COMMON ORTHOPEDIC PROBLEMS I

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DEVELOPMENTAL DYSPLASIA OF THE HIP

Tom F. Novacheck, MD

The purpose of this article is to emphasize the essential points regarding screening for and detection of developmental dysplasia of the hip (DDH). Background information is provided as necessary to understand why this entity presents in a variable fashion. This article is not intended to comprehensively cover treatment methodology for the orthopedist. Instead, the information provided is meant to aid the primary care practitioner in identification of the problem and in supporting the family during the treatment process when an abnormality is detected. The term developmental dysplasia of the hip is necessarily general and encompasses the many facets of the condition. DDH is variable at presentation but is defined as an abnormal formation of the hip joint occurring between organogenesis and maturity as a result of instability. It is intentionally nonspecific to include the entire spectrum of the disorder. The author finds it helpful to think of DDH as a spectrum in both time and severity. In other words, the individual findings depend extensively on the age of the child. They also depend heavily on the severity of that childs problem. This spectrum does not include hip abnormalities caused by other diseases, such as cerebral palsy or myelodysplasia. As in many other conditions, delays in detection lead to more severe consequences for treatment and prognosis; however, not all individuals at the same age of presentation have the same degree of

From the Motion Analysis Laboratory, Gillette Childrens Hospital; and the Department of Orthopaedic Surgery, University of Minnesota, St. Paul, Minnesota

PEDIATRIC CLINICS OF NORTH AMERICA

VOLUME 43 NUMBER 4 * AUGUST 1996

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abnormality. Therefore, the concept of DDH as a spectrum in time and severity should be useful in organizing one's understanding of this rather enigmatic problem. DDH was formerly known as congenital dislocation of the hip. The disease did not change, but there are some very good reasons to adopt the new terminology.' The change from congenital to developmental is crucial because clear evidence has shown that not all cases are diagnosable at birth.8,3,13 Hips that are found to be normal at birth (and even in the first few months of life) can subsequently be found to be abnormal later. In addition, this terminology acknowledges that with the passage of time, changes occur. In other words, a subluxatable hip, if untreated, may progress to become dislocatable or ultimately dislocated. The choice of the term dysplasia rather than dislocated acknowledges a wider spectrum of cases rather than only the more uncommon, most severe cases. To persist with the label dislocation is misleading and under-emphasizes the milder forms of DDH. This change in terminology has some obvious important legal implications. In the spectrum of instability, some hips are subluxatable, or "loose," in which the femoral head slides in the acetabulum. At rest they lie in the normal position against the floor of the acetabulum. Subluxated hips lie in a position away from the floor of the acetabulum. They also slide within the acetabulum. Dislocafable hips lie within the acetabulum at rest but have even more laxity and can be manually displaced from the acetabulum (dislocated) with a palpable "clunk." Dislocated hips rest in the dislocated position, that is, the femoral head rests outside of the acetabulum. The ability to reduce the hip depends on the age of the patient and length of time that the hip has been dislocated. Another term that warrants definition is dysplasia. In addition to its use in the naming of DDH, it can refer to the abnormal formation of tissue, including the femur, acetabulum, or soft tissues. It is a general term, and because it is nonspecific, one must be wary of how it is used.

EPIDEMIOLOGY In general, the incidence in white neonates is 1.0% for dysplasia and 0.1% for dislocated hips.lo Incidence varies with race. There is an increased incidence in the Lapp and native American populations and a decreased incidence in the black, Korean, and Chinese populations. There is a fourfold increased likelihood in females, and the left side is involved in 60% of cases (right 20%, bilateral 20%). Associated birth factors include breech presentation, torticollis, talipes equinovarus, metatarsus adductus, and being first born. These fac-

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tors are believed to be packaging issues. Although 2% to 3% of all babies are breech presentations, 16% to 25% of DDH patients are born breech. Risk is relative to intrauterine position with cephalic 0.7%, footling breech 2.0%, and frank breech 20.0%. There is a familial predisposition that is believed to be multifactorial. DDH is present in 2% to 10% of siblings and in 1%to 2% of biologic parents. Idleberger7has documented a 34% risk for paternal twins and 3% for fraternal twins.

