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Developmental dysplasia of the hip: Clinical features and diagnosis

Developmental dysplasia of the hip: Clinical features and diagnosis
Author:
Scott B Rosenfeld, MD
Section Editor:
William A Phillips, MD
Deputy Editor:
Diane Blake, MD
Literature review current through: Jan 2024.
This topic last updated: Oct 24, 2022.

INTRODUCTION — Developmental dysplasia of the hip (DDH) describes a spectrum of conditions related to the development of the hip in infants and young children. It encompasses abnormal development of the acetabulum and proximal femur and mechanical instability of the hip joint (table 1).

Newborns often have physiologic laxity of the hip and immaturity of the acetabulum during the first few weeks of life. In most cases, the laxity resolves, and the acetabulum proceeds to develop normally. With assessment of risk factors, serial physical examination of the hips, and appropriate use of imaging studies, most children with pathologic hips can be correctly diagnosed and treated without long-term sequelae. (See "Developmental dysplasia of the hip: Treatment and outcome".)

Typical DDH, which generally occurs in otherwise healthy infants, will be the focus of this topic review. Hip dysplasia and instability also occur in association with other conditions. Teratologic hip dysplasia occurs in association with various syndromes (eg, Ehlers-Danlos, Down syndrome, arthrogryposis), and neuromuscular hip dysplasia occurs when there is weakness and/or spasticity in some or all of the hip muscle groups (eg, in spina bifida or cerebral palsy). The diagnosis and management of teratologic and neuromuscular hip dysplasia differ from the diagnosis and management of hip dysplasia in otherwise healthy infants.

The clinical features and diagnosis of DDH in otherwise healthy children will be reviewed here. The epidemiology, pathogenesis, treatment, and outcome are discussed separately. (See "Developmental dysplasia of the hip: Epidemiology and pathogenesis" and "Developmental dysplasia of the hip: Treatment and outcome".)

CLINICAL FEATURES — The clinical features of DDH depend upon the age of the child and the severity of the abnormality (table 2). The spectrum of presentation ranges from instability on the newborn examination, to subtle limited abduction in the infant, to asymmetric gait in the toddler, to activity-related pain in the adolescent, to osteoarthritis in the adult. The earlier DDH is detected, the simpler and more effective the treatment and the better the long-term outcome [1,2]. (See "Developmental dysplasia of the hip: Treatment and outcome".)

History — Thorough review of the child's medical and family history helps to identify risk factors for DDH and exclude other congenital or neuromuscular causes of hip instability. The most important risk factors are female sex, breech positioning at ≥34 weeks gestation (whether or not external cephalic version is successful) [3], family history of DDH, and tight lower extremity swaddling [2]. Conditions that are associated with DDH, but are not proven to increase the risk of occurrence, include torticollis, plagiocephaly, metatarsus adductus, clubfoot, being the firstborn infant, oligohydramnios, birthweight >4 kg, and multiple gestation pregnancy. (See "Developmental dysplasia of the hip: Epidemiology and pathogenesis", section on 'Risk factors'.)

Infants and young children with untreated hip dislocation rarely have pain or other limitations. Most affected children begin to walk and reach other developmental milestones at the appropriate time.

Examination

Overview — We agree with professional society guidelines that recommend examination for DDH beginning in the newborn period and continuing at health supervision visits until approximately nine months of age and/or the child is walking independently [2-5]. The newborn examination and the office examinations at two to four weeks of age are particularly important for early detection of DDH.

The pertinent examination findings of DDH vary according to age (table 2). The most important aspects of the examination are hip instability in the young infant and range of motion in the older infant and child [2]. It is important to explain the hip examination to the caregivers as it is being performed and to document the results at each visit. (See 'Hip instability' below and 'Range of motion' below.)

Examination for DDH at the newborn visit and at subsequent health supervision visits is recommended by the American Academy of Pediatrics (AAP) [2], the American Academy of Orthopaedic Surgeons [4], the Pediatric Orthopaedic Society of North America [5], and the Canadian Task Force on DDH [6]. (See 'Screening for DDH' below.)

General examination — Important aspects of the general examination for DDH include the neurologic examination and examination of the spine and distal lower extremities, looking for abnormalities associated with DDH and other causes of hip instability.

The neurologic examination should include evaluation of spontaneous movement of all four extremities and assessment of spasticity. (See "Neurologic examination of the newborn" and "Detailed neurologic assessment of infants and children".)

The spine examination should include cervical range of motion (looking for torticollis (picture 1)) and cutaneous manifestations of spinal dysraphism (eg, dimples and hairy patches). (See "Myelomeningocele (spina bifida): Anatomy, clinical manifestations, and complications".)

Examination of the extremities should include examination of the feet for metatarsus adductus (picture 2). (See "Lower extremity positional deformations", section on 'Metatarsus adductus'.)

Age-specific findings of DDH — The pertinent examination findings vary according to age (table 2):

In infants younger than three months, it is most important to evaluate the stability of the hips using the Ortolani maneuver [2]. The Barlow maneuver, the Galeazzi test (also called the Allis or Perkins test), and the Klisic test also may be useful. Decreased abduction generally is not present at this age. (See 'Hip instability' below and 'Asymmetry' below and 'Klisic test' below.)

