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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences Developmental Dysplasia of the Hip: Beyond the Clinical Diagnosis Tanya L. Tivorsak, B.A., Kalpesh Patel, M.D., Kimberly Carney, M.D., Nina B. Klionsky, M.D., Gary B. Tebor, M.D., Johnny U.V. Monu, M.D. University of Rochester School of Medicine, Departments of Imaging Sciences and Orthopedics, Rochester, NY, United States Page 1 of 65

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Page 1: Developmental Dysplasia of the Hip: Beyond the … · Presentation material is for education purposes only. ... Developmental Dysplasia of the Hip: Beyond the ... oligohydramnios,

Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Developmental Dysplasia of the Hip: Beyond the

Clinical Diagnosis

Tanya L. Tivorsak, B.A., Kalpesh Patel, M.D., Kimberly Carney, M.D., Nina B. Klionsky, M.D., Gary B. Tebor, M.D., Johnny U.V. Monu, M.D.

University of Rochester School of Medicine, Departments of Imaging Sciences and Orthopedics,

Rochester, NY, United States Page 1 of 65

Page 2: Developmental Dysplasia of the Hip: Beyond the … · Presentation material is for education purposes only. ... Developmental Dysplasia of the Hip: Beyond the ... oligohydramnios,

Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Objectives

!! Review the spectrum of DDH !! Learn the current imaging criteria !! What the surgeon needs to know from the images !! Compare DDH versus hip dysplasia/dislocation in

congenital disorders !! Current management options

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Introduction

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Definition !! Formerly known as congenital dislocation of the hip joint

– but term changed since DDH is a developmental process and is not always detectable at birth.

!! The term dysplasia tends to be used for hips with a positive Ortolani sign (hip that can be dislocated or a dislocated hip that is able to be relocated).

!! The term dislocation tends to be used for hips with a negative Ortolani sign (unreducible hip).

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Wide Spectrum of Conditions

(1)! Primary dysplasia without instability

(2)! Instability (subluxable and dislocatable)

(3)! Subluxed

(4)! Dislocated

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Wide Spectrum of Conditions

!! Teratologic hip conditions are considered a different entity from DDH. They arise earlier in fetal development and are associated with other malformations.

!! However, there is conflicting literature in

whether teratologic hips are part of the DDH spectrum or not.

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Etiology

!! Multifactorial – genetic + intrauterine environmental factors

!! Usually unilateral (80% of the time), occurs

more frequently in the left hip – since the left hip of the fetus usually lies posteriorly against the mother s L-spine, limiting abduction

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Risk Factors

!! Native-Americans !! Family history

!! Females

!! Breech delivery !! Oligohydramnios

!! First born

!! Persistent hip asymmetry

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Embryology – Periods of Risk for Hip Dysplasia

After last menstrual period in fetal development: !! 12 weeks – lower limb rotates medially after hip joint arises at 7-11

wks – dysplasias are teratologic !! 18 weeks – hip muscle development – dysplasias due to

neuromuscular disorders !! Between perinatal period and 1st few weeks of birth – femoral head

grows faster than acetabulum, minimal coverage of head – dysplasias due to mechanical factors - oligohydramnios, breech position

!! Postnatal period – labral growth more rapid (more coverage of femoral

head) – dysplasias tend to be due to functional factors instead – increased estrogens causing ligamentous laxity, swaddling

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Natural History

!! Loss of tight fit between acetabulum and femoral head in the hip " may result in dysplasia or dislocation

!! Findings – shallow acetabulum with femoral

anteversion

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Natural History

Normal Hip – labrum is everted

Subluxed Hip – some inversion of labrum

Dislocated Hip – inversion of labrum, which becomes hypertrophied " called limbus, which may prevent

hip reduction

L

C

L = Labrum, C = Capsule

C

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Complications – If Untreated

!! Pain !! Early osteoarthritis !! Limb length discrepancy !! Decreased agility !! Abnormal gait/limping

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Clinical Diagnosis

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Clinical Diagnosis !! Ortolani s (reduction) and Barlow s (dislocation) maneuver

!! Shortened leg with limited abduction when flexed (6-8 wks of age) !! Asymmetry of thigh folds (rare) !! Galeazzi s sign (6-8 wks) - uneven knee levels when the supine

infant's feet are placed together on the exam table with the hips/knees flexed Ð usually seen in unilateral DDH.

