Jessica Hernandez, MD Attending Physician, Department of Emergency Medicine, Einstein Healthcare

Network
Background
Legg-Calv-Perthes disease is the eponym given to idiopathic osteonecrosis of the femoral head. It was
described approximately 100 years ago as a unique disease entity affecting the pediatric population. [1]
Legg-Calv-Perthes disease can lead to hip deformities and severe degenerative arthritis.

Pathophysiology
The pathophysiology and temporal sequence of events in Legg-Calv-Perthes disease remains
unclear; however, the following scenario is generally accepted:
1. The blood supply to the femoral head is interrupted.[2]
2. Bone infarction and necrosis affects the articular cartilage, subchondral bone, and the
bony epiphysis.[3]
3. Revascularization occurs and new bone ossification starts. In some cases, patients may
have normal bone growth and development.
4. With progression of the disease, bone resorption, delayed bone formation, and
subchondral fracture occurs. This microdamage is usually the result of normal physical
activity, not direct trauma.
5. This may result in deformities in the femoral head, epiphyseal growth plate, and possible
lesions in the metaphysis.[3]
Epidemiology
Frequency
United States

One in 1200 children younger than 15 years is affected by Legg-Calv-Perthes disease. The
disease is familial approximately 10% of the time.[4]
Mortality/Morbidity

Legg-Calv-Perthes disease is a self-limited disease if not treated. Outcome widely varies. In 1520% of patients with Legg-Calv-Perthes disease, involvement is bilateral.

Race

Whites are affected more frequently than persons of other races.

Sex

Males are affected 4-5 times more often than females.

Age

Legg-Calv-Perthes disease most commonly is seen in persons aged 3-12 years, with a median
age of 7 years.

Legg-Calve-Perthes Disease Imaging

Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR Consultant Radiologist and Honorary Professor, North
Manchester General Hospital Pennine Acute NHS Trust, UK

Overview

Legg-Calv-Perthes (LCPD) disease is a childhood hip disorder that results in infarction of the
bony epiphysis of the femoral head. LCPD represents idiopathic avascular necrosis of the
femoral head. The disease is bilateral in 10-20% of patients and usually affects children aged 4-8
years. When both hips are involved, they are usually affected successively, not simultaneously. A
family history is present in 6% of patients. In adults, the corresponding condition is termed
Chandler disease.
Although the etiology is unclear, certain risk factors have been identified in children, including
sex, socioeconomic group, and the presence of an inguinal hernia and genitourinary tract
anomalies. More specifically, boys are affected 3 to 5 times more often than girls, and the
incidence increases in low socioeconomic groups and in children with low birth weight.
Determining the prognosis is important at the time of presentation, because more than 50% of
patients with LCPD do not require treatment.[1, 2, 3]
The radiologic features of LCPD are demonstrated in the images below.

Legg-Calv-Perthes disease. Stage II disease. Note the

slight widening of the left hip joint, representing a small joint effusion. Joint
widening can also be secondary to hypertrophy of the cartilage.

Legg-Calv-Perthes disease. Plain radiograph in an adult

patient with residual coxa magna and plana deformity with superimposed joint

changes.
Legg-Calv-Perthes disease. Axial nonenhanced
CT scan through the hip joints in the same patient as in the previous image more
clearly shows the loss of structural integrity of the right femoral head.

Legg-Calv-Perthes disease. Nonenhanced axial CT

section through the hip joints obtained at a different level in the same patient as in
the previous 2 images. Once again, the scan shows the loss of structural integrity of
the right femoral head. Note the acetabular subchondral sclerosis.

Legg-Calv-Perthes disease. Coronal reconstruction

shows flattening, sclerosis, and early fragmentation of the right femoral head (same
patient as in the previous 3 images).
Staging

Several staging schema are used to determine severity of disease and prognosis; these include the
Catterall, Salter-Thomson, and Herring systems.[4]
The Catterall classification is based on radiographic appearances and specifies 4 groups during
the period of greatest bone loss.
Catterall staging is as follows:

Stage I Histologic and clinical diagnosis without radiographic findings

Stage II Sclerosis with or without cystic changes with preservation of the

contour and surface of femoral head

Stage III Loss of structural integrity of the femoral head

Stage IV Loss of structural integrity of the acetabulum in addition

The Salter-Thomson classification simplifies the Catterall classifications by reducing the groups
to 2. The first, called group A, includes Catterall groups I and II; for patients in this group, less
than 50% of the head is involved. The second, called group B, includes Catterall groups III and
IV; for patients in this group, more than 50% of the head is involved. For both classifications, if
less than 50% of the ball is involved, the prognosis is better, whereas if more than 50% is
involved, the prognosis is potentially poor.
The Herring classification addresses the integrity of the lateral pillar of the head. In lateral pillar
group A, there is no loss of height in the lateral one third of the head, and there is little density
change. In lateral pillar group B, there is a lucency and less than 50% loss of lateral height.
Sometimes, the head is beginning to extrude from the socket. In lateral pillar group C, there is a
more than 50% loss of lateral height.
Preferred examination

