Developmental dysplasia of hip: etiology, pathogenesis, investigation, treatment

1. Developmental dysplasia of hip
Presenter: Dr souvik paul,
Dr Prince raina
Moderator: Prof. Dr. Shobha S Arora

2. Normal Development
• Embryonic
– 7th week - acetabulum and hip formed from same
mesenchymal cells
– 11th week - complete separation bet two
– Prox femur ossific nucleus - 4-7 months

3. Incidence
 Incidence per 1000 live births :0.06 in Africans
76.1 in Native Americans
0.47–9.2 # INDIANS
• Loder RT et al: higher centre edge angle in asians than white
race
• Clinical finding (2.3/100 births)
• Ultrasound abnormality (8/100 births)
# Singh M et al.Ind J Pediatr.2006;.
Gupta A.K.et al. Nat Med J India. 2002;

4. Etiology
• Multifactorial
– Genetics and Syndromes
• Ehler’s Danlos
• Arthrogryposis
• Larsen’s syndrome
– Intrauterine environmental factors
• Teratogens
• Positioning (oligohydramnios)
– Neurologic Disorders
• Spina Bifida

5. Pathoanatomy
• Ranges from mild dysplasia --> frank dislocation
• Bony changes
– Shallow acetabulum with superolateral slope to roof
– Degenerative changes lateral roof -subluxation
– Osteophytes and cysts along acetabular rim- labral tears
– Femoral anteversion & valgus neck shaft angle

6. Pathoanatomy
Intraarticular
– Inverted Labrum
– Hypertrophied & lengthened Lig. teres
– Pulvinar fat thickening
– Contracted transverse acetabular ligament
– Neolimbus
• Extraarticular
– Tight adductors, iliopsoas

7. Genetics in DDH
Susceptiblity genes subjects authors
GDFs (growth ⁄ differentiate
factor 5)
338 case and 620 control Dai et al.
TBX4 (T-box 4) 505 case subjects and 551 control Wang et al.
ASPN (Asporin) 370 case and 445 control Shi et al.
IL-6 & TGF-b1 28 case and 20 control Kolundzic et al.
PAPPA2( pregnancy-associated
plasma protein-A2)
310 case and 487 control Jia et al.
Li et al. : DDH locus in chromosome 17q21.31-17q22
Wei Tian et al. association study: positive association between HOXD9 gene
and DDH.

8. Diagnosis
• Family history, sibling history of DDH
• Birth history(breech delivery)
• History of birth asphyxia, fever, NICU admission history to
exclude other possibilities

9. Newborn screening
A. New born- routine examination:
– Warm, quiet environment with removal of diaper
– Ortolani’s and Barlow’s maneuvers with a thorough history and
physical
– Head to toe exam to detect any associated conditons (Torticollis,
Ligamentous Laxity etc.)
– Baseline Neuro and Spine Exam

10. Presenting symptoms at various ages
B. Infant-
1) Limited abduction
2) Galleazi sign
3) Proximal location of GT
4) Asymmetry of thigh folds
5) Pistoning of hip
6) Klisic test(B/L DDH)

11. • C. Toddler- Limp detected for the first time, unilat/bilat
• D. Older child- school going age
Limp
Incresed lumber lordosis
Peritrochanteric ( abductor fatigue) &Groin pain
Trendelenburg gait
Galeazzi's sign

12. Specific Clinical signs
• Telescopy: Gross telescopy, hyper mobile hip, moves in all
directions- Tomsmith hip
• Telescopy- moderately positive- ? DDH

13. Differential diagnosis
• Neonatal septic arthritis- infants and newborn
• Tb hip- walking children
• Untreated posterior dislocation hip
• Paralytic dislocation of hip- MMC, spastic CP
• Tomsmith hip

14. Choi classification of Tomsmiths’ hip

15. Neglected posterior dislocation hip
• Symp: pain, deformity, and limp
• O/E: Limb -flexion, adduction and internal rotation
Kumar S, Jain AK. Clin Orthop Relat Res.2005 Feb

16. Imaging
• X-rays
– Femoral head ossification center
• 4 -7 months
• Ultrasound
– Operator dependent, safe
• CT
• MRI
• Arthrograms

18. Radiological findings in older children
• Smaller or absent proximal femoral epiphysis
( equal size- ? Traumatic)
• Hypoplastic/ deformed proximal femur- Tomsmith choi type 1

19. Severin Classification

20. USG
• Sensitive and without radiation exposure
• Intersection of roofline and baseline forms the alpha angle.
• Intersection of the inclination line and baseline forms the beta
angle.

