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KEYWORDS
Perthes disease;
Clinical features;
Investigation;
Treatment
Introduction
Perthes disease is idiopathic avascular necrosis of
the femoral head in childhood. It was described
independently by Legg in the USA, by Jacques Calve!
in France and by Georg Perthes in Austria during
the first decade of the 20th century. The description of Perthes disease followed rapidly after the
intervention of the radiograph. Prior to that, it had
been thought to be a self-limiting infection,
possibly tuberculosis. In many countries the disease
!
is known as Legg-Calve-Perthes
disease, or LCP. In
Britain the condition is generally referred to as
Perthes because he had recognised the fact that
this was a form of avascular necrosis.
*Tel.: 44-115-924-9924.
Epidemiology
Even the incidence of Perthes disease is controversial. Incidences are quoted per 100,000 of the
0268-0890/$ - see front matter & 2004 Elsevier Ltd. All rights reserved.
doi:10.1016/j.cuor.2004.06.001
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population but also per 100,000 children.1 It is
probably 1.5 to 4 cases per 100,000 of the population, leading to a prevalence of between 6 and 12
per 100,000 of the child population. Higher incidences of Perthes disease outside this range are
quoted, particularly for some urban areas.2 The male
to female ratio is 6:1. The incidence of bilateral
Perthes disease is approximately 25% and in the
majority of these cases the disease does not occur
synchronously in each hip. If it does, alternative
causes should be considered, the most common of
which would be multiple epiphyseal dysplasia.
J.B. Hunter
with poverty and Perthes disease.1 Add to this that
there are several studies suggesting that there is no
relationship between the environment and Perthes
disease and the situation is even more confusing. It
does seem to be true that the urban clustering
previously described is in fact a feature of population density rather than true effect and this has
been most satisfactorily demonstrated in Northern
Ireland, where population mobility is more limited
and therefore long-term follow-up for epidemiology more easy to achieve.
Trauma
Aetiology
Theories on the aetiology of Perthes are multiple
and difficult to disentangle.
Growth patterns
A constant feature in papers that describe the
epidemiology and aetiology of Perthes is that
patients are relatively immature in that they have
delayed bone age. This was noted particularly by
Wynne-Davis and Gormley in Scotland, but also in
Birmingham and more recently in Coventry.3,4 The
delayed bone age has been reflected in the short
stature of patients and some have found evidence
of reduced growth factors, such as somatomedins,
in patients with Perthes disease. Although true
bilaterality only occurs in 25%, some investigators
have found abnormalities of the contra-lateral hip,
which they believe represent the growth abnormalities that cause the condition.
Genetic factors
The role of genetic factors is controversial. WynneDavis originally felt that the factors affecting the
incidence of Perthes disease were environmental
rather than genetic. Clustering in families has,
however, been described on several occasions. It is
clear, however, that Perthes is not a straightforward genetic condition with a predictable mode of
inheritance.
Environment
A number of different environmental influences
have been suggested. In both Scotland and Liverpool, poverty has been suggested as an aetiological
factor and this came to be associated with an urban
environment.5 More recently, it has been suggested
that a rural environment might be associated more
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Legg Calve
Perthes disease
Irritable hip
Episodes of transient synovitis were thought to be
associated with an increased incidence of Perthes
disease. In fact, the relationship is the other way
round. The initial presentation of Perthes is that of
an irritable hip, sometimes with synovitis and
effusion. The synovitis is triggered by the subchondral fracture and the initial collapse. It is certainly
not worthwhile following up all cases of transient
synovitis to see if they were early Perthes disease.
Natural history
The natural history of Perthes is normally described in terms of the progression of the radiological appearances. The clinical course of the
disease is very unpredictable. Some cases do not
present until they are well into the healing phase
and some restriction of movement or an awkward
gait has been noted, whereas others present with
pain and stiffness before any but the most subtle
radiological signs are visible.
