is not permanently dislocated, that it is = dislocatable

13. *the majority of this hips become stable spontaneously within the 1 st 2 months *if a vulnerable hip is maintained in extension  it tends to remain dislocated/ subluxated *persistent dislocation/ subluxation cause progressive secondary changes in all the structures in and around the hip joint *those secondary changes (important): Dysplasia (abnormal development of acetabulum  which becomes maldirected Increase in the normal femoral neck anterversion Hypertrophy of the elongated capsule Contracture & shortening of the muscles that cross the hip joint  esp. adductor ms , iliopsoas ms Newborn is tightly wrapped in a blanket that maintains the hips in harmful position of extension and adduction = 1 factor that causes initial dislocation in congenitally unstable hip

14. *each & every one of the progressive secondary changes: increases the difficulty in reducing the hip + in maintaining its reduction *as time goes by, those changes becomes progressively more marked + progressively less reversible *all facts above emphasize the extreme importance of: early diagnosis  responsibility lies on: family physician, obstetrician, pediatrician who are the first to see and examine the infant *possible that if a newborn infant’s hip are never passively extended and never maintained in extension during the first few months of life, the great majority of genetically vulnerable hips could be prevented from dislocating/ subluxating  so would go develop normally

15. Fetal position in utero Sudden passive extension of the previously flexed hips immediately after birth  avoid! 1 factor in the initial dislocation of a congenitally unstable hip

16. Diagnosis & Treatment Clinical dx, RO dx, and ortho tx vary so greatly with the child’s age  so is considered in specific group Importance of very early diagnosis & very early treatment! General principles of tx : Gentle reduction of the hip Maintenance of the reduction with the hip in stable position, until the various components of the hip has become stable even in the position of weightbearing

17. Birth – 3 Months Note, in this period: The most important period of greatest opportunity The abnormality is never obvious  it means that doctor must seek it out by careful examination of every infant you see Physical examination: Barlow provocative test (+) Ortolani sign (+) Limitation passive abduction of the flexed hip Extra skin creases on the inner side of the thigh External rotation of lower limb *4,5: make us least suspicious of DDH, even both can present in normal infants Imaging: USG of the hip by Graf, Harcke & Kumar  preferred to plain x-ray, because much of acetabular roof and all of femoral head is still cartilaginous, which is radiolucent

18. Barlow Provocative Test Aim: to detect instability of the disloctable hip Proc.: Flexed hips are alternately Adducted while pressing the femur downward Abducted while lifting the femur upward Result in the presence of instability, you will feel and see Adducted while pressing the femur downward: hip dislocate posteriorly Abducted while lifting the femur upward: hip reduce (+) Barlow test = hip is dislocatable , but not dislocated

19. The Barlow Test Test: flexed hip is adducted slightly while pressing downward along the axis of the femur (+): femoral head slide posteriorly out of acetabulum Test: then, flexed hip is abducted slightly while lifting the femur upward and pressing forward on GT (+): femoral head is suddenly reduced into the acetabulum with a ‘jerk’

20. Radiograph Findings (infant): The left hip is dislocated in the extended position  see: slight upward & lateral displacement of the adducted femur Acetabulum has not yet become dysplastic Findings (2 mo baby): Upward & lateral displacement of right femur Delayed development (dysplasia) of the bony part of the right acetabulum

21. The Ortolani Sign In the already dislocated hip Aim: to see whether the already dislocated hip is reducible (+): femoral head lies posterior to the acetabulum when the hip is in the flexed position, and can be reduced by abduction while lifting the femur forward.

