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Developmental dysplasia of the hip (DDH) is a spectrum of disorders of development of the hip that present in different forms at different ages. The common etiology is excessive laxity of the hip capsule with a failure to maintain the femoral head within the acetabulum. The syndrome in the newborn consists of instability of the hip such that the femoral head can be displaced partially (subluxated) or fully (dislocated) from the acetabulum by an examiner. The hip may also rest in a dislocated position and be reducible on examination. Over time, the femoral head becomes fully dislocated and cannot be reduced by changing the position of the hip. In some infants, the clinical examination results are negative, but abnormalities found with the use of ultrasonography and radiographic studies portend later hip dysplasia. The syndrome may manifest later during childhood or adolescence as a dislocated hip or during adolescence as a hip with poorly developed acetabular coverage; the latter is termed dysplasia of the hip.
DDH is a disorder that evolves over time. The structures that make up the hip are normal during embryogenesis and gradually become abnormal for a variety of reasons, the chief being the fetal position and presentation at birth (e.g., malposition of the femoral head, abnormal forces acting on the developing hip) and the laxity of the ligamentous structures around the hip joint.
The older term congenital dislocation of the hip has gradually been replaced by developmental dysplasia, which was introduced during the 1980s to include infants who were normal at birth but in whom hip dysplasia or dislocation subsequently developed. The American Academy of Pediatrics defines DDH as a condition in which the femoral head has an abnormal relationship to the acetabulum. The abbreviation DDH has been used to denote both dislocation and dysplasia of the hip, and it is used in both senses in this chapter. Dislocation is defined as the complete displacement of a joint, with no contact between the original articular surfaces. Subluxation is defined as the displacement of a joint with some contact remaining between the articular surfaces. Dysplasia refers to the deficient development of the acetabulum.
Teratologic dislocation of the hip is a distinct form of hip dislocation that usually occurs with other disorders. The hips of patients with this condition are dislocated before birth, have a limited range of motion, and are not reducible on examination. Teratologic dislocation of the hip is usually associated with other neuromuscular syndromes, especially those related to muscle paralysis (e.g., myelodysplasia, arthrogryposis). The pathologic process, natural history, and management of teratologic dislocation are discussed separately.
In 1832, Guillaume Dupuytren described the condition of dislocation of the hip at birth and called it “original or congenital dislocation of the hip.” At the turn of the 20th century, Adolph Lorenz demonstrated his vigorous techniques of closed reduction of the hip ; however, because his reductions were so forceful, he has been called the “father of avascular necrosis.” Putti recommended early treatment, before the patient was 1 year old.
In 1937, Ortolani—another person who is famously associated with hip dislocation—described both a “click” or “jerk sign” of dislocation and a “click” or “jerk sign” of reduction. LeDamany actually described something similar in 1912.
Probably one of the most important treatment advances was the introduction by Arnold Pavlik in 1946 of the harness that bears his name. The simple stirrup device allowed for active movement to guide the dislocated hip into the socket. Pavlik’s work was stimulated by dissatisfaction with the rates of avascular necrosis (AVN) that he saw with existing treatment methods. In 1959, he reported the management of 1424 hips without a single case of AVN.
The incidence of DDH is difficult to determine because of disparities in the definition of the condition, the type of examinations used to detect hip abnormalities, the differing skill levels of examiners, and the populations being studied ( Box 13.1 ). Estimates of the incidence of some degree of hip instability in the newborn have ranged from a low of 1 per 1000 to a high of 3.4 per 100. Higher incidences are reported when screening involves both clinical examination and ultrasonography.
Dislocation: 1.4 per 1000 births
Clinical finding: 2.3 per 100 births
Ultrasound abnormality: 8 per 100 births
The data regarding the incidence of actual dislocation of the hip are more consistent, with reports ranging from 1.0 to 1.5 cases per 1000 live births. a
a References , , , , , , .
Geographic and racial variations in the incidence of DDH are marked; some areas of the world have a high endemic incidence, whereas in other areas the condition is virtually nonexistent. The reported incidence on the basis of geography ranges from 1.7 per 1000 live births in Sweden to 75 per 1000 live births in what was then Yugoslavia to 188.5 per 1000 live births in a certain district in Manitoba, Canada ( Table 13.1 ). An interesting study of more than 6000 specimens from a medieval British cemetery found evidence of DDH in 2.7 “cases” per 1000, which is a rate similar to that found by modern studies. Certain racial groups have a low incidence of DDH (e.g., African Bantu, Chinese), whereas other groups have a high incidence (e.g., Navajo Native American children).
Study, Year | Geographic Area: Population | Incidence per Thousand |
---|---|---|
Walker, 1973 | Island Lake Region (Manitoba, Canada): Canadian Indians | 188.5 |
Klisic, 1975 a | Belgrade, Yugoslavia | 75.1 |
Coleman, 1956 | Utah | 20.0 |
Hiertonn and James, 1968 | Adademiska Sjulchuset, Uppsala, Sweden | 20.0 |
Stanisavljevic, 1961 b | Detroit, Michigan | 10.0 |
Paterson, 1976 c | Adelaide, Australia | 6.2 |
Von Rosen, 1962 d | Malmö, Sweden | 1.7 |
Barlow, 1962 | Salford, England | 1.5 |
Hoaglund et al, 1981 | Hong Kong: Chinese | 0.1 |
Edlestein, 1966 | Africa: Bantus | 0.0 |
a Klisic P. Congenital dysplasia of the hip in the first year of life: incidence, diagnosis, and spontaneous evolution [in Serbian]. Srp Arh Celok Lek . 1968;96:961.
b Stanisavljevic S. Clinical findings in examination of hips in newborn babies. Henry Ford Hosp Med Bull . 1962;10:245.
c Paterson D. The early diagnosis and treatment of congenital dislocation of the hip. Aust N Z J Surg . 1976;46:359.
d von Rosen S. Diagnosis and treatment of congenital dislocation of the hip joint in the new-born. J Bone Joint Surg Br . 1962;44:284.
