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Discuss the Pathology and
Management of Developmental
Dysplasia of the Hip
By
Dr Kabiru SALISU
NOH Dala, Kano.
Outline
• Introduction
• Embryology
• Pathology
• Diagnosis
• Treatment
• Complications
• West African experience
• Conclusion
• References
Introduction
Developmental Dysplasia of the Hip (DDH)
Is a spectrum of intra-capsular hip pathology
that ranges from instability, subluxation or
Dislocation of the Hip; before, during or after
birth.
• CHD- Hippocrates
• Guillaume Dupuytren 1832
• Klisic 1989
• Ortolani -Early 1900
• Galeazzi- LLD
DDH is the commonest Orthopaedic disorder
in new born. It has excellent prognosis when
detected at birth. However, Poses a treatment
challenge when present late.
Classification
• Type I
• Type II
• Type III
Spectrum of disease ranges from
• Dysplasia
• Instability
• Subluxation
• Dislocation
• Teratologic hip
• Late (adolescent) dysplasia
Embryology of DDH
PATHOLOGY OF DDH
Epidemiology
• 1 per 1000 live births
• Barlow (1962) described an incidence of
instability 1:60 however 60 per cent stabilized
by one week and 88 per cent by 8 weeks
• Common in native Americans and Lapps
• Female: Male = 6:1
• Left: Right = 5:1
• Bilateral in 20%
Risk factors
- First born
- Female
- Faulty intrauterine position (eg Breech)
- Family history
- OTHER condition
Oligohydramnios
Torticollis 15- 20%
metatarsus adductus 1.5 -10%
Aetiopathogenesis
• Genetic factors
• Hormonal Factors
• Mechanical factors (Intrauterine malposition)
• Primary dysplasia
• Postnatal factors
Cradle board
Backing babies
Pathological changes in DDH
• At Birth - Normal, dysplasia & Stretched
capsule
• During infancy- Adaptation to persistent
instability and dislocation
• After weight bearing commences;- Persistent
dislocation and adaptation to lording
CHANGES IN THE BONES
• Acetabulum
• The femoral Head
• The neck of the femur
• The pelvis
CHANGES IN THE SOFT TISSUES
• The capsule
• Ligamentum teres and TAL
• The Muscle – Palvifemoral ;- Shortened
- Pelvitrochantric ;-
- Gluteal
• Secondry barries to
reduction include;
- Elongated and thicken
LT&TAL
- Everted labrum
- Limbus
- Neolimbus
- Pulvinar
- Hour glass shaped capsule
- Contracted muscles
- Femoral neck anteversion
MANAGEMENT OF DDH
Clinical presentation
• HISTORY
- Sex
- Race/ ethnicity
- Prenatal history
- Natal –breech
- Post natal
- Family history
- Difficulty in applying diaper
- Delay walking
- Limping & tip toeing
- Limb shortening
- Late presentation
Painless limp
Hip pain
Back pain /deformity
Examination
Test for sign of hip
instability
- Ortolani’s test
• Barlow’s test
- Asymmetry in groin and
gluteal folds
- Galleazi test
• Limitation of abduction
• Klisic test
- +ve Trendelenburg sign/ gait
- Hyperlordosis
- Compensatory Scoliosis
INVESTIGATION
Radiologic investigation
1 - ULTRASOUND SCAN
• Static (Graf technique)
- To determine dysplastic changes in the hip
• Dynamic (Hercke and Clarke)
- To determine instability
Alpha >60 & Beta <55
Graf classification
Class Alpha angle Beta angle Definition Treatment
I >60 <55 Normal -
II 43-60 55-77 Abnormal Observe/
harness
III <43 >77 Subluxated Pawlik harness
IV unmeasurable unmeasurable Dislocated Pawlik harness/
closed vs open
reduction
2- X-RAY
• X-ray feature became
more apparent from the
6 month
Shenton’s line
Hilgenreiner’s and Perkin’s lines
Acetabular index
Von Rosen’s lines
The Wilberg center–edge angle
3- Arthrography
• Used to confirm reduction after closed
reduction
• Help identify possible blocks to reduction
4- CT scan / MRI
TREATMENT OF DDH
Screening
Non Operative Care
• 1- Close observation
Indication;- USS feature of instability
No Dysplasia
No dislocation
Weekly USS or clinical Examination for 6weeks
