Orthopedics Book


Developmental Dysplasia of the Hip

Aka: Developmental Dysplasia of the Hip, Congenital Hip Dislocation, Hip Dislocation in the Newborn, Congenital Hip Dysplasia
  1. See Also
    1. Congenital Abduction Contracture of the Hip
    2. Teratologic Congenital Hip Dislocation
    3. Ortolani Test
    4. Barlow's Test
  2. Definitions
    1. Developmental Dysplasia of the Hip (DDH)
      1. Acetabulum or femoral head dysgenesis resulting in hip incongruity in infants
      2. Spectrum from hip laxity and instability to hip subluxation and dislocations
  3. Epidemiology
    1. Incidence
      1. Hip instability at birth: 0.1 to 0.3%
    2. Girls 9 times more often affected than boys
    3. Usually unilateral, but bilateral is common
  4. Risk Factors
    1. Breech Presentation (Odds Ratio 6)
      1. Unstable hips found in >10% of Breech infants
    2. Female gender (Odds Ratio 4.3)
    3. First born (Odds Ratio 2.7)
    4. Family History (positive in up to one third of cases)
      1. One affected sibling: 6% risk
      2. One affected parent: 12% risk
      3. One affected sibling and one affected parent: 36%
    5. Oligohydramnios
    6. Large for Gestational Age infant
  5. Pathophysiology
    1. Femoral head dislocates from acetabulum
    2. Results from Acetabular Dysplasia (shallow acetabulum)
      1. Results in subluxed, dislocated or unstable hip
    3. Left hip is affected in 60% of cases
      1. Remainder are right (20%) and bilateral (20%)
  6. Types
    1. Classic Congenital Hip Dislocation
    2. Congenital Abduction Contracture of the Hip
    3. Teratologic Congenital Hip Dislocation
      1. Severe, prenatal fixed dislocation
      2. Associated with genetic and neuromuscular disorders
  7. Associated Conditions
    1. Congenital Torticollis
    2. Breech Presentation in utero
    3. First degree relative with hip dysplasia history
    4. Clubfoot
    5. Metatarsus Adductus
    6. Torticollis
  8. Symptoms
    1. Painless limp in toddler (best if diagnosed earlier)
  9. Signs
    1. Dislocation and Relocation maneuvers
      1. Useful only in first few weeks to months of life
        1. Accuracy decreases as ligamentous laxity resolves
      2. Repeat exam in 2 weeks if equivocal results
      3. Tests
        1. Ortolani Test (relocate hip into acetabulum)
        2. Barlow's Test (attempt to sublux unstable hip)
    2. Pelvis symmetry
      1. Galeazzi's Sign (compare the 2 femur lengths)
      2. Observe for asymmetric skin folds
    3. Hip Range of Motion
      1. Abduction tested with hips flexed to 90 degrees
      2. Abnormal if abduction <60 degrees or less than opposite side by at least 20 degrees difference
  10. Imaging
    1. Dynamic Hip Ultrasound (infant aged 1-6 months)
      1. Diagnostic for Congenital Hip Dislocation
      2. Evaluates for subluxation and reducibility
      3. High False Positive Rate in age <6 weeks
        1. However, 90% Negative Predictive Value
      4. Grading based on Graf System
        1. Grade I: No abnormality
        2. Grade IV: Frank dislocation
    2. Hip XRay
      1. Not diagnostic for dislocation until >6 months
        1. Femoral head not calcified under age 4-6 months
        2. Diagnostic for Acetabular Dysplasia
          1. Abnormal acetabular fossa will be seen
      2. Evaluated with reference lines drawn over AP XRay
        1. Hilgenreiner's Line
          1. Horizontal line through triradiate cartilages
        2. Perkin's Line
          1. Vertical line along each lateral acetabulum
        3. Shenton's Line
          1. Femoral neck medial border