Although there is no single cause of DDH, a number of predisposing factors have been identified ( Box 13.2 ). These factors include ligamentous laxity, prenatal positioning, postnatal positioning, and racial predilection. The etiology of DDH is clearly multifactorial, and it is influenced by hormonal and genetic elements.
Ligamentous laxity (often inherited)
Breech position (especially footling)
Postnatal positioning (hips swaddled in extension)
Primary acetabular dysplasia (unlikely)
Ligamentous laxity is related to DDH in several ways. The condition is associated with the development of DDH when laxity is a familial trait. In fact, the racial incidence of laxity may parallel racial predilections for DDH. The newborn’s response to maternal relaxin hormones may explain the higher incidence of DDH among girls. These hormones, which produce the ligamentous laxity that is necessary for the expansion of the maternal pelvis, cross the placenta and induce laxity in the infant. This effect is much stronger in female than in male children.
In 1970, during an extensive genetic study of DDH, Wynne-Davies proposed that heritable ligamentous laxity was one of two major mechanisms for the inheritance of DDH ( Fig. 13.1 ). She believed that this was an autosomal dominant characteristic with incomplete penetrance. The fact that the risk of DDH is 34% in identical twins (i.e., both twins having DDH) but only 3% in fraternal twins also suggests a genetic influence. In Coleman’s study of Navajo families, hip dysplasia in one family member increased the risk for other family members fivefold. Newborns with DDH have also been found to have a higher ratio of collagen III to collagen I as compared with control subjects, which suggests a connective tissue abnormality in those with DDH. In a study of laxity by distraction of the symphysis pubis, infants with DDH had twice the amount of distraction of the symphysis pubis as compared with control subjects.
Several animal studies are relevant to the issue of laxity of the hip capsule. When the hip capsule and the ligamentum teres were removed from dogs, the animals frequently developed dislocated hips. Dislocation would also result if the capsule was mechanically stretched but not removed. Alternatively, removal of the acetabular roof resulted not in hip dislocation but only in a shallower acetabulum than normal. In a classic study of male and female newborn rabbits, only the female rabbit hips dislocated when the knees were splinted in extension; this observation supports the concept of hormonally induced laxity.
Prenatal positioning is strongly associated with DDH. Although only 2% to 3% of infants are born in breech presentation, 16% of infants with DDH are born in breech presentation. Neonates who were carried in certain breech positions in utero have a significantly higher risk of DDH ( Fig. 13.2 ). The breech effect is most notable when the knees are extended, with an incidence of 20% seen for a single or frank breech. Alternatively, the footling breech position, in which the hips are flexed, is associated with only a 2% incidence of DDH. Lowry and co-workers have shown a significant reduction in the incidence of DDH when the baby is delivered preterm by cesarean section (3.7%) as compared with those delivered vaginally (8.1%).
Experimental studies in which newborn rabbits’ knees were splinted in extension showed a high incidence of hip dislocation. If, in the same rabbits, the hamstring tendons were transected, the hips did not dislocate; this suggests that the pull of the hamstring across the flexed hip was the dislocating factor. The hip is affected by intrauterine position, and delivery by cesarean section does not alter the likelihood of hip dislocation. The incidence of DDH is also higher among first-born children and in those pregnancies that are complicated by oligohydramnios. , , These findings suggest that there is an intrauterine crowding effect on the developing hip. This argument is bolstered by the increased incidence of other postural abnormalities (e.g., torticollis, metatarsus adductus) in children with DDH. In addition, the left hip is more often involved than the right. Because the most common intrauterine position has the left hip adducted against the maternal sacrum, some authors believe that this position places the left hip at greater risk for dislocation as compared with the right hip. ,
Postnatal positioning is another factor that is associated with DDH. People who wrap their newborn babies in a hip-extended position (e.g., Native Americans who use cradleboards: Fig. 13.3 ) have a much higher incidence of DDH as compared with other populations. , , The mechanism of action is believed to be the placement of the hips in full extension against the normal neonatal hip flexion contracture. An archaeologic study of prehistoric adults found a high incidence of DDH (46/1000) which correlated with head molding from cradleboard immobilization. By contrast, people who usually carry their infants astride the hip or in a wrap that flexes and abducts the hips have a lower incidence of DDH as compared with other groups. A group studied more than 40,000 children in Malawi where a back-carry position similar to the Pavlik position is universally used. Over a 10-year period no cases of DDH were found. Postnatal positioning programs have been considered. In one such approach, new parents were given a set of “abduction pants” and wide diapers; a 65% decrease in the incidence of DDH was noted and attributed to the program.
The primary failure of acetabular development has been proposed as a cause of DDH. Early cadaver studies noted that the acetabulum is shallower at birth than during the earlier fetal period , , ; full coverage does not occur until 3 years of age. Beals believes that acetabular dysplasia is inherited and that it may be a precursor of dislocation. After birth, the acetabulum becomes deeper throughout childhood, and it eventually covers the head completely. However, other authors have rejected the primary acetabular dysplasia hypothesis. ,
Racial predilection apparently plays a role; certain ethnic groups seem to be predisposed to DDH, whereas others appear to be somewhat immune. Blacks and Asians have relatively low incidences of DDH (0.1 per 1000 to 5 per 1000), , whereas whites and Native Americans have higher incidences (15 per 1000).
Certain conditions, particularly postural abnormalities, are associated with DDH more commonly than chance alone would dictate ( Box 13.3 ). The association of DDH with torticollis is strong ( Fig. 13.4 ). In a child with torticollis, the likelihood of DDH ranges from 5% to 20%; this is thought to result from intrauterine crowding. b
b References , , , , , , , .
A relationship has also been noted between DDH and metatarsus adductus ( Fig. 13.5 ), with the incidence of concurrence ranging from 1.5% to 10%. , Clubfoot has not been shown to have a significant relationship with DDH. , Pregnancies that are complicated by oligohydramnios have been associated with an increased incidence of DDH. , First-born white infants have a higher incidence of DDH. c
c References , , , , , .
First-term hyperthyroidism has also been associated with increased incidence of DDH.