22. Limitation to passive abduction of flexed hip Caused by: contracture of adductor muscles Most apparent >1 mo Important sign Does not necessarily indicate abnormal hip, should always be investigated by further radiographic examination Findings: Limitation to passive abduction of right flexed hip Asymmetry of skin fold of the medial aspect of the thighs

23. USG By Graf Harcke & Kumar Indication: not recommended routinely All infants < 6 mo with (+) clinical findings All infants < 6 mo who are at high risk of having DDH: (+) family history Breech presentation Generalized ligamentous laxity Dynamic USG is more reliable than static USG  in detecting dislocability / subluxability of the hip

24. *> 6 mo : ossific nucleus of femoral head has usually appeared + ossification of the acetabular roof is more advanced  so, plain radiograph is preferable to USG USG by Harcke & Kumar (?). Note the white dot (over the center of femoral head), alpha angle, and beta angle. Normal: femoral head is in normal relationship with acetabulum (is in the socket), normal alpha angle (65 deg ) and beta angle (53 deg ) Head of femur is dislocated laterally and proximally from the acetabulum (is out of the socket)

25. Treatment Principle: Gentle reduction of the hip  at this time is usually not difficult, then Maintenance of the hip in stable position = flexion & abduction (splint) A period of 4 months’ protection Methods of maintenance of reduction (choice of splint) Frejka pillow splint Pavlik harness Occasionally, after 3 weeks of tx , hip is still too unstable to be reduced by Frejka / Pavlik  tx : Gentle closed reduction of the hip, then Plaster hip spica cast in ‘human position’ A period of 4 months’ protection

26. * A period of 4 months’ protection: to Allow capsule to become tighter Allow the reduced femoral head to stimulate development of the hip  so to reverse the secondary changes *effects of tx must be assessd clinically & ultrasonographically Frejka Pillow Splint Characteristic: Keeps the hip in stable position of flexion & abduction + Allows some active movements of the hips

27. Pavlik Harness Characteristic: Alternative of tx during the first 3-4 mo Maintains hips in flexion (prevents active and passive extension of the hip) + permits all other movements  so helps to stimulate the development of the reduced hip If properly used: produces excellent result, even for frankly dislocated hip, with relatively few complications Complication of inappropriate use: avascular necrosis of femoral head

28. 3 mo – 18 mo Clinical Limitation of passive abduction Apparent & real shortening of the involved lower limb Prominence of hip Galleazi’s sign/ Allis’ sign  only in unilateral dislocation Phenomenon of telescoping Ortolani test (+)  but becomes more difficult to elicit *Because adduction contracture is more marked, so 1,2,3 become progressively more obvious *presence of dislocation, not dislocatable (?)

29. (1) Prominence of right hip; (2) External rotation of lower limb; (3) Shortening of lower limb, caused by: Adduction contracture of right hip causing apparent shortening of the right lower limb + Dislocation causing true shortening Limitation of passive abduction of right hip in extension Limitation of passive abduction of right hip in extension

30. Galeazzi sign/ Alles ’ sign Test: flex the both hips to 90 deg , compare the level of knees Pathop .& result: when the hips are flexed to 90 deg  femoralhead lies posterior to the acetabulum  thigh of dislocated side is shortened (see: lower level of the knee) Telescoping Phenomenon In the presence of complete dislocation, push pull maneuver in the femur will cause femur to moves to & fro within the thigh Test: the involved hip is flexed, push-pull maneuver is applied Result: when femur is dislocated at the hip, it will move to and fro within the thigh

31. Ortolani Test (+): feeling the hip go in and go out of the joint during ortolani test *(+) = presence of dislocation * ortolani sign here becomes: Progressively more difficult to elicit the longer the hip has remained out of joint Cannot be elicited with subluxation Radiographic Examination Findings: Excessive slope of ossified portion of the acetabulum/ delayed ossification of acetabulum = indicates acetabular dysplasia & maldirection Delayed ossification of the femoral head Varying degrees of upward & lateral displacement of the head of femur

32. Plain x ray can be performed in child > 3 mo Acetabular index = n < 25 o Perkins line = n ossific nucleus of femoral head is medial to this line Shenton line = n intact CEA (center-edge angle) of Wiberg = n >20 o Delayed ossification of femoral head >> appears later. Normal begins to appear between 4 – 6 mo CEA: formed by Perkin line and a line from the center of the femoral head to the lateral edge of the acetabulum >> only in > 5 yo CT, MRI is performed after closed reduction, to determine concentric reduction

