High Yield Topics

Perthes Disease

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Ovid: 5-Minute Orthopaedic Consult

Editors: Frassica, Frank J.; Sponseller, Paul D.; Wilckens, John H.
Title: 5-Minute Orthopaedic Consult, 2nd Edition
> Table of Contents > Perthes Disease

Perthes Disease
Kris J. Alden MD, PhD
Simon C. Mears MD, PhD
Basics
Description
  • Perthes disease is a self-limited, idiopathic disorder of the hip in young children.
    • Stages include ischemia, resorption, repair, and ultimately collapse of the femoral head.
    • Although most patients do well after
      healing in childhood, it may lead to collapse of the femoral articular
      surface and subsequent degenerative joint disease.
    • Up to 50% of affected patients develop disabling arthritis in their mid-50s (1).
  • All classification systems are based on radiographs showing femoral head involvement.
    • Catterall staging system (2):
      • Stage I: Involvement of the anterior femoral head alone
      • Stage II: Involvement of the entire medial dome
      • Stage III: Progression into the lateral column of the head
      • Stage IV: Involvement of the whole head
    • Lateral pillar staging system (3):
      • Group A: <10% involvement of the
        lateral pillar with incongruent joint; evaluates appearance of the hip
        at maturity to forecast the potential for hip arthrosis; uses several
        radiographic parameters (femoral head sphericity, femoral neck length,
        acetabular slope, and presence of coxa magna)
      • Group B: >50% of the lateral pillar is maintained.
      • Group C: <50% of the lateral pillar is maintained.
    • Stulberg classification (used to predict onset of degenerative changes) (4):
      • Class 1: Normal hip
      • Class 2: Spherical femoral head short neck
      • Class 3: Ovoid or mushroom-shaped head
      • Class 4: Flat femoral head with congruous acetabulum
      • Class 5: Flat femoral head ion
  • Synonyms: Legg-Calvé-Perthes disease; Osteochondritis deformans juvenilia
Epidemiology
  • Most common in children 4–10 years old, but reported from 2 years old to teens (5)
  • Males affected 4–5 times more often than females
  • More common in Caucasians and Asians than in African Americans and Native Americans
  • Bilateral involvement is present in 10% of cases, but rarely is it synchronous (5).
  • Symmetric involvement suggests a more general disorder (e.g., hypothyroidism or multiple epiphyseal dysplasia).
Incidence
<1% of the population (2)
Risk Factors
  • Small stature
  • Low socioeconomic level
  • Increased parental age
  • Living in urban areas
  • Ethnicity (Asian, Inuit, Central European)
Genetics
A positive family history is noted in 2–10% of cases (5).
Etiology
  • The cause is unknown, but most current theories are based on vascular compromise of the femoral head.
  • Proposed causes of the limited blood supply:
    • Trauma
    • Intraosseous venous hypertension or obstruction
    • Hypercoagulable disorders
  • Perthes disease in an African American child should prompt a hypercoagulability workup.
  • Minor trauma may have role in initiating events in any of these theories.
Associated Conditions
  • Short stature
  • AVN of the tarsal navicular vessels (Köhler disease)
Diagnosis
Signs and Symptoms
  • Limited abduction and internal rotation
  • Child often leaning the trunk over the affected limb while walking (Trendelenburg sign)
  • In long-standing disease, mild atrophy of the thigh, calf, and buttock
  • Leg-length inequality (late finding)
History
  • Insidious onset of a limp (most common presentation)
  • Pain either absent or mild
  • When pain is present, usually related to activity and relieved by rest
  • Pain usually in the groin area, but can be referred to the anteromedial thigh or knee
Physical Exam
  • Observe the child’s gait for indications of pain: Short, quick steps or shift of the shoulders with each stride.
  • Check for limitation of internal or external rotation compared with contralateral side.
  • Check abduction while holding the child’s pelvis still.
Tests
Lab
  • No diagnostic lab tests
  • On occasion, laboratory studies may be indicated to rule out hyperthyroidism, septic arthritis, or sickle cell disease.
Imaging
  • Radiographs of the hip are the most useful imaging study and show several stages of disease:
    • Initial phase:
      • Affected epiphysis is smaller than that on the contralateral side, with widening of the medial joint space.
      • Physeal irregularity and subchondral radiolucency also may be present.
    • Fragmentation phase:
      • The bony epiphysis begins to fragment.
      • Mixed lucent and dense areas appear in the epiphysis.
    • Reparative phase:
      • Normal bone density returns.
      • Alterations in the shape of the femoral head and neck become obvious.
    • Healed phase:
      • Residual deformity is revealed.
    • 4 head-at-risk signs:
      • Gage sign: A radiolucent “v” in the lateral portion of the epiphysis
      • Calcification lateral to the epiphysis
      • Lateral subluxation of the femoral head
      • Horizontal physis
  • MRI detects infarction but does not give information on the stage of the disease (no specific indication for this test).
  • Arthrography is useful for assessing femoral head deformity and congruency.
Pathological Findings
  • The physeal plate shows the formation of histologic clefts.
  • Cartilage clusters are present, extending into the metaphysis.
Differential Diagnosis
  • Fracture
  • Gaucher disease
  • Glycogen storage diseases
  • Hypothyroidism
  • Juvenile rheumatoid arthritis
  • Multiple epiphyseal dysplasia
  • Multiple or spondyloepiphyseal dysplasia
  • Osteoid osteoma
  • PVNS
  • Septic arthritis
  • Septic hip
  • Sickle cell disease
  • SCFE
  • Steroid arthropathy
  • Transient synovitis of the hip
  • Traumatic AVN node
  • Tuberculous arthritis
  • Tumors

