Developmental Dysplasia of the Hip Dr.Abdulmonem Al Siddiky Assistant professor & Consultant Ped.Ortho.,Ped.Spine & Spinal deformities KKUH , KSU

Overview Introduction Normal Development of the Hip Etiology and Pathoanatomy Epidemiology and Diagnosis Treatment Complications

Introduction Developmental Dysplasia of the Hip DDH - preferred term Teratogenic hips Subluxation Dislocation-usually posterosuperior (reducible vs irreducible) Dysplasia

Summary Risk Factors 1/1,000 born with dislocated hip 10/10,000 born with subluxation or dysplasia 80% Female First born children Family history (6% one affected child, 12% one affected parent, 36% one child + one parent) Oligohydramnios Breech (sustained hamstring forces) Native Americans (swaddling cultures) Left 60% (left occiput ant), Right 20%, both 20% Torticollis or LE deformity

Normal Development Embryonic 7th week - acetabulum and hip formed from same mesenchymal cells 11th week - complete separation between the two Prox fem ossific nucleus - 4-7 months

Normal Hip Tight fit of head in acetabulum Transection of capsule Still difficult to dislocate Surface tension

Pathoanatomy Ranges from mild dysplasia --> frank dislocation Bony changes Shallow acetabulum Typically on acetabular side Femoral anteversion

Pathoanatomy Soft tissue changes Intraarticular Usually secondary to prolonged subluxation or dislocation Intraarticular Labrum Inverted + adherent to capsule (closed reduction with inverted labrum assoc with increased Avascular Necrosis) Ligamentum teres Hypertrophied + lengthened Pulvinar Fibrofatty tissue migrating into acetabulum

Pathoanatomy Soft Tissue (Intraarticular) Extraarticular Transverse acetabular ligament Contracted Limbus Fibrous tissue formed from capsular tissue interposed between everted labrum and acetabular rim Extraarticular Tight adductors (adductor longus) Iliopsoas

Tough Reductions… Obstacles to reduction Extraarticular Intraarticular Tight iliopsoas and adductors Intraarticular Labrum Ligamentum teres Transverse acetabular ligament Pulvinar Redundant capsule (hourglass) +/- limbus

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

Diagnosis Newborn screening Ortolani’s and Barlow’s maneuvers with a thorough history and physical Warm, quiet environment with removal of diaper Head to toe exam to detect any associated conditons (Torticollis, Ligamentous Laxity etc.) Baseline Neuro and Spine Exam

Diagnosis Key physical findings Asymmetry Limb length- Galeazzi Abduction ROM Skin folds Limp Waddilng gait / hyperlordosis - bilateral involvement

Ortolani’s Maneuver * After 3 months of age tests become negative

Barlow’s Maneuver

Diagnosis Some cases still missed At risk groups should be further screened AAP Recs further imaging (e.g. US) if exam is “inconclusive” AND First degree relative + female Breech Positive provocative maneuver (Ortolani or Barlow) Referral to Orthopaedist

Imaging X-rays Ultrasound CT MRI Arthrograms Femoral head ossification center 4 -7 months Ultrasound Operator dependent CT MRI Arthrograms Open vs closed reduction

Imaging Radiographs

Imaging Radiographs

Imaging Radiographs

Imaging Radiographs

Imaging Acetabular Index

Imaging Acetabular Index

Imaging Acetabular Index < 30 wnl

Imaging

Imaging

Imaging

Imaging

Radiographs Summary Femoral head appears 4 - 7 months Shenton’s line Perkin’s and Hilgenreiner’s lines Inferomedial quadrant Center Edge Angle (< 20 abnormal) Acetabular index Normal < 30 (Weintroub et al) Tear drop* Abnormal widening in DDH *may be only sign in mild subluxation

Imaging Ultrasound Introduced in 1978 for eval of DDH Operator dependent Useful in confirming subluxation, identifying dysplasia of cartilaginous acetabulum, documenting reducibility Prox Femoral Ossification Center interferes Requires a window in spica cast (avoid)