Torticollis: 15%–20%
Metatarsus adductus: 1.5%–10%
Oligohydramnios
The hip joint begins to develop at approximately the seventh week of gestation, when a cleft appears in the mesenchyme of the primitive limb bud ( Fig. 13.6 ). These precartilaginous cells differentiate into a fully formed cartilaginous femoral head and acetabulum by the eleventh week of gestation. , , The concave shape of the acetabulum is determined by the structure (femoral head) within the acetabulum. If there is a failure during the normal embryogenesis of the hip, the consequence is a major anomaly (e.g., proximal femoral focal deficiency).
At birth, the neonatal acetabulum is completely composed of cartilage, with a thin rim of fibrocartilage called the labrum ( Fig. 13.7 ). The hyaline cartilage of the acetabulum is continuous with the triradiate cartilages, which divide and interconnect the three osseous components of the pelvis (i.e., the ilium, the ischium, and the pubis). The surface of the acetabular cartilage, which abuts the bone of the pelvis, is made up of epiphyseal cartilage in the shape of a hemisphere; it functions as a major growth plate. The growth of this physis is essential for acetabular development, and any damage to the periacetabular area may induce a growth disturbance. , , The labrum also contributes significantly to the development of acetabular depth; thus any excision of the labrum during the treatment of DDH is ill advised. The majority of acetabular shape development is determined by approximately 8 years of age. Late acetabular development during adolescence is enhanced by the growth of the secondary acetabular centers such as the os acetabulum.
The proximal femur has a complex and often misunderstood growth pattern. In the neonate, the entire upper femur is a cartilaginous structure in the shape of a femoral head and the greater and lesser trochanters. The development of the proximal segment of the femur occurs through a combination of appositional growth on the surfaces of the upper femur and epiphyseal growth at the juncture of the cartilaginous upper femur and the femoral shaft. In the normal femur, an ossification center appears in the center of the femoral head between the fourth and seventh months of postnatal life. This center grows until physeal closure during late adolescence; at this time, it has become the adult femoral head, and it is covered with a thin layer of articular cartilage. During the period of growth, the thickness of the cartilage surrounding this bony nucleus gradually decreases, as does the thickness of the acetabular cartilage. The thickness of the cartilage accounts for the widened radiographic appearance of a normal hip in a child.
As the child matures, three acetabular epiphyseal centers develop and are responsible for the final contours of the hip socket ( Fig. 13.8 ). The os acetabulum, which is the largest of the three, appears at approximately 8 years of age and forms along the anterior wall as part of the pubis. The acetabular epiphysis, which also ossifies at approximately 8 years of age, forms along the superior edge of the acetabulum as part of the ilium; it fuses when the child is approximately 18 years old. The third center is a small epiphysis in the posterior or ischial area, which develops when the child is 9 years old and fuses when he or she is 17 years old.
Excessive pressure on the cartilaginous upper femur can cause a loss of vascular perfusion, which results in the necrosis of the chondrocytes. Various portions of the femoral head and growth plate can be injured, with the resulting patterns of deformity corresponding with the areas of injury. The greater trochanteric area is usually unaffected; it will continue to grow normally, gradually becoming more proximal than the femoral head. This “trochanteric overgrowth” is actually normal trochanteric growth in the presence of upper femoral “undergrowth.” , ,
Muscle imbalance can also significantly affect the growth and morphology of the upper femur. Excessive adductor pull or inadequate abductor muscle function results in a valgus deformity of the upper femur. , ,
DDH is a gradually progressive disorder that is associated with distinct anatomic changes, many of which are initially reversible. It is a malformation of anatomic structures that have developed normally during the embryologic period. Relatively gentle forces, persistently applied, are probably the cause of such deformations. At birth, the affected hip will spontaneously slide into and out of the acetabulum. For this to occur, the posterosuperior rim of the acetabulum has to have lost its sharp margin and become flattened and thickened in the area over which the femoral head slides ( Fig. 13.9 ). As the head rides in and out of the socket, a ridge of thickened articular cartilage (called the neolimbus by Ortolani) arises along the posterosuperior acetabular wall ( Fig. 13.10 ). The sliding of the head in and out produces a “clunk.” The neolimbus is the structure that produces this feel as the head slides over it. , , ,
Some hips that are unstable at birth spontaneously reduce and become normal, with complete resolution of the aforementioned anatomic changes. Other hips eventually remain out of the socket permanently, and many secondary anatomic changes take place gradually. The frequency of spontaneous reduction as compared with progressive dislocation is not known.
In those hips that remain dislocated, secondary barriers to reduction develop. In the depths of the acetabulum, the fatty tissue known as the pulvinar thickens and may impede reduction ( Fig. 13.11 ). The ligamentum teres also elongates and thickens, and it may take up valuable space within the acetabulum. The transverse acetabular ligament is often hypertrophic as well, and it may impede reduction. More important, the inferior capsule of the hip assumes an hourglass shape, eventually presenting an opening that is smaller in diameter than the femoral head. The iliopsoas, which is pulled tight across this isthmus, contributes to this narrowing ( Fig. 13.12 ). The capsule also narrows through a “Chinese finger-trap” mechanism. The femoral changes are minimal and include an increase in anteversion and some flattening of the femoral head as it lies against the ilium.
When an attempt is made to reduce the hip against the narrowed hip capsule, the femoral head abuts the cartilaginous acetabular lip, and it tends to push this rim into the acetabulum. It is extremely important to realize that the acetabular structure is not impeding the femoral head from entering the acetabulum. Rather, the constricted hip capsule is forcing the head against the acetabular rim, and the capsule must be released or stretched to allow the head to move beneath the acetabular rim and enter the acetabulum. Clinicians often use the term labrum for this blocking structure, and sometimes they excise it. However, the actual labrum is a thin fibrocartilaginous rim around the periphery of the acetabular cartilage. The blocking structure encountered in patients with DDH is not only the labrum but also a significant portion of the cartilaginous acetabulum itself. This vital cartilaginous acetabular anlage is essential for the normal growth and development of the acetabulum, and it should not be excised.