2- Abduction splinting and bracing (Pavlik
Herness)
Indication ;- Child < 6month with
-Instability + dysplasia or dislocation
- Persistent instability > 6weeks
Success rate;- 90%
• Hip Flexion 100 - 1200
• Abducted 30-400
• Internal rotation 10-20 0
• 6weeks
• Weekly examination
• Other forms of Abduction splinting are;
- Double Napkins
- Abduction pillow
- Backing
3- Closed Reduction and Hip Spica or Traction
Indications
– DDH in 6 – 18mths
– Failure of Pavlik treatment
• Reduce dislocation
• 6weeks spica or traction
• 6month removable splint
Operative care
Indication
• DDH > 2 yr with residual hip dysplasia
• Failed closed reduction
• Persistent subluxation
• Soft tissue interposition
• Femoral anteversion, coxa valga
• Increased acetabular index
• Degenerative changes
1- Adductor Tenotomy
and traction
2- Open
reduction and
hip Spica
3- Open reduction +
femoral osteotomy
• To correct excessive
femoral anteversion
and/or valgus
• Prevent AVN
4- open reduction
+ pelvic
osteotomy
Or
Acetabuloplasty
Redirection Osteotomies
Reshaping Osteotomies
Salvage Osteotomies
• 5- Hip replacement
Complications
• Failed reduction
• AVN of femoral head
• Persistent Dislocation in adult
• Recurrence
• Transient femoral nerve palsy
West African experience
• Poor knowledge of risk factors
• Lack of screening programs
• Late presentation with complications
Conclusion
• DDH is one of the commonest Orthopaedic
conditions at the neonatal period with good
prognosis when identified early.
• Identification of babies at risk, screening at
birth and early institution of treatment are
known to improve outcome.
References
• John AH. Tachjian’s Paediatric Orthopaedics5th edition. Elservier
Sounders;2014. Chapter 16; Developmental Dysplasia of the Hip:483-535
• Duthei RB. Mercer’s orthopaedic surgey 9th edition. Arnold. Chapther 3.
Congenital Malformations: 152- 172
• Solomon L, Ganz R, Leunig M, Mousell F, Learmonh L. Apley’s system of
orthopaedics and fractures 9th edition. Holder Arnold.Chapter 19; The Hip;
Developmental Dysplasia of the hip: 498-504
• Cris S. Developmental dysplasia of the hip. Orthobullet
• Developmental Dysplasia of the hip. www.orthinfo.org
• Agrawal U. Developmental Dysplasia of the hip. Ppt
• Agarwal A, Gupta N. Risk factor and diagnosis of DDH in children. J clin
Orthop trauma. 2012;3:10-14
• Thawrani D. Pelvic osteotomies. Medscape;2015
Thank you

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Management of ddh

Editor's Notes

  1. The term congenital dislocation of the hip dates back to the time of Hippocrates. This condition, also known as hip dysplasia or developmental dysplasia of the hip (DDH), has been diagnosed and treated for several hundred years. Most notably, Ortolani, an Italian pediatrician in the early 1900s, evaluated, diagnosed, and began treating hip dysplasia. Galeazzi later reviewed more than 12,000 cases of DDH and reported the association between apparent shortening of the flexed femur and hip dislocation. Since then, significant progress has been made in the evaluation and treatment of DDH
  2. DDH encompasses a spectrum of disease that includes  dysplasia  a shallow or underdeveloped acetabulum subluxation dislocation teratologic hip dislocated in utero and irreducible on neonatal exam presents with a pseudoacetabulum associated with neuromuscular conditions and genetic disorders commonly seen with arthrogryposis, myelomeningocele, Larsen's syndrome late (adolescent) dysplasia  mechanically stable and reduced but dysplastic
  3. 4th week from single mesenchyme Separation occur 7-8wks Development is completed 11wks Acetabulum & femoral head are mainly cartilaginous at birth Development continue post op.
