LCPD SUPPLEMENT
A Brief History of Legg-Calvé-Perthes Disease
Dennis R. Wenger, MD and Nirav K. Pandya, MD
Abstract: Legg-Calvé-Perthes (LCP) disease has an extensive
history that has provided an ongoing intellectual challenge for
the orthopaedic community. Debate around etiology and treatment of LCP disease continues even after its initial description in
the early 1900s. In order for modern day clinicians to have a full
understanding of the condition, one must be a scholar of its
development. The purpose of our review will be to discuss the
scientific communities’ understanding of presentation, etiology,
and treatment of LCP disease over time.
Level of Evidence: Level V.
Key Words: Legg-Calvé-Perthes disease, pediatric hip, history,
treatment
(J Pediatr Orthop 2011;31:S130–S136)
L
egg-Calvé-Perthes (LCP) disease has provided a
continual intellectual challenge for the orthopaedic
community, with ongoing debate about etiology, pathogenesis, and treatment. From the time of its initial description by Legg, Calvé, and Perthes (1909 to 1910), the
condition has puzzled clinicians across the globe (Fig. 1).
To better understand LCP disease, one can benefit from a
review of the development of our knowledge of the
disorder.
PRE-RADIOGRAPHIC ERA
The 19th century brought the rapid development of
orthopaedic knowledge, particularly in Germany, France,
and Austria despite radiographs not having been discovered. With the increasing use of general anesthesia,
direct surgical exploration of the hip led to an expanded
understanding of hip pathology. Despite these efforts,
clarification of different childhood hip diseases remained
rather limited beyond hip sepsis and tuberculosis (TB);
both of which were easily recognized owing to the
significant destruction of the hip joint. It is from the
study of hip infections in children that much of the early
work centering on what we now know as LCP disease was
unknowingly performed.
From the Department of Orthopaedic Surgery, Rady Children’s
Hospital San Diego and the University of California-San Diego,
Children’s Way, San Diego, CA.
The authors declare no conflict of interest.
Reprints: Dennis R. Wenger MD, Department of Orthopaedic Surgery,
3030 Children’s Way, San Diego, CA 92103. E-mail: OrthoEdu@
rchsd.org.
Copyright r 2011 by Lippincott Williams & Wilkins
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While performing a detailed histological analysis of
hip TB in the late 1800s, Rokitansky, the renowned
Viennese bone pathologist, described a milder form of
childhood hip disease that closely mirrored what we now
call LCP disease.1 Thomas, Baker, and Wright also
reported on what they believed to be infectious processes
affecting the pediatric hip whose presentation was retrospectively noted to be very similar to LCP.2–4
As a result, in lieu of radiographs, the proposed
etiologies of the irritated pediatric hip in the 19th century
(which were difficult to differentiate) were generally
grouped into infection, coxitis/synovitis, slipped capital
femoral epiphysis,5 osteochondritis,6 or pseudocoxalgia.7
Further analysis in the study of childhood hip
disease awaited the radiographic era.
THE EARLY RADIOGRAPHIC ERA
Roentgen’s late 19th century discovery of the x-ray
revolutionized the orthopaedic world, particularly the
understanding of childhood hip disease.8 Within months
after his report (around Christmas time, 1895 in Wurzburg, Germany), x-ray machines were installed in
hospitals in most major European cities, confirming that
truly important new scientific developments do not
require advertisement and self-promotion. A revolution
of eponymic attachment to orthopaedic disorders (Kohler,
Sever, Osgood, Schlatter, Scheurmann) soon followed in
an era described by Mercer Rang as “osteochondritismanship.” It was during this era in 1905 that Kohler
made an initial description of the x-ray changes we now
know as LCP disease,9 however his report was not widely
recognized.