After the femoral head has been reduced, the acetabular rim may still impede the deep seating of the femoral head because the rim has become thicker than normal. If the head is maintained within the acetabulum, this thickened cartilage will usually flatten out gradually and allow the head to seat deeply. Known clinically as “docking the head,” this phenomenon was described by Severin in 1941. The femoral head itself is usually deformed into a globular shape as a result of pressure against the lateral portion of the acetabulum, and it may not be congruous with the acetabulum at the time of reduction; however, this anatomic situation also eventually resolves if reduction is maintained.
When a stable reduction is obtained, the acetabulum gradually remodels. This remodeling increases the depth of the acetabulum, and the acetabular angle gradually becomes more horizontal. During the acetabular remodeling period, secondary ossification centers often appear prematurely in the acetabulum.
If the hip remains dislocated, additional changes occur during the growth and development of the acetabulum. The acetabular roof becomes progressively more oblique, the concavity gradually flattens and eventually presents a convex surface, and the medial wall of the acetabulum thickens. The acetabulum has been noted to be excessively anteverted in patients with DDH, thus providing diminished coverage of the femoral head. , Medial twisting of the whole wing of the pelvis has been demonstrated by magnetic resonance imaging (MRI) in patients with untreated DDH. Medial wall thickening is seen radiographically as a thickening and alteration of the shape of the teardrop body. Although acetabular anteversion is present in the young hip, retroversion of the acetabulum has been found in adolescents and adults with hip dysplasia.
To a point, these changes are reversible, but the exact upper age at which hip reduction will result in normal acetabular development is uncertain. Harris suggested that a hip reduced by the time a patient was 4 years old could achieve “satisfactory” acetabular development. He found that significant acetabular growth continued through 8 years of age.
In adults, the fully dislocated femoral head may lie well above the acetabular margin in a markedly thickened hip capsule; this is the so-called “high-riding dislocation” ( Fig. 13.13 ). The adult dislocated femoral head is oval and flattened medially. The acetabulum is filled with fibrous tissue, hypertrophied ligamentum teres, and thickened transverse acetabular ligament, and the articular cartilage is either atrophic or absent. The muscles that insert at the proximal femur are foreshortened and more horizontally oriented ( Fig. 13.14 ). Fully dislocated adult hips may remain free from degenerative changes for many years, even for the individual’s lifetime.
In some untreated hips, the femoral head retains some contact with the acetabulum. These subluxated hips have an unstable contact area that allows the head to slide proximally and distally against a widened and oblique acetabular surface. This instability produces degenerative changes that often become apparent during late adolescence and that usually progress rapidly within a few years to severe degeneration. Late radiographic changes include subchondral sclerosis and cyst formation in the acetabulum and the femoral head, osteophyte formation, and the loss of articular cartilage. The reorientation of the acetabulum and the redirection of the forces across the hip can ameliorate degenerative changes if they are performed before severe degeneration occurs.
The fate of the unstable hip remains an enigma. How often an unstable hip spontaneously reduces—or, alternatively, becomes dislocated, subluxated, or dysplastic—remains a subject of controversy. A primary problem is the definition of an unstable hip. Traditionally, instability has been defined by a positive result on the Ortolani or Barlow test. However, the classification has been complicated by the inclusion of hips that are clinically stable but that have abnormal ultrasonographic characteristics. Thus the criteria that an investigator uses to define abnormal hips must be taken into consideration when evaluating any study of hip instability.
Barlow found 1 hip in 60 that he examined to exhibit his instability sign; 60% normalized within 1 week, and 88% were corrected within 2 months without treatment. Coleman’s natural history study in Navajo children found that 5 of 23 Ortolani-positive hips spontaneously corrected, with the remainder being dysplastic, subluxated, or dislocated. Yamamuro followed 52 newborns with untreated instability. Three of 12 Ortolani-positive hips resolved, and 24 of 42 subluxatable hips also resolved.
The term dysplasia refers to a radiographic finding of increased obliquity and the loss of the concavity of the acetabulum, with an intact Shenton line ( Fig. 13.15 ). The term subluxation is used when the femoral head is not in full contact with the acetabulum ( Fig. 13.16 ). The radiographic findings of subluxation include a widened teardrop femoral head distance, a reduced center–edge angle, and a break in the Shenton line. The term dislocation specifies that the femoral head is not in contact with the acetabulum. Both subluxated and dislocated hips have dysplastic changes.
Dysplasia is a direct result of abnormal forces across the acetabulum. The lateralization of the femoral head results in increased forces over a smaller unit area with an increase in the sheer vector. Increased sheer vectors affect the physes of the acetabulum. With an acetabular inclination of more than 15 degrees, the sheer and lateralization forces exceed the medialization forces, and progressive subluxation is inevitable. Roof osteophytes will form at synovial attachment sites. These progress, forming a pseudoacetabulum that increases the contact surface area of fibrocartilage at the acetabular margin. Joint contact area determines the cartilage stress. Stress may rise from 20 to 320 kp/cm 2 .
An abductor lurch is an effective adaptation in that the abductor forces are reduced to the point that only body weight forces are carried through the hip. When the pelvis drops, however, the head coverage decreases, and this has a negative biomechanical effect.
Dysplastic hips without subluxation usually become painful and develop degenerative changes over time. These hips often become subluxated as the degenerative disease progresses. Cooperman and associates reported that all dysplastic hips without subluxation with a center–edge angle of less than 20 degrees sustained osteoarthritic changes over 22 years of follow-up ; however, no direct correlation of the center–edge angle with the development of arthritis was demonstrated. It has been shown that a hip with an acetabular angle of 35 degrees or more 2 years after reduction has an 80% probability of becoming a Severin class III or IV hip, which will likely require later hip replacement. It is estimated that 20% to 50% of cases of degenerative arthritis of the hip are the result of subluxation or residual acetabular dysplasia. d
d References , , , , , , , .
The only guarantee of a lifetime of normal hip function is a completely normal radiographic appearance of the hip.