ETIOLOGY

Three theories have been postulated to account for the disease process of DDH: (1) mechanical, (2) primary acetabular dysplasia, and (3) ligamentous laxity. The mechanical theory helps to explain the increased incidence in firstborn and breech position as well as the association with the other packaging problems. It implies abnormal intrauterine positioning as a cause. The theory of primary acetabular dysplasia has two main proponents: (1) Faber5 and (2) Wynne-Davis.15 They believe that there is a primary acetabular dysplasia that predisposes to dislocation and an increased rate of acetabular dysplasia in biologic parents of patients with late DDH. The theory of ligamentous laxity is supported by Howorth and Massie.6 Wynne-Davis also has found an increased incidence of ligamentous laxity in neonatal DDH patients and their firstdegree relatives. The hormonal effects of relaxin on the collagenous structures of the infant are similar to those on the mother. This author believes that ligamentous laxity with or without associated mechanical factors is the basis for the pathologic changes and that the genetic aspects are related to familial laxity. A combination of this tendency with mechanical factors from malpositioning can cause the femoral head to move out of the confines of the acetabulum. The changes that occur in the acetabulum are secondary to these primary problems and are the result, rather than the cause, of the dysplasia.

PATHOLOGIC CHANGES IN PATIENTS WITH DDH

The pathologic changes in patients with DDH are the basis for understanding DDH as a spectrum in time and severity. Changes begin in the hip joint capsule. Laxity of this structure allows the femoral head to begin to migrate out of the acetabulum. Redundancy is present superolaterally. The labrum is a cartilaginous structure that normally enlarges the capacity of the bony acetabulum. With subluxation, the

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labrum everts. The ligamentum teres elongates. At this point, the femoral head is subluxated or subluxatable. With further laxity, the femoral head dislocates and the labrum inverts (Fig. 1A). At this point, intra-articular structures, including the ligamentum teres and the pulvinar (fibrofatty tissue in the floor of the acetabulum), may begin to hypertrophy. The labrum becomes pathologic in size and shape and is then referred to as the limbus. With these changes, the femoral head can no longer be relocated because of the obstruction to reduction caused by these soft tissues. The psoas tendon coursing across the front of the joint capsule causes a narrowing at the isthmus (Fig. 1B). The transverse acetabular ligament, capsule, limbus, pulvinar, and ligamentum teres all may have a role in preventing relocation of the femoral head, and at this point the dislocation is fixed. With the passage of time, contractures can develop in the iliopsoas and hip adductors, further prohibiting positioning of the leg and efforts to reduce the hip. Ultimately, because the growth forces have been abnormal, the cartilage models of the proximal femur and acetabulum deform with several possible consequences, including flattening of the femoral head, valgus neck-shaft angle, excessive femoral anteversion, and acetabular dysplasia.", l2

Figure 1. A, Inverted limbus. Infolding of the labrum prevents the femoral head from entering the acetabulum. A redundant and hypertrophied ligamentum teres and contracture of the transverse acetabular ligament also may hinder reduction. B, Hourglass configuration. The isthmus is caused by stretching and narrowing of the joint capsule and the iliopsoas tendon. (From Morrissy RT [ed]: Lovell and Winter's Pediatric Orthopaedics, ed. 3, vol. 2. Philadelphia, JB Lippincott, 1990, p 821; with permission.)

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NATURAL HISTORY

One should consider both the short-term and long-term outcomes for dysplastic hips. Barlow2 found that 1 in 60 infants at birth had evidence of instability, but that 58% became stable within 3.5 days. They had no residual abnormalities, and their hips went on to develop normally. The remaining children went on to develop hip dysplasia, either subluxation or dislocation. Hips that are abnormal after the immediate newborn period and remain untreated develop the long-term complications, including: osteoarthritis pain abnormal gait leg length discrepancy decreased agility Wedge and Wasylenko14 found that pain develops in subluxated hips earlier than in dislocated hips and therefore actually have a poorer long-term prognosis than hips with complete dislocation (Fig. 2). Osteoarthritis eventually develops in all, but pain develops in adolescents with

Figure 2. Anteroposterior radiograph of the left hip of a 35-year-old woman with pain in the left hip and marked early degenerative arthrosis caused by severe acetabular dysplasia.