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Treatment
General Measures
  • The position of best containment of the femoral head within the acetabulum is determined by plain radiographs or arthrography.
  • Containment of the femoral head:
    • If containable, it is maintained in this position by abduction orthosis or casts or by femoral or acetabular osteotomy.
    • If not containable, salvage procedures can reduce pain, equalize leg length, and improve movement.
  • Surgery is not recommended for children <6 years old unless substantial subluxation is shown.
  • Restoration of joint motion is critical to maintaining synovial and, therefore, cartilage nutrition.
  • Containment of the femoral head in the acetabulum prevents deformity.
  • A cast or brace may be used to abduct the leg and contain the femoral head.
Activity
  • If weightbearing is painful, protect the child with the use of crutches.
  • If marked stiffness occurs, the patient should rest until stiffness resolves.
Special Therapy
Physical Therapy
  • May be helpful for maintaining ROM of the hip.
  • Most critical for maintaining abduction motion.
Medication
First Line
Pain should be treated with medications such as acetaminophen or NSAIDs based on the child’s age and weight.
Surgery
  • Osteotomy of the femur or acetabulum (or
    both) repositions the femoral head into a contained place within the
    acetabulum and allows growth and remodeling to occur in a spheric,
    congruous fashion.
  • Internal fixation (e.g., a plate) is used to stabilize the osteotomy so early motion may be started.
    • Hardware should be removed because future procedures are likely.
  • Up to 50% of patients develop end-stage arthritis in their mid-50s and are treated with total hip arthroplasty.
Follow-up
Disposition
Issues for Referral
Patients with Perthes disease should be followed by an orthopaedic surgeon or pediatric orthopaedic surgeon.
Prognosis
  • The younger the child is at the healing stage, the better the potential for remodeling of the femoral head.
  • Poor results are rare in children <6 years old.
  • Almost always, symptoms resolve after the healing phase, and patients have freedom from pain during the teenage years.
  • Factors for a poorer prognosis (6):
    • Children present at ≥8 years old.
    • Involvement of the lateral column of the femoral head
    • Poor ROM
    • Nonconcentric or noncontained femoral head after healing
  • ~50% of patients with Perthes disease as a child will need hip replacement by later adulthood (50–60 years old) (1).
Complications
  • Loss of sphericity or collapse causes the following:
    • Early degenerative joint disease
    • Leg-length discrepancy
    • Loss of ROM with flexion and adduction contractures
Patient Monitoring
  • Brace or cast containment usually
    averages 6–18 months or until the reossification stage occurs and the
    risk of femoral head collapse has passed.
  • Examine the child out of the brace to check ROM.
  • Take radiographs with the child in the brace.
  • See the patient every 4–8 weeks until reossification occurs.
References
1. McAndrew MP, Weinstein SL. A long-term follow-up of Legg-Calvé-Perthes disease. J Bone Joint Surg 1984;66A:860–869.
2. Weinstein
SL. Legg-Calvé-Perthes disease. In: Morrissy RT, Weinstein SL, eds.
Lovell and Winter’s Pediatric Orthopaedics, 6th ed. Philadelphia:
Lippincott-Raven, 2006:1039–1083.
3. Herring JA, Neustadt JB, Williams JJ, et al. The lateral pillar classification of Legg-Calvé-Perthes disease. J Pediatr Orthop 1992;12:143–150.
4. Stulberg SD, Cooperman DR, Wallensten R. The natural history of Legg-Calvé-Perthes disease. J Bone Joint Surg 1981;63A:1095–1108.
5. Wynne-Davies
R, Gormley J. The aetiology of Perthes’ disease. Genetic,
epidemiological and growth factors in 310 Edinburgh and Glasgow
patients. J Bone Joint Surg 1978;60B:6–14.
6. Herring
JA, Kim HT, Browne R. Legg-Calvé-Perthes disease. Part II: Prospective
multicenter study of the effect of treatment on outcome. J Bone Joint Surg 2004;86A:2121–2134.
Miscellaneous
Codes
ICD9-CM
732.1 Perthes disease
Patient Teaching
  • Maintaining ROM is important.
  • Parents should be told that the disease often takes ~2 years to heal.
  • Patients usually have a largely pain-free childhood after healing, even though problems may develop later in adulthood.
  • Healing of the disorder cannot be accelerated.
  • No effective means of prevention exists.
  • Early detection is important, so containment can be achieved before substantial deformity of the femoral head occurs.
Activity
Activity should be restricted if the hip is painful.
Prevention
Surgery may be helpful in increasing the containment of
the femoral head, which can lessen or prevent later arthritic changes
in the hip.
FAQ
Q: How likely is the development of later arthritis?
A:
Children who develop Perthes disease by 6 years of age have a better
prognosis than those who present at ≥8 years old. ~50% of patients
develop arthritis in their 50s.

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