The subluxated hip always leads to symptomatic degenerative hip disease. , , , , The affected individual often presents with gradually increasing pain in one or both hips but no history of hip symptoms or treatment. After the pain begins, it tends to progress rapidly over a period of months. Severe subluxation leads to symptoms during the second decade of life, moderately subluxated hips become painful during the third and fourth decades, and the least severely subluxated hips become symptomatic during the fifth and sixth decades. , In one study, the hips with well-developed false acetabula had the highest incidence of pain and disability.
A completely dislocated hip causes symptoms much later than a subluxated hip; in some individuals, these hips never become painful. No strict linear relationship between center–edge angle, acetabular angle, and osteoarthritis has been found. , Hip, knee, and back pain have been noted in approximately half of the patients with untreated DDH. Other studies have emphasized cases in which there were no symptoms or few symptoms despite lifelong hip dislocation. , ,
Other degenerative and functional problems develop in people with untreated dislocated hips. , , , , Unilateral dislocations cause limb length inequality, ipsilateral valgus knee deformity, an abnormal gait, decreased agility, and postural scoliosis. Bilateral cases are associated with significant back pain as a result of increased lumbar lordosis.
DDH in the neonate is diagnosed by eliciting Ortolani or Barlow sign or from significant changes seen in the sonographic morphology of the hip. The unstable hip may either stabilize spontaneously or become dysplastic or dislocated over a period of several months.
The hip examination ( Box 13.4 ) of the neonate requires an artful approach in which the setting must be controlled and the examiner experienced. The first requisite is a relaxed child. To achieve this, the infant may need a bottle; the examination surface should be warm and comfortable, and the room should be reasonably quiet. A firm examination surface is best, but if the parent’s lap keeps the child more comfortable, it will suffice.
The “feel” of this examination is most important, and it is not unlike palpation of the liver. Movement of the hip in and out of the socket is a delicate event that is best appreciated with a very light touch. The examiner holds the child’s knees, one in each hand, and examines one hip at a time.
In the test for Barlow sign, the examiner attempts to slide the femoral head out of the acetabulum ( Fig. 13.17 ). The hip is adducted, and a gentle push is applied to slide the hip posteriorly. The examiner’s fingers are positioned over the greater trochanter, and the trochanter is allowed to move laterally. In a positive test, the hip will be felt to slide out of the acetabulum. As the examiner relaxes the proximal push, the hip can be felt to slip back into the acetabulum.
The Ortolani test is the reverse of the Barlow test: the examiner attempts to reduce a dislocated hip ( Fig. 13.18 ). The examiner grasps the child’s thigh between the thumb and the index finger and, with the fourth and fifth fingers, lifts the greater trochanter while simultaneously abducting the hip. When the test result is positive, the femoral head will slip into the socket with a delicate “clunk” that is palpable but not audible. The examiner should repeat this sequence four or five times to be certain of the findings, alternating the Barlow test and the Ortolani test in a gentle arc of motion. The other hip is then examined in the same manner. During the newborn period, there are usually no other signs of abnormality.
This examination is subject to many factors that can affect its effectiveness and reliability. The hurried examiner usually fails to appreciate the instability. It is possible to examine a hip throughout 15 maneuvers and to feel the instability only the sixteenth time that the hip is moved. The explanation is that this “feel” is quite delicate and requires just the right degree of relaxation on the part of the examiner as well as the infant. Many examiners report a “click”: a high-pitched snap, often felt at the extremes of abduction. This click may originate in the ligamentum teres or occasionally in the fascia lata or psoas tendon, and it usually does not indicate a significant hip abnormality ; however, one study reported a 9.35-fold increase in the incidence of abnormal results on ultrasonography in children with simple clicks, and another found a 1.5% incidence of DDH when a click was the only finding. One study of 256 patients found no abnormalities with this finding.
It is important to plan appropriate follow-up for children after the initial evaluation. Occasionally, patients who had a negative clinical examination during the neonatal period present at an older age with dysplasia ( Fig. 13.19 ). Imaging studies—ultrasonography for the infant and pelvic radiography for the child who is older than 6 months—should be done in children with risk factors that include breech presentation in a girl and positive family history. Whether imaging is necessary for all children who are referred remains unclear.
As the child enters the second and third months of life, other signs of DDH appear (see Box 13.4 ). It is important to recall that the progression from instability to dislocation during the newborn period is a gradual process. In some children, an irreducible dislocation develops within a few weeks, whereas in others the hip dislocation remains reducible until they are 5 or 6 months old. When the hip is no longer reducible, specific physical findings appear, including limited abduction, shortening of the thigh, proximal location of the greater trochanter, asymmetry of the thigh folds, and pistoning of the hip.
The limitation of abduction, which is the most reliable sign of a dislocated hip, is best appreciated by abducting both hips simultaneously with the child on a firm surface ( ). A unilateral dislocation produces a visible reduction in abduction on the affected side as compared with the normal side ( Fig. 13.20 ). Shortening of the thigh (Galeazzi sign) is best appreciated by placing both hips in 90 degrees of flexion and comparing the height of the knees, again looking for asymmetry ( Fig. 13.21 ). Because the thigh is foreshortened, there will be more thigh folds on the affected side than on the normal side ( Fig. 13.22 ). Although this sign is always present with a unilateral dislocation, extra thigh folds are a common normal variant and do not necessarily indicate hip dislocation.
A potentially perilous situation for the unwary examiner is the child with bilateral hip dislocation. This child has no asymmetry of abduction, and the flexed knees are at the same level. Combined abduction is limited, but this is difficult to detect because the limitation is symmetric. One test that can help the examiner to recognize a bilateral dislocation is the Klisic test, in which the examiner places the third finger over the greater trochanter and the index finger on the anterior superior iliac spine. An imaginary line drawn between the fingers should point to the umbilicus. When the hip is dislocated, the more proximal greater trochanter causes the line to point approximately halfway between the umbilicus and the pubis ( Fig. 13.23 ).
