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Developmental Dysplasia of the Hip

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Topic updated on 06/13/14 12:59pm
Introduction
  • A disorder of abnormal development resulting in dysplasia and possible subluxation or dislocation of the hip secondary to capsular laxity and mechanical factors
  • DDH encompasses a specturm 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
  • Epidemiology
    • incidence
      • most common orthopaedic disorder in newborns
      • dysplasia is 1:100
      • dislocation is 1:1000
    • location
      • most common in left hips in females
      • bilateral in 20%
    • demographics
      • more commonly seen in Native Americans and Laplanders
      • rarely seen in African Americans
    • risk factors
      • first born
      • female (6:1 over males)
      • breech
      • family history
      • oligohydramnios
  • Pathophysiology
    • initial instability thought to be caused by maternal and fetal laxity, genetic laxity, and intrauterine and postnatal malpositioning
    • pathoanatomy
      • initial instabiltiy leads to dysplasia
      • dysplasia leads to gradual dislocation
    • typical deficiency is anterior or anterolateral acetabulum
      • in spastic cerebral palsy, acetabular deficiency is posterior-superior q
  • Associated conditions
    • associated with "packaging" deformities which include
      • congenital muscular torticollis (20%)
      • metatarsus adductus (10%)
      • congenital knee dislocation
    • conditions characterized by increased amounts of type III collagen
Classification
  • Can be classified as a spectrum of disease involvement (phases)
    • dislocated
      • Ortolani-positive early when reducible; Ortolani-negative late when irreducible
    • dislocatable
      • Barlow-positive
    • subluxatable
      • Barlow-suggestive
Presentation
  • Physical exam (< 3 months)
    • mainstay of physical diagnosis is palpable hip subluxation/dislocation on exam 
      • Barlow 
        • dislocates a dislocatable hip by adduction and depression of the flexed femur
      • Ortolani  
        • reduces a dislocated hip by elevation and abduction of the flexed femur
      • Galeazzi (Allis)  
        • apparent limb length discrepancy due to a unilateral dislocated hip with hip and knee flexed at 90 degrees
        • femur appears shortened on dislocated side
    • hip clicks are nonspecific findings
    • Barlow and Ortolani a rarely positive after 3 months of age because of soft-tissue contractures about the hip
  • Physical exam (> 3 months)
    • limitations in hip abduction
      • most sensitive test once contractures have began to occur
    • occurs as laxity resolves and stiffness begins to occur
    • decreased symmetrically in bilateral dislocations
    • leg length discrepancy predominate
  • Physical exam (> 1 year - walking child)
    • pelvic obliquity
    • lumbar lordosis
      • in response to hip contractures resulting from bilateral dislocations in a child of walking age
    • Trendelenburg gait 
      • results from abductor insufficiency
    • toe walking
      • compensate for relative shortening of affected side
Imaging
  • Radiograph
    • become primary imaging modality at 4-6 mo after the femoral head begins to ossify
      • hip dislocation
        • Hilgenreiner's line   
          • horizontal line through right and left triradiate cartilage
          • femoral head ossification should be inferior to this line
        • Perkin's line  
          • line perpendicular line to Hilgenreiner's through a point at lateral margin of acetabulum
          • femoral head ossification should be medial to this line
        • Shenton's line  
          • arc along inferior border of femoral neck and superior margin of obturator foramen
          • arc line should be continuous
        • delayed ossification of the femoral head is seen in cases of dislocation
      • hip dyplasia
        • acetabular index (AI)  
          • angle formed by a line drawn from point on the lateral triradiate cartilage to point on lateral margin of acetabulum and Hilgenreiners line
          • should be less than 25° in patients older than 6 months
        • center-edge angle (CEA) of Wiberg 
          • angle formed by a vertical line from the center of the femoral head and a line from the center of the femoral head to the lateral edge of the acetabulum
          • less than 20° is considered abnormal
          • reliable only in patients over the age of 5 years
        • acetabular teardrop not typically present prior to hip reduction 
          • development of teardrop after reduction is thought to be good prognositc sign for hip function
  • Ultrasound
    • evaluates for acetabular dyspasia and/or the presence of a hip dislocation
      • useful before femoral head ossification (<4-6 mos)
      • may produce spurious results if performed before 4-6 weeks of age
    • allows view of bony acetabular anatomy, femoral head, labrum, ligamentum teres, hip capsule
      • alpha angle 
        • angle created by lines along the bony acetabulum and the ilium
        • normal is greater than 60°
      • beta angle
        • angle created by lines along the labrum and the ilium
        • normal is less than 55°
      • femoral head is normally bisected by a line drawn down from the ilium