  4. The reported incidence of neonatal hip instability in northern Europe is approximately 1 per 1000 live births, but this is dependent on the definition of ‘instability’. Barlow (1962) described an incidence of1:60; however 60 per cent stabilized by one week and 88 per cent by 8 weeks
  5. Aetiology and pathogenesis Genetic factors must play a part in the aetiology, for DDH tends to run in families and even in entire populations (e.g. in countries along the northern and eastern Mediterranean seaboard). Wynne-Davies (1970) identified two heritable features which could predispose to hip instability: generalized joint laxity (a dominant trait), and shallow acetabula (a polygenic trait which is seen mainly in girls and their mothers). However, this cannot be the whole story because in 4 out of 5 cases only one hip is dislocated. Hormonal factors (e.g. high levels of maternal oestrogen, progesterone and relaxin in the last few weeks of pregnancy) may aggravate ligamentous laxity in the infant. This could account for the rarity of instability in premature babies, born before the hormones reach their peak. Intrauterine malposition (especially a breech position with extended legs) favours dislocation; this socalled ‘packaging disorder’ is linked with the higher incidence in first-born babies, among whom spontaneous version is less likely. Unilateral dislocation usually affects the left hip; this fits with the usual vertex presentation (left occiput anterior) in which the left hip is adjacent to the mother’s sacrum, placing it in an adducted position. Other manifestations of intrauterine crowding, including plagiocephaly, congenital torticollis and postural foot deformities, are also associated with a higher than usual incidence of DDH. Postnatal factors may contribute to persistence of neonatal instability and acetabular maldevelopment. Dislocation is very common in Lapps and North American Indians who swaddle their babies and carry them with legs together, hips and knees fully extended, and is rare in southern Chinese and African Negroes who carry their babies astride their backs with legs widely abducted. There is also experimental evidence that simultaneous hip and knee extension leads to hip dislocation during early development (Yamamuro and Ishida, 1984
  6. At birth the hip, though unstable, is probably normal in shape but the capsule is often stretched and redundant.
  7. After weightbearing commences, these changes are`intensified. Both the acetabulum and the femoral neck remain anteverted and the pressure of the femoral head induces a false socket to form above the shallow acetabulum. The capsule, squeezed between the edge of the acetabulum and the psoas muscle, develops an hourglass appearance. In time the surrounding muscles become adaptively shortened Acetabulum – shallow Posterior superior defect Poor acetabular cartilage Covered by fibrocartilage Pulvin The femoral Head - Relatively bigger the acetabulum - later became flattened on it medial and posterior aspect The neck of the femur - Marked shortening Anteversion increase to up to 900 The pelvis- tilted forward - hyper lordosis
  8. The capsule Stretched, laxed and redundant Assume hour glass appearance Ligamentum teres and TAL elongated and hypertrophied Palvifemoral;- shortened and
  9. secondary barriers to reduction develop. In the depths of the acetabulum, the fatty tissue known as the pulvinar thickens and may impede reduction (Fig. 1. The ligamentum teres also elongates and thickens, and it may take up valuable space within the acetabulum. The transverse acetabular ligament is often hypertrophic as well, and it may impede reduction.112 More important, the inferior capsule of the hip assumes an hourglass shape, eventually presenting an opening that is smaller in diameter than the femoral head. The iliopsoas, which is pulled tight across this isthmus, contributes to this narrowin. The capsule also narrows through a “Chinese finger-trap” mechanism. The femoral changes include increase anteversion and flattening of the femoral head
  10. Prenatal history ; suggestive oligohydramnios
  11. In Ortolani’s test, the baby’s thighs are held with the thumbs medially and the fingers resting on the greater trochanters; the hips are flexed to 90 degrees and gently abducted. Normally there is smooth abduction to almost 90 degrees. In congenital dislocation the movement is usually impeded, but if pressure is applied to the greater trochanter there is a soft ‘clunk’ as the dislocation reduces, and then the hip abducts fully (the ‘jerk of entry’). If abduction stops halfway and there is no jerk of entry, there may be an irreducible dislocation Barlow’s test is performed in a similar manner, but here the examiner’s thumb is placed in the groin and, by grasping the upper thigh, an attempt is made to lever the femoral head in and out of the acetabulum during abduction and adduction. If the femoral head is normally in the reduced position, but can be made to slip out of the socket and back in again, the hip is classed as ‘dislocatable’ (i.e. unstable).