After three months of age, limited abduction, apparent thigh-length discrepancy (in unilateral cases), and the Galeazzi (for unilateral cases) and Klisic tests are better indicators of DDH than instability. By three months of age, tests of instability are of little value because unstable hips have usually stabilized (the once-dislocatable hip stabilizes in the reduced position; the once-reducible hip stabilizes in the dislocated position). (See 'Range of motion' below and 'Asymmetry' below and 'Klisic test' below.)

In walking-aged children with unilateral DDH, weakness of the hip abductors on the affected side may be indicated by a positive Trendelenburg pelvic tilt test (inability to maintain the pelvis horizontally while standing on the ipsilateral leg) (figure 1) and the presence of a Trendelenburg lurch while walking. (See 'Asymmetry' below.)

Hip instability — Each hip should be examined individually for reducibility and instability (table 1) [7-9]. The infant should be on a stable surface in the supine position, with the hip flexed to 90° and in neutral rotation. The examination should occur when the infant is calm and not crying. The hips are best examined with all clothing and diapers removed from the lower extremities.

Ortolani maneuver – The thigh is grasped loosely with the examiner's index and middle finger along the greater trochanter and the thumb on the inner thigh. From an adducted position, the hip is gently abducted while lifting or pushing the trochanter anteriorly. Extreme abduction should be avoided because it decreases the sensitivity of the Ortolani maneuver. If the hip is dislocated, the Ortolani maneuver may reduce it and is accompanied by a palpable clunk. A positive Ortolani maneuver assumes a dislocated hip that is reducible.

Barlow maneuver – The hip is held in the same manner as for the Ortolani maneuver. The thigh is grasped loosely with the examiner's index and middle finger along the greater trochanter and the thumb on the inner thigh. The hip is gently adducted and, in a change from previous recommendations, no downward pressure is applied, and the head is palpated to detect moving out of the back of the acetabulum. The examiner should not attempt to forcefully dislocate the femoral head [2]. If the hip is dislocatable, posterior movement and a palpable clunk may be detected as the femoral head exits the acetabulum (the "jerk of exit") [2,7-9]. A subluxatable hip is characterized by a subtle sliding movement or a feeling of looseness, like a tennis ball moving in a soup bowl. A positive Barlow maneuver assumes a reduced hip that is subluxatable or dislocatable [2].

The sensation of reduction or dislocation is distinct and best described as a "jerk" or "clunk." This sensation is different from that of the high pitch benign joint popping, clicking, or snapping caused by the snapping of tendons or ligaments in and around the hip and knee. The term "hip click" is misleading and is best avoided; isolated hip clicks are not suggestive of DDH [10-12].

The combination of the Barlow and Ortolani maneuvers has a high specificity (estimated to be approximately 98 to 99 percent) in the detection of hip instability [13]. The sensitivity varies depending upon the skill of the examiner, the number of examinations performed, and the diagnostic standard (eg, ultrasonography, radiographs). Sensitivity is increased in experienced hands (ranging from 87 to 97 percent) [14-18].

Asymmetry — Asymmetric examination findings may be helpful in unilateral DDH. However, as many as 37 percent of cases are bilateral [19]. Lack of asymmetry does not exclude DDH and may delay the diagnosis.

Apparent shortening of one femur is an important sign of unilateral dislocation and can be elicited with the Galeazzi test. This is performed with the infant supine, hips flexed to 90°, knees flexed, and feet flat on a level surface and side by side, with the heels in apposition to the buttocks. In this position, the knees are normally at the same level. In unilateral dislocation, the head of the femur is displaced posteriorly, functionally shortening the thigh, and the ipsilateral knee will be lower than the other knee (figure 2). A positive Galeazzi test is not specific for DDH. Other causes of leg-length discrepancy must be considered (eg, hemihypertrophy, femoral hypoplasia, coxa vara).

Asymmetry in the position or number of the inguinal, thigh, or gluteal skin folds (figure 3A-B) may be a clue to the diagnosis of unilateral DDH. In the case of DDH, the increased folds are caused by "bunching" of the skin and muscle around a functionally shortened femur. Asymmetric skin folds are less helpful than other signs in predicting DDH. In a series of 105 children referred for asymmetric skin folds, only two had pathologic DDH; both had a positive Galeazzi test and limited hip abduction [20]. Asymmetric skin folds are sensitive but not specific for DDH, since skin fold asymmetry is present in approximately 24 percent of all infants [21].

In the child who is walking, gait asymmetry may provide a clue to unilateral DDH. The abnormal gait is usually caused by leg-length discrepancy, resulting in gait changes, such as toe-walking on the affected side or vaulting over the longer contralateral leg [22]. A child with unilateral hip dislocation will have a Trendelenburg lurch on the affected side (figure 1).

Range of motion — In a child older than two to three months, limitation of abduction (<45°) is the most reliable sign of DDH. Normal range of motion in a supine infant with the pelvis stabilized is >75° for abduction and at least 30° past the midline for adduction [23]. In a 10-year prospective longitudinal study that included 2876 infants, unilateral limited hip abduction after eight weeks of age had a sensitivity of 78 percent and specificity of 93 percent in the detection of radiographically confirmed DDH [24]. In another review of 683 infants older than three months, unilateral limited hip abduction had a sensitivity of 69 percent and a specificity of 54 percent [25]. Infants younger than two to three months may have normal adduction because they have not had enough time to develop an adduction contracture. (See "Developmental dysplasia of the hip: Epidemiology and pathogenesis", section on 'Embryology and pathogenesis'.)