The first formal description of the disease occurred
nearly simultaneously in 1909 and 1910 by Legg in
Boston, Calvé in France, and Perthes in Germany; all of
whom postulated different disease etiologies. Legg presented a series of limping patients with flattened femoral
heads, which he believed were due to trauma.10 In contrast, Calvé reported on 10 patients with noninflammatory hip pain and a flattened femoral head that he felt was
due to abnormal osteogenesis.11 Finally, Perthes reported
on 6 patients with hip pain that he felt was due to an
inflammatory condition.12 Concurrently, Waldenstrom
from Sweden described the radiographic features of this
condition although he mistakenly thought it was due to
TB.13
The different theories regarding the etiology of the limping children in these early papers not only demonstrated the
rapid impact of Roentgen’s discovery in helping to
provide a reproducible visual picture of a disease, but
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History of Legg-Calvé-Perthes Disease
FIGURE 1. The “founding fathers” who first described the condition. A, A. LeggFUnited States, B, J. Calvé–France, C, G.
PerthesFGermany.
also that how little was known as to the cause of these
flattened femoral heads as seen on x-ray.
ETIOLOGIC THEORIES AND DISEASE
CONFIRMATION
Initially, the disease described by Legg, Calvé, and
Perthes was lumped into the general category of childhood hip osteochondritis (what all abnormalities of bone
growth and shape in childhood came to be referred to at
the time). Yet, the true etiology remained unknown. Was
it because of an embolic disorder, hyperemia, trauma,
endocrine dysfunction, infection, or a blood supply
deficiency?
Histological studies provided a better understanding
of the pathophysiology of LCP disease, and a subsequent
understanding of the radiographic stages of the disease.
In 1921, Phemister in Chicago reported on his histologic
findings after curettage of a proximal femur of a 10-yearold boy with several months of hip symptoms.14 He found
a combination of necrotic and healing bone intermingled
with granulation tissue and osteoclasts. This confirmed
that the radiographic changes seen in LCP disease were
likely owing to aseptic necrosis followed by a healing
phase in which revascularization occurred. This histological sequence was described as schleichend ersetzung by
the Germans (subsequently interpreted by Phemister as
“creeping substitution”) in which bone was first resorbed
by osteoclasts and then replaced with new bone by
osteoblasts. The concept of osteoblasts and osteoclasts
working simultaneously in the proximal femur was
further supported by the work of Riedel in 1922.15 Yet,
Phemister felt that his findings were due to both an
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inflammatory and infectious process, and the true etiology remained unanswered. Multiple other theories were
entertained, including bacillary embolism,16 arterial hyperemia,17 transient synovitis,18 endocrine dysfunction,19 and
external toxins.20
FIGURE 2. Diagrammatic depiction of femoral head circulation. All etiologic theories focus on how this vascularity is
somehow temporarily disturbed.
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A disruption of the blood supply to the femoral
head was also entertained, and has now come to the
forefront as the most important etiologic factor in Perthes
disease (Fig. 2). Konjetzny was the first to show in 1926
that an interruption to the vascular supply to the femoral
head was present in LCP disease.21 This vascular work
was furthered by Theron (obstruction of the superior
retinacular artery),22 Atsumi et al (lateral epiphyseal
artery interruption),23 Sanchis et al and Inoue et al
(double infarction theory),24,25 and Kleinman and Bleck
(increased blood viscosity).26 Currently, thrombophilia
(owing to dysfunction in the levels of protein C and S) is
being postulated as a possible factor leading to vascular
thrombosis and subsequent osteonecrosis.27
The above histological studies provided the framework for a better understanding of LCP pathophysiology,
and the reasons for the changes in femoral head and neck
shape that occurred during the disease process. Early,
acute pain in LCP was thought to be the result of a shear
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fracture between the necrotic bone segment and the more
elastic living bone under the cartilage surface (Caffey’s
sign).28 Growth in the femoral neck width (owing to appositional bone growth) during a period in which longitudinal growth had slowed or stopped created a short/
wide femoral neck. The femoral head widened as well,
leading to decreased coverage by the acetabulum with the
acetabular rim impinging on the femoral head. Weightbearing on this “biologically” plastic femoral head led to
further deformity, hinge abduction, and finally an
incongruent joint, which leads to premature arthritis.