33. Treatment Preliminary lengthening of the tight adductor and hamstring muscles = by continuous tape traction for few weeks, at home whenever feasible Subcutaneous adductor tenotomy + gentle closed reduction if the hip under GA Maintenance of reduced hip in a hip spica cast in stable position = marked flexion + moderate abduction (‘human position’) Protection of reduction = by Large Frejka pillow splint/ 2 long leg casts separated by abduction bar  allow some movement of the hip within a safe range  so provide further stimulation for development of acetabulum & femoral head

34. Continuous skin traction with adhesive tape on the lower limb For few weeks Aim: gradually stretches the shortened muscles around the hip in preparation for a safe and gentle closed reduction Bilateral hip spica plaster cast Applied after tenotomy & hip is reduced, + perfection of reduction is confirmed radiologically Change every 2 months  until RO reveals satisfactory development of both acetaulum & femoral head Period of immobilization is needed to bring about reversal of secondary changes will vary depends on number of months the hip had been dislocated before tx  usually 5 – 8 months Earlier diagnosis and tx would have shortened the period of immobilization

35. Hip spica cast In human position, as opposed to frog position Postreduction RO in cast may be difficult to interpret  CT will provide more accurate image of relationship between femoral head & acetabulum

36. Prognosis Result of gentle & careful closed tx during 3 – 18 mo : good in 80% px % of good result is much higher if started in 3 mo than in 18 mo If closed reduction fails/ immobilization fails to maintain obtained reduction: open reduction + iliopsoas muscle release + capsular repair  through anterior approach *< 1 yo : medial approach is reasonable alternative  this approach does not allow a capsular repair, but such repair is considered to be less necessary in this young age group, by some experts

37. 18 mo – 5 yo Notes: the secondary changes are more severe + less reversible Clinical ( px is walking) Limitation of passive abduction Apparent & real shortening of the involved lower limb Prominence of hip Galleazi’s sign/ Allis’ sign  only in unilateral dislocation Phenomenon of telescoping Ortolani test (+)  but becomes more difficult to elicit Typical limp Trendelenburg sign (+) *1-6: more marked than in 3 mo-18 mo

38. Trendelenburg Sign (B): The child is standing with both of feet on the floor, findings: The dislocation is not apparent Slight shortening in the lower limb (C): The child is standing on the normal hip, finding: because hip abductors have a normal fulcrum, pelvis level is held. Test (A): ask px to stand in one foot (on the side of dislocated hip) (+): because hip abductor muscles have no fulcrum, it cannot hold the pelvis level  so it drops on the opposite side  the child will be in effort to maintain balance, so she shifts her trunk toward the involved side

39. Important, Trendelenburg sign is also present in: DDH Coxa vara Paralyzed hip abductors Painful conditions around the hip

40. Limp When the dislocation is unilateral: the child walks as though the lower limb on that side is too short + shifts the trunk toward in the involved side when weight is borne to that hip When the dislocation is bilateral: the child shifts the trunk from one side to other while walking  gives impression of waddling like a duck **with a subluxation, : Trendelenburg sign & limp is not nearly as apparent as in dislocation, but More readily detected when the muscles are fatigued, e.g. after period of long walking

41. Treatment Associated with difficulties, dangers, disappointments even in the most experienced hands Treatment: Overcome the very resistant muscle contracture Reduction of dislocation Maintaining the reduction

42. (1) Overcome the very resistant muscle contracture By: longer period of tape traction + subcutaneous adductor tenotomy For children > 3 yo with high dislocation: femoral shortening is a reasonable alternative to preoperative traction

43. (2) Open Reduction (Operative Reduction) For children > 18 mo : the likelihood of perfect reduction becomes progressively less  so, open reduction (operative reduction) is indicated *during this operative reduction, the secondary soft tissue abnormalities (esp. (1) tight iliopsoas ms (2) elongated joint capsule) is dealt with *main problem in this age group: not the reduction, but the maintenance of reduction Difficulty in the maintenance of reduction is caused by: significant instability of reduced, but poorly developed, hip joint *what component that caused those significant instability and poorly developed joint? Abnormal direction in which the acetabulum faces