Plain radiography remains the major modality for the evaluation of LCPD. Staging of the disease
is based on plain radiographic findings.[1, 5, 6]

Scintigraphy is a useful technique in early disease when plain radiographic findings may be
normal; with scintigraphy, abnormalities become apparent earlier in the course of disease than
they do with plain radiography.
Computed tomography (CT) scans allow early diagnosis of bone collapse and curvilinear zones
of sclerosis early in the disease process when plain radiography is less sensitive. CT scans can
also demonstrate subtle changes in the bone trabecular pattern.
Ultrasonography is useful in the preliminary diagnosis of transient synovitis of the hip and the
onset of LCPD.[7] Hip effusion with capsular distension is well depicted on sonographic images.[8,
9, 10, 11, 12]

Magnetic resonance imaging (MRI) is as sensitive as isotopic bone scanning and allows more
precise localization of involvement than conventional radiography.[13, 14, 15, 16, 17, 18]
Limitations of techniques

Plain radiographic findings may be entirely normal in early symptomatic disease.

Although abnormalities become apparent with scintigraphy earlier in the course of disease than
they do with radiography, abnormal scintigraphic findings are nonspecific; findings may be
positive in patients with trauma, synovitis, and infections.
The use of CT scanning is limited by the comparatively higher radiation dose.
Ultrasonographic diagnosis of LCPD is based on a demonstration of hip effusion, which is a
nonspecific finding.
On MRI scans, the changes seen as bone marrow edema and joint effusions are nonspecific.
Angiography, venography, and arthrography are invasive procedures and do not provide
significantly better clinical information for guiding therapeutic options.
Radiography

Early radiographic signs of LCPD include the following:

Small femoral epiphysis (96%)

Sclerosis of the femoral head with sequestration and collapse (82%)

Slight widening of the joint space caused by thickening of the cartilage,

failure of epiphyseal growth, the presence of joint fluid, or joint laxity (60%)

(see the images below)

Legg-Calv-Perthes
disease. Stage II disease. Note the slight widening of the left hip joint,
representing a small joint effusion. Joint widening can also be secondary to

hypertrophy of the cartilage.

Legg-Calv-Perthes
disease. Stage II disease. Note the slight widening of the left hip joint
representing a small joint effusion. Joint widening can also be secondary to

hypertrophy of the cartilage.

Legg-Calv-Perthes
disease. The left joint effusion is apparent. The femoral head is smaller on the
left than the right. The femoral head is also considerably denser on the left
side. Joint widening can also be secondary to hypertrophy of the cartilage.

Legg-Calv-Perthes disease. The left joint effusion

is apparent. The femoral head is smaller on the left than on the right. The
femoral head is also considerably denser on the left side. Joint widening can
also be secondary to hypertrophy of the cartilage.

An absence of destruction of the articular cortex, as occurs in bacterial

arthritis (destruction of articular cartilage never occurs in LCPD)

Late signs of LCPD on radiographs include the following:

Delayed osseous maturation of a mild degree, a radiolucent crescent line

representing a subchondral fracture

Femoral head fragmentation and femoral neck cysts from intramedullary

hemorrhage or extension of physeal cartilage into metaphysis, loose bodies,

and coxa plana (see the images below)

LeggCalv-Perthes disease. Image shows flattening and early fragmentation of the
left femoral head with the presence of femoral neck cysts. The femoral head

is obviously smaller on the left than on the right.

Legg-Calv-Perthes disease. Image shows flattening and early fragmentation
of the left femoral head with the presence of femoral neck cysts. The femoral
head is obviously smaller on the left than on the right.

Legg-Calv-Perthes disease. Image shows

flattening and early fragmentation of the left femoral head with the presence
of femoral neck cysts. The femoral head is obviously smaller on the left than
on the right.

Coxa magna, or remodeling of the femoral head, which becomes wider and
flatter, similar in appearance to a mushroom (see the image below)

Legg-Calv-Perthes disease. Plain radiograph in an

adult patient with residual coxa magna and plana deformity with
superimposed joint changes.