Ultrasound Femoral head Abductors Ilium

Ultrasound Femoral head Abductors Ilium

Ultrasound Femoral head Abductors Ilium

Ultrasound Femoral head Abductors Ilium

Ultrasound Graf’s alpha angle

Ultrasound Graf’s alpha angle >60 = normal *line w/ ilium bisects head 50/50

Natural History Newborn Variable > 6 months more aggressive tx required due to more extensive pathology and decreased potential for acetabular remodeling Abnormal Gait, Decreased Abduction and Strength, Increased DJD Unilateral worse than Bilateral Subluxation worse than Dysplasia

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

Birth to Six Months Triple-diaper technique Pavilk harness (1944) Prevents hip adduction “Success” no different in some untreated hips Pavilk harness (1944) Experienced staff* Very successful Allows free movement within confines of restraints *posterior straps for preventing add. NOT producing abd.

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

Pavlik Harness Failures Poor parent compliance Improper use by the physician Inadequate initial reduction Failure to recognize persistent dislocation Viere et al 1990 Bilateral dislocation Absent Ortolani’s sign > 7weeks of age

Pavlik Harness Complications Avascular necrosis Femoral nerve palsy Forced hip abduction Safe zone (abd/adduction and flexion/extension) Femoral nerve palsy Hyperflexion *Be aware of Pavlik Harness Disease *Follow until skeletal maturity

Birth - Six months Closed reduction + Spica Failure after 3 weeks of Pavlik trial

Birth - Six months Closed reduction General anesthesia Arthrogram Safe zone - avoid AVN +/- adductor tenotomy Open reduction if concentric reduction not possible Usually teratogenic hips in this age group

Open Reduction Medial approach Antero -lateral Pectineus / adductor longus + brevis Cannot address simeoultaneous bony work Antero -lateral Smith-peterson Sartorius / Tensor Fascia lata

Open Reduction

6 months - 4 years Present a more difficult problem 6 - 18 months Prolonged dislocation Contracted soft tissues 6 - 18 months Closed reduction +/- adductor tenotomy Spica in human position of 100 degrees of flexion and about 55 degrees abduction (3 months) Abduction Orthosis 4 wks full time/4 wks nighttime Open reduction (if closed fails) Capsulorraphy CT scan Spica for 6 wks followed by PT

6 months - 4 years 18 months - 4 years Closed reduction Open redcution Reducibile - check arthrogram and medial dye pool Irreducible - Open reduction Open redcution Tight - femoral shortening Stable - +/- pelvic osteotomy

Femoral Shortening Schoenecker + Strecker 1984 Traction vs. Femoral shortening 56% AVN in traction group 0% AVN in femoral shortening

Pelvic Osteotomy Persistent instability + dysplasia after open reduction + femoral shortening Requires concentric reduction of a reasonably spherical femoral head Usually based on surgeon preference Salter and Pemberton 2 m/c in US

Pelvic Osteotomy Volume changing Pemberton Dega’s Hinges on triradiate Requires remodeling of “new” incongruity Provides more anterolateral coverage Dega’s

Pemberton

Pelvic Osteotomy Redirecting Salter Triple innominate Ganz Dial Osteotomy thru sciatic notch Hinge thru pubic symphysis Triple innominate Ganz Dial

Pelvic Osteotomy Redirecting Salter Triple innominate Ganz Dial Osteotomy thru sciatic notch Hinge thru pubic symphysis Triple innominate Ganz Dial

Salter Osteotomy

Salter Osteotomy

Salter Osteotomy

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

Chiari Osteotomy

Chiari Osteotomy

Chiari Osteotomy

Chiari Osteotomy

Chiari Osteotomy

Avascular Necrosis

Avascular Necrosis Most common Not part of the natural history of DDH Iatrogenic Etiology unknown Femoral head compression Injury to blood supply Excessive abduction Sullivan et al 1997 Sig  blood flow w/ increasing abd angle

TX 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

Summary Femoral shortening better than traction Pelvic osteotomies Dega, Pemberton Salter, triple innominate, Ganz Chiari

Questions

Evaluation of a 4week infant who has a hip click reveals a positive Ortolani sign. Treatment should include: A. traction, closed reduction, and spica casting. B. triple diapers and reassessment in 1 month C. an AP pelvis x-ray at age 4 months D. Fitting of a Pavlik harness and reassessment in 2 weeks E. an US of the hip, fitting of a Pavlik, and reevaulation in 3 months.