    • used as an adjunct only in patients at 3-4 weeks in patients who are considered high risk (family history and/or have an equivocal physical exam)
    • normal ultrasound in patients with soft-tissue 'clicks' will have normal acetabular development
    • allows for monitoring of reduction during Pavlik harness treatment
    • is not cost effective for routine screening
  • Arthrogram  q 
    • used to confirm reduction after closed reduction under anesthesia
    • help identify possible blocks to reduction
      • inverted labrum
        • labrum enhances the depth of the acetabulum by 20% to 50% and contributes
          to the growth of the acetabular rim
        • in the older infant with DDH the labrum may be inverted and may mechanically block concentric reduction of the hip
      • inverted limbus
        • represents a pathologic response of the acetabulum to abnormal pressures caused by superior migration of the head
        • consists of fibrous tissue
      • transverse acetabular ligament
      • hip capsule is constricted by iliopsoas tendon causing hour-glass deformity of the capsule  
      • pulvinar
      • ligamentum teres
  • CT
    • CT study of choice to evaluate reduction after closed reduction and spica casting
  • MRI
    • does not play significant role in primary diagnosis
Screening
  • All infants require screening
    • Physical exam
      • succesful 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
Treatment in Children
  • Nonoperative
    • 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 brace treatment if not successful after 3-4 weeks 
    • closed reduction and spica casting 
      • indications
        • DDH in 6 - 18 months of age
        • failure of Pavlik treatment
  • Operative
    • open reduction and spica casting
      • indications
        • DDH in patient >18 months of age
        • failure of closed reduction 
    • open reducion 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
    • 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
Techniques
  • Abduction splinting/bracing (Pavlik harness)
    • goals
      • treatment is based on early concentric reduction in order to prevent future degeneration of the hip
      • risk, complexity and complications are increased with delays in diagnosis
    • position in bracing
      • goal is 90-100° flexion (controled by anterior straps) and abduction of 50° (controled by posterior straps)
    • extreme positions can cause
      • AVN due to impingement of the posterosuperior retinacular branch of the medial femoral circumflex artery
        • seen with extreme abduction (> 60°)
        • placement of abduction within 'safe zone'
      • transient femoral nerve palsy
        • seen with hyperflexion
    • discontinue if hip is not reduced by 3-4 weeks to prevent Pavlik disease
      • erosion of the pelvis superior to the acetabulum and prevention of the development of the posterior wall of the acetabulum
    • worn for 23 hours/day for at least 6 weeks or until hip is stable
      • wean out of harness over 6-8 weeks after hip has stabilized until normal anatomy develops
    • confirm position with ultrasound or xray and monitor every 4-6 week
  • Closed reduction and spica casting
    • performed under general anesthesia
      • excessive force can result in AVN
    • arthrogram used to confirm reduction
      • concentric reduction must be obtained with less than 5mm of contrast pooling medial to femoral head and the limbus must not be interposed
      • the arthrogram will also help identify anatomic blocks to reduction:
    • spica casting
      • following reduction immobilize in a spica cast with hip flexion of 100 deg. and abduction of 45 deg with neutral rotation for 3 months
        • 'human position'
        • change cast at 6 weeks
      • adductor tenotomy performed if patient has an unstable safe zone
        • used if excessive abduction required to maintain the reduction
      • confirm reduction with CT scan in spica cast with selective cuts to minimize radiation to the child
  • Open reduction
    • anterior approach (Smith-Peterson) most common to decrease risk to medial femoral circumflex artery 
      • capsulorrhaphy can be performed after reduction
      • used if patient is older than 12 months
    • other possible approaches include
      • medial adductor appoach, variation of of Ludloff
        • Pros
          • directly addresses block to reduction
          • can be used in patients under 12 months of age
          • less blood loss
        • Cons
          • unable to perform a capsulorrhaphy
          • higher association of AVN
      • anteromedial approach 
      • posteromedial approach
    • remove possible anatomic blocks to reduction
      • iliopsoas contracture, capsular constriction, inverted labrum, pulvinar, hypertrophied ligamentum teres
    • adductor tenotomy performed if patient has an unstable safe zone
      • if excessive abduction required to maintain the reduction
    • immobolize in functional position of 15° of flexion, 15° of abduction and neutral rotation
  • Femoral Osteotomy 
    • 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
  • Pelvic Osteotomies 
    • increase anterior or anterolateral coverage
    • used after reduction is confirmed on abduction-internal rotation views and satisfactory ROM has been obtained