  12. .
  13. A potentially perilous situation for the unwary examiner is the child with bilateral hip dislocation. This child has no asymmetry on abduction, and the flexed knees are at the same level. Combined abduction is limited, but this is difficult to detect because the limitation is symmetric. One test that can help the examiner to recognize a bilateral dislocation is the Klisic test, in which the examiner places the third finger over the greater trochanter and the index finger on the anterior superior iliac spine. An imaginary line drawn between the fingers should point to the umbilicus.
  14. With each step, the pelvis drops as the dislocated hip adducts, and the child leans over the dislocated hip; this is known as an abductor lurch or Trendelenburg gait
  15. - Femur head ossifies at 4-6month thus uss is 100% sensitive and specific shows the soft anatomy of the hip and the relationship of the femoral head and the acetabulum very well. Technical advances have improved image quality, and dynamic techniques add significant information to that obtained from static images.
  16. Graf Technique – Morphologic assessment, relies on anatomic landmarks 3 lines- Baseline- line of ilium as it intersects bony and cartilaginous portions of acetabulum Inclination line – Line along the margin of cartilaginous acetabulum Acetabular roofline – Along the bony roof Angle between roof and base line – Alpha - >60 , evaluates bony acetabulum Angle between inclination and base line- Beta - <55 , evaluates cartilaginous acetabulum
  17. On Xrays- Hilgenreiners line - Perkins line - Shenton’s line - Acetabular Index - Centre-edge angle of wilberg - Acetabular depth to width – normally >38% - Widened acetabular tear-drop Von-Rosen’s view – with hip abducted internally rotated, and extended
  18. In a normal hip, the medial beak of the femoral metaphysis lies in the lower, inner quadrant produced by the juncture of the Perkin and Hilgenreiner lines. The Shenton line is smooth in the normal hip. In the dislocated hip, the metaphysis lies lateral to the Perkin line; the Shenton line is broken because the femoral neck lies cephalic to the line from the pubis
  19. Another useful measurement is the acetabular index, which is an angle formed by the juncture of the Hilgenreiner line and a line drawn along the acetabular surface. In normal newborns, the acetabular index averages 27.5 degrees. At 6 months of age, the mean is 23.5 degrees. By 2 years of age, the index usually decreases to 20 degrees. Thirty degrees is considered the upper limit of normal
  20. Von Rosen’s lines: with the hips abducted 45° the femoral shafts should point into the acetabula. In each case the left side is shown to be abnormal. A helpful radiographic projection is the Von Rosen view, in which both hips are abducted, internally rotated, and extended.292 In the normal hip, an imaginary line extended up the femoral shaft intersects the acetabulum. When the hip is dislocated, the line crosses above the acetabulum
  21. The Wilberg center–edge angle, which is the angle that is formed between the Perkin line and a line drawn from the lateral lip of the acetabulum through the center of the femoral head. This angle, which is a useful measure of hip position in older children, is considered normal if it is more than 10 degrees in children between the ages of 6 and 13 years. It increases with age.