          2. Superior border of obturator foramen
  11. Evaluation: Exam Findings
    1. Hip click
      1. Palpable or audible, high-pitched click on Ortolani or Barlow Maneuver
      2. Hip clicks are caused by soft tissue movement and are not related to hip instability or dislocation
      3. Distinguish the benign hip click from the pathologic Hip Clunk (dislocation)
        1. Hip clicks are benign and require no further evaluation
        2. Hip Clunks are managed as Developmental Dysplasia of the Hip (see below)
    2. Hip Clunk
      1. Distinct, palpable/audible shift of the femoral head as it is relocated (Ortolani) or dislocated (Barlow)
      2. Hip Clunk suggests Developmental Dysplasia of the Hip, with dislocation or subluxation
    3. Hip instability or laxity
      1. May feel as a tennis ball might move within a cereal bowl without frankly dislocating (no Hip Clunk)
      2. Represents a loose fit of the femoral head with the acetabulum (without subluxation or dislocation)
      3. Relatively mild findings on the spectrum of Developmental Dysplasia of the Hip
    4. Limited Hip Range of Motion
      1. Reduced hip abduction (<60 degrees or more than 20 degrees difference between sides)
      2. Requires additional evaluation for Developmental Dysplasia of the Hip
    5. Hip subluxation
      1. Femoral head approaches the edge of the acetabulum but does not fully dislocate
      2. A soft clunk may be palpable on Ortolani or Barlow Maneuvers
    6. Hip Dislocation
      1. Femoral head completely escapes the acetabulum
      2. Most severe on the spectrum of Developmental Dysplasia of the Hip
  12. Evaluation: Increased Developmental Dysplasia of the Hip Risk
    1. Indications
      1. Breech Presentation OR
      2. Two or more DDH risk factors (Female gender, first-degree relative Family History)
    2. Approach
      1. Equivocal or positive exam results
        1. Refer to orthopedics
      2. Normal exam
        1. Consider Hip Ultrasound at age 4-6 weeks
        2. Repeat exam in 2 weeks and well child exam
        3. Refer to orthopedics for findings suggestive of DDH
  13. Evaluation: Standard Developmental Dysplasia of the Hip Risk
    1. Approach to significant findings (hip subluxation, Hip Dislocation or age over 6 months with findings)
      1. Referral to orthopedics
    2. Approach to mild instability or equivocal exam findings
      1. Repeat exam in 2 weeks
      2. Hip subluxation or dislocation
        1. Refer to orthopedics
      3. Persistent mild hip instability
        1. Obtain Hip Ultrasound or repeat exam every 2 weeks
  14. Management
    1. Management indicated for hip instability beyond 5 days of life
    2. Step 0: Observation
      1. Indicated only in mild instability for age <6 weeks
      2. Repeat examinations every 2 weeks for first 6 weeks of life
      3. Persistent instability or other DDH findings prompt orthopedic referral for Pavlik Harness
    3. Step 1: Pavlik Harness
      1. Indicated as first-line if age <6 months for frankly dislocated or dislocatable hips
      2. Start with harness trial for 3-4 weeks
      3. Splints hips in flexed and abducted position
      4. Long-term effectiveness: 95% (80% if frank dislocation)
      5. Ultrasound should demonstrate reduction at 3 weeks
        1. Reduced: Continue harness for >6 weeks
        2. Not Reduced: Go to Step 2
      6. Avascular necrosis risk: 0-14% overall, <2% for infants with early Splinting
    4. Step 2: Closed Reduction and Casting by Orthopedics
      1. Indications
        1. No reduction with Pavlik Harness in 3-4 weeks
        2. Children over age 6 months