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subluxation during the second decade, whereas persons with complete dislocation may not experience significant pain until their late third or early fourth decade. Regardless, both have a poor long-term outcome with pain and disability in early adulthood.

PRESENTATION

Because of the differences in severity of the dysplasia and the progressive changes that occur over time, presentation and the method of confirming the diagnosis are variable. Before walking age, the diagnosis is based on physical examination. Initially, the examination in the newborn nursery is the critical screening tool to detect loose, dislocatable, and dislocated hips. Detection up until approximately 4 to 6 months of age still depends primarily on the Barlow and Ortolani maneuvers as part of routine well-baby examinations. At approximately 6 months of age, the family may notice a difference in the range of motion with some difficulty with diapering. Occasionally the family may notice or the examination may indicate a leg length difference or an asymmetric appearance of the two legs (asymmetric skinfolds). The importance of frequent, routine screening hip examinations until walking age by an experienced examiner cannot be overemphasized. With the onset of walking, gait asymmetry (generally related to leg length discrepancy) is the hallmark for identifying an abnormal hip. Occasionally, asymmetric intoeing or outtoeing is the clue. If the hip abnormality remains undiagnosed, it will likely be the onset of pain or more noticeable gait asymmetry that draws attention to the hip as the child becomes older, larger, and more well developed. These various presentations are highlighted later in case presentations.

DIAGNOSIS

History

In recording the patient's medical history, one should have a higher index of suspicion if one or more of the many risk factors for DDH, including race, sex, family history, being first-born, and breech position, are present. None of these factors is sufficient alone to make the diagnosis, but their presence may cause sufficient concern to pursue further evaluation with ultrasonography, radiography, or pediatric orthopedic consultation. In the presence of one or more of these risk factors, further caution should be exercised and another level of screening considered.

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Examination
Physiologic hip flexion, abduction, and external rotation contractures are normal findings in neonatal hip examinations. Knee flexion contractures are also typical. These, of course, are related to normal intrauterine positioning. An asymmetric examination may be a subtle clue to the presence of hip dysplasia. As mentioned previously, the mainstay screening examination still consists of the Barlow provocative test and Ortolani reduction maneuver (Fig. 3). A helpful trick to remember is that the 0 Ortolani stands in for the hip is out. The examination is a reduction maneuver to place it back into a normal position. The Barlow provocative test, then, detects the subluxatable or dislocatable hip. The key to detection is not a click but rather a clunk. One tries to detect abnormal movement between the femoral head and the acetabulum. The author finds it helpful to minimize the movements of abduction and adduction and to emphasize the push of the Barlow test to try to move the femoral head posteriorly and the pull of the Ortolani reduction maneuver to draw the femoral

Flgure 3 A, The Ortolani (reduction) maneuver. In this technique, the hip is abducted . slightly. Then, with the index and long fingers over the greater trochanter, the thigh is raised (pulled forward) to gently reduce the hip. When performing the test, one hand should always be used to stabilize the pelvis; therefore, only one hip at a time can be examined. 3, The Barlow (provocative) test. The thumb is placed on the inner aspect of the thigh near the lesser trochanter. The hip is adducted. Longitudinal pressure is exerted on the thigh with the thumb, pushing it toward the table. The examiner again uses one hand to stabilize the pelvis, testing one hip at a time. (From Lovell WW, Winter RB [eds]: Pediatric Orthopaedics, ed. 2, vol. 2. Philadelphia, JB Lippincott, 1986, p 707; with permission.)

