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119
Pictorial
Hemophilia: Evaluation of Musculoskeletal with CT, Sonography, and MR Imaging George
Hermann,1
Marvin
Gilbert,2
S.
bleeding
in soft tissues.
CT is useful
in evaluating
pseudotumors. and regression
subtle
the joints leads to hemophilic arthropathy. Bleeding into musdes causes joint contractures, and bleeding into bone and adjacent soft tissues results in osseous and soft-tissue pseudotumors.
bony
Sonography of soft-tissue
Hemophilic
Arthropathy
Repeated decade Hemophilia is a defect in coagulation by a deficiency of clotting factor or clotting that is nonfunctional. Deficiencies of factor VIII (antihemophilic factor) lead to classic hemophilia (hemophilia A), and deficiencies offactor IX (plasma prothromboplastin component) lead to Christmas disease (hemophilia
B). These
genetic
disorders
Involvement
and I. Fikry Abdelwahab1
The purpose of this essay is to illustrate features of the musculoskeletal complications of hemophilia as shown by CT, sonography, and MR imaging. MR can be used to detect early synovial and cartilaginous changes that may not be evident on conventional radiography and to differentiate between acute and chronic erosion and intra- and extraosseous is valuable in following progression hematomas.
Essay
are X-Iinked
recessive.
They
primarily affect males, but are transmitted by females. The prevalence of hemophilia in the United States is 1 :1 0,000 for factor VIII deficiency and 1 :50,000 to 1 :1 00,000 for factor IX deficiency [1 ]. The clinical and radiologic features of patients with classic hemophilia and Christmas disease are virtually identical. Hemorrhage most often occurs in the synovial joints. In descending order, the knee, ankle, elbow, shoulder, and hip are involved [11. Repetitive bleeding into the musculoskeletal system is the most common complication of both conditions. Bleeding into
oflife.
early treatment
bleeding
into the joints often occurs
Joint degeneration
is progressive,
can slow the process,
destruction
(SE) MR images
(e.g.,
500/20
[TR/TE])
and intermediate
signal intensity on 12-weighted images (e.g., 1600/80) (Fig. 1). Cartilage has low signal intensity on both Ti - and 12weighted images [3] (Fig. 2). Hemorrhage first causes thickening of the synovia and later leads to thinning of the cartilage. As long as the subchondral
requests to G. Hermann, Box 1234. 2 Department of Orthopaedics, The Mount Sinai Medical Center, City University of New York, New York, NY 10029-6574, January
1992 0361 -803x/92/1
581 -01 19 © American
Roentgen
of the joint
is unavoidable. Sonography, CT, and MR imaging each have specific advantages as diagnostic tools. Although they all can be used to evaluate soft-tissue masses, intraarticular processes are best assessed by using MR. MR is capable of showing synovial abnormalities, ligamentous tears, periarticular bleeding, cartilaginous and osseous abrasions, and joint space narrowing [2]. Hypertrophied synovia resulting from repeated intraarticular bleeding has low signal intensity on Ti -weighted spin-echo
Received May 20, 1991 : accepted after revision August 26, 1991. I Department of Radiology, The Mount Sinai Medical Center, City University of New York, One Gustave L. Levy Pt., New York, NY 10029-6574.
AJR 158:119-123,
in the first
and although
Ray Society
Address reprint
Downloaded from www.ajronline.org by NYU Langone Med Ctr-Sch of Med on 04/21/15 from IP address 128.122.253.212. Copyright ARRS. For personal use only; all rights reserved
120
HERMANN
hemoshows extremely hypertrophied synovial tissue (arrowheads) with intermediate signal intensity and eroslons of patella. Posterior cruciate ligament (arrow) is stretched but Intact.
Fig. 2.-32-year-old
Fig. 1.-22-year-old hemophiliac with philic arthropathy of right knee. MR image
Fig. 3.-13-year-old hemophiliac with mild knee Plain radiograph was normal. MR image shows thinning of femoral cartilage with irregular pain.
outline.
A and B, Proton
show decreased
El
AL.
