DEVELOPMENTAL DYSPLASIA OF THE HIP Board Question: 2008 PREP, Q180 A LGA newborn girl who was born via C/S for breech presentation is ready for discharge from the newborn nursery at 72 hours of age. She has had an entirely normal newborn course. Of the following, you are MOST likely to advise the mother that her infant is at increased risk for: a) developmental dysplasia of the hip b) positional deformity of the foot c) positional plagiocephaly d) recurrent hypokalemia e) recurrent hypoglycemia CASE 1: 2 week old baby girl here for her weight check. Birth hx is significant for breech presentation necessitating a c/s. No further complications. PE in newborn nursery was documented as stable hips. What are important features of your physical exam today? What follow-up is important? CASE 2: 1 month old baby girl here for her WCC. Birth history is full term C/S for breech presentation. No complications. Mom and baby discharged from hospital on day of life 3. Baby has been exclusively BF. She regained birth weight by two weeks of age and has been gaining 35gm/day since your last visit. Today’s PE is notable for positive Barlow and negative Ortolani maneuvers. What is your next step? What is Developmental dysplasia of the hip? This refers to a spectrum of disorders of the hip that may be present at birth or develop during childhood. The hip may be completely dislocated with the femoral head outside of the acetabulum or subluxated with the femoral head partially outside the acetabulum. The hip may be dislocatable or subluxable which means it’s in normal position at rest but can be dislocated/subluxated with Barlow/Ortolani maneuvers. The hip may also be reducible where it is dislocated at rest, but the femoral head can be positioned into the acetabulum with manipulation. Finally, the hip may have dysplasia or abnormality of the shape of the femoral head and acetabulum (shallowness of the acetabulum). DDH can lead to premature degenerative joint disease, impaired walking and pain. What is the incidence? Hip instability can be detected in 1:100 babies; actual dislocation 1-2:1000 children In one study: at 1-3 days of life- incidence 5.5% but the same babies at 2-6 weeks of life incidence of 0.5%: 2nd study: 60-80% newborn abnormal hip by exam and 90% detected by US in newborn period resolved spontaneously without intervention. DDH is detected in 1 in 5000 infants at 18 months of age 1 Increased incidence in Native Americans and Lapp populations which use swaddling clothes and cradle boards that limit hip mobility and position the hip in adduction and extension. (25-50 per 1000 live births) 20% bilateral involvement. When unilateral, left is 3 times more often affected than right because in normal fetal positioning it is forced into adduction against the mother’s sacrum. What are the Risk factors? Breech Presentation (Girls 12%; Boys 3%): 7x higher than non-breech Females 4 times more common than males Positive family history: Risk of recurrence in subsequent children 6% with one affected child; 12% with one affected parent; 36% with affected parent and child Crowded intrauterine environment (Oligo, LGA, 1st born, multiple gestation) What is the pathogenesis? The pathogenesis of DDH is multifactorial. Ligamentous laxity allows the femoral head to move outside of the acetabulum under mechanical forces during development. The dysplasia results from the femoral head not having proper contact with the acetabulum during formation/development. What are the Clinical Features of DDH? Hip instability: o Barlow & Ortoloni maneuvers- should be performed in infants <8-12 weeks. The combination of Ortolani and Barlow maneuvers has a specificity of 98% and sensitivity 87-99% depending on the experience of the examiner. Barlow- dislocates the femoral head from the acetabulum by adducting a flexed hip with a gentle posterior force. Ortolanireduces a dislocated hip by abducting a flexed hip with a gentle anterior force. The sensation of dislocation or reduction, which is best described as a “jerk” or “clunk”. Hip clicks usually originate from the psoas tendon or ligamentum teres and do not signify abnormality. Asymmetry: o Galeazzi test- is performed with the infant supine, hips flexed to 90°, knees flexed, and feet flat on the examination table. The knees should be the same height, but in unilateral dislocation, the head of the femur will be displaced posterior therefore the ipsilateral knee will be lower. o Skin folds- Look for asymmetry of inguinal, thigh, and gluteal skin