Patients with unilateral DDH may have increased internal rotation of the hip because increased femoral anteversion is often associated with DDH [26]. Internal hip rotation is assessed with the patient lying prone on the examination table and the knees flexed; the lower leg is rotated away from the axis of the body (rotating the hip internally) (figure 4). (See "Approach to the child with in-toeing", section on 'Focused neurologic examination'.)

Klisic test — A positive Klisic test is suggestive of DDH. The Klisic test is performed by placing the index finger on the anterosuperior iliac spine and the middle finger on the greater trochanter. An imaginary line between these points passes through or above the umbilicus in a child without DDH (negative Klisic test). The line passes below the umbilicus if the hip is dislocated (positive Klisic test) because the greater trochanter is in a more superior position (figure 5). The Klisic test may be particularly helpful in bilateral dislocations when abduction is symmetrical and the Galeazzi sign is negative (ie, the knees are at the same height), because the result is evaluated independently for each side and does not rely on comparison with the contralateral limb.

Bilateral dislocation — Bilateral DDH occurs in as many as 37 percent of cases [19,27]. In infants younger than three months, the tests for instability (ie, the Ortolani and Barlow maneuvers) may be useful in detecting bilateral DDH. However, as DDH progresses, dislocated hips become irreducible and instability is no longer detected. Findings related to symmetry (eg, Galeazzi sign, thigh folds) are not helpful in bilateral dislocation [23]. (See "Developmental dysplasia of the hip: Epidemiology and pathogenesis", section on 'Embryology and pathogenesis'.)

In children older than three months, the most important examination finding for bilateral dislocated hips is symmetric limited abduction (<45°). Other signs may include widening of the perineum, bilateral positive Klisic tests, and short thigh segments relative to the child's size (figure 6). Once the child begins to walk, hyperlordosis and a waddling Trendelenburg gait are classic findings (table 2 and figure 1).

Dysplasia without dislocation — Dysplasia without dislocation usually has no clinical findings and is asymptomatic until adolescence or early adulthood. It is often found only through diligent screening of risk factors or incidentally when radiographs are obtained for other concerns (eg, an abdominal radiograph for evaluation of bowel obstruction, cystography (image 1)) or for assessment of activity-related hip pain in an adolescent or young adult.

Natural history — The natural history of untreated DDH depends upon the age of the patient and the severity of DDH [28-31]. Most hip instability in newborns stabilizes soon after birth, as physiologic laxity decreases and the femoral head and acetabulum grow. In observational studies, there is a high rate of resolution of neonatal hip instability without intervention [4,7,32-35]. In a cohort of 11,989 infants examined by a single examiner, 60 percent of hips that demonstrated instability on physical examination at birth stabilized in the first week of life, and nearly 90 percent stabilized by two months [7]. These hips were functionally and radiographically normal at 12 months of age. Similar results were reported in a study of routine ultrasonographic screening of more than 14,000 infants: 6 percent had abnormalities soon after birth, 90 percent of which became normal by nine weeks of age [35].

Infants and young children with untreated hip dislocation rarely show signs of pain or other limitations. Most children reach developmental milestones at the appropriate time. A dislocated hip generally does not delay the age at which the child begins to walk. A dislocated hip may function well for many years [31]. However, weakness in the hip abductor musculature may result in the classic Trendelenburg gait pattern. Over time, there may be gradual progression of functional disability, pain, and accelerated degenerative hip disease. The risk of these complications is not well-defined but may be associated with the development of a false acetabulum [36]. Patients with unilateral dislocations may have leg-length discrepancy, ipsilateral knee problems, scoliosis, and gait disturbance. Patients with bilateral dislocations may develop back pain (perhaps related to increased lumbar lordosis).

Dysplasia in a reduced or subluxed hip may be diagnosed incidentally when radiographs are obtained for other reasons. The clinical course for patients with this presentation is variable. These hips are often asymptomatic in infants and young children, and radiographic abnormalities may improve with time, as the acetabulum still has excellent remodeling potential [36-39]. After school age, the likelihood of spontaneous improvement is low. Patients with persistent dysplasia without dislocation may remain asymptomatic for some time but may develop activity-related hip pain or premature degenerative joint disease in adolescence or young adulthood. This occurs as a result of increased cartilage contact stress at the anterior and lateral margins of the acetabulum. Pain may start soon after skeletal maturity, or, in females, during the first or second pregnancy, or at menopause. Patients 40 to 60 years old who present with hip osteoarthritis are often found to have mild dysplasia as a contributing cause [28-31].

DIAGNOSTIC IMAGING

Ultrasonography — Ultrasonography is the primary imaging technique for assessing the morphology and stability of the infant hip. It is an important adjunct to the clinical evaluation until four to six months of age [40-42]. Ultrasonography can be helpful in confirming physical examination findings and evaluating infants with risk factors who have normal examination findings. The major drawback of ultrasonography is that accurate interpretation requires training and experience [43].

Ultrasonographic criteria for DDH have been established for static imaging (which includes coronal and transverse planes) and dynamic imaging of the flexed hip with and without a modified Barlow stress maneuver [42,44]. The combination of static and dynamic imaging permits evaluation of hip morphology, position, and stability.