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head anteriorly back into place. One tries to detect translational movement between the femoral head and the pelvis. In the author's experience, if a lot of rotational movement occurs simultaneously (i.e., flexion/ extension or abduction/adduction), then detection of subtle translational movement is difficult. The child should be at rest and not struggling against the examiner. The author takes the approach to assume the hip is abnormal and to prove that it is not. If he is sure of the results of the examination, then no other evaluation is warranted. If not, then further assessment is indicated. The presence of asymmetric skinfolds is a sensitive but nonspecific indicator of abnormality. If the folds are symmetric, then it would be very unlikely that the hip is abnormal. Asymmetry is common and is present in approximately 30% of all infants. It can be used as an indicator to raise one's index of suspicion. This finding in the absence of any other abnormalities is insignificant. As time passes and secondary soft tissue contractures develop, the Barlow and Ortolani maneuvers become less dependable as the hip becomes fixed in its abnormal position. Limited abduction, skinfold asymmetry, and the Galeazzi sign (relative shortening of the femoral segment) become more important. Bilateral hip disease is especially difficult to identify because no asymmetry is present. At younger ages, the Barlow and Ortolani examinations are valid. After the development of secondary soft tissue contractures, findings on examination include widening of the perineum, symmetric limited abduction (less than 45"), and the appearance of abnormally short thigh segments for the child's overall size. After walking age, hyperlordosis and a waddling gait are the classic findings. Radiography Radiographs before 4 months of age can be fraught with problems and may provide a false sense of security if they seem normal. Because the secondary center of ossification of the femoral head has not yet developed, an abnormal relationship between the upper end of the femur and the acetabulum may not be apparent. In addition, unless the femoral head lies in an abnormal position at rest, the radiograph may provide false-negative information. Therefore, the only way that the radiograph can be useful is if it is abnormal. A normal radiograph before 4 months of age cannot rule out pathology. Figure 4 depicts several of the useful measurements that can be made to identify hip abnormalities. The reader should refer back to it for the case presentations later in the article. The better screening radiographic test prior to femoral head ossifi-

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B
Figure 4. A, Hilgenreiners line is a horizontal drawn through the triradiate cartilage. Perkins line is drawn perpendicular to it at the lateral edge of the acetabulum. With these lines, the area about each hip is divided into four quadrants. The ossific nucleus of the femoral head (if present) or the medial beak of the metaphysis should be in the inner lower quadrant. The appearance of the ossific nucleus often is delayed in hip dislocation. H = distance from highest point of femoral neck to Hilgenreiners line (decreased in hip dislocation); D = distance from triradiate cartilage to intersection of H with Hilgenreiners line (increased A in hip dislocation). 6, normal acetabular index (angle) of the left hip (<30 at birth) as compared with an abnormal acetabulum (and angle) on the right. (from Wenger DR, Rang M (eds): The Art and Practice of Childrens Orthopaedics. New York, Raven Press, 1993, pp 268-269; with permission of Lippincott-Raven Publishers.)

cation is ultra~onography.~ in any ultrasound assessment, its value As depends on the experience and ability of the operator. If the primary care practitioner has a good ultrasonographer available, then this tool can be valuable in detecting subtle abnormalities. After the appearance of femoral head ossification (typically at 4 to 6 months of age), the radiograph is more useful because abnormalities

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and asymmetries are more apparent. CT, bone scan, and MR imaging have no role in the detection of DDH.
TREATMENT

The essential, basic goals of treatment are the same regardless of the age at diagnosis. The treatment necessary to achieve those goals varies and depends on the age at diagnosis and the degree of abnormality. The goals are hierarchic and are as follows:
1. The hip must be concentrically reduced. The cartilage of the surface of the femoral head must lie directly on the cartilage of the floor of the acetabulum. There can be no intervening space or soft tissue between them. 2. When step 1 is achieved, stability must be obtained so that when the leg is allowed to move freely without support, it does not move away from the socket floor (subluxate) or dislocate. 3. All of the dysplastic features of bone and cartilage must resolve. These are the most long-lasting changes and the slowest to correct. For resolution to occur, the femoral head must be stable and located in the acetabulum. These dysplastic features include flattening of the femoral head, a misshapen acetabulum, and abnormal shape of the proximal femur (excessive femoral anteversion and increased neck-shaft angle).