Barlow Ortolani

Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences Pics from www.zadeh.co.uk Page 14 of 65

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Clinical Diagnosis

!! If hip dysplasia or dislocation is suspected, the patient should be examined to rule out any underlying medical or neuromuscular disorder.

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Recommendations for screening

Abnormal exam Refer to orthopedist

Inconclusive exam (i.e. hip click that seems benign/uncertain)

Do follow-up exam in 2 wks – if the exam is positive or still inconclusive, refer to orthopedist and recommend US

Negative exam + risk factors

Recommend imaging at 4-6 wks

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Imaging in DDH

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Imaging Modalities in DDH

!! Ultrasound – for screening, useful in neonatal period !! Radiographs – useful after the femoral head starts

to ossify (3-4 months of age)

!! MRI – evaluation of difficult cases and complications of DDH

!! CT – most commonly used post-treatment to view

reduction

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Ultrasonography

!! Dynamic Standard Minimum Examination – 1. Static (Graf method) assessment in coronal plane with the hip at rest, based on shape and depth of acetabulum by morphology and angular measurements 2. Dynamic (Harcke method) assessment in transverse plane with the hip under stress

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Static

Normal

ilium Femoral head

labrum Coronal view – hip at rest

To get this view – the transducer is placed in coronal orientation over the lateral aspect of the hip, with the infant supine or in the lateral decubitus position.

S

L

S = superior L = lateral

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Normal Graf Measurements

•! ! angle (normal = > 60°) indicates angle of bony acetabulum.

•! " angle (normal = < 55°),

indicates angle of cartilaginous femoral head coverage.

•! Femoral head coverage

by acetabulum – normal radio of d/D is > 50%.

!

"

Normal

D d

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Dynamic

Axial/transverse flex stress view on US: Femoral head ossification visualized (open arrow) with ischium (thin arrow). Compare anatomy with CT (similar positioning). This hip was nonsubluxable. If the hip subluxes posteriorly on this view, it is abnormal.

Normal

To get this view: Place U/S transducer over femoral head transverse to pelvis, flex hip, and exert posterior stress on knee.

Triradiate cartilage

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Graf vs Harcke Classification

!! Harcke's methods are more equivocal for evaluation since they depend on hip stability tests – many DDH cases may not yield positive results in Harcke s test (i.e. not subluxable).

!! Graf's classification gives a better indication

of the normal and dysplastic conditions.

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U/S Graf classification** Class Features Treatment I - mature Good bony modeling, ! angle > 60° None

IIa(+) -physiologic immaturity

Satisfactory bony modeling, ! angle = 50°- 59° Follow-up, no Tx

IIa(-) – maturational deficit < 3 mo old

•! ! angle = 50°- 59°, " Angle < 55° •! Deficient bony modeling •! Cartilaginous acetabular roof is still broad and covers femoral head

Pavilk harness or if borderline - just follow-up

IIb – delayed osseous development > 3 mo

-------------------Same as IIa(-)--------------------------- Pavlik harness

IIc – critical zone hip IId – decentering hip

•! ! angle 43°-49°, " Angle < 77° in IIc, > 77° in IId •! Deficient/highly deficient bony modeling •! Cartilaginous acetabular roof is still broad

Pavlik harness

IIIa, IIIb, IV – Eccentric hip

•! ! angle < 43°, " Angle > 77° •! Poor bony modeling, flattened bony promontory •! Displaced cartilage roof triangle

Pavlik ( > 95% successful in IIIa/b, 50% successful in IV), possible reduction

(Adapted from Graf, 1987)

**Classification does not take into account the position of the femoral head.

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"

! 50°-60°

> 55°

Graf IIa – 3 week old female with FHx of DDH

Femoral head coverage by acetabulum ~ 50%

IIa – Physiologic Immaturity Page 25 of 65

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Graf IIc – 3 week old with hip click

!