AJR:158,
January
1992
hemophiliac with hemophilic arthropathy.
density-weighted
(SE 1600/30)(A)
and T2-weighted
signal intensity, which represents
arrows), and fibrosis. Femoral condyle in B (solid arrows) indicates effusion.
is thinned
subarticular
and eroded.
(SE 1600/80)(B)
lesion, detached
Synovium
is thickened.
MR images (open Bright signal
cartilage
Fig. 4.-18-year-old
hemophiliac with severe repeated hemarthrosis. TI-weighted (SE 500/20) coronal MR image (A) of knee shows enlarged epiphyses. Articular surface is destroyed. Large subarticular cyst formation is present on medial side (A, arrowheads). Central area of low signal intensity in A (arrow) is surrounded by high-signalA and B, Relatively
intensity structure that remains bright on T2-weighted (SE 2300/80) image (B, short wide arrow). Arrowheads in B point to bloody fluid. Femoral condyle shows subarticular defect as well (B, long thin arrows).
bone is intact, findings on conventional radiographs are normal. MR is capable of detecting early erosion of the cartilage
Soft-Tissue
(Fig. 3). Subchondral cysts usually bearing area as a result of fissuring
Bleeding into the large muscles is common. The most commonly involved muscles are the iliopsoas quadriceps and gastrocnemius. A permanent contraction results if the hematoma is replaced by fibrous tissue. When bleeding in a large muscle fails to resolve, it becomes encapsulated and forms a pseudotumor. Because sonography is convenient, inexpensive, and in-
Fluid may penetrate
from
imaging
to differentiate
clear
can be used
fluid before
radiographs
the cyst
(Figs.
bone resorption
develop at the weightof the articular cartilage.
the joint
blood
becomes
4 and 5). Bone
are well visualized
into the cyst
cysts
[3].
in the cyst
obvious caused
MR from
on the plain by trabecular
on CT (Fig. 6).
Hematomas
AJA:i58,
January
MUSCULOSKELETAL
i992
INVOLVEMENT
IN HEMOPHILIA
121
with severe hemo(5E 800/20) cownal MR image shows depression of medial plateau. Large area of destruction laterally (arrow) was not
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Fig. 5.-42-year-old patient philia. Moderately Ti-weighted
seen on conventional
radiograph.
Fig. 6.-26-year-old throsis
of right
man with recurrent
hemar-
shoulder.
CT scan of shoulder shows periarticular erosion (arrow) and subarticular cystlike formation of glenoid (arrowheads).
Fig. 7.-30-year-old
hemophiliac
with left-sided
pelvic mass. A, Sonogram of pelvis shows echo-free lesion in left psoas muscle (arrow). B, Sonogram obtained 3 weeks after A shows lesion is markedly smaller (arrow). Echoes have increased.
A Fig. 8.-72-year-old philia and numerous
B
patient with severe hemopseudotumors. CT scan
shows huge mass in left iliac fossa bulging into abdomen. Smaller pseudotumor with dense calcification
is present
at right iliac fossa.
Fig. 9.-34-year-old
patient
with severe
hemo-
philia and pelvic mass. CT scan of pelvis shows large pseudotumor in right iliac fossa. Mass displaces contrast-filled bowel (solid arrows). Note low attenuation
at center
of mass
(open
arrow).
volves no radiation, it is the method of choice for following the progression and regression of soft-tissue hematomas [4] (Fig. 7). Hemophilic pseudotumor is a rare complication that occurs in 1 -2% of hemophiliacs. Most develop in the muscles of the pelvis and lower extremity, where the large muscles have a rich blood supply, or in bone following intraosseous bleeding.
Sonography, Cl, and MR imaging have important roles in detecting pseudotumors, especially when they are in the pelvis. Cl is useful in detecting both the extent of soft-tissue masses and the involvement of bone. Pseudotumors contain coagulated blood and are surrounded by a thick wall (Fig. 8). Contrast-enhanced Cl is useful in determining the thickness of the wall. In the acute
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122
HERMANN
El
AL.
AJA:158,
January
1992
C
A Fig. lO.-36-year-old patient with severe hemophilia and mass in thigh. A, CT scan of thigh shows thick-walled low-density mass. B, Transaxial upside-down sonogram shows central anechoic area (arrow) C, Longitudinal sonogram shows extent of mass. Irregular echoes (arrow)
with enhancement
(E) of echoes
behind
mass,
indicating
fluid content.
represent solid material.