Static views include a coronal image with the infant in the lateral decubitus position and the hips flexed at 30 to 45° [45]. In this position, the ossified ilium is viewed as a straight white line above the femoral head and superior acetabulum (figure 7 and image 2). In practice, these images are often oriented as if the baby is lying in the lateral position, with the ilium pointed to the left of the femoral head, instead of above it. The alpha angle is measured from the lateral wall of the ilium and the bony roof line. The beta angle is the angle formed by the lateral wall of the ilium and the cartilaginous roof line. These angles are used to classify DDH according to the Graf system (figure 8) [45]. Another important parameter is the percentage of the femoral epiphysis that is covered by the acetabular roof. For infants younger than four months of age, we generally consider coverage of >50 percent to be normal [46]. A systematic review of 28 studies found high variability and low reproducibility for all ultrasonographic dysplasia metrics [47].

The dynamic technique uses axial and coronal images with real-time stress of the femoral head, similar to the instability test maneuvers [48]. In the first few days of life, 4 to 6 mm of laxity is considered normal. Dynamic imaging is usually omitted when the hips are examined during treatment [44].

Radiographs — Radiographs may be helpful in the evaluation of DDH after four to six months of age [40,42]. Before that, radiographs have limited value because the femoral head and acetabulum are cartilaginous and unossified. However, they may be helpful if there are concerns for other bony abnormalities (eg, congenital coxa vara, proximal femoral focal deficiency, and sacral agenesis). (See "Radiologic evaluation of the hip in infants, children, and adolescents", section on 'Imaging studies for specific clinical settings'.)

When a hip radiograph is requested in a newborn, it should consist of a single anteroposterior (AP) view with the hips held in 20 to 30° flexion; the frog lateral view is unnecessary and increases radiation exposure. The flexion is necessary to accommodate the physiologic flexion contracture of the newborn hip.

After four to six months, when AP radiographs are obtained, the hips should be in the neutral position [42]. Radiographic lines and angles are used to assess acetabular development and hip position. Radiographic findings consistent with DDH include:

Lateral and superior positioning of the ossified portion of the femoral head and neck (image 3 and figure 9A-B)

Increased acetabular index (figure 10 and image 4); normal values for the acetabular index are as follows: <35° at birth; <25° at 1 year; <20° at 2 to 3 years; <15° at 6 to 7 years; <11 to 12° at 10 years; and <10° at 15 years [49]

Delayed appearance of the femoral ossific nucleus on the involved side or asymmetric sizes of the femoral ossific nuclei (image 3)

Other imaging — Other imaging modalities, such as arthrography, computed tomography (CT), and magnetic resonance imaging (MRI), are not useful in the diagnosis of DDH but may be used to evaluate postsurgical reduction. Arthrography (injection of radiopaque contrast into the hip joint) and three-dimensional imaging, such as MRI or CT, are frequently obtained to confirm successful reduction and to determine the position of the femoral head within the acetabulum after casting. Pediatric protocols that involve reduced doses of radiation should be used.

MRI is not typically used in the diagnostic evaluation of the young child with DDH, but its use is becoming more common in the evaluation of postsurgical reduction [50]. Compared with CT, MRI exposes the patient to no radiation and provides information about vascularity of the femoral head [51]. It is also helpful for evaluating long-term sequelae of partially treated or untreated DDH, such as labral tears and arthritis [51]. (See "Radiologic evaluation of the hip in infants, children, and adolescents", section on 'Developmental dysplasia of the hip' and "Developmental dysplasia of the hip: Treatment and outcome", section on 'Outcome'.)

DIAGNOSIS — The diagnosis of DDH in infants is usually made by physical examination demonstrating hip instability (table 1), asymmetry (in unilateral cases), and/or limited abduction. Examination findings of DDH vary according to age (table 2). (See 'Hip instability' above and 'Asymmetry' above and 'Range of motion' above.)

Diagnostic imaging can be used to make the diagnosis in infants with inconclusive examination findings. Diagnostic imaging also may be used to make the diagnosis in infants who have risk factors and normal examination. (See 'Diagnostic imaging' above and 'Suboptimal or inconclusive examination' below and 'Normal examination and risk factors' below.)

Ultrasonography generally is preferred for infants younger than four to six months of age and radiography for infants older than four to six months. Ultrasonographic measurements (figure 7) are used to classify DDH according to the Graf system (figure 8). Radiographic findings of DDH include lateral and superior positioning of the ossified portion of the femoral head and neck (image 3 and figure 9A-B), increased acetabular index (figure 10), and delayed appearance of the femoral ossific nucleus on the involved side or asymmetric sizes of the femoral ossific nuclei. (See 'Ultrasonography' above and 'Radiographs' above.)

APPROACH TO DIAGNOSIS AND REFERRAL — Our approach to diagnosis and referral for DDH varies with the clinical examination findings, the age of the infant, and risk factors. It is generally consistent with the American Academy of Orthopaedic Surgeons (AAOS) clinical practice guideline for the detection and nonoperative management of DDH [4,52], the American Academy of Pediatrics (AAP) clinical practice guideline for early detection of DDH [2], the American College of Radiology (ACR) Appropriateness Criteria for DDH [42], and the American Institute of Ultrasound in Medicine guideline for detection and assessment of DDH [44]. Local practices and preferences of the treating orthopedic surgeons may vary, particularly outside of the United States.

Positive Ortolani or limited/asymmetric abduction — For infants (of any age) with a positive Ortolani maneuver (ie, a reducible dislocated hip) or limited or asymmetric abduction (ie, a nonreducible dislocated hip), we suggest referral to an orthopedic surgeon who is experienced in the diagnosis and treatment of DDH for further evaluation and management. Decisions regarding the imaging studies can be deferred to the consultant, who may use a variety of studies during management and follow-up.