RADIOGRAPHIC CLASSIFICATION
Armed with a better understanding of disease
etiology as gained from histological studies, new classification systems were developed after the initial era of radiographic discovery. In 1922, Waldenstrom (Fig. 3A, B)
FIGURE 3. A, H. Waldenstrom (Sweden) also described the radiographic features now known as LCP disease but thought the
changes were due to tuberculosis. B, The chronologic radiographic stages of LCP as described by Waldenstrom: initial,
fragmentation, re-ossification, and healed. The chronologic sequence occurs over an approximate 2-year time period (Illustration
courtesy of Stuart Weinstein, MD). LCP indicates Legg-Calvé-Perthes.
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History of Legg-Calvé-Perthes Disease
FIGURE 4. Alternative imaging methods to analyze LCP disease. A, Bone scan, B, MRI, C, 3-D CT Scan.
described 4 chronologic radiographic stages of LCP: initial,
fragmentation, re-ossification, and healed.29 He noted that
femoral head deformity developed and worsened during the
initial and fragmentation stage. During the re-ossification
A
stage, femoral head deformity could worsen, improve, or
remain unchanged.
Nearly 50 years later (1971), Catterall30 developed a
prognostic radiographic classification based on the extent
B
FIGURE 5. A, Cardiff, WalesFwhere A.O. Parker and Brian McKibben developed the concept of abduction cast containment. B,
Modern fiberglass cast used for the nonoperative treatment of LCP disease. LCP indicates Legg-Calvé-Perthes.
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FIGURE 6. Traditional brace methods to provide femoral head containment. A, Traditional abduction brace, B, Toronto brace
(Bobechko), C, Atlanta brace.
of the femoral head infarct (I: anterior epiphysis involvement, II: central segment fragmentation and collapse,
III: anterior, central, and lateral involvement; IV: entire
head involvement). In addition, he defined 4 head “atrisk” signs, including lateral subluxation, calcification
lateral to the epiphysis, Gage’s sign (V shaped defect
laterally), and a horizontal growth plate. A fifth at risk
sign was later contributed by Smith in 1982 (presence of a
metaphyseal cyst).31
In 1980, Salter and Thompson32 recognizing that
Catterall groups I and II were distinct from groups III
and IV, developed a simpler classification dividing
patients into 2 groups (A: less than 1=2 of the head
involved, B: greater than 1=2 of the head involved). The
extent of femoral head involvement was determined by
the extent of the subchondral fracture (Caffey’s sign).
Seventy years after Waldenstrom (1992), Herring
et al33 developed another prognostic classification based on
the height of the lateral epiphyseal pillar during the fragmentation stage of disease (group A: no collapse, group
B: lateral pillar with >50% of original height, group C:
lateral pillar with <50% of original height). They later
added a “B-C” border classification, making the system as
complex as that of Catterall.34
These classification systems with follow-up analysis
clarified that patients with “at-risk” signs and/or Catterall
stages III and IV (Salter and Thompson B), and Herring
group C patients were destined to have poor outcomes
owing to progressive head deformity and joint incongruity. Stulberg et al (1981) developed an important
5-part classification system, which could be made at the
time of femoral head healing, clarifying which patients
FIGURE 7. Surgical containment methods for LCP (A) Femoral varus osteotomy, (B) Salter osteotomy. LCP indicates Legg-CalvéPerthes.
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would have premature arthritis.35 The use of alternative
imaging modalities in addition to plain radiograph have
become increasingly important in defining disease severity
(Fig. 4A–C).
