بسم الله الرحمن الرحيم

CDH Congenital Dislocation of the Hip Mamoun Kremli Professor / Consultant Pediatric Orthopedics College of Medicine & King Khalid University Hospital

CDH The most common disorder affecting the hip in children Spectrum of diseases/abnormalities of the hip with different etiologies, pathologies, and natural histories affecting the proximal femur and acetabulum Initial pathology is congenital, progresses if untreated. Does not always result in dislocation.

CDH Definition A progressive deformation of previously normally formed structures during the embryonic period NOT A malformation arising during the period of organogenesis

CDH Nomenclature CDH Congenital Dislocation of the Hip DDH Developmental Dysplasia of the Hip CDH Congenital Dysplasia of the Hip CHD Congenital Heart Disease !

CDH Spectrum Teratologic Hip : Fixed dislocation Occurrs prenatally Often with other anomalies Dislocated Hip : Completely out May or may not be reducible Subluxated Hip : Only partially in Unstable Hip : Femoral head can be dislocated Acetabular Dysplasia : Shallow Acetabulu Head Subluxated or in place

Have underlying hip dysplasia CDH Incidence Hip Instability at Birth : 0.5 – 1 % of infants Classic CDH : 0.1 % of infants Mild Dysplasia : Substantial Contributing to adult Osteoarthritis Up to 50 % of Hip Arthritis in Ladies Have underlying hip dysplasia

CDH Incidence Area Incidence per 1000 Canadian Indians 188.5 Hungary 28.7 Uppsala, Sweden 20 USA Caucaseans Blacks 15.5 4.9 Malmo, Sweden 2.18 Chinese, Hong Kong 0.1 Bantus, Africa 0.0 among (16678)

CDH Etiology Multi-factorial

CDH Etiology Physiologic Factors Ligament Laxity : Hormonal : ( Estrogen, Relaxin) Females Familial hyper laxity : mild - moderate - Ehler Danlos ADD Picture of knee hyperextension

Genetic Factors Gender : Female Most studies: Twin studies: CDH Etiology Genetic Factors Gender : Female Most studies: Females > 4-6 X than males Twin studies: Monozygotic 38 % Dizygotic 3 % (similar to siblings)

Family Incidence and Genetic Counselling CDH Etiology Family Incidence and Genetic Counselling Affected At risk Risk One sibling Siblings 1 in 17 One parent Children 1 in 8 One parent, one sibling 1 in 3 2nd degree relative Nieces, nephews 1 in 100

CDH Etiology Mechanical Factors Prenatal : - Breech position - Oligohydramnious - Primigravida - Cong. Knee recurvatum/dislocation - Metatarsus adductus - Torticollis Postnatal : - Swaddling / Strapping – Knees extended

CDH Etiology Mechanical Factors Breech Presentation : Normally 2 –4 % CDH 16 % The Breech position In Utero Extended knees and flexed hips

CDH Etiology Environmental & Mechanical Factors Swaddling / strapping ( Mihad ): Knees extended & Hips adducted Proven experimentally Proven statistically American Indians. Eskimos, and Saudi Arabia Mechanics Hip adduction and extension

CDH Patients At Risk Positive Family History : increases risk 10X A baby girl : increases risk 4-6 times Breech Presentation : increases risk 5-10 X Torticollis : CDH in 10-20 % cases Foot Deformities : ( calcaneovalgus & metatarsus adductus) signs of intrauterine crowding Knee Deformities : ( hyperextension & dislocation ) associated with Teratologic type

CDH Risk Factors When Risk Factors Are Present The infant should be examined repeatedly The hip should be imaged ( by U/S or X-ray )

CDH Neonatal Examination The infant should be quiet and comfortable

CDH Neonatal Examination LOOK : External rotation attitude Lateralized contour Wide perineum ( in bilateral )

CDH Neonatal Examination anterior posterior LOOK : Asymmetric thigh folds

CDH Clinical Examination Look : Shortening ( not in neonates ) - in supine - Galeazzy sign

CDH Neonatal Examination FEEL : Empty groin Weak Femoral pulse

CDH Neonatal Examination MOVE : Hip instability in early infancy Limited hip abduction in flexion - later (careful in bilateral) if <600 on both sides: request imaging

Cerebral palsy Clinical Assessment Hip Flexion Deformity Thomas Test SPECIAL : Loss of fixed flexion deformity of hips ( early infancy ) Normally FFD newborn 28o at 6 weeks 19o at 6 months 7o FFD Normal No FFD ?CDH

CDH Neonatal Examination Ortolani Feel a Clunk Not hear a click !