Positive Barlow — For infants (of any age) with a positive Barlow maneuver (ie, a dislocatable or subluxatable hip), we suggest hip surveillance (ie, serial follow-up examinations) to ensure the development of hip stability. Hip surveillance may be performed by the primary care clinician or an orthopedic surgeon who is experienced in the diagnosis and management of DDH according to local practice and preference.

Suboptimal or inconclusive examination

Newborn – If the newborn examination is suboptimal (eg, the infant is not relaxed enough for a reliable assessment of motion and stability) or inconclusive (eg, no evidence of instability, but with joint clicks or thigh asymmetry), the infant is re-examined at the next health supervision visit (usually at two to four weeks of age).

Two to four weeks – For two- to four-week-old infants with inconclusive examination (eg, no evidence of instability, but with joint clicks or thigh asymmetry), we suggest referral to an orthopedic surgeon who is experienced in the diagnosis and treatment of DDH for further evaluation and management. Alternative courses of action, depending upon the level of suspicion, include [2,44]:

Ultrasonography at six to eight weeks (adjusted for prematurity), at which point "minor" ultrasonographic abnormalities found in newborns should be resolved (see 'Ultrasonography' above and 'Natural history' above)

Continuing to follow clinically (see 'Examination' above)

Four weeks and older – For infants four weeks and older with subtle or nonspecific findings, we suggest imaging or referral. We perform ultrasonography if the infant is younger than six months and radiographs if the infant is older than six months of age. (See 'Diagnostic imaging' above.)

Normal examination and risk factors — Decisions regarding imaging of infants with normal physical examination and risk factors for DDH are best made on a case-by-case basis [53]. The risk of dislocation is greatly reduced if the newborn examination is normal [2].

We obtain imaging (by ultrasonography at four to six weeks of age [adjusted for prematurity]) for infants with any of the following risk factors:

Male and female infants with breech positioning at ≥34 weeks gestation (whether or not external cephalic version was successful or was necessary)

Family history of DDH (including hip replacement for dysplasia before age 40 years in a close relative)

History of clinical instability on examination

If ultrasonography is unavailable or a child with a risk factor presents at six months or older, screening may be done with a radiograph of the hips and pelvis. (See 'Ultrasonography' above and 'Radiographs' above.)

The hips of infants with risk factors for DDH who have normal examination and normal imaging studies at six weeks (ultrasonography) or four months (radiographs) should continue to be examined at subsequent health supervision visits until the child is walking independently. Normal clinical examination at six weeks of age does not definitively exclude DDH [54]. The pertinent examination findings vary depending on age (table 2). (See 'Examination' above and 'Age-specific findings of DDH' above.)

Normal ultrasonography at six weeks of age is not necessarily predictive of absence of dysplasia. In observational studies, 7 to 29 percent of infants with breech presentation and normal hip ultrasonography at six weeks of age had hip dysplasia on follow-up radiographs [55,56]. This suggests that infants with breech presentation require vigilant monitoring for signs of DDH (table 2) until the child is walking normally.

Given regional variability in performance and interpretation of hip ultrasonography and thresholds for treatment, it is reasonable for clinicians to develop regional protocols in collaboration with a consulting pediatric orthopedic surgeon and pediatric radiologist [2].

Our strategy for selective screening with ultrasonography is consistent with the ACR Appropriateness Criteria [42]. The AAP clinical practice guideline recommends that imaging before six months of age be considered for male or female infants with suspicious or inconclusive physical examination or normal hip examination and any of the following: history of breech presentation in the third trimester (regardless of mode of delivery); positive family history; history of previous clinical instability or tight lower extremity swaddling; or caregiver concern [2]. The AAOS clinical practice guideline recommends imaging for infants with breech presentation, family history of DDH, or history of clinical instability on examination [4]. (See "Developmental dysplasia of the hip: Epidemiology and pathogenesis", section on 'Risk factors'.)

Normal examination and no risk factors — The hips of infants with normal examination should continue to be examined for signs of DDH at subsequent health supervision visits. Normal findings at age six to eight weeks do not definitively exclude DDH [54]. The pertinent examination findings vary with age (table 2). (See 'Examination' above and 'Age-specific findings of DDH' above.)

DIFFERENTIAL DIAGNOSIS — The major considerations in the differential diagnosis of DDH in infants include other causes of leg-length discrepancy (in infants with a positive Galeazzi test (figure 2)), such as [57]:

Proximal femoral focal deficiency, an uncommon congenital condition with a spectrum that ranges from hypoplasia of the femoral head to congenital absence of all but the distal femoral epiphysis (see "Radiologic evaluation of the hip in infants, children, and adolescents", section on 'Proximal femoral focal deficiency')

Coxa vara, defined by an angle of less than 120° between the femoral neck and shaft, which results in elevation of the greater trochanter (figure 11) (see "Radiologic evaluation of the hip in infants, children, and adolescents", section on 'Developmental coxa vara')

Hemihypertrophy or hemihyperplasia (eg, Beckwith-Wiedemann syndrome) (see "Beckwith-Wiedemann syndrome")

Sacral agenesis with limb deformity (see "Closed spinal dysraphism: Pathogenesis and types", section on 'Caudal regression or sacral agenesis')

SCREENING FOR DDH — Recommendations for screening newborn infants for DDH vary from country to country. Some countries recommend screening of all infants, whereas others recommend screening only those with selective risk factors [58]. The age at which the child is screened and the approach to screening (eg, clinical examination versus ultrasonography versus an approach based on risk stratification) also varies from country to country [36,58].