folds. With asymmetry of the thigh or popliteal creases, the abnormality is on the side where the crease is most proximal. The 2 inguinal crease should not pass the anal aperture. This should replace your Barlow/Ortolani examination in infants >8 weeks. Decreased range of Motion- An infant lying supine should have hip abduction of >75% and adduction of 30% past the midline. An infant >3 mo with < 45% abduction is a sign of DDH. Dislocation- Klisic test can be used to detect dislocation, especially after 3 months. Place index finger on anterior superior iliac spine & the middle finger on the great trochanter. An imaginary line between these 2 points should point towards or above the umbilicus. If dislocated will point below. Abnormal Gaito Bilateral DDH is very difficult to diagnose but may be evident once a child begins to walk with hyperlordosis, a waddling Trendelenburg gait, and unilateral toe-walking. Dysplasia without dislocation- No clinical findings. Usually detected incidentally by radiograph. Can lead to premature osteoarthritis of the hip. How do you Diagnose DDH? ****HIP EXAMINATION SHOULD OCCUR AT EVERY WCC UNTIL THE CHILD IS WALKING NORMALLY (AROUND 2 YEARS) PER AAP GUIDELINES*********** Physical Exam: o Barlow & Ortolani (<8-12 weeks) o Galeazzi & skin folds o Decreased ROM (including limited abduction), abnormal gait Ultrasound: Assesses the morphology and stability of the hip (static and dynamic imaging) until infant is 3-5 months of age. US is more accurate after 4 weeks of life. Accurate interpretation requires training and experience. Radiographs: More valuable after 4-6 months of age when the ossification center develops in the femoral head. 3 Who do you Screen/Refer for DDH? Please hold a discussion about both recommendations with the resident(s) & discuss which recommendations you follow & why AAP recommendations (2000): see algorithm below Newborn: If positive Ortolani/BarlowRefer to Peds Ortho. Do not order US. Treatment decisions are not based on US but PE findings. If soft signs (asymmetry of creases, hip clicks), then re-exam at 2 weeks. 2 week: If positive Ortolani/BarlowRefer to Peds Ortho. If other signs/ suspicion for DDH present then refer to Peds Ortho or perform US at 1 month. If hips normal continue routine screening at WCC. Breech: AAP recommends all girls should be screened by US at 6 weeks or Radiograph at 4-6 mo (risk 120/1000); Consider screening all breech boys (risk 26/1000). + FHX: Consider screening girls by US at 6 weeks or Radiograph at 4 months (risk 44/1000), boys with family history risk (9.4/1000) should follow periodic schedule unless positive findings. 4 Table 1. Previous Recommendations by the American Academy of Pediatrics and the Canadian Task Force for DDH for Newborns and Infants* Level of Evidence** III Quality of Evidence CTF: “Fair” AAP: "Good" Increases splint rate without decreasing late surgery rate II-1, III Both: "Fair" BOTH RECOMMEND AGAINST CTF: (D) AAP: "Strong" Ultrasound screening in high-risk infants Does not affect many infants and does not reduce the operative rate II-1, III CTF: “Fair” AAP: “Strong” CTF RECOMMENDS AGAINST (D) AAP RECOMMENDS “Strong” Routine radiographic screening at ages 3 to 5 months Ultrasound or radiograph if positive clinical exam Test is unreliable III CTF: “Fair” CTF RECOMMENDS AGAINST (D) AAP: “Poor” AAP RECOMMENDS AGAINST “Strong” AAP: "Good" AAP RECOMMENDS "Strong" None AAP was "Mixed" AAP: “Poor” AAP RECOMMENDS AGAINST “Strong” Screening Interventions Serial clinical examination of the hips by a trained clinician during the periodic health examination in all infants (until walking independently) Summary Findings Decreases the operative rate from 1–2 per 1000 infants to 0.2– 0.7 per 1000 Ultrasound screening (static or dynamic method) in all infants Does not influence management Positive screen: refer to orthopedist Equivocal screen: follow-up in 2 weeks Negative screen: follow-up in 2 months Suspicious physical, not positive: refer to orthopedist or obtain ultrasound 3-4 weeks Treatment Interventions Triple diapering Effectiveness unknown, may delay definitive therapy, though may aid in ensuring follow-up Abduction therapy Effectiveness unknown. Causes avascular necrosis of the hip (in 1%–4% of treated infants) III Observation before intervening DDH resolves spontaneously in many cases I Recommendations BOTH RECOMMEND CTF: (B) AAP: "Strong" CTF DOES NOT RECOMMEND (C) CTF: “Good” CTF RECOMMENDS (A) “I”, controlled trial with randomization; “II-1”, controlled trial without randomization; “III”, expert opinion; AAP – American Academy of Pediatrics; CTF – Canadian Task Force *For more details on quality and recommendation coding, please see Patel (2001) & Lehmenn (2000). 