  22. CT- scan- delineation of acetabular and femoral anatomy in older age group MRI- further delineation of block to reduction
  23. All infants require screening Physical exam successful screening requires repetitive screening until walking age Ultrasound ultrasound screening of all infants occurs in many countries, however, it has not been proven to be cost effective USA recommendations is to perform ultrasound at 4 to 6 weeks in patients with risk factors positive physical findings utilized to follow Pavlik treatment or for equivocal exams
  24. abduction splinting/bracing (Pavlik harness)    indications DDH < 6 months of age and reducible hip Pavlik harness treatment is contraindicated in teratologic hip dislocations is a dynamic splint that requires normal muscle function for successful outcomes contraindicated in patients with spina bifida or spasticity outcomes overall Pavlik harness has success rate of 90% dependent upon age at initiation of treatment and time spent in the harness abandon pavlik harness treatment if not successful after 3-4 weeks  If pavlik harness fails, convert to semi-rigid abduction brace with weekly ultrasounds for an addition 3-4 weeks before considering further intervention
  25. The three golden rules of splintage are: (1) the hip must be properly reduced before it is splinted; (2) extreme positions must be avoided; (3) the hips should be able to move
  26. closed reduction and spica casting  indications DDH in 6 - 18 months of age failure of Pavlik treatment
  27. depending on radiological evidence of satisfactory acetabular development. The concentrically reduced hip is held in a plaster spica at 60 degrees of flexion, 40 degrees of abduction and 20 degrees of internal rotation. After 6 weeks the spica is changed and the stability of the hips
  28. open reduction and spica casting  indications DDH in patient >18 months of age failure of closed reduction
  29. open reduction and femoral osteotomy  indications DDH > 2 yr with residual hip dysplasia  anatomic changes on femoral side (e.g., femoral anteversion, coxa valga) femoral head should be congruently reduced with satisfactory ROM, and reasonable femoral sphericity best in younger children (< 4 yr) after 4 yr, pelvic osteotomies are utilized used to correct excessive femoral anteversion and/or valgus femoral osteotomy and shortening may be needed to prevent AVN decrease tension produced by reduction of a previously dislocated hip
  30. open reduction and pelvic osteotomy  indications DDH > 2 yr with residual hip dysplasia  severe dysplasia accompanied by significant radiographic changes on the acetabular side (increased acetabular index)  used more commonly in older children (> 4 yr) decreased potential for acetabular remodeling as child ages
  31. Pemberton (pericapsular) osteotomy The Pemberton osteotomy is used for anteriorly and laterally deficient acetabula in patients older than 18 months who still have an open triradiate cartilage. A Smith-Peterson approach is used to access the inner and outer table of the iliac wing. The outer iliac cortex is osteotomized, with the cut started just above the anterior inferior iliac spine and carried out posteriorly and parallel to the capsule in the direction of the triradiate cartilage. The inner table is osteotomized if anterior coverage is necessary. Care must be taken to remain halfway between the anterior edge of the sciatic notch and the posterior rim of the acetabulum Dega osteotomy The concept of the Dega osteotomy [37] is similar to that of the Pemberton osteotomy—that is, to change the configuration or shape of the capacious acetabulum by hinging the fragment through the open triradiate cartilage. However, unlike the Pemberton osteotomy, which increases anterolateral coverage, the Dega osteotomy involves cutting only the lateral aspect of the ilium and thus provides posterolateral coverage
  32. The Chiari osteotomy is made just above the acetabulum, in the region of insertion of the hip capsule.‖ The osteotomy curves to match the acetabular contour, and it slopes upward from lateral to medial. The level of the osteotomy and the slope of the cut must be precise for the displaced iliac wing to cover the femoral head without impinging on it. The acetabular fragment is displaced medially almost the full width of the ilium at that level, and it is held there with pin or screw fixation
  33. Osteonecrosis seen with all forms of treatment increased rates associated with excessive or forceful abduction previous failed closed treatment repeat surgery diagnosis based on radiographic findings that include failure of appearance or growth of the ossific nucleus 1 year after reduction broadening of femoral neck  increased density and fragmentation of ossified femoral head residual deformity of proximal femur after ossification Delayed diagnosis bilateral dislocations patients typically functions better if hips are not reduced if 6 years of age or older unilateral dislocation better outcomes without surgical treatment if patient is 8 years of age or older epiphysiodesis can be performed for treatment of limb length discrepancy Recurrence approximately 10% with appropriate treatment requires radiographic follow-up until skeletal maturity Transient femoral nerve palsy seen with excessive flexion during Pavlik bracing