The American Academy of Orthopaedic Surgeons clinical practice guideline recommends against universal ultrasound screening of infants for DDH [4]. The Pediatric Orthopaedic Society of North America (POSNA) acknowledges the lack of data to support screening [5,59] but points to the value of early diagnosis in preventing long-term complications. POSNA recommends following the American Academy of Pediatrics clinical practice guideline with clinical assessment for DDH at birth and every well-child visit until the child is walking normally and selective imaging of infants with risk factors [2]. This approach is supported by a decision analysis that recommends screening of all infants with physical examination combined with selective use of ultrasound in infants that are high risk [60]. This is especially relevant, given reports that missed diagnosis of DDH is a common cause for malpractice suits against pediatricians [61]. The United States Preventive Services Task Force no longer addresses screening for DDH [62].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Developmental dysplasia of the hip".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient education" and the keyword[s] of interest.)

Basics topic (see "Patient education: Developmental dysplasia of the hip (The Basics)")

SUMMARY AND RECOMMENDATIONS

Terminology – Developmental dysplasia of the hip (DDH) describes a spectrum of conditions related to the development of the hip in infants and young children. It encompasses abnormal development of the acetabulum and proximal femur, as well as mechanical instability of the hip joint (table 1). (See "Developmental dysplasia of the hip: Epidemiology and pathogenesis", section on 'Terminology'.)

Hip examination – Hip examination should occur soon after birth and at every health supervision visit until approximately nine months of age and/or the child is walking independently. The symptoms and signs of DDH vary with age and severity (table 2). (See 'Overview' above and 'Age-specific findings of DDH' above.)

Physical examination techniques to detect hip instability use abduction and elevation to feel for reducibility (the Ortolani maneuver) and gentle adduction without downward pressure to feel for dislocatability (the Barlow maneuver). The sensation of reducibility or dislocatability is distinct and best described as a "jerk" or "clunk." (See 'Hip instability' above.)

Examination findings that are less specific for DDH include asymmetry (of femur length, skin folds (figure 3A-B), or gait) and decreased hip abduction. (See 'Asymmetry' above and 'Range of motion' above.)

Diagnostic imaging – Diagnostic imaging is helpful in confirming physical examination findings, evaluating infants with risk factors, and making treatment decisions. During the first four months of life, ultrasonography is the primary imaging technique for assessing the morphology and stability of the hip. Ultrasonographic measurements (figure 7) are used to classify DDH according to the Graf system (figure 8). (See 'Ultrasonography' above.)

Diagnosis – The diagnosis of DDH in infants is usually made by physical examination demonstrating hip instability, asymmetry (in unilateral cases), and/or limited abduction. Absence of asymmetry does not exclude DDH because DDH is bilateral in as many as 37 percent of cases. Examination findings of DDH vary according to age (table 2). Diagnostic imaging can be used to make the diagnosis in infants with inconclusive examination findings. Diagnostic imaging also may be used to make the diagnosis in infants who have risk factors and normal examination. (See 'Diagnosis' above.)

Approach to diagnosis and referral – Our approach to diagnosis of and referral for DDH varies with the examination findings, age of the infant, and risk factors for DDH:

For infants with a positive Ortolani maneuver or limited or asymmetric abduction, we suggest referral to an orthopedic surgeon who is experienced in the diagnosis and treatment of DDH. (See 'Positive Ortolani or limited/asymmetric abduction' above.)

For infants with a positive Barlow maneuver, we suggest serial follow-up examinations by the primary care clinician or an orthopedic surgeon who is experienced in the diagnosis and treatment of DDH to ensure development of hip stability. (See 'Positive Barlow' above.)

For newborns with suboptimal or inconclusive examination, we suggest re-examination at the next health supervision visit. For infants two to four weeks of age with inconclusive examination, we suggest referral to an orthopedic surgeon who is experienced in the diagnosis and treatment of DDH. For infants older than four weeks with inconclusive examination, we suggest imaging or referral. (See 'Suboptimal or inconclusive examination' above and 'Diagnostic imaging' above.)

We obtain ultrasonography at four to six weeks (adjusted for prematurity) of age for infants with normal examination and any of the following risk factors (see 'Normal examination and risk factors' above):

-Breech positioning at ≥34 weeks gestation

-Family history of DDH (including hip replacement for dysplasia before age 40 years in a close relative)