All of these steps must be accomplished while avoiding complications. The two most important complications are failure to achieve all of these goals and aseptic necrosis (loss of the blood supply to the femoral head). Aseptic necrosis may be common, and its outcome can be worse than the initial problem. Failure to achieve the three goals also leads to a poor long-term outcome. Because of the progressive changes that occur over time, treatment options are frequently divided into age categories.
Zero to Six Months of Age

A small subset of children with mild detectable laxity during the first 3 days of life may not require immediate intervention because many resolve spontaneously as the effects of maternal hormones fade. If reevaluation at 3 to 5 days of age shows that the hips are completely stable, no treatment is necessary. Triple diapering is ill-advised in any situation because it promotes hip extension, which is an unfavorable position for normal hip development. For children who do need treatment, the mainstay choice is generally the Pavlik harness (Fig. 5). It is a dynamic splint that does not

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'

Figure 5. Application of the Pavlik harness. A, The chest halter is positioned at the nipple line and fastened with the Velcro closure. The crossed shoulder straps then stabilize the halter at this position. 6, The leg and foot are set into the stirrups and fastened with the Velcro straps. Care must be taken to ensure that the flexion and abduction straps are oriented anteriorly and posteriorly with respect to the child's knee. C, The anterior (flexion) stirrup straps are connected to the halter. The straps are adjusted so that hip flexion is between 100" and 110" to achieve optimum position of the femoral head relative to the acetabulum (the hips should be able to flex higher than this). One should think of these straps as checkreins that prevent hip extension. The insertion of the anterior stirrup straps on the halter must be at the child's anterior axillary line. 0, Lastly, the posterior (abduction) stirrup straps are attached to the halter. The insertion point of these straps on the halter should be located over the child's scapula. The posterior stirrup straps should be adjusted so that there are approximately 5 to 8 cm between the knees when both hips are adducted and the hips have free abduction. Likewise, these straps should be considered checkreins preventing adduction. (From Mubarak S, Garfin S, Vance R, et al: Pitfalls in the use of the Pavlik harness for treatment of congenital dysplasia, subluxation, and dislocation of the hip. J Bone Joint Surg 63A:1244, 1981; with permission.)

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rigidly immobilize the hips. Rather, the straps act as checkreins to not allow hip extension or adduction. By maintaining the hips in flexion and abduction, unstable hips can "tighten up," and dislocated hips can be reduced. It is generally a safe treatment with a high success rate and few complications. The length of treatment is variable, but a minimum of 6 weeks is usually necessary. Because of the rapid growth rate at this age, frequent adjustments (i.e., every 2 weeks) are necessary to maintain ideal position. If unsuccessful, a hip spica cast is usually the next choice for treatment for this age group. Because the Pavlik harness is a dynamic brace, it is not appropriate for older, stronger, or larger infants because they may overpower it.

Six to Eighteen Months of Age

In general, hips are not manually reducible in this age group. The author prefers to use traction to allow for some stretching of secondarily contracted soft tissues prior to attempted closed reduction. It is debatable whether prereduction traction protects against aseptic necrosis, but the author feels that the benefit obtained in soft tissue relaxation facilitates reduction and may allow nonoperative reduction in some children who may otherwise have needed an open, operative reduction. Skin traction safely can be accomplished at home9 and is generally used for 3 weeks. The child is then admitted for attempted closed reduction under general anesthesia. An arthrogram is used to confirm adequacy of reduction. If the reduction is acceptable, a hip spica cast is applied with the hips gently abducted and flexed 90" to 100". The hip at this point may not be concentrically reduced (a small residual pool of arthrogram dye remains between the cartilage of the femoral head and the floor of the acetabulum). The hip is not immediately stable in this position. In general, each of these problems improves with casting and is confirmed at the time of the first cast change at 4 to 6 weeks. Casts are changed while the child is under anesthesia every 6 weeks until the hip is stable and reduced, at which time casting is discontinued and a flexionabduction brace is applied. In general, two or three casts are needed. If the reduction is inadequate, surgical (open) reduction is necessary. If the family is aware ahead of time that an arthrogram is to be performed and the appropriateness of nonoperative treatment will be assessed with it, then one can proceed with surgery, if needed, while the child is under the same anesthetic. At this age, it is likely that the only surgery that will be necessary is to relieve soft tissue obstructions to reduction. In this age group, casting and the passage of time usually