CDH Neonatal Examination Barlow

CDH Neonatal Examination Ortolani / Barlow clunk Ortolani Barlow

CDH Neonatal Examination Ortolani / Barlow

CDH Neonatal Examination Hamstring Stretch Sign Flex hip and knee 900 each. Keep hip flexed and gradually extend the knee Normally a resistance is felt towards the end of knee extension (caused by the hamstrings which are pulled from both ends) In cases of CDH, no resistance is felt (when the hip is dislocated, the origin of the hamstrings are not pulled by hip flexion)

CDH Neonatal Examination Hamstring Stretch Sign

CDH Clinical Examination Neonate (up to 2-3 months) : - Instability/ Ortolani-Barlow - Thomas test Infant ( > 2-3 months) : - Limited abduction - Shortening ( Galeazzi ) - Hamstring stretch sign Toddler : - Limited abduction Walking : - Trendelenburgh

CDH Clinical Examination

CDH Clinical Examination

CDH Clinical Examination The Walking Child Trendelenburgh: unilateral / bilateral (waddling)

CDH Screening Program Clinical screening proven to be effective Performed by Trained personnel Must be DYNAMIC with periodic examination till walking Adjunctive use of U/S controversial

CDH Ultrasound Screening Incidence of hip instability declines rapidly to 50 % within the first week of neonatal life Better to delay U/S screening

CDH Ultrasound Screening Early U/S screening not recommended Delayed U/S screening : - Older than 6 weeks - Those at risk only - by History Clinical exam

CDH Ultrasound Referral If hip normal : no need If hip clearly unstable : no need If suspicious : U/S appropriate If at risk factors : U/S appropriate

CDH Ultrasound Too sensitive detects a lot of hip anomalies most of which would develop normally Operator dependant Static Vs Dynamic

CDH Radiography Early infancy : not reliable By 2-3 months of age : reliable AP view - neutral position - draw reference lines - acetabular index - in early infancy < 30o : normal 30o – 40o : questionable > 40o : abnormal Von Rosen view : 45o abduction

CDH Radiography

CDH Radiography

CDH Radiography

CDH Radiography in out out in Von Rosen view

CDH Radiography 39o 27o

CDH Radiography out in

CDH Treatment Aims Obtain and Maintain concentric reduction In an Atruamatic fashion Without disrupting the blood supply

CDH Treatment Method depends on Age The earlier started, the easier the treatment The earlier started, the better the results Should be detected EARLY

CDH Treatment Birth to 6 months : Pavlik harness or hip spica cast 6 months – 12 months : closed reduction UGA and hip spica casts 12 months – 18 months : possible closed / possible open reduction Above 18 months : open reduction and ? Acetabuloplasty Above 2 years : open reduction,acetabulplasty, and femoral osteotomy Above 8 years : open reduction,acetabulplasty cutting three bones, and femoral osteotomy

CDH Treatment Hip instability in the neonatal period Most resolve spontaneously Observation Pavlik harness Double /triple diapers ??

CDH Treatment Hip instability in the neonatal period Double / Triple Diapers Often inadequate : therefore inappropriate Gives illusion patient is in “treatment” while wasting valuable time Most hip instability improves spontaneously in early infancy , giving this ineffective management credit

CDH Treatment Birth – 6 months Hip instability (dislocatable) Established dislocation (reducible) Should be actively treated until hip is normal clinically and radiographically Pavlik harness Hip Spica Cast

CDH Treatment Birth – 6 months Pavlik harness

CDH Treatment Birth – 6 months Other Devices - Frejka pillow - Craig - Von Rosen splint Soft abduction splints: Not good enough Rigid abduction splints: Risk AVN

Initially non operative – closed reduction CDH Treatment 6 – 12 months Initially non operative – closed reduction Reduction under anesthesia and immobilization in hip spica cast Position: Human Avoid severe abduction Avoid Frog position Must be stable and concentrically reduced otherwise needs open reduction Better Picture

Possibly closed reduction !! CDH Treatment 12 – 18 months Possibly closed reduction !! when hip stable and concentrically reduced Probably open reduction when hip unstable or not concentrically reduced Arthrography guided:

CDH Treatment Arthrography Closed Reduction Too lateralized Acceptable

CDH Treatment Above 18 months Open reduction ? and acetabulplasty ? And femoral shortening – if high

CDH Treatment Above 3 years Open reduction And acetabulplasty And femoral shortening

Redirectional Acetabuloplasty Salter’s Add Picture with K wires

Need for a lot of improvement in cover Pemberton’s Need for a lot of improvement in cover

Triple Steel

CDH When Not to Treat ?! Bilateral High Posterior Dislocation good function – not painful

CDH When Not to Treat ! وخيرٌ من بعض ِ الدواءِ الداءُ Painful stiff left hip Painful stiff right hip in adduction

CDH When Not to Treat ! وخيرٌ من بعض ِ الدواءِ الداءُ Painful right hip & ankylosed left hip

Complex multi-factorial, endemic– treatable. CDH Summary Complex multi-factorial, endemic– treatable. Dr’s awareness and health education. Screening programs are needed. Learning proper examination methods. Identify at-risk groups. repeat examination & imaging. Efficient referral system. Proper management in referral centers.