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Congenital dislocation of the hip is the condition of hip instability at or soon after birth in which the hip becomes dislodged from it’s socket and subsequently becomes permanently dislocated and deformed. The condition usually leads to a limp in childhood and premature arthritis. Early treatment relies upon early recognition and active management. Early treatment can in many cases relocate the hip into its socket and if the hip is maintained in this position then normal function can be achieved.
The term congenital dislocation of the hip dates back to the time of Hippocrates. This condition, also known as hip dysplasia or developmental dysplasia of the hip (DDH). Ortolani, an Italian paediatrician evaluated, diagnosed, and began treating hip dysplasia in the early 1900s (Ortolani, 1976). Galeazzi later reviewed more than 12,000 cases of DDH and reported the association between apparent shortening of the flexed femur and hip dislocation.
Typically, the term CDH is used when referring to patients who are born with dislocation or instability of the hip, which may then result in hip dysplasia. Abnormal development of the hip includes the osseous structures, such as the acetabulum and the proximal femur, and the labrum, capsule, and other soft tissues. It may occur at any time, from conception to skeletal maturity.
Terms used in CDH:
Subluxation is incomplete contact between the articular surfaces of the femoral head and acetabulum.
Dislocation is complete loss of contact between the articular surface of the femoral head and acetabulum.
Instability is the ability to subluxate or dislocate the hip with passive manipulation.
Congenital dislocation of the hip CDH is dislocation of the hip occurring around birth and early childhood.
Developmental dysplasia of the hip DDH is a name often used for CDH.
The overall frequency is usually reported as approximately 1 case per 1000 individuals, However the incidence at birth may be considerably higher with many hips stabilising in the first week of childhood (Barlow, 1962).
The exact cause of CDH is not clear, but hip dysplasia does appear to be related to a number of different factors. One factor is racial background with a very low incidence among southern Chinese and African American populations (Getz, 1955; Hoaglund, 1973; Rabin, 1965; Skirving, 1979). An underlying genetic disposition also appears to exist in that a 10-fold increase in the frequency of hip dysplasia occurs in children whose parents had DDHcompared with those whose parents did not (Bjerkreim, 1978). Eighty percent of persons with DDH are female (Wilkinson, 1972). Other factors possibly related to DDH include intrauterine positioning in that the prevalence of DDHin females born in breech position is as high as 1 case in 15 persons (Ramsey, 1976). The left hip is more commonly associated with DDH than the right, and this is believed to be due to the common intrauterine position of the left hip against the mother’s sacrum, forcing it into an adducted position (Dunn, Clin Orthop 1976 119:11-22). Ligamentous laxity is also believed to be associated with hip dysplasia, although this association is less clear. Children in cultures in which the mother swaddles the baby, forcing the hips to be adducted, also have a higher rate of hip dysplasia (Kutlu, 1992). At birth, white children do tend to have a shallow acetabulum (McKibbin, 1970; Ralis, 1973). This may provide a susceptible period in which abnormal positioning or a brief period of ligamentous laxity may result in hip instability.
The normal growth of the acetabulum depends on normal epiphyseal growth of the triradiate cartilage and on the 3 ossification centres located within the acetabular portion of the pubis (os acetabulum), ilium (acetabular epiphysis), and ischium. The presence of the spherical femoral head within the acetabulum is critical for stimulating normal development of the acetabulum (Harrison, 1961). The anatomy of the dislocated hip, especially after several months, often includes formation of a ridge called the neolimbus. Closed reduction is often unsuccessful at a later date, secondary to various obstacles to reduction. These include adductor and psoas tendon contraction, ligamentous teres, a transverse acetabular ligament, and pulvinar and capsular constriction. With long-standing dislocations, interposition of the labrum can also interfere with reduction (Dunn, Clin Orthop 1976 119:23-7; Fleissner, 1994; Noble, 1978; Ponseti, 1978).
Symptoms of Presentation
Early clinical manifestations of DDH are identified during examination of the newborn. In the UK each child is usually examined by a trained paediatrician. If any suspicion arises from the examination by an Orthopaedic Surgeon and ultrasound investigation is usually undertaken. The classic examination finding is revealed with the Ortolani manoeuvre; a palpable “clunk” is present when the hip is reduced in and out of the acetabulum and over the margin of the hip. Ortolani originally described this clunk as occurring with either subluxation or reduction of the hip (in or out of the acetabulum). More commonly, the Ortolani sign is referred to as a clunk, felt when the hip reduces into the acetabulum, with the hip in abduction.