These examinations are capricious, and the clinician should use imaging studies to evaluate infants with questionable findings and those with risk factors that are associated with DDH. These risk factors include a family history of DDH, breech position, oligohydramnios, torticollis, and metatarsus adductus. The significantly higher frequency of DDH among girls as compared with boys must also be considered. The reexamination of a child a few months later helps to decrease the possibility of missing a dislocation. It is imperative that experienced orthopaedic practitioners provide education to primary care providers regarding examination for DDH.
The unilateral dislocated hip produces distinct clinical signs in a walking child ( ; see Box 13.4 ). Although some authors have suggested that children with DDH are late to start walking, more recent studies have shown no significant delay. , The affected side appears to be shorter than the normal extremity, and the child toe-walks on the affected side. With each step, the pelvis drops as the dislocated hip adducts, and the child leans over the dislocated hip; this is known as an abductor lurch or Trendelenburg gait ( Fig. 13.24 ). When the child attempts to stand on that foot with the other elevated off of the floor, he or she leans toward the affected side (Trendelenburg sign). As in the younger child, there is limited abduction on the affected side, and the knees are at different levels when the hips are flexed (Galeazzi sign).
In the walking child, bilateral dislocation is more difficult to recognize than unilateral dislocation. There is usually a lurching gait on both sides, but some children mask this rather well, showing only an increase in the dropping of the pelvis during the stance phase. Excessive lordosis is common, and it is often the presenting complaint ( Fig. 13.25 ). The lordosis is the result of hip flexion contracture, which is usually present. The knees are at the same level, and abduction is symmetric but limited. There is usually an excessive internal and external rotation of the dislocated hips.
The neonate’s hip is a difficult structure to image with standard radiographic techniques because the hip is composed primarily of cartilage and soft tissue. Ultrasonography is a modality which gives the examiner a detailed view of soft and hard tissues about the hip, both in static and mobile modes. A number of technics are in use and many studies have defined parameters of normal, and indications for treatment.
Graf in 1980 first described his technique using a lateral imaging technique with the transducer placed over the greater trochanter ( Fig 13.26 ). He subsequently developed a classification of abnormalities which is in use today ( Table 13.2 ).
Class | Alpha Angle | Beta Angle | Description | Treatment |
---|---|---|---|---|
Standard Classification | ||||
I | >60° | <55° | Normal | None |
IIa | 50°–60° | 55°–77° | Immature (<3 mo) | Observation |
IIb | >50°–60° | 55°–77° | >3 mo | Pavlik harness |
IIc | 43°–49° | >77° | Acetabular deficiency | Pavlik harness |
IId | 43°–49° | >77° | Everted labrum | Pavlik harness |
III | <43° | >77° | Everted labrum | Pavlik harness |
IV | Unmeasurable | Unmeasurable | Dislocated | Pavlik harness/closed vs. open reduction |
Simplified Classification | ||||
I | >60° | <55° | Normal | None |
II | 43°–60° | 55°–77° | Delayed ossification | Variable |
III | <43° | >77° | Lateralization | Pavlik harness |
IV | Unmeasurable | Unmeasurable | Dislocated | Pavlik harness/closed vs. open reduction |
Harcke and Kumar in the 1980s reported dynamic studies in which the hip is moved to reproduce the Barlow and Ortolani maneuvers, and the degree of subluxation is documented with ultrasonography. , During the first few days of life, 4 to 6 mm of motion is considered normal, and definite treatment indications developed on the basis of stress views are still evolving. ,
Tersen in 1989 described an ultrasonic measure of femoral head coverage which has also had wide usage, with a 50% coverage considered to be normal.
A number of recent reviews have compared the several classification systems and their reproducibility. ,
Graf’s classification system is based on the angles formed by the sonographic structures of the hip ( Fig. 13.26D ). The “baseline” is the line of the ilium as it intersects the bony and cartilaginous portions of the acetabulum. The “inclination line” is the line along the margin of the cartilaginous acetabulum. The third line is the “acetabular roofline” along the bony roof. The intersection of the roofline and the baseline forms the alpha angle, whereas the intersection of the inclination line and the baseline forms the beta angle. A smaller alpha angle indicates a shallower bony acetabulum. A smaller beta angle indicates a better cartilaginous acetabulum. In other words, as the femoral head subluxates, the alpha angle decreases, and the beta angle increases. The classification is illustrated in Table 13.2 .
In the Graf classification class I hips are normal, class II hips are either immature or somewhat abnormal, class III hips are subluxated, and class IV hips are dislocated. Class I hips need no follow-up, whereas class III and IV hips usually require treatment. Class II hips form the group in which the degree of abnormality and the need for treatment are less clear. Graf subdivided class II in several ways in different publications (see Table 13.2 ). He noted that stage IIc is the most important to identify because it represents a preluxation-phase hip that will subsequently dislocate. He emphasized that the probe should be perpendicular to the acetabulum as well as to the cut in the center of the acetabulum. , ,
Treatment philosophies regarding abnormalities in Graf class II hips vary widely (some authors treat only those hips with clinical instability), regardless of sonographic findings. Others treat all class II hips with abduction devices. Exact treatment guidelines are lacking. Graf recommends treating IIa hips, while others begin treatment for IIb hips but not IIa. In the newborn period the ultrasound images show transient abnormalities, and in most centers the most useful exam is done at age 6 weeks. There is wide agreement that treatment decisions should be based on ultrasonography examinations performed at 6 weeks of age or later to allow for hip maturity.
The Harcke method uses four views, the frontal neutral view, the frontal flexion view, the transverse neutral view, and the transverse flexion view. With his method the instability is evaluated with a combination of two perpendicular planes, with and without stress. Thus the position, stability, and morphology of the hip joint are assessed. The findings are reported as normal, subluxated, slightly dislocated, or dislocated.
The main finding of the Terjesen method is the percentage of coverage of the femoral head. The lower normal limit for the coverage value is 50% in infants older than 1 month of age. In addition, the alpha angle of Graf is measured, and the shape of the lateral bony rim is defined as normal, defective, or rounded. The exact percentage of head coverage which requires treatment is as yet not clearly defined ( Figs. 13.27 and 13.28 ).