NONOPERATIVE TREATMENT
Early treatment approaches for LCP focused on
long periods of hospitalization and complete nonweightbearing, either by bed rest or a wheelchair. Subsequent
methods focused on childhood mobility and included a
wide variety of special types of braces, including the
“patten-ended caliper” designed by Hugh Owen Thomas,
and other braces and belts designed to prevent weightbearing and unload the hip for up to 2 years to allow for
femoral head healing.36
This period of bracing and belts was followed by the
abduction plaster cast era brought on by A. O. Parker
and Brian McKibben in Cardiff, Wales (Fig. 5A).37 They
found that patients could be placed in wide abduction
plaster casts for an extensive period of time (often 1 year
or more) to provide femoral head containment within the
acetabulum during revascularization using the acetabulum as a mold for the “soft” femoral head. The abduction
casting concept was popularized in North America by
Gordon Petrie 1971 at the Montreal Shriner’s Hospital.38
Although demanding and rigorous to apply, the Petrie
cast method showed that weight-bearing with the femoral
head contained was not harmful (Fig. 5B).
Problems with Petrie casting were common, including multiple clinic visits and even hospitalizations for
repeating casting, regaining hip and knee range of
motion, and dealing with femoral condylar flattening
that sometimes was seen with prolonged treatment. As a
result, abduction casting was replaced by more civilized,
removable braces that mimicked Petrie casts (Fig.
6A–C).39,40 The Toronto brace (developed by Bobechko),
which allowed full knee flexion while maintain hip
internal rotation proved too cumbersome to be practical.
The Atlanta brace, which initially became popular owing
to its ease of wear, soon fell out of favor because it failed
to internally rotate the femur (and thus could not
predictably contain the femoral head). The ineffectiveness
of this brace style was shown by the work of both Meehan
et al and Martinez et al.41,42
OPERATIVE CONTAINMENT
By the early 1960s, it became clear that keeping a
child in a cast or brace for a prolonged period was not only
difficult and psychologically stressful to a child, but also
often ineffective because the child would not dutifully wear
the brace. The demise of government/charity hospitals
where a child could be hospitalized for weeks or even
months was also drawing to a close. This caused surgeons
to consider operative methods to contain the femoral head.
The concept of surgical containment of the femoral
head via femoral varus osteotomy was first introduced by
Soeure in Belgium 1952,43 followed by Axer in Israel in
1965,44 and Lloyd-Roberts in London in 1976.45 The
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History of Legg-Calvé-Perthes Disease
femoral varus osteotomy centered the head in the acetabulum, allowing the acetabulum to serve as a mold (Fig. 7A).
In 1962, Salter added the innominate osteotomy as
a method for containment in Perthes disease (a method
that he initially used for developmental dysplasia of the
hip, Fig. 7B).46 He felt that acetabular rotation provided
a better method for femoral head containment while
avoiding the potential limb shortening and limp often
associated with femoral osteotomy. Subsequently it has
been noted that the Salter osteotomy alone cannot provide adequate acetabular rotation to fully contain the
femoral head in older patients with extensive necrosis.
These older children with severe head involvement sometimes benefit from advanced containment methods such
as the combination of femoral osteotomy plus Salter
osteotomy or triple pelvic osteotomy.47,48
FUTURE DIRECTIONS
Although LCP disease has been recognized for a
century, full agreement on its etiology and treatment remains controversial. Despite a lack of full understanding
of the disease, modern treatment methods allow most
children to be treated without lengthy hospitalization,
casting, or bracing. Advanced imaging modalities such as
3-dimensional CT scan and dynamic MRI have given us
greater insight into the anatomy and growth patterns of
this disorder.49 Surgical containment is generally successful, but there are cases, which fail and require further
surgery. Older patients who may develop femoral acetabular impingement after advanced coverage procedures
can later be treated by the methods developed by Ganz,
including femoral head neck recontouring or femoral
head reduction.50 Future treatment of LCP is also likely
to include biological and pharmacologic methods.51
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