Evaluation of a 4week infant who has a hip click reveals a positive Ortolani sign. Treatment should include: A. traction, closed reduction, and spica casting. B. triple diapers and reassessment in 1 month C. an AP pelvis x-ray at age 4 months D. Fitting of a Pavlik harness and reassessment in 2 weeks E. an US of the hip, fitting of a Pavlik, and reevaulation in 3 months.

A healthy 5-mo-old infant w/ DDH of the L hip has been treated in a Pavlik for 3 months. Exam shows limited ABD and a Galeazzi sign. Radiographs in harness were taken. Management should consist of: A. an arthrogram and closed reduction B. a change of the Pavlik to a Frejka pillow C. no further Rx until the child is 6mos D. Adjustment of the Pavlik and continuation of Rx E. open reduction through a medial approach and spica

A healthy 5-mo-old infant w/ DDH of the L hip has been treated in a Pavlik for 3 months. Exam shows limited ABD and a Galeazzi sign. Radiographs in harness were taken. Management should consist of: A. an arthrogram and closed reduction B. a change of the Pavlik to a Frejka pillow C. no further Rx until the child is 6mos D. Adjustment of the Pavlik and continuation of Rx E. open reduction through a medial approach and spica

A healthy 5-mo-old infant w/ DDH of the L hip has been treated in a Pavlik for 3 months. Exam shows limited ABD and a Galeazzi sign. Radiographs in harness were taken. Management should consist of: A. an arthrogram and closed reduction B. a change of the Pavlik to a Frejka pillow C. no further Rx until the child is 6mos D. Adjustment of the Pavlik and continuation of Rx E. open reduction through a medial approach and spica

This is an x-ray of a 9-month-old infant who has intoeing This is an x-ray of a 9-month-old infant who has intoeing. Exam of the hips show ABD of the Left hip to 75 degrees and the Right to 90 degrees. Both the Ortolani and Barlow signs are negative. Management should include: A. observation B. application of a Pavlik harness C. closed reduction of the Left hip D. open reductino of the Left hip E. open reduction of the left hip with innominate osteotomy

This is an x-ray of a 9-month-old infant who has intoeing This is an x-ray of a 9-month-old infant who has intoeing. Exam of the hips show ABD of the Left hip to 75 degrees and the Right to 90 degrees. Both the Ortolani and Barlow signs are negative. Management should include: A. observation B. application of a Pavlik harness C. closed reduction of the Left hip D. open reductino of the Left hip E. open reduction of the left hip with innominate osteotomy

This is an x-ray of a 9-month-old infant who has intoeing This is an x-ray of a 9-month-old infant who has intoeing. Exam of the hips show ABD of the Left hip to 75 degrees and the Right to 90 degrees. Both the Ortolani and Barlow signs are negative. Management should include: A. observation B. application of a Pavlik harness C. closed reduction of the Left hip D. open reductino of the Left hip E. open reduction of the left hip with innominate osteotomy

The x-ray shows a AP pelvis of a 6yo girl who presents with a limp and intermittent pain in the right groin. Management should include: A. A varus derotational osteotomy of the right femur B. open reduction and adductor tenotomy C. open reduction with femoral and pelvic osteotomies D. PT for muscle strengthening and ROM exercises E. longitudinal traction, closed reduction, and adductor tenotomy

The x-ray shows a AP pelvis of a 6yo girl who presents with a limp and intermittent pain in the right groin. Management should include: A. A varus derotational osteotomy of the right femur B. open reduction and adductor tenotomy C. open reduction with femoral and pelvic osteotomies D. PT for muscle strengthening and ROM exercises E. longitudinal traction, closed reduction, and adductor tenotomy

The x-ray shows a AP pelvis of a 6yo girl who presents with a limp and intermittent pain in the right groin. Management should include: A. A varus derotational osteotomy of the right femur B. open reduction and adductor tenotomy C. open reduction with femoral and pelvic osteotomies D. PT for muscle strengthening and ROM exercises E. longitudinal traction, closed reduction, and adductor tenotomy

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