21. Graf classification
Class Alpha Angle Beta Angle Description Treatment
I > 60° < 55° Normal None
IIa 50°–60° 55°–77° Immature (<3
mo)
Observation
IIb >50°–60° 55°–77° >3 mo Pavlik harness
IIc 43°–49° >77° Acetabular
deficiency
Pavlik harness
IId 43°–49° >77° Everted labrum Pavlik harness
III <43° >77° Everted labrum Pavlik harness
IV Unmeasurable Dislocated Pavlik
harness/closed
vs. open
reduction

22. MRI
• Mao C et al. (Acta Radiol. 2016 Jun) :Compared with 3D CT, MRI is
more safe, precise, reliable and reproducible
• Fukiage K et al. (J Pediatr Orthop B.2015 Jul) found Femoral head
volume in 3D MRI :indicates severity of DDH
• Fukuda A et al. (J Child Orthop. 2016 Jun) Used ultrafast MRI to
diagnose DDH without sedation.
• E. G. MCNALLY et al. (J Bone Joint Surg 2007) MRI accurately
depicted acetabular anatomy and confirmed reduction in 12
patients.

23. Treatment Options
• Age of patient at presentation
• Family factors
• Reducibility of hip
• Stability after reduction
• Amount of acetabular dysplasia

26. Birth to Six Months
• Triple-diaper technique
– Prevents hip adduction
• Pavilk harness (1944)
– Very successful
– Allows free movement within
confines of restraints

27. Birth to Six Months
• Pavlik harness
– Indications
• Fully reducible hip
• Child not attempting to stand
• Close regular follow-up (every 1-2 weeks)
• For imaging and adjustments
• Duration
• Childs age at hip stability + 3 months

28. Pavlik Harness
• Failures
– Poor parent compliance
– Improper use by the physician
• Inadequate initial reduction
• Failure to recognize persistent dislocation
Treated with CR f/b hip spica after 3 weeks of Pavlik trial

29. Pavlik Harness
• Complications
– Avascular necrosis
• Forced hip abduction
Safe zone (Ramsey pl et al. JBJS Am 1976)
– Femoral nerve palsy
• Hyperflexion
25 to 30 degrees from
maximum abduction
50-90 degrees of flexion

30. Ucar d et al.
Journal of pediatric orthop.:march 2004
• prospectively studied results of pavlik harness f/b abduction
brace in patients of Graf type 2c/ severe hips.
• 22 hips :mean age 14.8(6-26) weeks
• follow up : 24.2(10-45) months.
• 90 % hip: reduced
• AVN :2 hips

31. Open Reduction
• Antero -lateral
– Smith-peterson
– Sartorius / TFL
• Medial approach(can be used)
– Pectineus / adductor longus + brevis
– Cannot address simeoultaneous bony work

32. 6 months - 2 years
– Closed reduction +/- adductor tenotomy
– Spica in position of 100 degrees flexion and about 55
degrees abduction (3 months)
– Abduction Orthosis 4 wks full time/4 wks nighttime
– Open reduction (if closed fails)

33. 2 Years of Age and Older
 Present a more difficult problem
– Prolonged dislocation
– Contracted soft tissue
Open redcution
• Tight - femoral shortening
• Stable - +/- pelvic osteotomy

38. Femoral shortening procedures
Indication:
1. excessive pressure needed on femoral head in
reduction
2.when a dislocated hip is reduced in a child older than 2
years of age#
• # Schoenecker PL et al.J Bone Joint Surg Am 1984