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lead to stability of the hip in the located position with subsequent resolution of dysplastic features of bone and cartilage.
More than 18 Months of Age

In general, the secondary changes by this age are too severe to respond adequately to nonoperative intervention. Closed reduction is seldom successful, and open, surgical reduction is needed. Traction is less effective also. Instead a femoral shortening osteotomy is a useful option to avoid the excessive forces associated with aseptic necrosis. Derotation and varusization can be incorporated into the osteotomy to immediately eliminate dysplastic changes in the shape and alignment of the femur. Femoral osteotomy may help with the stability of the reduction and improve acetabular remodeling. An adductor tenotomy can be added at any age to improve the range of motion and joint stability of the hip at the time of open or closed reduction. At older ages, acetabular dysplasia may be too extensive, with insufficient remaining remodeling potential necessitating simultaneous acetabular redirectional osteotomy (Salter or Pemberton). Beyond 4 years of age in bilateral cases and 8 years in unilateral cases, reduction should not be attempted for two reasons: (1) the likelihood of aseptic necrosis is high, and (2) the potential for a poor result is high because of a mismatch between the shape and size of the femoral head and acetabulum with insufficient remodeling potential to accommodate to one another.
CASE PRESENTATIONS

This discussion emphasizes how the variable pathologies define how the child presents, the diagnosis is made, and treatment is chosen.
Case 1: Newborn Girl

A full-term girl was born via normal vaginal delivery, vertex presentation. Family history was negative. The Ortolani test was positive for a dislocatable hip on initial examination. The remainder of the examination was normal. The anteroposterior radiograph of the pelvis was normal (Fig. 6). A Pavlik harness led to stability of the hip within 2 weeks. At 6 weeks, it was discontinued.

Teaching Points

1. The diagnosis of dislocatable hip is made at this age by screening physical examination.

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Figure 6. Anteroposterior radiograph of the pelvis o a newborn infant. The proximal femur f and femoral head are nonossified. Drawing Hilgenreinersand Perkins lines would reveal that the proximal medial femoral metaphysis lies in the inferomedial quadrant (normal). Shentons line is intact. No apparent acetabular dysplasia is present. This radiograph i normal. s

2. The radiograph in this case is not helpful in identifying or ruling out problems because the hip is reduced when the child is at rest. In addition, the entire proximal femur is cartilaginous at this age, making interpretation of the empty space between the femoral shaft and the pelvis difficult at best. 3. Because of prompt treatment and early success, there are few, if any, dysplastic features of bone and cartilage. At this point, the child requires intermittent observation only and a followup radiograph at 6 and 12 months of age to confirm normal development.
Case 2: Seven-Month-Old Girl An otherwise healthy child was seen for routine well-baby examination and was found to have asymmetric skinfolds and limited abduction of the left hip. Ortolani and Barlow examinations were negative. A radiograph (Fig. 7) revealed left hip dislocation, delayed ossification of the left femoral head, and acetabular dysplasia. Skin traction at home for 3 weeks was followed by successful closed reduction confirmed by arthrography. Twelve weeks of casting led to stability and was followed by bracing until the acetabular dysplasia resolved.

Teaching Points
1. Physical examination remains the method by which problems are detected at this age.

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Figure 7. Anteroposterior radiograph of the pelvis of a 7-month-old girl. The left hip is completely dislocated. The left femur is displaced proximally and laterally compared with the right. Ossification of the left femoral head is delayed. Shenton's line is broken on the left and intact on the right. Marked acetabular dysplasia is present with an acetabular angle exceeding 30".