Reconstructive Pelvic Osteotomies
 q
Indication
Technique
 
Salter

Younger patients typically with open triradiate cartilage

Single cut above acetabulum through the ilium to sciatic notch. Acetabulum hinges through the pubic symphysis. The redirectional osteotomy can provide 20-25° lateral and 10-15° anterior coverage (coverage limitations in anterolateral head). May lengthen leg up to 1 cm.


Triple(Steele)

Favored in older children because their symphysis pubis does not rotate well. Performed when open triradiate cartilages are present

Salter osteotomy plus additional cuts through superior and inferior pubic rami. Acetabular reorientation procedure.

PAO (Ganz) 

Triradiate cartilage must be closed in order to perform 

Involves multipe osteotomies in the pubis, ilium, and ischium near the acetabulum. This allows improved three-dimentional correction of the acetabulum configuartion. It is technically the most challenging. Posterior column and pelvic ring remain intact and pateints are allowed to weight bear early  

  
Pemberton For moderate to severe DDH; most versatile; triradiate cartilage must be open

Osteotomy starts approximately 10 to 15 mm above the AIIS, procedes posteriorly, and ends at the level of the ilioischial limb of the triradiate cartilage (halfway between the sciatic notch and the posterior acetabular rim). Osteotomy hinges at the triradiate cartilage posteriorly and the symphysis pubis anteriorly.  This osteotomy does do not enter sciatic notch and is therefore stable and does not need internal fixation. Reduces acetabular volume

Dega Favored in neuromuscular dislocations (CP) and patients with posterior acetabular deficiency; for severe cases

Osteotomy from acetabular roof to triradiate cartilage (incomplete cuts through pericapsular portion of the innominate bone). The acetabular configuration changes by hinging through the triradiate cartilage. This osteotomy does do not enter sciatic notch and is therefore stable and does not need internal fixation. Reduces the acetabular volume

 
Dial Technically difficult and rarely used

The dial or spherical osteotomy leaves the medial wall or teardrop in its original position and, as a result, is intra-articular. 

 
Salvage pelvic osteotomies
Shelf Salvage procedure performed in patients older 8yr

Add bone to the lateral weight bearing aspect of acetabulum by placing an extra-articular buttress of bone over the subluxed femoral head. Depends on fibrocartilge metaplasia for successful results.

Chiari Salvage procedure for pateints with inadequate femoral head coverage and  when a concentric reduction can not be obtained

Make cut above acetabulum to sciatic notch and shift ileum lateral beyond edge of acetabulum. Depends on fibrocartilge metaplasia for successful results. Medializes the acetabulum via iliac osteotomy.

  
 
 
Complications
  • 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 

 

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Qbank (12 Questions)

TAG
(SBQ07.100) Figures A-E show a series of radiographic lines used in the assessment of a paediatric hip joint. Which of the following figures shows Perkin's line? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶
TAG
(OBQ11.142) Which of the following is true regarding the structure outlined in Figure A? Topic Review Topic
FIGURES: A          

1. It is comprised of the iliopectineal eminence and quadrilateral surface
2. In normal hips, all children usually have this radiographic figure by 18 months of age
3. This figure is usually present in children with developmental dysplasia of the hip prior to reduction
4. The structure is a result of the radiographic superimposition of the ilioischial and Iliopectineal lines
5. It is comprised of the cotyloid fossa and iliopectineal eminence

PREFERRED RESPONSE ▶
TAG
(OBQ11.187) A 6-week-old female infant is referred to your practice for concerns of developmental dysplasia of the hip. On physical exam, you note a positive Ortolani test on the left side. Pavlik harness treatment is initiated. Which of the following imaging modalities should be utilized at the two week follow-up visit? Topic Review Topic

1. Magnetic Resonance Imaging (MRI)
2. Computed Tomography (CT)
3. Ultrasound (US)
4. Plain Radiographs
5. Arthrogram and Dynamic Fluroscopy

PREFERRED RESPONSE ▶
TAG
(OBQ11.235) Which of the following best describes the radiographic measurement labeled #1 on Figure A. Topic Review Topic
FIGURES: A          