"

< 50% coverage of femoral head by acetabulum

41° - 46°

61° - 65°

Lateral and superior displacement of femoral head

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

Graf III: 2 day-old female with high risk FHx (mother, sister with severe hip dysplasia) and negative Ortolani and Barlow s tests (dislocated hip unable to be reduced)

Pulvinar (P) = fibrofatty tissue between acetabulum and femoral head, more evident in DDH due to femoral head not pressing against it in the acetabulum.

!

pulvinar

"

37°-49°

68°-80°

30% coverage of left femoral head by acetabulum

Cartilaginous acetabular roof superiorly displaced

P

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Pelvic Radiographs !! Views - supine frontal or standing frontal (older children) USEFUL FOR: !! After 3-6 mo of age – femoral head ossification visible !! Evaluating abnormalities of lower L-spine, sacrum, proximal femur !! Assessment of hip flexion in Pavlik harness placement in infants

!! Maintenance of reduction

!! Track resolution of acetabular dysplasia

!! Monitor for AVN Page 28 of 65

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Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences

1.! Acetabular Angle (useful for assessment of resolution)

2.! Femoral head position with reference to Hilgenreiner s and

Perkin s (H and P) lines

3.! Shenton s arc

4.! Center-edge angle (Wiberg and Ogata)

Reliable Radiographic Parameters

These parameters are useful to the orthopedic surgeon in evaluating DDH.

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Acetabular Angle !! Angle between Hilgenreiner s line (line through triradiate

cartilages) and line through the superior acetabular roof

20°° 19°° Hilgenreiner s line

Normal pelvis radiograph in 10 mo old male

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Acetabular Angle – Normal Values

!! < Age 2: 17°- 30° !! > Age 2: 18° ± 4° !! Angle decreases with age due to modeling of

the acetabulum by the femoral head and/or bone maturation along the acetabular roof.

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H line

Femoral Head Position and Shenton s Arc

P line (Perkins) Vertical, tangent to lateral rim of acetabulum, perpendicular to H line

1)! Normal ossified capital femoral epiphysis in lower inner quadrant (H- and P-lines)

2)! Shenton s arc

Normal Hip Radiograph Presentation material is for education purposes only. All rights reserved. ©2006 URMC Imaging Sciences Page 32 of 65

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Femoral head center

Center-Edge (CE) Angle of Wiberg

Line parallel to longitudinal body axis

Line to most lateral point of acetabular roof

Quantitates lateral coverage of femoral head by the acetabulum

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Center-edge (CE) angle of Wiberg

#!Normal values: > 19° in 5-8 years > 25° in 9-12 years > 26°- 30° in 13-20 years #!More reliable in children over age 5 since

femoral head center is difficult to define in children under age 5

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Refined CE Angle of Ogata

Image modified from Omeroglu et al., 2002

Femoral head center

Ogata Wiberg

Lateral pt of bony condensation

Similar to CE angle of Wiberg except the lateral line is tangent to the lateral point of bony condensation

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Femoral head center

Line for CE angle of Ogata = - 5°- 0°

Line for CE angle of Wiberg = 10°

Wiberg Ogata

DDH

Comparison of CE Angles in DDH vs. Normal Hip

Femoral head center

Lateral pt of bony condensation

Normal Hip

Wiberg and Ogata

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Refined CE Angle of Ogata Problems with CE angle of Wiberg: #! May overestimate lateral femoral head coverage in severe

cases. #! Subchondral bony condensation in acetabular roof represents

magnitude of compressive stresses, not lateral point of acetabulum.

#! A poor acetabular cover has shown to be present in hips with a

normal CE angle of Wiberg but in which the lateral point of bony condensation is short of the lateral rim of acetabular roof.

#! Refined CE angle of Ogata addresses these problems.

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Radiograph: Late-diagnosed DDH in a 7 year-old female from Mexico

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Radiograph: Late-diagnosed DDH in a 7 year-old female from Mexico

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Arthrogram – 6 month-old female with left DDH

Left hip: Medial pooling of contrast in the joint space. The femoral head is

subluxed superiorlaterally.

Left hip: Femur appears laterally displaced.

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CT !! Most commonly used to document reduction if child is

placed in spica cast. !! Can be performed preoperatively in the older child in

severe cases to help the surgeon in planning treatment procedures.