Later clinical examination, when the child is aged 3-6 months, is quite different. At this point, the hip, if dislocated, is often dislocated in a fixed position (Bjerkreim, 1978). The Galeazzi sign is a classic identifying sign for unilateral hip dislocation. This is performed with the patient lying supine and the hips and knees flexed. Examination should demonstrate that one leg appears shorter than the other. Although this is usually due to hip dislocation, realizing that any limb length discrepancy results in a positive Galeazzi sign is important. Additional physical examination findings for late dislocation include asymmetry of the gluteal thigh or labral skin folds, decreased abduction on the affected side, standing or walking with external rotation, and leg length inequality.
Ultrasound investigation have been of significant benefit in the assessment and treatment of children with hip dysplasia. However the benefit of screening all children with ultrasonography is controversial (Falliner, 1999; Paton, 1999). Even with ultrasound screening, children with hip dysplasia can be missed.
Standard radiographic views include a standing anteroposterior view of the pelvis, with the hips in neutral position, and a false profile view in which the patient is standing angled at 65° from the x-ray plate. The radiograph is then taken, profiling the anterior aspect of the acetabulum. If any evidence of hip subluxation is present, an abducted internal rotation view can help determine if the hip reduces and better determines the true neck-shaft angle of the proximal femur. A CT scan can also be helpful in determining femoral anteversion and in determining the extent of posterior acetabular coverage and may be used in older children. These may be summated into three-dimensional images to visualize the overall shape of the acetabulum. Numerous radiographic measurements have been used in the evaluation of DDH.
Arthrography is performed by injecting dye into the hip joint and examining the patient with aid of fluoroscopy, usually with the patient under anaesthesia and it is commonly used during hip reduction.
Treatment modalities – non-operative
The natural history of hip dysplasia depends, in part, on the severity of the disease, and whether or not a false acetabulum has formed (Wedge, 1978). Unilateral dislocations result in significant leg length inequality, with a gait disturbance and possibly associated hip and knee pain. The development of a false acetabulum is associated with a poor outcome in approximately 75% of patients. Indications for treatment depend on the patient’s age and the success of the previous techniques. Children younger than 6 months with instability upon examination are treated with a form of bracing, usually a Pavlik harness. If this is not effective or if the hip instability or dislocation is noted when the child is older than 6 months, closed reduction is typically recommended, often with traction prior to the reduction. When the child is older than 2 years or with failure of the previous treatment, open reduction is considered. If the patient is older than 3 years, femoral shortening is performed instead of traction, with additional varus applied to the femur if necessary. A patient with residual acetabular dysplasia who is older than 4 years should be treated with an acetabular procedure.
Treatment for DDH that is diagnosed when the patient is a young adult can be considered for residual acetabular dysplasia. Unfortunately, radiographic characterization of DDH that is severe enough to lead to early osteoarthrosis is difficult. Radiological parameters and X-ray measurements are commonly used in the assessment (Wiberg, 1939, Cooperman, 1983).
Treatment modalities – operative
Relative contraindications to surgery include older age (>8 y for a unilateral hip dislocation or >4-6 y for bilateral hip dislocation, especially if a false acetabulum is not present). Other contraindications to surgery include a neuromuscular disorder, such as a high myelomeningocele or spinal cord injury, or cerebral palsy in a patient who has had a hip dislocation for more than 1 year.
Open reduction is the treatment of choice for children older than 2 years at the time of initial diagnosis or for children in whom attempts at closed reduction have failed. Pelvic osteotomy may be needed for residual hip dysplasia. When open reduction is performed, a spica cast is worn for 6 weeks. The patient is then placed in an abduction orthosis. Numerous possible complications can occur, including redislocation, stiffness of the hip, infection, blood loss, and, possibly the most devastating, necrosis of the femoral head (Fogarty, 1981; Schoenecker, 1978).
Overall, the prognosis for children treated for hip dysplasia is very good, especially if the dysplasia is managed with closed treatment. If closed treatment is unsuccessful and open reduction is needed, the outcome is less favourable, although short-term outcome appears to be satisfactory. If secondary procedures are needed to obtain reduction, then the overall outcome is significantly worse.
Early diagnosis is the most crucial aspect of the treatment of children with DDH. The use of ultrasonography and other diagnostic imaging modalities and the implementation of improved educational programs will most likely decrease the number of children with DDH diagnosed late.
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