Plain radiographs have a sensitivity of 97% and a specificity of 78% in the detection of LCPD.
Severe osteoarthritis and infective arthritis may mimic the disease.
Computed Tomography

Early signs of LCPD on CT scans include the following:

Bone collapse

Curvilinear zones of sclerosis

Subtle changes in bone trabecular pattern

Disruption of an area of condensation of bone formed by a compressive group

of trabeculae (abnormal asterisk sign)

Late signs of the disease on CT scans include the following:

Central or peripheral areas of decreased attenuation

Intraosseous cysts

Coronal reconstructions can show subchondral fractures, subtle buckling, or collapse of the
articular surface.
Degree of confidence

When CT scanning is employed, the staging of LCPD determined on the basis of plain
radiographic findings is upgraded in 30% of patients. CT scanning is not as sensitive as nuclear
medicine or MRI, but it may be used for follow-up imaging in patients with LCPD.
False positives/negatives

CT-scan findings of osteoarthritis and infective arthritis may mimic those of LCPD.

Magnetic Resonance Imaging

Early in the course of LCPD, irregular foci of low signal intensity or linear segments replace the
normal high signal intensity of bone marrow in the femoral epiphysis on T1- and T2-weighted
images. Other findings include an intra-articular effusion and a small, laterally displaced
ossification nucleus, labral inversion, and femoral head deformity. The MRI characteristics of
LCPD are demonstrated in the images below.[13, 14, 15, 16, 17, 18]

Legg-Calv-Perthes disease. Coronal T2-weighted MRIs

show irregularity and flattening of cortical margins of the left femoral epiphysis. Also
note a mild joint effusion and subluxation and hinge deformity of the left femoral

head.
Legg-Calv-Perthes disease. Coronal T1-weighted
MRIs show the loss of normal high signal intensity in the left femoral epiphysis,

which now has low signal intensity.

Legg-Calv-Perthes
disease. Axial T1-weighted MRIs through the femoral heads show low signal
intensity in the left femoral head.

Fat-suppressed or short-tau inversion recovery (STIR) sequences are more accurate than plain
radiographs are in showing degenerative changes of the articular cartilage. These MRIs
demonstrate the influx of fluid into areas of articular cartilage irregularity.
The asterisk sign is defined as findings of areas of low signal intensity on T1-weighted images
and high signal intensity on T2-weighted images in marrow. The double-line sign occurs in as
many as 80% of patients and represents the sclerotic rim, which appears as a signal void. This
sign is demonstrated as a line between necrotic and viable bone edges with a hyperintense rim of
granulation tissue.

Jaramillo et al found in a study that multipositional MRI with an open magnet was comparable to
arthrography for demonstrating containment of the congruency of the articular surfaces of the
hip.[13] However, in the evaluation of deformity or loss of the spherical nature of the femoral
head, open MRI performed less well.
Sebag et al showed dynamic gadolinium-enhanced subtraction MRI to be a simple and promising
means of early recognition of ischemia in LCPD.[14]
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate
dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK],
gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic
fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the
eMedicine topic Nephrogenic Systemic Fibrosis. The disease has occurred in patients with
moderate to end-stage renal disease after being given a gadolinium-based contrast agent to
enhance MRI or MRA scans.
As of late December 2006, the FDA had received reports of 90 such cases of NSF/NFD.
Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating
and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning,
itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes;
joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the
hip bones or ribs; and muscle weakness. For more information, see Medscape.
Degree of confidence

MRI is as sensitive as isotopic bone scanning, and it allows more precise localization of
involvement than does conventional radiography. MRI is preferred for evaluating the position,
form, and size of the femoral head and surrounding soft tissues.
False positives/negatives

The differential diagnosis includes severe osteoarthritis, infective arthritis, and other causes of
bone marrow edema and joint effusions.
Ultrasonography

Ultrasonography is useful in establishing the diagnosis of transient synovitis of the hip and the
onset of LCPD.
Hip effusion, which results in capsular distension, is accurately documented on sonograms.
(Capsular distension lasting longer than 6 weeks is associated with LCPD.) Ultrasonography
allows aspiration of joint fluid for laboratory examination. Together, the results of clinical
evaluation, radiography, and sonography determine the need for sonography-guided aspiration.
Ultrasonography-guided aspiration allows the selection of only those patients with septic arthritis

for surgical drainage and shortens the procedure. Negative sonographic findings allow the
exclusion of septic arthritis but not osteomyelitis.
A chronologic, 4-part staging of LCPD has been proposed on the basis of the ultrasonographic
findings. The stages reflect the degree of flattening and fragmentation and the reconstitution of
the femoral head. Thickening of articular cartilage, associated synovitis, and lateral extrusion of
the femoral head can be documented. Joint effusion is present in 74% of patients in stages I-II.
Lateral extrusion increases from stage II onward until the healing stage.[8, 9, 10, 11, 12, 19]
Degree of confidence

Although not performed routinely, ultrasonographic evaluation of patients with LCPD is a simple
and standardized procedure that can be useful for staging the disease and monitoring its course. It
can also spare the patient from radiation exposure and lower treatment costs. Lateral extrusion
and the onset of healing in patients with LCPD can be shown earlier with sonograms than with
radiographs.
Hip ultrasonography seems to be a reliable method for monitoring the containment of the
femoral head in LCPD.[20]
False positives/negatives

Changes similar to those of LCPD can be found in transient synovitis and other conditions that
cause hip joint effusions. Moreover, joint effusion is not always present in patients with LCDP.
Nuclear Imaging

Technetium-99m diphosphonate uptake depends on the stage of the disease, but it does play a
role in the diagnosis. Characteristic features include a photopenic void in proximal femoral
epiphyses (seen in the first 2 images below), as compared with the contralateral side, which
usually can be seen by using a pinhole camera with the hip in maximal medial rotation, obviating
the need of single-photon emission CT (SPECT).