-History of clinical instability on examination

  1. Weinstein SL, Mubarak SJ, Wenger DR. Developmental hip dysplasia and dislocation: Part II. Instr Course Lect 2004; 53:531.
  2. Shaw BA, Segal LS, SECTION ON ORTHOPAEDICS. Evaluation and Referral for Developmental Dysplasia of the Hip in Infants. Pediatrics 2016; 138.
  3. Lambeek AF, De Hundt M, Vlemmix F, et al. Risk of developmental dysplasia of the hip in breech presentation: the effect of successful external cephalic version. BJOG 2013; 120:607.
  4. American Academy of Orthopaedic Surgeons. Detection and nonoperative management of pediatric developmental dysplasia of the hip in infants up to six months of age. Evidence-based clinical practice guideline. September 2014. http://www.aaos.org/research/guidelines/DDHGuidelineFINAL.pdf.
  5. Schwend RM, Schoenecker P, Richards BS, et al. Screening the newborn for developmental dysplasia of the hip: now what do we do? J Pediatr Orthop 2007; 27:607.
  6. Patel H, Canadian Task Force on Preventive Health Care. Preventive health care, 2001 update: screening and management of developmental dysplasia of the hip in newborns. CMAJ 2001; 164:1669.
  7. BARLOW TG. EARLY DIAGNOSIS AND TREATMENT OF CONGENITAL DISLOCATION OF THE HIP. Proc R Soc Med 1963; 56:804.
  8. Ortolani M. Congenital hip dysplasia in the light of early and very early diagnosis. Clin Orthop Relat Res 1976; :6.
  9. COLEMAN SS. Diagnosis of congenital dysplasia of the hip in the newborn infant. J Am Med Assoc 1956; 162:548.
  10. Bond CD, Hennrikus WL, DellaMaggiore ED. Prospective evaluation of newborn soft-tissue hip "clicks" with ultrasound. J Pediatr Orthop 1997; 17:199.
  11. Kane TP, Harvey JR, Richards RH, et al. Radiological outcome of innocent infant hip clicks. J Pediatr Orthop B 2003; 12:259.
  12. Nie K, Rymaruk S, Paton RW. Clicky hip alone is not a true risk factor for developmental dysplasia of the hip. Bone Joint J 2017; 99-B:1533.
  13. Dezateux C, Brown J, Arthur R, et al. Performance, treatment pathways, and effects of alternative policy options for screening for developmental dysplasia of the hip in the United Kingdom. Arch Dis Child 2003; 88:753.
  14. Baronciani D, Atti G, Andiloro F, et al. Screening for developmental dysplasia of the hip: from theory to practice. Collaborative Group DDH Project. Pediatrics 1997; 99:E5.
  15. Poul J, Bajerova J, Sommernitz M, et al. Early diagnosis of congenital dislocation of the hip. J Bone Joint Surg Br 1992; 74:695.
  16. Fulton MJ, Barer ML. Screening for congenital dislocation of the hip: an economic appraisal. Can Med Assoc J 1984; 130:1149.
  17. Burger BJ, Burger JD, Bos CF, et al. Neonatal screening and staggered early treatment for congenital dislocation or dysplasia of the hip. Lancet 1990; 336:1549.
  18. Groarke PJ, McLoughlin L, Whitla L, et al. Retrospective Multicenter Analysis of the Accuracy of Clinical Examination by Community Physicians in Diagnosing Developmental Dysplasia of the Hip. J Pediatr 2017; 181:163.
  19. Dunn PM. Perinatal observations on the etiology of congenital dislocation of the hip. Clin Orthop Relat Res 1976; :11.
  20. Anderton MJ, Hastie GR, Paton RW. The positive predictive value of asymmetrical skin creases in the diagnosis of pathological developmental dysplasia of the hip. Bone Joint J 2018; 100-B:675.
  21. Ando M, Gotoh E. Significance of inguinal folds for diagnosis of congenital dislocation of the hip in infants aged three to four months. J Pediatr Orthop 1990; 10:331.
  22. Screening for the detection of congenital dislocation of the hip. Arch Dis Child 1986; 61:921.
  23. Novacheck TF. Developmental dysplasia of the hip. Pediatr Clin North Am 1996; 43:829.
  24. Choudry Q, Goyal R, Paton RW. Is limitation of hip abduction a useful clinical sign in the diagnosis of developmental dysplasia of the hip? Arch Dis Child 2013; 98:862.
  25. Castelein RM, Korte J. Limited hip abduction in the infant. J Pediatr Orthop 2001; 21:668.
  26. Sankar WN, Neubuerger CO, Moseley CF. Femoral anteversion in developmental dysplasia of the hip. J Pediatr Orthop 2009; 29:885.
  27. Loder RT, Skopelja EN. The epidemiology and demographics of hip dysplasia. ISRN Orthop 2011; 2011:238607.
  28. Wedge JH, Wasylenko MJ. The natural history of congenital dislocation of the hip: a critical review. Clin Orthop Relat Res 1978; :154.
  29. Wedge JH, Wasylenko MJ. The natural history of congenital disease of the hip. J Bone Joint Surg Br 1979; 61-B:334.
  30. Weinstein SL. Natural history of congenital hip dislocation (CDH) and hip dysplasia. Clin Orthop Relat Res 1987; :62.
  31. Crawford AH, Mehlman CT, Slovek RW. The fate of untreated developmental dislocation of the hip: long-term follow-up of eleven patients. J Pediatr Orthop 1999; 19:641.
  32. Bialik V, Bialik GM, Blazer S, et al. Developmental dysplasia of the hip: a new approach to incidence. Pediatrics 1999; 103:93.
  33. Castelein RM, Sauter AJ. Ultrasound screening for congenital dysplasia of the hip in newborns: its value. J Pediatr Orthop 1988; 8:666.
  34. Terjesen T, Holen KJ, Tegnander A. Hip abnormalities detected by ultrasound in clinically normal newborn infants. J Bone Joint Surg Br 1996; 78:636.
