Legg-Calvé-Perthes disease
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
with acute pain
supportive care
Any acute pain should be managed with nonsteroidal anti-inflammatory drugs 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 are only allowed following this. A short and variable period (up to 6 weeks) in abduction plaster(s) may be required if symptoms are severe.
Primary options
acetaminophen: children <12 years of age: 10-15 mg/kg orally every 4-6 hours when required, maximum 75 mg/kg/day; adults: 325-1000 mg orally every 4-6 hours when required, maximum 4000 mg/day
OR
ibuprofen: children <12 years of age: 10 mg/kg orally every 6-8 hours when required, maximum 40 mg/kg/day; adults: 400-800 mg orally every 6-8 hours when required, maximum 2400 mg/day
under 5 years
mobilization plus monitoring
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.
nonsurgical containment
Treatment recommended for ALL patients in selected patient group
If the radiographs suggest lateral extrusion, nonsurgical containment is considered as children up to 5 years of age can typically tolerate it well.
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.
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]Rich MM, Schoenecker PL. Management of Legg-Calvé-Perthes disease using an A-frame orthosis and hip range of motion: a 25-year experience. J Pediatr Orthop. 2013 Mar;33(2):112-9. http://www.ncbi.nlm.nih.gov/pubmed/23389562?tool=bestpractice.com 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. Patients are then weaned back to normal activities once lateral epiphyseal reossification is evident.
5 to 7 years
mobilization plus monitoring
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.
surgical containment
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.
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.
In selected cases, where the femoral head involvement exceeds 50% but the head is not deformed, an innominate osteotomy may be performed. In severe cases a combination of both these procedures may be considered.
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]Nguyen NA, Klein G, Dogbey G, et al. Operative versus nonoperative treatments for Legg-Calvé-Perthes disease: a meta-analysis. J Pediatr Orthop. 2012 Oct-Nov;32(7):697-705. http://www.ncbi.nlm.nih.gov/pubmed/22955534?tool=bestpractice.com
[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].
over 7 years to 12 years
surgical containment
The remodeling potential is decreasing in this age group and there is controversy regarding the benefit of surgical intervention. It is important to get the hip contained as early as possible to allow for remodeling.
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.
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.
In selected cases, where the femoral head involvement exceeds 50% but the head is not deformed, an innominate osteotomy may be performed. In severe cases a combination of both these procedures may be considered.
Shelf acetabuloplasty provides a good or fair Stulberg outcome when performed in early Perthes stages as a containment surgery; however, caution is advised in performing the shelf procedure in children older than 10 to 11 years of age.[76]Kadhim M, Holmes L Jr, Bowen JR. The role of shelf acetabuloplasty in early and late stages of Perthes disease: a meta-analysis of observational studies. J Child Orthop. 2012 Oct;6(5):379-90.
[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].
salvage procedure
Containment is contraindicated and a salvage procedure is performed.
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]Hsu JE, Baldwin KD, Tannast M, et al. What is the evidence supporting the prevention of osteoarthritis and improved femoral coverage after shelf procedure for Legg-Calvé-Perthes disease? Clin Orthop Relat Res. 2012 Sep;470(9):2421-30. http://www.ncbi.nlm.nih.gov/pubmed/22194022?tool=bestpractice.com A proximal valgus femoral osteotomy is also a useful salvage procedure to redirect the femoral head to improve congruence between two misshapen surfaces.
Shelf acetabuloplasty is associated with less favorable outcomes when used for reconstructive-salvage purposes in late Perthes disease stages. Caution is advised in performing the shelf procedure in children older than 10 to 11 years of age.[76]Kadhim M, Holmes L Jr, Bowen JR. The role of shelf acetabuloplasty in early and late stages of Perthes disease: a meta-analysis of observational studies. J Child Orthop. 2012 Oct;6(5):379-90.
over 12 years
salvage procedure
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.
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. These techniques aim to increase the load-bearing area by attempting to improve congruence. 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]Hsu JE, Baldwin KD, Tannast M, et al. What is the evidence supporting the prevention of osteoarthritis and improved femoral coverage after shelf procedure for Legg-Calvé-Perthes disease? Clin Orthop Relat Res. 2012 Sep;470(9):2421-30. http://www.ncbi.nlm.nih.gov/pubmed/22194022?tool=bestpractice.com A proximal valgus femoral osteotomy is also a useful salvage procedure to redirect the femoral head to improve congruence between two misshapen surfaces.
Shelf acetabuloplasty is associated with less favorable outcomes when used for reconstructive-salvage purposes in late Perthes disease stages. Caution is advised in performing the shelf procedure in children older than 10 to 11 years of age.[76]Kadhim M, Holmes L Jr, Bowen JR. The role of shelf acetabuloplasty in early and late stages of Perthes disease: a meta-analysis of observational studies. J Child Orthop. 2012 Oct;6(5):379-90.
replacement arthroplasty after skeletal maturity
It is estimated that 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]Hanna SA, Sarraf KM, Ramachandran M, et al. Systematic review of the outcome of total hip arthroplasty in patients with sequelae of Legg-Calvé-Perthes disease. Arch Orthop Trauma Surg. 2017 Aug;137(8):1149-54. http://www.ncbi.nlm.nih.gov/pubmed/28674737?tool=bestpractice.com The essential deciding factors for replacement arthroplasty are the disability and symptoms. 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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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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