Bialik and colleagues provided some useful guidelines for the use of ultrasonography with their protocol to reduce the number of hips that are treated unnecessarily by delaying the start of treatment pending sonographic reexamination. Neonates with hips that were stable during their initial examination were reexamined clinically and with ultrasonography at 6 weeks of age, whereas those with unstable hips were reexamined at 2 weeks of age. If the ultrasonographic study showed no improvement of the unstable hips at the second examination, treatment with the Pavlik harness was begun. At the end of the established waiting periods, 90% of the abnormal hips had become normal without treatment. Only 3% of the Graf IIa hips failed to normalize without treatment, whereas 17% of Graf III hips and 25% of Graf IV hips failed to normalize. Slightly more than half of the hips treated had no clinical instability. Their outcome, had they not been treated, remains speculative. A review of 5 years of experience with a universal screening program in Germany found that the need for surgical treatment was reduced but not eliminated for children with DDH. Other authors have reported a more dramatic reduction in surgical rates in response to universal screening.
A number of authors believe that ultrasonography is too sensitive and results in the overtreatment of hips that would otherwise develop normally. Screening studies have shown that only 0.012% of hips that are normal on clinical examination have evidence of dysplasia later and that most Graf IIa hips normalize without treatment. When ultrasonography was used for screening, the treatment rate doubled as compared with using clinical findings alone. Another study found that only 9.5% of infants with abnormal ultrasound scans had clinical signs of DDH.
It is now clear that ultrasonography is a valuable adjunct to the detection of neonatal hip abnormalities. The experienced examiner will frequently encounter babies whose hip exams are normal, but whose ultrasound exams show definite abnormality. Studies suggest that screening with ultrasonography does pick up clinically silent hips without increasing the rate of treatment for minor abnormalities that would resolve spontaneously. , The exact indications for treatment by ultrasonographic criteria are still being refined.
Ultrasonography is also very useful for detecting early treatment failures when using the Pavlik harness or other treatment modalities. It is important to note that a normal result with ultrasonography does not completely preclude later abnormalities. Several cases of dysplasia at walking age have been reported in children who had normal ultrasonographic findings during the neonatal period. Imre studied 300 babies who were born breech; of those with normal examinations and ultrasound studies, 29% later had abnormal radiographs of the hips at 5 months of follow-up. Thus we also must conclude that a “normal” ultrasound at 6 weeks of age does not guarantee a normal hip later in life.
Plain radiography of the pelvis usually demonstrates a frankly dislocated hip in individuals of any age. In newborns with typical DDH, however, the unstable hip may appear radiographically normal. As the child reaches 3 to 6 months of age, the dislocation will be evident radiographically, but the examiner must be familiar with the landmarks of the immature pelvis to recognize the abnormality. In the infant, the upper femur is not ossified, and most of the acetabulum is cartilaginous. The triradiate cartilage lies between the ilium, the ischium, and the pubis.
Several classic lines are helpful when evaluating the immature hip ( Figs. 13.29–13.31 ). The Hilgenreiner line is a line through the triradiate cartilages. The Perkin line, which is drawn at the lateral margin of the acetabulum, is perpendicular to the Hilgenreiner line. The Shenton line is a curved line that begins at the lesser trochanter, goes up the femoral neck, and connects with a line along the inner margin of the pubis. In a normal hip, the medial beak of the femoral metaphysis lies in the lower, inner quadrant produced by the juncture of the Perkin and Hilgenreiner lines. The Shenton line is smooth in the normal hip. In the dislocated hip, the metaphysis lies lateral to the Perkin line; the Shenton line is broken because the femoral neck lies cephalic to the line from the pubis.
Another useful measurement is the acetabular index, which is an angle formed by the juncture of the Hilgenreiner line and a line drawn along the acetabular surface (see Fig. 13.30 ). In normal newborns, the acetabular index averages 27.5 degrees. At 6 months of age, the mean is 23.5 degrees. By 2 years of age, the index usually decreases to 20 degrees. Thirty degrees is considered the upper limit of normal. , , The acetabular index of the weight-bearing zone or the sourcil is normally less than 15 degrees. ,
In the older child, the center–edge angle is a useful measure of hip position (see Fig. 13.31 ). This angle is formed at the juncture of the Perkin line with a line that connects the lateral margin of the acetabulum to the center of the femoral head. In children who are 6 to 13 years old, an angle of more than 19 degrees has been reported as normal; in children who are 14 years old and older, an angle of more than 25 degrees is considered normal.
A helpful radiographic projection is the Von Rosen view, in which both hips are abducted, internally rotated, and extended. In the normal hip, an imaginary line extended up the femoral shaft intersects the acetabulum. When the hip is dislocated, the line crosses above the acetabulum.
The acetabular teardrop figure, as seen on an anteroposterior (AP) radiograph of the pelvis, is formed by several lines. It is derived from the wall of the acetabulum laterally, the wall of the lesser pelvis medially, and a curved line inferiorly, and it is formed by the acetabular notch. The teardrop appears between 6 and 24 months of age in a normal hip and later in a dislocated hip. In a study by Smith and associates, the teardrop did not appear until hips were reduced, but the teardrop was present in dislocated hips by 29 months of age in a study by Albiñana and associates. When the hip is dislocated or subluxated, the acetabular portion of the teardrop loses its convexity, and the teardrop is wider from the superior to the inferior directions. The reduced hip remodels the acetabulum, and the teardrop gradually narrows. Hips in which the teardrop appears within 6 months of reduction have a better outcome than hips in which the teardrop appears later. Four types of teardrop bodies have been noted: open, closed, crossed, and reversed. The teardrops have also been described as U- or V-shaped, with a V-shaped teardrop being associated with a dysplastic hip and a poor outcome ( Fig. 13.32 ).
Another measure of acetabular dysplasia is the acetabular index of depth to width in which the depth of the central portion of the acetabulum is divided by the width of the acetabular opening, with normal being more than 38%. The femoral head extrusion index represents the percentage of the femoral head that lies outside of the acetabulum.