  35. Marks DS, Clegg J, al-Chalabi AN. Routine ultrasound screening for neonatal hip instability. Can it abolish late-presenting congenital dislocation of the hip? J Bone Joint Surg Br 1994; 76:534.
  36. Dezateux C, Rosendahl K. Developmental dysplasia of the hip. Lancet 2007; 369:1541.
  37. Harris NH, Lloyd-Roberts GC, Gallien R. Acetabular development in congenital dislocation of the hip. With special reference to the indications for acetabuloplasty and pelvic or femoral realignment osteotomy. J Bone Joint Surg Br 1975; 57:46.
  38. Schwend RM, Pratt WB, Fultz J. Untreated acetabular dysplasia of the hip in the Navajo. A 34 year case series followup. Clin Orthop Relat Res 1999; :108.
  39. Wood MK, Conboy V, Benson MK. Does early treatment by abduction splintage improve the development of dysplastic but stable neonatal hips? J Pediatr Orthop 2000; 20:302.
  40. Lehmann HP, Hinton R, Morello P, Santoli J. Developmental dysplasia of the hip practice guideline: technical report. Committee on Quality Improvement, and Subcommittee on Developmental Dysplasia of the Hip. Pediatrics 2000; 105:E57.
  41. Elbourne D, Dezateux C, Arthur R, et al. Ultrasonography in the diagnosis and management of developmental hip dysplasia (UK Hip Trial): clinical and economic results of a multicentre randomised controlled trial. Lancet 2002; 360:2009.
  42. Expert Panel on Pediatric Imaging:, Nguyen JC, Dorfman SR, et al. ACR Appropriateness Criteria® Developmental Dysplasia of the Hip-Child. J Am Coll Radiol 2019; 16:S94.
  43. Dias JJ, Thomas IH, Lamont AC, et al. The reliability of ultrasonographic assessment of neonatal hips. J Bone Joint Surg Br 1993; 75:479.
  44. AIUM-ACR-SPR-SRU Practice Parameter for the Performance of an Ultrasound Examination for Detection and Assessment of Developmental Dysplasia of the Hip. J Ultrasound Med 2018; 37:E1.
  45. Graf R. New possibilities for the diagnosis of congenital hip joint dislocation by ultrasonography. J Pediatr Orthop 1983; 3:354.
  46. Harcke HT, Pruszczynski B. Hip ultrasound for developmental dysplasia: the 50% rule. Pediatr Radiol 2017; 47:817.
  47. Quader N, Schaeffer EK, Hodgson AJ, et al. A Systematic Review and Meta-analysis on the Reproducibility of Ultrasound-based Metrics for Assessing Developmental Dysplasia of the Hip. J Pediatr Orthop 2018; 38:e305.
  48. Harcke HT, Grissom LE. Performing dynamic sonography of the infant hip. AJR Am J Roentgenol 1990; 155:837.
  49. Hensinger RN. Standards in Pediatric Orthopedics: Tables, Charts, and Graphs Illustrating Growth, Lippincott Williams & Wilkins, Philadelphia 1986. p.68.
  50. Desai AA, Martus JE, Schoenecker J, Kan JH. Spica MRI after closed reduction for developmental dysplasia of the hip. Pediatr Radiol 2011; 41:525.
  51. Jaramillo D, Villegas-Medina O, Laor T, et al. Gadolinium-enhanced MR imaging of pediatric patients after reduction of dysplastic hips: assessment of femoral head position, factors impeding reduction, and femoral head ischemia. AJR Am J Roentgenol 1998; 170:1633.
  52. Mulpuri K, Song KM, Gross RH, et al. The American Academy of Orthopaedic Surgeons Evidence-Based Guideline on Detection and Nonoperative Management of Pediatric Developmental Dysplasia of the Hip in Infants up to Six Months of Age. J Bone Joint Surg Am 2015; 97:1717.
  53. Pacana MJ, Hennrikus WL, Slough J, Curtin W. Ultrasound Examination for Infants Born Breech by Elective Cesarean Section With a Normal Hip Exam for Instability. J Pediatr Orthop 2017; 37:e15.
  54. Reidy M, Collins C, MacLean JGB, Campbell D. Examining the effectiveness of examination at 6-8 weeks for developmental dysplasia: testing the safety net. Arch Dis Child 2019; 104:953.
  55. Morris AR, Thomas JMC, Reading IC, Clarke NMP. Does Late Hip Dysplasia Occur After Normal Ultrasound Screening in Breech Babies? J Pediatr Orthop 2019; 39:187.
  56. Imrie M, Scott V, Stearns P, et al. Is ultrasound screening for DDH in babies born breech sufficient? J Child Orthop 2010; 4:3.
  57. Godley DR. Assessment, diagnosis, and treatment of developmental dysplasia of the hip. JAAPA 2013; 26:54.
  58. Shipman SA, Helfand M, Moyer VA, Yawn BP. Screening for developmental dysplasia of the hip: a systematic literature review for the US Preventive Services Task Force. Pediatrics 2006; 117:e557.
  59. Broadhurst C, Rhodes AML, Harper P, et al. What is the incidence of late detection of developmental dysplasia of the hip in England?: a 26-year national study of children diagnosed after the age of one. Bone Joint J 2019; 101-B:281.
  60. Mahan ST, Katz JN, Kim YJ. To screen or not to screen? A decision analysis of the utility of screening for developmental dysplasia of the hip. J Bone Joint Surg Am 2009; 91:1705.
  61. McAbee GN, Donn SM, Mendelson RA, et al. Medical diagnoses commonly associated with pediatric malpractice lawsuits in the United States. Pediatrics 2008; 122:e1282.
  62. United States Preventive Services Task Force. Developmental hip dysplasia: Screening. Available at: https://www.uspreventiveservicestaskforce.org/BrowseRec/InactiveTopic/224 (Accessed on October 15, 2018).
Topic 6288 Version 28.0

References

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