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Developmental Dysplasia of Hip in Prader-Willi Syndrome - Detection & Treatment Kyungjei Woo Dept. of Orthopedic Surgery Samsung Medical Center

Developmental Dysplasia of Hip (DDH) in Prader-Willi Syndrome (PWS)

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Developmental Dysplasia of Hip in Prader-Willi Syndrome- Detection & Treatment

Kyungjei Woo

Dept. of Orthopedic SurgerySamsung Medical Center

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Musculoskeletal Abnormalities

• Scoliosis & Kyphosis – Scoliosis : 63.9 %

• Mean Cobb angle – 15.1°

• Thoracic : 6

• Thoracolumbar : 8

• Lumbar : 4

• Double curve : 6

– Kyphosis : 16.7 %• Mean kyphotic angle – 30.8°

(Shim et al. Journal of Pediatric Orthopaedics, 2010)

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Musculoskeletal Abnormalities

• Lower Limb Alignment Abnormalities

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Musculoskeletal Abnormalities

• Foot Abnormalities– Bilateral pes planus : 36.1 % – Bilateral pes cavus – Metatarsus adductus – Bilateral hallux valgus

(Shim et al. Journal of Pediatric Orthopaedics, 2010)

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Musculoskeletal Abnormalities

… and DDH

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Why in PWS?

• Contributing Factors– Hypotonia

– Ligamentous laxity

– But, still…

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• Detection & Treatment are not quite different between patients with and without PWS.

DDH in PWS

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• Types of DDH• Dysplasia

• Subluxation

• Dislocation

Developmental Dysplasia of the Hip

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• Incidence– DDH : 0.1%

DDH in PWS

10 % (X 100) West et al. Journal of Pediatric Orthopaedics 2004

~22.2 % (X 200) Shim . Journal of Pediatric Orthopaedics, 2010

DDH in PWS

10 % (X 100) West et al. Journal of Pediatric Orthopaedics 2004

~22.2 % (X 200) Shim . Journal of Pediatric Orthopaedics, 2010

Developmental Dysplasia of the Hip

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Phenomenon of combined disease– Torticolis– Adductus metatarsus– Plagiocephaly

Developmental Dysplasia of the Hip

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Diagnosis

• Clinical Diagnostic Test– Ortolani & Barlow : clunk

• Reduction & Provocation test

– Limitation of Range of Motion• Abduction

– Leg Length Discrepancy• Galeazzi sign or Allis sign

– Skin Crease

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Barlow testBarlow testOrtolani test Ortolani test

Diagnosis

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Diagnosis

• Clinical Diagnostic Test– Ortolani & Barlow : clunk

• Reduction & Provocation test

– Limitation of Range of Motion• Abduction

– Leg Length Discrepancy• Galeazzi sign or Allis sign

– Skin Crease

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– Static assessment• Inter-observer & intrao-bserver variations

– Dynamic assessment• Ortolani & Barlow test

• Operator dependent

• Subjective assessment

Diagnosis - US

αβ

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after 4~6 months or 1y

Diagnosis - Radiographs

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Male / 8moTreatment

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Closed reduction Open reduction

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After 6 wks

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Again 6 wks (POD 3m) cast off & abd. brace

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After 9m – still good

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after 6m / POD 1y4m

• 아이가 힘들어해서 밤에 보조기 안 하고 있어요 .• 아직 못 걸어요 . (2 years old)

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아직 헐겁습니다 .아이가 보채도 보조기 하는 것이 좋습니다 .보조기 못 하더라도 다리 벌리는 자세 자꾸 해주세요

after 6m / POD 1y4m

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POD 2y6m (3 years old)

혼자서 걸을 수 있다 . 2개월 되었다

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POD 3y4m (4 years old)

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• Varus derotation osteotomy

• Peri-acetabular osteotomy

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POD 7m (4ma)

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POD 11m / slip down

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POD 2wks

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Take Home Messages

• High incidence of hip dysplasia Hypotonia combined with ligamentous laxity

• Early detection and treatment Improve long-term results

Serial radiographic monitoring of the hips should be performed early in life in these children