1. Tonnis line
2. Galeazzi's line
3. Hilgenreiner's line
4. Shenton's line
5. Perkin's line

PREFERRED RESPONSE ▶
TAG
(OBQ11.249) A 15-year-old soccer player complains of bilateral hip pain. The pain is worse with activity and she notices that she has fatigue and pain that extends to the thighs and knees following a soccer match. She is nontender at the pubis symphysis and has no pain with resisted abdominal crunches. She has no pain with adduction of the hip. Hip flexion and rotation is normal. A radiograph of the right hip is shown in Figure A. Which of the following surgical interventions is best indicated? Topic Review Topic
FIGURES: A          

1. Single innominate osteotomy (Salter)
2. Double innominate osteotomy
3. Peri-acetabular osteotomy (Ganz)
4. Triple innominate osteotomy (Steele)
5. Dega osteotomy

PREFERRED RESPONSE ▶
TAG
(OBQ10.86) Which of the following concepts regarding pediatric hips is true? Topic Review Topic

1. The proximal femoral physis and greater trochanteric apophysis develop from different cartilaginous physes
2. The proximal femoral physis grows at a rate of 9 mm per year
3. Normal infant femoral anteversion is between 10-20 degrees
4. The ossific nucleus of the proximal femur is visable on radiographs by 6 months of age in most children
5. Slipped capital femoral epiphysis (SCFE) typically occurs through the zone of proliferation

PREFERRED RESPONSE ▶
TAG
(OBQ09.87) Failure to achieve reduction of a dislocated hip in an otherwise healthy 4 month old infant which did not reduce after 3 weeks in a Pavlik harness and 3 weeks in an abduction brace is best treated with which of the following? Topic Review Topic

1. Adjusting the harness to 75 degrees of abduction and maintaing 90 degrees of hip flexion
2. Adjusting the harness to 75 degrees of abduction and increasing hip flexion to 120 degrees
3. Closed reduction with hip arthrogram, adductor tenotomy if necessary, and hip spica casting
4. Open reduction and femoral shortening osteotomy
5. Open reduction, femoral shortening osteotomy, and pelvic acetabular osteotomy

PREFERRED RESPONSE ▶
TAG
(OBQ08.42) A 2-week-old infant girl is referred for a hip clunk noticed by the pediatrician. She was the product of a normal spontaneous vaginal delivery and is otherwise healthy. Examination demonstrates a right hip Ortolani sign. A coronal ultrasound is shown in figure A. What is the most appropriate next step in treatment? Topic Review Topic
FIGURES: A          

1. Observation with repeat ultrasound in 1 month
2. Pavlik harness application
3. Closed reduction and spica casting
4. Open reduction and spica casting
5. Open reduction, acetabular osteotomy, femoral shortening, and spica casting

PREFERRED RESPONSE ▶
TAG
(OBQ08.150) In patients older than 12-months of age with developmental dysplasia of the hip, all of the following physical exam findings are likely present EXCEPT? Topic Review Topic

1. Limited hip abduction
2. Positive Ortolani maneuver
3. Abnormal leg lengths on Galeazzi testing
4. Trendelenburg gait
5. Pelvic obliquity

PREFERRED RESPONSE ▶
TAG
(OBQ05.83) In infants with developmental dysplasia of the hip (DDH), anatomic closed reduction may be prevented by all of the following anatomic structure EXCEPT. Topic Review Topic

1. Interposition of gluteus medius
2. Limbus formed by fibrous tissue and hyaline cartilage
3. Ligamentum teres and prominent fibrofatty pulvinar tissue
4. Contracted transverse acetabular ligament
5. Inverted acetabular labrum

PREFERRED RESPONSE ▶
TAG
(OBQ04.175) A five-year-old boy with cerebral palsy presents to the clinic with a dislocated right hip, what quadrant of the acetabulum is most likely deficient? Topic Review Topic

1. Anterior-inferior
2. Anterior-superior
3. Posterior-superior
4. Posterior-inferior
5. Anterior-inferior and anterior-superior

PREFERRED RESPONSE ▶
TAG
(OBQ04.215) Figure A depicts an ultrasound of a newborn infant's hip. Which of the following structures (1 through 5) represents the labrum? Topic Review Topic
FIGURES: A          

1. 1
2. 2
3. 3
4. 4
5. 5

PREFERRED RESPONSE ▶
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