2. Secondary changes in the soft tissues lead to limited motion (decreased abduction) and prevent the relocation or movement of the hip (negative Ortolani and Barlow). 3. Treatment is more aggressive and longer in duration because of these secondary changes, but nonoperative management is still successful because the changes are not severe and growth potential is good, allowing for significant remodeling.
Case 3: 14-Month-Old Boy
An otherwise healthy child started walking at 12 months but presented with an asymmetric gait and "outtoeing" on the right. Physical examination subsequently revealed leg length discrepancy (left side short), asymmetric skinfolds, and limited abduction on the left. Ortolani and Barlow maneuvers were negative. Radiography (Fig. 8) showed the dislocation, delayed femoral head ossification, and dysplastic changes in the acetabulum. Prereduction traction allowed for sufficient relaxation to avoid femoral shortening, but attempted closed reduction was unsuccessful. Open reduction was followed by hip spica casting. Acetabular development was inadequate necessitating acetabular reconstruction 14 months later. The prognosis is good.

Teaching Points

1. After walking age, hip dysplasia generally is identified not by routine examination but by parental complaint when walking is found to be abnormal.

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Figure 8. Anteroposterior radiograph of the pelvis o a 14-month-oldboy. Complete dislocaf tion of the left hip is shown with lateralization and proximal migration of the left femur. Shenton's line is broken on the left and intact on the right. The acetabular angle is slightly increased on the left, indicating acetabular dysplasia. Ossification of the left femoral head is delayed.

2. Frequently, parents note the outtoeing on the normal side and believe that it is abnormal. It is the normal side while the dislocated left side is carried in a more neutral position. 3. Traction is still useful at this age, eliminating the need for osteotomy of the femur.
Case 4: 24-Month-Old Boy
An otherwise healthy boy was evaluated for an increased arch in his lower back. In addition to his hyperlordosis, his examination revealed a waddling gait. Leg lengths were equal, but hip abduction is limited on both sides to 30". A radiograph (Fig. 9) revealed bilateral hip dislocations and abnormal femoral head development bilaterally. Acetabular development was not grossly deficient. Bilateral open reduction combined with femoral shortening, varus, and derotation restored normal development.

Teaching Points

1. The lack of asymmetry in cases of bilateral dislocation make diagnosis more difficult at younger ages and is not uncommonly delayed as a result. 2. One must have a high index of suspicion to identify the characteristic gait and hyperlordosis.

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Figure 9. Anteroposterior radiograph of the pelvis of a 24-month-old boy. Both hips are dislocated. Shentons line is broken. Both femoral heads would lie in the upper, outer quadrant formed by Hilgenreiners and Perkins lines. Femoral head ossification is abnormal but present. One can see why there would be no leg length difference or asymmetry in abduction given this radiographic appearance.

Case 5: Five-Year-Old Girl

A child presented for evaluation of a painful limp with activities. Her gait was antalgic. The remainder of her examination was remarkably normal with only a slight decrease in abduction on the left (loo less than the other side). A radiograph (Fig. 10) revealed hip subluxation on the left (Shentons line broken, lateralization of the femoral head in the acetabulum) and acetabular dysplasia.

Teaching Points

1. This childs abnormality was so subtle at all ages that detection

would be nearly impossible until pain develops. Unfortunately, this child has the worst prognosis: subluxation leading to acetabular dysplasia with onset of pain and disability in childhood. 2. The presence of leg pain and gait asymmetry in childhood without a significant history of trauma should not be ignored because significant developmental problems may be the cause. 3. Treatment must address all of the dysplastic issues at this late age. Open reduction, femoral shortening and derotation, and Pemberton pelvic osteotomy were performed simultaneously with a good result.
Case 6: 17-Year-old Male Athlete

This teenager had no noted problems until 18 months prior to presentation, when he began to notice discomfort in his left hip at the end of the day

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Figure 10. Anteroposterior radiograph of the pelvis of a 5-year-old girl. The left femoral head is subluxated. There is an asymmetry in Shentons line, with the left one being more abnormal. The left femoral head is flattened. Erosion and cyst formation in the acetabulum are already present at this young age.

after strenuous activities. Evaluation was sought because the symptoms were increasing in frequency and severity. He could recall no trauma. He had a morning appointment. His gait and examination were normal. In particular, no tenderness to palpation, swelling, restriction of hip motion, guarding, or leg length discrepancy was present. A radiograph (Fig. 11) revealed acetabular dysplasia with lateral uncoverage of the femoral head. A Ganz periacetabular

Figure 11. Anteroposterior radiograph of the pelvis of a 17-year-oldmale patient. Shentons line is abnormal on the left. Uncoverage of the lateral portion of the femoral head is present bilaterally but is worse on the left. Acetabular dysplasia is also worse on the left. There is no thinning of the acetabular cartilage at this point.