The natural history was originally described by
Waldenstrom, whose classification is interesting, in
that the active stages of the disease were all
crammed into what he described as the evolutionary stage of the disease. He felt that the
disease was prolonged in extent and this certainly
can be the case. More recently, Josephs group from
India have popularised the use of a classification
modified from the Elizabethtown classification,
which divides the condition into four stages;
sclerotic, fragmentation, healing and healed
(Table 1 and Fig. 1).11 This natural history study
also records the median duration of each stage and
the morphological changes as they occur, recording
the timing of epiphyseal extrusion, metaphyseal
widening and the appearance of adverse changes,
suggesting that deformation of the epiphysis occurs
during the late stage of fragmentation or in the
early stage of revascularisation. This has led to
their recommendation that any containment surgery be performed before the late stage of
fragmentation. This is an important study and
together with its companion piece on the timing
275
Days
I Sclerotic
A no loss height B height loss
II Fragmentation
A early B late
III Healing
A peripheral B41/3 epiphysis
IV Healed
220
240
550
Pathology
The information on the pathology of Perthes is
poor because it is a non-lethal condition with active
stages during childhood. Few full pathological
specimens are available and much of the information comes from small biopsies and curettings.13 It
was fairly well established that there is thickening
of the articular epiphyseal cartilage. The bony
epiphysis and the deep layers of epiphyseal
cartilage are affected by the ischaemic process
and infarct, whilst the superficial layers of the
articular cartilage continue to gain nourishment
from the joint and synovial fluid. In severe
Perthes, the physis itself is disrupted with distortion of the physeal columns and the formation of
irregular cell columns and cartilage. The physeal
distortion is seen radiologically as the metaphyseal
cysts and Gage sign. Both of these cysts in fact
contain unossified columns of cells from the
distortion of the physis. The fragmentation of the
bony epiphysis is the beginning of the repair
process, with infarcted and damaged structures
being removed prior to reossification.
Prognosis
The long-term complication for patients with
Perthes disease is osteoarthritis. The long-term
follow-up from Iowa shows that at 40 years of
follow-up (i.e. patients in their late 40s and 50s),
40% had had a joint replacement, 10% had disabling
arthritis that warranted a joint replacement and a
further 10% had Iowa hip scores less than 80.
Groups of patients followed up for less than 40
years appear to be functioning well. Between 20
and 40 years of follow-up, the majority of patients
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J.B. Hunter
Figure 1 The stages of Perthes (a) Sclerotic with a sub-chondral fracture, (b) early fragmentation (femoral osteotomy
performed), (c) early healing, peripheral only, (d) late healing, (e) head healed, result is Stulberg 1, Mose good.
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and the concentric ring method of Mose17,18 (Figs. 2
and 3, Table 2). Neither is completely reliable.
Doubts have been cast on the intra- and interobserver reliability of the Stulberg classification
and in the Iowa series, even completely round
femoral heads, as judged by Mose, had severe
arthritis by the middle of their seventh decade,
although no arthritis in their 30s.19,20
The classic results of Catterall suggested that
less severe disease carried a better prognosis.
Follow-up was to six years (so not extensive) and
the outcome grade was that devised by Sundt,
which has not been widely used. The results suggest
that 50% or more of patients with Perthes do
extremely well with no intervention and this has
led to the development of severity gradings to be
applied during the course of the disease to select
appropriate patients for intervention.21
Figure 2 Example Stulberg outcome grades. (a) Grade 2, (b) Grade 3, (c) Grade 4, and (d) Grade 5.
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Table 4
Investigations
Plain X-rays
AP and lateral X-rays of the hips are the mainstay of
investigation for Perthes. Perthes is a protracted
disease and the repeatability of plain X-rays
enables the course of the disease to be charted.
MRI
Several classifications of Perthes disease based on
MRI appearances have been proposed. At this stage,
none has gained acceptance, because they have not
proved definitively prognostic and the necessity for
repeated imaging studies in Perthes is inconvenient and expensive in the context of Perthes.
Bone Scan
Many groups have described the benefits of using
isotope bone scanning in Perthes. The groups in
Montpelier and Chicago suggest that pinhole collimation (which provides a far more detailed picture)
is useful in recording the viability of the lateral
column of the epiphysis. This in turn may predict
deformity. Conventional bone scanning without
collimation would appear to have no role.26,27
Arthrography
The arthrogram is an important investigation of the
child for whom intervention is being considered.