5 • • US Preventive Services Task Force (USPSTF) Recommendations (2006): The USPSTF concludes that evidence is insufficient to recommend routine screening for developm adverse outcomes. Grade: I Statement. This USPSTF screening recommendation applies only to infants who do not have obvious hip dislocations or other abnormalities evident without screening. Risk factors for DDH include female gender, family history of DDH, breech positioning, and in utero postural deformities. However, the majority of cases of DDH have no identifiable risk factors. Screening tests for DDH have limited accuracy. The most common methods of screening are serial physical examinations of the hip and lower extremities, using the Barlow and Ortolani procedures, and ultrasonography. The Barlow examination is performed by adducting a flexed hip with gentle posterior force to identify a dislocatable hip. The Ortolani examination is performed by abducting a flexed hip with gentle anterior force to relocate a dislocated hip. Data assessing the relative value of limited hip abduction as a screening tool are sparse and suggest the test is of little value in early infancy and is of somewhat greater value as infants age. What is the treatment for DDH? 0-6 months: abduction splinting (ie-Pavlik harness, Aberdeen splint, von Rosen splint). The pavlik harness prevents hip extension and adduction and permits flexion and abduction. It is adjusted weekly or biweekly to maintain ideal position while the infant grows. Treatment usually lasts 2-3 months. Treatment is discontinued after 3 weeks if the dislocated hip is not reduced. Only used in otherwise normal infants. Potential complications include femoral nerve compression, brachial plexus palsy, knee subluxation, skin breakdown, residual acetabular dysplasia, and avascular necrosis of the femoral head. Double or triple diapering is no longer a recommended therapy because it was not effective and it promoted hip extension. 6-18 months: Closed or open reduction in OR under anesthesia is usually necessary for infants 6 months or older. This is followed by 3-4 months of spica casting changed at 6 week intervals to maintain hip in position. 18 months and older: Open reduction in OR under anesthesia is usually necessary for children 18 months or older. This is followed by 3-4 months of spica casting changed at 6 week intervals to maintain hip in position. Risks for long-term problems such as failure of treatment, residual dysplasia, and avascular necrosis of the femoral head are greater the older the patient is at treatment. Outcome: Most neonatal hips with instability or dysplasia resolve spontaneously When DDH is diagnosed in newborns, treatment with abduction splinting is successful in the majority of patients Residual dysplasia, either following treatment or undiagnosed, may result in progression to premature degenerative disease. 6 Children treated often have annual radiographs until 6 looking for late complications, such as osteonecrosis Answers: Board question: A Case 1: Residents should discuss performing hip examination at each well child check including Galeazzi test, range of motion testing (including abduction), Barlow & Ortolani maneuvers (until 8-12 weeks), evaluating skin folds, & gait (when weight bearing) Case 2: Residents should refer to orthopedics, no need for imaging first. References: 1. US Preventive Services Task Force. Screening for Developmental Dysplasia of the Hip: Recommendation Statement. Pediatrics 2006; 117; 898-902. 2. American Academy of Pediatrics Committee on quality improvement, subcommittee on developmental dysplasia of the hip. Clinical Practice Guideline: Early detection of Developmental Dysplasia of the Hip. Pediatrics 2000; 105 (4); 896-905. 3. Goldberg, MJ. Early Detection of Developmental Hip Dysplasia: Synopsis of the AAP Clinical Practice Guideline. Pediatr. Rev. 2001;22;131. 4. Waanders, NA. Epidemiology and pathogenesis of developmental dysplasia of the hip. UpToDate;2011. 5. Waanders, NA. Clinical features and diagnosis of developmental dysplasia of the hip. UpToDate;2011. 6. Waanders, NA. Treatment and Outcome of the developmental dysplasia of the hip. UpToDate;2011. 7. Kliegman, RM et al. Nelson Textbook of Pediatrics 18th Edition. Philadelphia: WB Saunders Company, 2007. 7