44. Most reliable operation to reduce the dislocation: innominate osteotomy Proc.: redirecting the entire maldirected acetabulum  Principle: redirection of entire acetabulum in such way that a reduced hip, which previously was stable only in a position of flexion & abduction, is rendered stable with the limb in normal position of weightbearing

45. Prognosis of reduction in 18 mo – 5 yo Type of reduction Prognosis Closed reduction Depressing, because only 30% are excellent or good Careful open reduction + innominate osteotomy 87% excellent or good *but, still not good as the result of successful closed treatment instituted in the first 3 months of life = extreme importance in early dx & tx

46. > 5 yo About DDH in > yo Fortunately, only few children reach the age of 5 with untreated DDH  although the same cannot be said for congenital subluxation Secondary changes in complete dislocation is so marked The reversibility of secondary changes is so limited  that even an extensive operative procedure (incl. femoral shortening) cannot be expected to meet with success, particularly in: bilateral dislocation + >6 – 7 yo Beyond 6 – 7 yo : unwise to attempt reduction  because good result is not possible to obtain with any form of treatment  the child will be doomed to a disability for the rest of her life

47. Treatment Condition Notes Treatment Residual subluxation Is less difficult to treat than the dislocation Innominate osteotomy up to the end of the growing period and beyond Irreducible congenital dislocation of the hip Unfortunate children  Palliative & salvage types of operative procedures for relief of pain, in early adult life Take home messages: Early dx & gentle tx is still the most important aspect in DDH Neonatal screening for DDH in all infant is effective in reducing the incidence of missed dislocation  decrease number of children who require extensive surgery Subluxation is more difficult to detect at birth so routine PE & radiographic re-examination at 4 mo will be useful in the detection It is hoped that during your professional lifetime, you will never allow DDH in newborn infant to be unrecognized!

48. DDH – PRINCIPLES OF TREATMENT (LOVELL) According to age < 6 mo : Pavlik Harness (can be Frejka pillow, von Rosen splint) Prerequisite: Ortolani (+) 6 mo – 2 yo : Closed/ open reduction + maintenance of reduction with hip spica cast Can be preceded with skin/ skeletal traction for 2 weeks (now is rarely) > 18 yo : open reduction +/- femoral shortening 18mo – 3 yo (Gray zone): open reduction + femoral shortening/ acetabular procedure Acetabular procedure can be in conjunction with open reduction or 6-8 weeks after it Most common acetabular procedure: Salter innominate osteotomy, Pemberton innominate osteotomy > 3 yo : open reduction + femoral shortening + acetabular procedure Acetabular procedure can be in conjunction with open reduction or 6-8 weeks after it, of later

49. PAVLIK HARNESS Most commonly used Characteristic: prevents hip extension and adduction (that cause redislocation ), and but allow further flexion and abduction (read to reduction and stabilization) Use full time for 6 weeks > 95% of cases will resolve Can be used until the age of 6 mo After 6 mo : failure >50%

50. CONTRAINDICATION OF PAVLIK HARNESS Significant muscle imbalance > myelodysplasia, CP Significant stiffness of the joints > arthrogryphosis Excessive ligamentous laxity > Ehlers-Danlos syndrome

51. PAVLIK HARNESS DISEASE E/ failure of PH treatment, but physician still prolong the treatment using PH, so that the secondary pathologic changes occur An inappropriately applied harness is a failure of the physician, not a failure of the orthotic Diseases Damage of the femoral head Injury of acetabular cartilage Inferior dislocation Femoral nerve compressive neuropathy Skin problems If family is not compliant, closed reduction and cast is a more judicious approach Position Hip flexion: 100 – 110 degree Abduction within safe zone of Ramsey = arc of abduction and adduction, that is between redislocation and comfortable, unforced abduction Use 6 – 12 weeks

52. SKIN/ SKELETAL TRACTION Aim: to allow gradual relaxation of secondarily contracted muscles (iliopsoas and adductor longus) >> this will allow reduction without creating excessive joint force, thereby decreasing the risk of AVN and and reduce the need of open reduction

53. CLOSED REDUCTION Performed in operating room Closed reduction + adductor tenotomy (open/ percutaneous)