!! Protocol = 0.75 – 1.5 mm collimation and 0.5 – 1.0 mm

reconstructions, scan only through the hip joint. !! 3-D reconstruction for better hip analysis in complex

cases and for preoperative planning.

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CT reconstruction: Right hip dysplasia s/p varus osteotomy of proximal femur shaft

Flattened femoral head that is laterally subluxed, dysplastic and shallow acetabulum.

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MRI

!! Evaluation of difficult cases – if acetabular formation is inconclusive and subluxation still remains after conservative treatment.

!! Evaluation of newborns with cartilaginous

femoral heads (not ossified yet) in difficult cases.

!! Evaluate treatment complications (i.e. AVN).

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11 month old male with DDH

Axial Gradient Echo: Left hip: High-riding femoral head (1) abuts posterior lip of acetabulum

(arrow) and (2) is rotated and displaced posteriorlaterally. Right hip: Femoral head rotated and displaced anteriorlaterally.

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11 month-old male with DDH

T2 FSE Coronal Left hip: Femoral head abutting acetabulum (thin arrow), with deformed acetabular fossa (open arrow).

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Follow-up of 10 month old male with left hip dislocation, s/p closed reduction with spica cast

Coronal T2 Fat Sat Left Hip: Femoral head (thin arrow) located anteriormedially in shallow acetabulum (open arrow).

femur

ilium

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11 month old with DDH

Small dysplastic femoral head is posteriorly subluxed (arrow). The acetabulum is shallow and dysplastic (open arrow).

Coronal T2 Fat Sat Right Hip Sagittal T1 Right Hip

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Hip Dysplasia/Dislocation in

Congenital Disorders

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Hip Dysplasia/Dislocation in Congenital Disorders

!! Several congenital disorders may prompt further evaluation of the hips beyond the routine clinical exam for early diagnosis and management of hip dysplasia.

!! Hip dysplasia/dislocation present in congenital

disorders tend to be teratologic (not in the spectrum of DDH).

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Caudal Regression/Sacral Agenesis

19 year-old female with pelvis deformity including bilateral hip dislocation with pseudoacetabulum formation at the iliac bones and absent coccyx, sacrum, and lower two lumbar vertebrae.

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Morquio s Syndrome (mucopolysaccharidoses)

8 year-old female with bilateral high dislocated hips with acetabular dysplasia.

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5 yo male with bilateral DDH and Hx of absent ACL/PCL (Larsen s

syndrome?)

Left Hip: Superior and posterior dislocation of femur (thin arrow), head not situated in acetabulum, formation of pseudoacetabulum (open arrow).

Triradiate cartilage

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5 yo male with bilateral DDH and Hx of absent ACL/PCL (Larsen s syndrome?)

Right hip: Lateral and superior dislocation (thin arrows) with formation of pseudoacetabulum (open arrow).

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5 yo male with bilateral DDH and Hx of absent ACL/PCL (Larsen s syndrome?)

Pseudoacetabulum formation

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Other Congenital Abnormalities with Hip Dysplasia/Dislocation

!! Cerebral Palsy

!! Arthrogryposis multiplex

!! Congenital Myopathy

!! Ehlers-Danlos

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Differential Diagnosis of DDH

!! Various teratologic hip disorders

!! Proximal femoral focal deficiency (PFFD)

!! Septic hip

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Current Management

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Infants with SUBLUXABLE Hips

!! Subluxable hip – hip can move but cannot be completely dislocated (no clunk on exam)

!! May just follow with weekly ultrasound for 3

wks, if dysplasia persists – initiate Pavlik harness

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Infants with DISLOCATED or SUBLUXED Hips

1)! Apply Pavlik harness after clinical diagnosis, then obtain U/S.

2)! Then do clinical and U/S follow-up weekly.

3)! At 3 wks, if the hip is:

#! Reduced – Continue with harness until normal exam, U/S, and x- ray. Follow-up at 4-6 wks. #! Questionable (unstable but reducible): Do fixed abduction brace for 3 wks. Afterwards, if the hip is stable, then do Pavlik harness. If it is unstable, follow Not-Reduced protocol below. #! Not Reduced – Closed reduction + arthrogram, possible open reduction with cast.