39. Femoral Shortening and
Derotation Osteotomy
Combined with Open
Reduction of the Hip
Intertrochanteric Varus
Osteotomy and Internal
Fixation with a Blade
Plate

40. Pelvic Osteotomy
• To reduce point loading by increasing contact area,
• Relaxing the capsule and muscles about the hip,
• Improving moment arm of hip,
• Normalizing the forces of weight bearing

42. Types: 1. Volume changing
– Pemberton
• Hinges on triradiate
• Requires remodeling of “new” incongruity
• Provides more anterolateral coverage
– Dega’s
– San Diego

43. Pelvic Osteotomy
• Redirecting
– Salter
• Osteotomy thru sciatic notch
• Hinge thru pubic symphysis
– Triple innominate
– Ganz
– Dial

44. Salvage or Shelf procedures
• Chiari
– Requires capsular metaplasia
– Pain - main indication
– Treatment of chronic hip pain in adolescents

46. • Prospectively study
• 75 hips with late-diagnosed DDH
• Group 1: < 6 months ,37 hips
• Group 2: 6–11 months ,17 hips
• Group 3: 12months –3years,21 hips
• follow-up: 11 (6–18) years
• Procedure: 68 reduced by CR,
OR +-Salter innominate osteotomy :17 hips

47. • acetabular angle improved rapidly in the younger children
:group A
• femoral head continued to grow irrespective of age at
reduction and became normal in almost all cases.
• Salter’s innominate osteotomy : excellent result in cases with
increasing acetabular angle.
• all but 2 patients were asymptomatic.

48. Complications
Untreated:
• persistent limp on the affected side
• premature osteoarthritis
• lower back or hip pain
Treated :
• AVN
• Redislocation
• Residual Acetabular Dysplasia

50. Mean age of onset of sec OA: 34.5 yrs :dysplastic DDH, 32.5 yrs:low
dislocation, 40.2 yrs:high dislocation
Classification: Crowe, Hartofilakidis, Eftekhar #
Center of hip :center of triangle-ASIS, ischial tuberosity ,obturator foramen
Cup: close to the teardrop
Acetabular screws: posterosuperior quadrant
#Crowe et al.JBJS1980. Wasielewski Rcet al. Clin Orthop Relat Res. 2005

51. Redislocation
Risk factors:
1. Insufficient release of anteromedial capsule ,inferior articular str &
transverse acetabular ligament
1. Greater pubic width
2. Decreased abduction in spica cast.
3. Dysplasia of femoral head
4. Insufficiently corrected femoral version
Procedures :
• 1.transfer and tenodesis of the ligamentum teres #
• 2.percutaneous K-wire to stabilize hip after reduction. ##
# Wenger DR et al. J Child Orthop. 2008
## Castañeda P et al. J Pediatr Orthop. 2015

52. Sankar WNJ Pediatr Orthop. 2011 Apr-May
Risk factors for failure after open reduction for DDH:
a matched cohort analysis
• Retrospective match-controlled study
• Cohort 1:22 successful OR for DDH
• Cohort 2:22 revision OR after redislocation

53. • Radiographs compared :acetabular index, pelvic width, triradiate cartilage
width, height of dislocation, size of ossific nucleus, abduction angle in the
spica cast, Tönnis grade, and Severin grade.
• Cohort 2 :significantly larger pelvic width and lower abduction angle
(mean 39 degrees vs. 51 degrees in grp 1) (P=0.003 ).
• Reasons for failure: dysmorphic femoral head and abnormal femoral
version.

54. Summary
• Best if treated before 6 weeks of age
• 0 - 6 months of age
– Pavlik
• 6 - 18 months
– Closed vs open reduction and spica
• 18 - 48 months
– Closed
– Open +/- osteotomies
• Femoral shortening better than traction
• Pelvic osteotomies
– Dega, Pemberton
– Salter, triple innominate, Ganz
– Chiari

55. Thank you