      2. Attempted closed reduction under arthrogram
      3. Hip Spica Casting for 6 weeks
      4. Positioning confirmed by post-op MRI or CT
      5. Avascular necrosis risk similar to Pavlik Harness (0-14% overall, <2% for infants with early Splinting)
    5. Step 3: Surgical Open reduction
      1. Indicated in refractory cases
      2. Requires multi-step procedure
        1. Tendon lengthening
        2. Clearing tissues obstructing relocation
        3. Tightening hip capsule
        4. Osteotomy if performed after age 18 month
      3. Complications
        1. Re-disclocation
        2. Avascular necrosis (5-60% risk)
  15. Course
    1. Many unstable hips at birth stabilize by 5 days of life
  16. Prognosis
    1. Delayed treatment risks worse outcomes
    2. Monitor children with imaging until skeleton mature
  17. Complications
    1. Hip Osteonecrosis
    2. Premature Osteoarthritis of the hip as early as late teen
  18. Prevention
    1. Screening guidelines vary by organization (AAP, AAFP, USPTF)
      1. USPTF and AAFP found insufficient evidence for universal screening
    2. American Academy of Pediatrics (AAP) recommendations
      1. Screen all newborns with Ortolani Maneuver and Barlow Maneuver
      2. Repeat Hip Exam at routine visits for the first year of life
      3. High risk patients (e.g. Breech, Family History) should be screened with Hip Ultrasound
  19. References
    1. (2006) Pediatrics 117:898-902 [PubMed]
    2. Harding (1997) J Pediatr Orthop 17:1149-52 [PubMed]
    3. Jackson (2014) Am Fam Physician 90(12): 843-50 [PubMed]
    4. Storer (2006) Am Fam Physician 74(8):1310-6 [PubMed]
    5. Vitale (2001) J Am Acad Orthop Surg 9:401-11 [PubMed]

Hip Dislocation, Congenital (C0019555)

Definition (MSH) Congenital dislocation of the hip generally includes subluxation of the femoral head, acetabular dysplasia, and complete dislocation of the femoral head from the true acetabulum. This condition occurs in approximately 1 in 1000 live births and is more common in females than in males.
Concepts Congenital Abnormality (T019)
MSH D006618
ICD9 754.3
ICD10 Q65.2
SnomedCT 157001006, 268347007, 205061001, 205050009, 205337000, 52781008, 48334007
English Congenital Hip Dislocation, Congenital Hip Dislocations, Congenital Hip Dysplasia, Congenital Hip Dysplasias, Dislocation, Congenital Hip, Dislocations, Congenital Hip, Dysplasia, Congenital Hip, Dysplasias, Congenital Hip, Hip Dislocations, Congenital, Hip Dysplasia, Congenital, Hip Dysplasias, Congenital, CDH - Congen dislocation hip, Congen.dislocation hip NOS, Developmental displacement hip, Congenital dislocation of hip, unspecified, Congenital dysplasia of hip, Congenital dislocation of the hip, Congenital dislocation of the hips, Congenital hip dislocations, CDH, Hip Dislocation, Congenital, DISLOCATION CONGEN HIP, DYSPLASIA CONGEN HIP, HIP DYSPLASIA CONGEN, CONGEN HIP DYSPLASIA, CONGEN HIP DISLOCATION, HIP DISLOCATION CONGEN, congenital dislocation of hip, congenital dislocation of hip (diagnosis), developmental dysplasia of hip, developmental dysplasia of hip (diagnosis), developmental dislocation of pelvis, hip, or femur (diagnosis), developmental dislocation of pelvis, hip, or femur, Hip Dislocation, Congenital [Disease/Finding], congenital dislocation hip, developmental dislocation hip, developmental hip dislocation, congenital dysplasia hip, congenital hip dislocations, developmental hip displacement, Dislocation;hip;congenital, congenital hip dysplasia, congenital dysplasia of the hip, congenital hip dislocation, developmental dislocation hips, developmental dislocation of