Catterall emphasises the importance of the arthrogram being dynamic. The surgeon must certainly
view how the hip moves, as medial pooling occurs
both with a flattened head that moves congruently
and with hinge abduction (Fig. 6). An arthrogram
may demonstrate whether the hip is containable
(i.e. the area of disease can be located satisfactorily within the acetabulum) or in non-containable
hips may demonstrate the position of best fit prior
to valgus or valgus extension osteotomy.
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Theories of treatment
Relief from weight-bearing
Because the natural history of the condition
indicates collapse and extrusion to be associated
with femoral head deformity and therefore it is
assumed a poor result, the earliest treatments
available involved prolonged relief of weightbearing whilst healing was allowed to progress. In
some cases this resulted in many years of bed rest
with or without an abduction frame.28 Other
methods of relief of weight-bearing involved traction and the use of the Snyder sling, which kept the
affected leg in the Long John Silver position.
Containment
The main theory proposed for the treatment of
Perthes disease is that of containment. Essentially,
the injured femoral head is secured within the
socket and kept moving in order that after
regeneration it should be round and fully mobile.
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Salvage procedures
Children who have had Perthes and present with
deformed femoral heads, difficulties walking, stiffness and pain, require treatment, even if that
treatment cannot be with the objective of creating
a round femoral head at maturity. The most
frequent cause of pain and stiffness is hinge
abduction, when the extruded lateral segment of
epiphysis impinges on the lateral border of the
acetabulum.
Abduction osteotomy
This osteotomy is designed to remove the impinging
segment of epiphysis from the lateral wall, allowing
the foot to be placed comfortably underneath the
pelvis and a more normal gait. It is effective at
reducing pain. In the best circumstances, because
of the restoration of movement, remodelling of the
femoral head occurs and very good results have
been reported for this procedure from Catterall. It
is important that this procedure not be performed
Cheilectomy/recreation of offset
An alternative approach to the extruded fragment
is to remove it surgically. As originally proposed this
operation was done through an anterolateral
approach with simple excision of the fragment.37
Many orthopaedic surgeons have had poor experiences with this procedure. Stiffness of the hip joint
has been a frequent sequela and more seriously,
removal of the perichondral ring, together with the
fragment, has resulted in slipped upper femoral
epiphysis of the remaining epiphysis. This operation
had fallen into disuse until repopularised by the
Bern group, who have performed it in association
with trochanteric distalisation and surgical dislocation of the hip.38 When performed on a hip with an
open physis they have always stabilised the
epiphysis to prevent slipped upper femoral epiphysis. Reshaping of the femoral head and recreating
the femoral offset in the manner they describe is
certainly a more thorough approach than bumpectomy and the long-term results of this are
awaited.
Hip Arthroscopy
Occasionally in Perthes, healing of the epiphysis is
incomplete and patients complain of symptoms
suggestive of a loose body. If this is truly the case,
then hip arthroscopy can be very useful. It should
be noted that sometimes the loose body is not
loose at all and detaching it from the femoral head
arthroscopically can be extremely difficult.39
Conclusion
Many controversies remain in Perthes. These
algorithms are presented as a way through the
maze of contradictory and incomplete evidence
(Tables 5 and 6). Catterall has emphasised the
importance of clinical examination in the assessment of cases of Perthes and this is particularly
important in the younger group, many of whose
hips heal very round, despite rather adverselooking X-rays early on, because they have maintained an excellent, almost normal, range of
movements. The older child or early adolescent
with Perthes requires urgent attention, as the
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References
1. Kealey WD, Moore AJ, Cook S, Cosgrove AP. Deprivation,
urbanisation and Perthes disease in Northern Ireland. J
Bone Joint Surg Br 2000;82(2):16771.
2. Hall AJ, Barker DJ, Dangerfield PH, Taylor JF. Perthes
disease of the hip in Liverpool. Br Med J Clin Res
1983;287(6407):17579.
3. 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 Br
1978;60(1):614.
4. Harrison MH, Turner MH, Jacobs P. Skeletal immaturity in
Perthes disease. J Bone Jt Surg Br 1976;58(1):3740.
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