STEPS:

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Treatment in Older Children

!! 6 – 18 months of age: Surgical reduction if Pavlik and/or closed reduction have failed.

!! 18 months and older: Open reduction, femoral or

iliac osteotomies. !! Osteotomies re-position the acetabular roof for

improved coverage of the femoral head to encourage proper development of the hip joint.

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Post-op – Bilateral pelvic osteotomies (arrows)

Pre-op

Example of Osteotomies: DDH in 7 year-old female

2nd surgery, post-op: Revised left pelvic osteotomy, (thin arrow), new femoral osteotomy (open arrow) Presentation material is for education purposes only. All

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Treatment Complications - AVN

8 year-old male with history of previously treated DDH. The femoral head is wide and flattened with a short neck, consistent with avascular necrosis.

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Conclusion !! DDH is a wide spectrum of conditions that range from primary

dysplasia without instability to severe, unreducible dislocation with a multifactorial etiology.

!! Evaluation: Clinical diagnosis, ultrasound, and radiographic evaluation

are primary – imaging features helpful to the orthopedic surgeon are: alpha angle and femoral head position on US; acetabular/CE angles, head position, and Shenton s arc on radiographs.

!! CT is helpful for pre-op evaluation in older children or to document reduction; MRI helpful for difficult cases and treatment complications.

!! Hip dysplasia/dislocation may be associated with various congenital

disorders – usually contain teratologic hips. !! Treatment (hip relocation) includes Pavlik harness, fixed abduction

brace, closed reduction, and open reduction with cast (osteotomies).

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References !! DD Aronsson, MJ Goldberg, TF Kling, Jr, and DR Roy. Developmental

dysplasia of the hip. Pediatrics 1994, 94: 201-208. !! Vitale MG. Skaggs DL. Developmental dysplasia of the hip from six months to

four years of age. J Am Acad Orthop Surg 2001, 9: 401-11. !! Nelitz M, Guenther KP, Gunkel S, Puhl W. Reliability of radiological

measurements in the assessment of hip dysplasia in adults. Br J Radiol 1999, 72:331-4.

!! Gerscovich EO. A radiologist s guide to the imaging in the diagnosis and treatment of developmental dysplasia of the hip. Skeletal Radiol 1997, 26: 386-97.

!! Graf R. Guide to sonography of the infant hip. New York: Thieme, 1987. !! Wientroub S, Grill F. Ultrasonography in developmental dysplasia of the hip. J

Bone Joint Surg Am 2000, 82:1004-1018. !! American Academy of Pediatrics: Clinical Practice Guideline: Early Detection

of Developmental Dysplasia of the Hip. Pediatrics 2000, 105: 896-905. !! Murray KA, Crim JR. Radiographic imaging for treatment and follow-up of

developmental dysplasia of the hip. Seminars in ultrasound, CT, and MR 2001, 22: 306-44.

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References !! Omeroglu H, et al. Measurement of center-edge angle in developmental

dysplasia of the hip: a comparison of two methods in patients under 20 years of age. Skeletal Radiol 2002, 31: 25-29.

!! Broughton NS, et al. Reliability of radiological measurements in the assessment of the child s hip. JBJS (Br) 1989, 71-B:6-8.

!! Tonnis D. Normal values for the hip joint for the evaluation of x-rays in children and adults. Clin Orthop 1976, 119: 39-47.

!! Ogata S, et al. Acetabular cover in congenital dislocation of the hip. JBJS Br 1990, 72:190-96.

!! American Academy of Pediatrics: Clinical Practice Guideline: Early Detection of Developmental Dysplasia of the Hip. Pediatrics 2000, 105: 896-905 .

!! Fayad LF, Johnson P, Fishman EK. Multidetector CT of Musculoskeletal Disease in the Pediatric Patient: Principles, Techniques, and Clinical Applications. Radiographics 2005, 25:603-618.

!! Ogata S, Moriya H, Tsuchiya K, et al. Acetabular cover in congenital dislocation of the hip. J Bone Joint Surg [Br] 1990, 72:190–6.

!! Harcke HT, Grissom LE. Pediatric hip sonography. Diagnosis and differential diagnosis. Radiol Clin North Am. 1999, 37:787-96.

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