Legg-Calv-Perthes disease. Technetium-99m

diphosphonate bone scan shows a photon-deficient defect in the right femoral head.

Legg-Calv-Perthes disease. Zoomed images of the same

patient as in the previous image show the photopenic defect more clearly.
Legg-Calv-Perthes disease. Radiograph obtained at the same time as the
radionuclide scans in the previous 2 images illustrates the usefulness of isotope
bone scans. The radiographic changes of subchondral lucency are subtle.

Scintigraphy may be helpful in early diagnosis. Initially, uptake is decreased in the femoral head
because of an interruption in the blood supply. Later, uptake is increased in the femoral head as a
result of revascularization, bone repair, and degenerative osteoarthritis. In addition, acetabular
activity can be increased with associated joint disease.
Degree of confidence

The sensitivity of radionuclide scanning in the diagnosis of LCPD is 98%, and the specificity is
95%.
False positives/negatives

Similar activity patterns may occur with osteoarthritis or infective or inflammatory arthritis. The
presence of a large joint effusion can simulate diminished perfusion caused by osteonecrosis.
Angiography

Angiography is performed only in rare cases. Early in the disease process, opacification of the
joint with contrast material can reveal subtle flattening of the chondral surface of the femoral
head and widening of the joint space.
Angiographic findings may demonstrate an interruption in the superior capsular arteries and a
generalized decrease of blood flow in the affected hip. Later in the disease process, the size and
position of sequestered fragments can be identified by the distribution of revascularized osseous
segments despite the demonstration of a smooth cartilaginous surface. However, vascular
changes in LCPD are nonspecific on angiograms.

Penyakit Legg-Calv-Perthes
Posted in Info Penyakit by nugraad001 on January 25, 2007
DEFINISI
Penyakit Legg-Calv-Perthes (Coxa plana) adalah suatu keadaan yang ditandai dengan
hancurnya lempeng pertumbuhan pada leher tulang paha.
Penyakit ini ditemukan pada 1 diantara 1.000-5.000 anak yang berumur 5-10 tahun dan lebih
sering menyerang anak laki-laki.
Biasanya hanya menyerang satu sisi panggul.
PENYEBAB
Penyebabnya adalah berkurangnya aliran darah ke tulang paha, tetapi penyebab dari
berkurangnya aliran darah ini tidak diketahui.

Penyakit ini menyebabkan pendataran pada kepala tulang paha. Terjadi gangguan aliran darah
dan dalam waktu 1-3 minggu, ujung tulang paha akan mati.
Jika darah kembali mengalir ke daerah tersebut, maka sel-sel tulang yang baru akan muncul
dalam waktu 6-12 bulan. Penggantian tulang yang lama oleh tulang yang baru memerlukan
waktu sekitar 2-3 tahun.
GEJALA
Gejalanya berupa:
- nyeri lutut (bisa merupakan satu-satunya gejala yang timbul pada awal perjalanan penyakit)
- nyeri selangkangan atau nyeri paha yang sifatnya menetap
- penciutan otot paha bagian atas
- tungkai agak memendek atau tungkai kiri dan kanan panjangnya tidak sama
- kekakuan panggul sehingga pergerakan panggul terbatas
- gangguan berjalan, berjalan menjadi goyah
- jangkauan pergerakan berkurang.
Komplikasinya adalah osteoartritis.
DIAGNOSA
Pemeriksaan fisik menunjukkan berkurangnya pergerakan panggul.
Pada foto rontgen akan tampak bahwa kepala tulang paha mendatar.
PENGOBATAN
Tujuan pengobatan adalah untuk melindungi tulang dan persendian dari stres dan cedera lebih
lanjut.
Pada fase awal biasanya penderita diharuskan menjalani tirah baring atau memakai tongkat
penyangga.
Brace, gips atau pembidaian untuk imobilisasi digunakan pada saat pertumbuhan tulang yang
baru sedang berlangsung.
Mungkin perlu dilakukan pembedahan agar panggul tetap berada dalam kantungnya.
PROGNOSIS
Jika dilakuan pengobatan, prognosis biasanya baik, tulang akan kembali pulih disertai kelainan
bentuk yang minimal.