The false-profile radiographic view represents a lateral view of the acetabulum, and it is especially useful for evaluating anterior acetabular dysplasia. , , The patient is positioned 65 degrees obliquely to the x-ray beam, with the foot parallel to the cassette. The extent of anterior coverage is represented by a dense line of ossification known as the sourcil, the limit of which is sometimes difficult to define. An acetabular angle can be constructed; the mean value is 32.8 degrees, with a range of 17.7 to 53.6 degrees ( Fig. 13.33 ).
The Severin classification has been used for many years to specify outcome in hips that have been treated for DDH ( Table 13.3 ). However, in 1997, Ward and associates reported poor levels of intraobserver and interobserver reliability when the system was used. The interpretive ambiguities and lack of objective measures emphasize the need for a more reliable scheme.
Class | Radiographic Appearance | Center–Edge Angle (Age) |
---|---|---|
Ia | Normal | >19° (6–13 yr) |
>25° (≥14 yr) | ||
Ib | Normal | 15°–19° (6–13 yr) |
20°–25° (≥14 yr) | ||
IIa | Moderate deformity of femoral head, femoral neck, or acetabulum | Same as class I |
III | Dysplasia without subluxation | <15° (6–13 yr) |
<20° (≥14 yr) | ||
IVa | Moderate subluxation | ≥20° |
IVb | Severe subluxation | <0° |
V | Femoral head articulates with pseudoacetabulum in superior part of original acetabulum | |
VI | Redislocation |
Although parents may become concerned about radiation exposure during the course of their child’s management, the increase in carcinogenic risks from the cumulative radiographs taken to manage an average DDH case have been estimated to be less than 1%.
The arthrographic anatomy of the hip was well described by Severin in 1941. In the normal hip, the free border of the labrum is easily seen as a sharp “thorn” overlying the femoral head ( Fig. 13.34 ). A recess of joint capsule overlies this thorn. The capsule expands beyond this recess and is then constricted by the ringlike zona orbicularis. In a child with DDH, when the hip is in the dislocated position, the acetabular edge is seen, and the capsule is enlarged as it extends over the femoral head. The capsule is constricted at its middle portion into an hourglass shape by the iliopsoas tendon.
When the hip is placed into a reduced position, it may reduce fully against the acetabular wall, or it may “dock” against the labrum and the capsular constriction of the iliopsoas (see Fig. 13.48B ). When the reduction is deep, the labrum lies flat over the head and has a sharp border. When the head is docked, the labrum is blunted and interposed between the head and the acetabular wall. The ligamentum teres is seen within the joint, and it may be outlined by contrast material. A bulge in the acetabular cartilage beneath the labrum (the neolimbus) may be seen. If the reduction is stable and the hip is immobilized in a safe position, then the femoral head will gradually overcome the capsular tightness. Arthrography repeated 6 weeks later shows the head as being well seated in the acetabulum.
Arthrography should usually be performed with the patient under general anesthesia. We prefer the median, subadductor approach with image intensification ( Fig. 13.35 ). The needle is inserted just beneath the adductor longus, approximately 2 cm distal to its origin. If the starting point is too close to the adductor’s origin, the needle will encounter the inferior portion of the acetabulum rather than the joint itself. The needle is directed medially and aimed toward the contralateral sternoclavicular joint. When resistance is encountered, the position of the needle is noted on the image. The needle should be directed toward the joint space. A small amount of contrast material is injected to be certain that the joint has been entered; the contrast agent should flow freely around the femoral head. Another 1 mL of contrast agent is injected, and the needle is removed. Permanent films should be obtained for each significant position of the hip. It is important to note the positions of maximum stability and instability.
MRI affords excellent anatomic visualization of the infant hip, but it is not commonly used because of the expense involved and the need for sedation. Kashiwagi and associates proposed an MRI-based classification of hips with DDH. Group 1 hips had a sharp acetabular rim, and all were reducible with a Pavlik harness. Group 2 hips had a rounded acetabular rim, and almost all could be reduced with a Pavlik harness. Group 3 hips had an inverted acetabular rim, and none was reducible with the harness. MRI findings include the widening of the iliac bone, the lateral drift of the superior and posterior portions of the acetabular floor, the overgrowth of the acetabular cartilage, and the convexity of the posterior portion of the acetabular cartilage. ,
MRI with gadolinium-contrast arthrography is an important tool for the evaluation of the adolescent patient with hip dysplasia and pain. This technique allows for the evaluation of the condition of the labrum and the articular cartilage of the hip joint. Disruption and tears of the labrum, cartilage delamination, and articular cartilage loss can be identified with this technique. ,
All neonates should undergo a clinical examination for hip instability. Beyond that recommendation, there is a lack of consensus with regard to the need for further screening. Most authors agree that infants with risk factors associated with DDH should receive more careful screening that includes at least an examination by an experienced examiner and an ultrasound study of the hip. These risk factors include first-born female, a family history of DDH, and breech birth position. Clinical findings of torticollis, metatarsus adductus, and oligohydramnios may be associated with an increased incidence of hip instability with some studies showing a relationship and others not. , , , . First-born whites also have an increased risk for DDH relative to other racial groups. e
e References , , , , , .
The need for screening with ultrasonography remains controversial. In addition to the added cost, the disadvantage of ultrasound screening of all newborns is the identification of a large number of children with sonographic abnormalities for which there are no firm treatment guidelines. Some authors recommend ultrasonography in combination with clinical examination for all infants with appropriate risk factors, although others found a low yield of significant abnormalities in the absence of clinical findings, even in hips that were considered to be at risk. The American Academy of Pediatrics has issued a practice guideline that recommends radiographic screening (ultrasonography) for female infants who were either carried in the breech position or have a positive family history of DDH. Alternatively, the US Preventive Services Task Force—in accordance with a best-evidence review—concluded that the “net benefits” of screening could not be determined; they found that there was a high rate of spontaneous resolution of the abnormality and a lack of evidence of the effectiveness of intervention on functional outcome.
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