Approach
Treatment must be individualized and is based on the age at presentation, extent of epiphyseal involvement at the time of treatment, presence of lateral epiphyseal extrusion, the stage of the disease, and the range of hip motion.[69] The primary goal of treatment is to maintain pain-free and full range of motion at the hip joint throughout the course of the natural history of the condition. This is best achieved by allowing activities and full use of the joint.
Acute pain
Any acute pain should be managed with nonsteroidal anti-inflammatory drugs such as ibuprofen or acetaminophen. A short period of rest is recommended during acute exacerbations. This may be followed by a period of limited activities and/or sports until the pain resolves and hip motion is restored. Full resumption of normal activities is only allowed following this. A short and variable period (up to 6 weeks) in abduction plaster(s) may be required if symptoms are severe.
Ongoing treatment depending on patient age
The age of the child is the most important variable in planning treatment because, the healing potential is closely linked to that of growth and remodeling.
Children under 5 years: the healing potential is very good at this age and the outcome is very favorable. A full range of motion is maintained and the child is monitored. If the radiographs suggest lateral extrusion, nonsurgical containment is considered.
Children 5 to 7 years: the key factor in this age group is the extent of epiphyseal involvement.[63][65] If epiphyseal involvement is less than 50%, apart from symptomatic treatment for acute episodes, mobilization and maintaining hip movement is adequate. If epiphyseal involvement is 50% or greater, a surgical containment procedure is the treatment of choice. In patients aged under 6 years, pelvic osteotomies have better radiologic outcome than femoral osteotomies, whereas in patients aged 6 years and older, femoral and pelvic osteotomies are equally likely to yield a spherical congruent head.[70]
Children over 7 years and up to 12 years: the remodeling potential is decreasing in this age group and there is controversy regarding the benefit of surgical intervention. The essential factor in deciding the treatment in this group is based on the clinical findings and the stage of the disease according to the Catterall classification.[63] For the initial and fragmentation stages (I and II), surgical containment is the primary line of treatment.[71] It is important to get the hip contained as early as possible to allow for remodeling. In the latter stages of the disease (III and IV), containment is contraindicated and a salvage procedure is performed to increase the load bearing area.
Children over 12 years: limited remodeling potential indicates that containment is not an option in this age group. Salvage procedures may be considered to improve congruence of the hips. It is estimated that 50% of untreated hips develop disabling arthritis by the sixth decade. This can be treated with a replacement arthroplasty.
Containment and salvage procedures
Containment brings in an increased area of femoral head articular cartilage under the weight-bearing portion of the acetabulum. This ensures maximal contact between the immature femoral head and the acetabulum during the period of growth. Containment is warranted in the presence of predictors of poor outcome such as recurrent synovitis, lateral subluxation, more than 50% femoral head involvement and a collapsed lateral pillar. The position of optimal containment is determined by an arthrogram and screening of the affected hip under general anesthesia. It can be achieved either by splints or surgically.
Nonsurgical containment: a dynamic radiographic screening arthrogram allows assessment of the optimal position in which to contain the hip. This position is then maintained in an abduction cast, splint, or brace, or a combination of all 3 where a spica and adductor tenotomy is followed by a period in an abduction brace.[72] It is essential to maintain motion during the course of the disease process, therefore a prolonged hip spica is not favored. Bracing should be balanced with a daily range of motion exercises to minimize stiffness. Children up to 5 years of age can typically tolerate nonsurgical containment. Patients are then weaned back to normal activities once lateral epiphyseal reossification is evident.
Surgical containment: surgical containment brings the femoral articular surface under the weight-bearing part of the acetabulum. This allows normal development of the hip joint with growth. The optimum position of containment of the hip is assessed by hip arthrogram and screening under general anesthesia. Surgical containment is recommended for children between the ages of 5 and 7 years with more than 50% epiphyseal involvement and in children between 7 and 12 years with Catterall stages I and II disease. Proximal femoral osteotomy is a versatile procedure in bringing the hip into a contained position. This is achieved by a lateral varus opening wedge osteotomy.[73] In selected cases, where the femoral head involvement exceeds 50% but the head is not deformed, an innominate osteotomy may be performed.[74] In severe cases a combination of both these procedures may be considered.
Salvage procedures are considered in patients between 7 and 12 years with stages III and IV disease and all children over 12 years of age. Salvage options address either the acetabulum to recreate or deepen the socket, or aim to improve congruence between the weight-bearing areas by altering the femoral head orientation with a femoral valgus osteotomy. Because the acetabulum is shallow and malformed, the salvaged hip does not have a roof lined by articular cartilage and the lateral part of the femoral head is uncovered. The purpose of surgery in these patients is to create a roof for the femoral weight-bearing portion. Shelf arthroplasty and the Chiari osteotomy are the 2 common acetabular procedures undertaken to salvage the hip joint with or without the addition of the femoral osteotomy. The Chiari osteotomy is a suitable salvage option in an older child with very little remodeling potential. Shelf arthroplasty is employed in severe disease to prevent subluxation and improve acetabular coverage, although evidence for this is lacking.[75]
Shelf acetabuloplasty provides a good or fair Stulberg outcome when performed in early Perthes stages (stages I and II) as a containment surgery, but less favorable outcomes were observed when shelf surgery was used for reconstructive-salvage purposes in late Perthes disease (stages III and IV). Caution is advised in performing the shelf procedure in children older than 10 to 11 years of age.[76][Figure caption and citation for the preceding image starts]: Surgical containmentFrom the personal collection of Jwalant S. Mehta, MS (Orth), MCh (Orth), FRCS, FRCS (Orth) [Citation ends].
Replacement arthroplasty
An estimated 50% of untreated hips develop disabling arthritis by the sixth decade. The mechanics of a symptomatic hip can be suitably restored by a hip replacement, with similar functional outcomes to those in patients having a hip replacement because of primary osteoarthritis, although the revision rate at mid-term follow-up is slightly increased.[77] The essential deciding factors for a hip replacement are the disability and symptoms and it is performed only after skeletal maturity. The techniques of replacement arthroplasty are constantly evolving. At present, cemented or uncemented components are used. Surface replacements are not suitable due to the femoral head deformity.
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