hip, congenital dislocation hips, hip congenital dislocation, acetabulum dysplasia, Hip, Dislocation Of, Congenital, Dislocation Of Hip, Congenital, Congenital Dysplasia Of The Hip, Hip Displacement, Congenital, Displacements, Congenital Hip, Displacement, Congenital Hip, Congenital Hip Displacement, Hip Displacements, Congenital, Congenital Hip Displacements, Disloc. hip - congen., Clicky hips, Congenital dislocation of hip NOS (disorder), Congenital dysplasia of the hip (disorder), Congen. disloc. hip, Congenital hip dislocation, Developmental hip dysplasia, Dislocation of hip congenital, Hip dysplasia, HIP DYSPLASIA, CONGENITAL, DDH, HIP DYSPLASIA, DEVELOPMENTAL, Congenital dislocation of hip, Congenital hip dysplasia, Dysplasia of acetabulum, CDH - Congenital dislocation of the hip, Developmental displacement of the hip, Congenital dysplasia of the hip, Congenital dislocation of hip (disorder), Congenital hip dysplasia (disorder), Developmental dislocation of hip, dislocation; hip, congenital, dysplasia; acetabulum, acetabulum; dysplasia, Congenital dislocation of hip, NOS, Congenital dislocation of hip NOS, Developmental dysplasia of the hip, congenital dislocation of the hip
Swedish Höftledsdislokation, medfödd
Czech kyčel - dysplazie kongenitální, kyčel - luxace kongenitální, Vrozená dislokace kyčle, Vývojová kyčelní dysplazie, Dislokace kyčle kongenitální, Kongenitální dislokace kyčle
Finnish Synnynnäinen lonkan sijoiltaanmeno
German Angeborene Luxation des Hueftgelenkes, nicht naeher bezeichnet, Entwicklungsbedingte Hueftdysplasie, kongenitale Hueftluxation, Hueftluxation, kongenital, Luxatio coxae congenita, Hüftdysplasie, kongenitale, Hüftluxation, kongenitale
Italian Displasia congenita dell'anca, Displasia evolutiva dell'anca, Lussazione congenita dell'anca
Korean 상세불명의 엉덩관절의 선천 탈구
Polish Zwichnięcie biodra wrodzone, Zwichnięcie stawu biodrowego wrodzone, Dysplazja stawu biodrowego wrodzona
Japanese 股関節脱臼-先天性, 股関節形成異常症-先天性, 発育性股関節形成不全, ハツイクセイコカンセツケイセイフゼン, センテンセイコカンセツダッキュウ, 先天性股関節形成異常症, 先天性股関節脱臼, 先天股脱, 先股脱
Norwegian Hofteleddsdysplasi, medfødt, Medfødt dislokasjon av hofte, Medfødt hofteleddsdysplasi, Luxatio coxae congenita
Spanish luxación congénita de cadera, SAI, luxación congénita de cadera, SAI (trastorno), Congenital dislocation of hip NOS, Luxación congénita de cadera, Displasia del desarrollo de la cadera, displasia congénita de la cadera (trastorno), displasia congénita de la cadera, displasia del acetábulo, luxación congénita de la cadera (trastorno), luxación congénita de la cadera, Displasia Congénita de la Cadera, Luxación Congénita de la Cadera
Dutch ontwikkelingsheupdysplasie, heupdislocatie, congenitaal, congenitale heupdislocatie, congenitale heupontwrichting, acetabulum; dysplasie, dysplasie; acetabulum, luxatie; heup, congenitaal, Congenitale heupluxatie, niet gespecificeerd, Congenitale heupdislocatie, Congenitale heupluxatie, Dislocatie, congenitale heup-, Heupdislocatie, Heupdislocatie, congenitale, Heupdysplasie, congenitale
French Dysplasie développementale de hanche, Luxation congénitale de hanche, Luxation de la hanche congénitale, Luxation congénitale de la hanche, Dysplasie luxante de la hanche, Maladie luxante de la hanche
Hungarian Veleszületett csípőízületi dislocatio, Congenitális csípőízületi dislocatio, A csípő fejlődési dysplasiája, Csípőízület veleszületett dislocatiója
Portuguese Luxação congénita da anca, Displasia da anca em desenvolvimento, Displasia Congênita de Quadril, Luxação Congênita de Quadril
Derived from the NIH UMLS (Unified Medical Language System)

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