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osteotomy was performed to realign the acetabular surface and improve the coverage of the femoral head. The pressure to which the joint cartilage is subjected decreases and the joint cartilage should be protected indefinitely.

Teaching Points
1. This young mans case is similar to that of the 5-year-old patient (Case 5). Abnormalities in early childhood prior to walking age were either minimal or nonexistent. 2. Symptoms developed only with increased weight, stature, and activity level. 3. His examination is normal in the office early in the day because the level of stress on his cartilage that day had not yet been sufficient to cause pain. Therefore, his typical compensatory Trendelenberg gait was not present. 4. He has a very poor prognosis without reconstructive surgery and is already developing early degenerative arthrosis.

CONCLUSION The goal of this article is to review the spectrum of hip dysplasia, emphasizing a methodology for screening for this disease process that is useful for primary care practitioners. Remembering the pathologic basis for the statement DDH represents a spectrum in time and severity helps to explain the disease process and improve ones ability to properly screen for hip abnormalities. The intent of this article is not to describe all of the details of complete evaluation and treatment; the reader is referred to other references for that information. Regardless of age at diagnosis, the goals of treatment remain the same: the femoral head must be reduced, it must be stable in the reduced position, and all dysplastic features of bone and cartilage must resolve (while complications are avoided). Early detection is important because these goals are easier to achieve at younger ages with less intervention and a lower likelihood of complications.

References
1. Aronsson DD, Goldberg MJ, Kling TF Jr, et a1 Developmental dysplasia of the hip. Pediatrics 94201, 1994 2. Barlow TC: Early diagnosis and treatment of congenital dislocation of the hip. J Bone Joint Surg Br 44:292, 1962 3. Bialik V, Fishman J, Katzir J, et a1 Clinical assessment of hip instability in the newborn by an orthopedic surgeon and a pediatrician. J Pediatr Orthop 6:703,1986

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4. Boa1 DK, Schwenkter EP: The infant hip: Assessment with real-time US. Radiology 157667, 1985 5. Faber A: Erbbiologische Untersuchungen iiber die Anlage zur angebore Huftverrenkung. Zeitschrift f i r Orthopadie und ihre Grenzgebiete, 66:140, 1937 6. Howorth MB, Massie WK: Congenital dislocation of the hip, Part 111. Pathogenesis. J Bone Joint Surg Am 33191,1951 7. Idleberger K The genetic patholow o so-called CDH. Munich, Urban and Schwar- f , zenberg 1951 8. Ilfeld FW.Westin GW. Makin M: Missed or develoumental dislocation of the hiu. Clinical 6rthopaedics and Related Research 203976, 1986 9. Hayes MAB Traction at home for infants with developmental dysplasia of the hip. Orthopaedic Nursing 1433, 1995 10. Morrissy RT (ed): Love11and Winters Pediatric Orthopaedics, ed 3, vol2. Philadelphia, JB Lippincott, 1990, p 815 11. Ogden JA, Moss H L Pathologic anatomy of congenital hip disease. Weil UH (ed): Progress in Orthopedic Surgery. Berlin, Springer-Verlag, 1978, p 3 12. Siffert Rs: Patterns of deformity of the developing hip. Clinical Orthopaedics and Related Research 16014-25,1981 13. Tredwell SJ, Bell HM: Efficacy of neonatal hip examination. J Pediatr Orthop 1:61,1981 14. Wedge JH, Wasylenko MJ: The natural history of CDH. J Bone Joint Surg Br 61:306, 1977 15. Wynne-Davis R Acetabular dysplasia and familial joint laxity: Two etiological factors in CDH. J Bone Joint Surg Br 52704,1970
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Address reprint requests to Tom F. Novacheck, MD

Department of Orthopaedics

Gillette Childrens Hospital 200 East University Avenue St. Paul, MN 55101-2598