Fig. 1 1.-56-year-old hemophiliac with numerous pseudotumors. A, Ti-weighted (SE 500/27) MR image of pelvis shows high-signal-intensity mass in left gluteal region (straight arrow). Large mass in left pelvic region shows inhomogeneous, mostly decreased signal intensity (arrowhead). B, T2-weighted (SE 2000/80)
image.
Mass
left gluteal region remained bright. Left-sided
in
pcI-
vic mass shows signal intensity
inhomogeneous, mostly increased (arrowhead). Hypointense rim of masses in both A and B (curved arrows) represents chronic pseudotumor.
Fig. 12.-22-year-old hemophiliac with pelvic pseudotumor. A, Moderately Ti-weighted MR image (SE 700/ 24) shows well-demarcated mass with bright signal intensity surrounded by low-signal-intensity mass (arrows). B, T2-weighted MR image (SE 2100/90) shows part of mass is bright (arrow). Other areas show decreased signal intensity, indicating presence of blood of different ages (arrowhead). A
B
Downloaded from www.ajronline.org by NYU Langone Med Ctr-Sch of Med on 04/21/15 from IP address 128.122.253.212. Copyright ARRS. For personal use only; all rights reserved
AJR:i58,
January
1992
MUSCULOSKELETAL
INVOLVEMENT
stage, the center of the pseudotumor is hypodense on CT, but the periphery is isodense and indistinguishable from surrounding muscle [5] (Fig. 9). In the acute and subacute stages, sonography shows a central anechoic area with increased echoes behind the lesion, caused by fluid in the pseudotumor. A distinct plane separates the mass from the surrounding muscles (Fig. 10). CT shows the thick wall more consistently than does sonography. Multiple irregular echoes represent solid material that cannot be documented on CT. Differential diagnosis from abscess may be difficult. The MR signal characteristics of soft-tissue hemorrhage vary with the age of the blood [2]. Hematomas in the acute stage (1 -6 days) have the same signal as muscle has on Ti weighted MR images and low signal intensity on 12-weighted images [6]. Subsequently, the Ti relaxation time shortens progressively and the signal intensity increases (Fig. i 1). After 7 days, the peripheral portion of the hematoma becomes hyperintense on Ti - and 12-weighted images (Fig. 1 2). The center gradually becomes hyperintense over the next several weeks, usually in a patchy distribution [7].
IN HEMOPHILIA
The hypointense rim of a pseudotumor 12-weighted images consists of fibrous hemosiderin [6, 7] (Fig. 1 1).
123
on both 11 - and tissue that contains
REFERENCES 1 . Aesnick D, Niwayama G. Bleeding disorders. In: Aesnick 0, Niwayama G. eds. Diagnosis of bone and joint disorders. Philadelphia: Saunders,
1989:2497-2522 2. Julish BS, Lieberman JM, Strandjord SE, Bryan PJ, Mulopulos MT. Hemophilic arthropathy: assessment with MR imaging.
GP, Modic Radiology
1987:164:759-762 3. Murphy WA. Magnetic resonance imaging. In: Resnick D. Bone and joint imaging. Philadelphia: Saunders, 1989:120-135 4. Aspelin P. Pettersson H, Sigurjonsson S, Nilsson IM. Ultrasonographic examination of muscle hematomas in hemophiliacs. Acta Radiol 1984; 25: 51 3-516 5. Hermann G, Yeh HC, Gilbert MS. Computed tomography and ultrasonography of the hemophilic pseudotumor and their use in surgical planning. Skeletal Radiol 1986:15:123-128 6. Wilson DA, Prince JR. Imaging of hemophilic pseudotumors. AJR
1988:150:349-350 7. Aubin JI, Gomori JM, Grossman Al, Gefter WB, Kressel HY. High-field imaging of extracranial hematomas. AJR 1987;148 :813-817
MR
119
Pictorial
Hemophilia: Evaluation of Musculoskeletal with CT, Sonography, and MR Imaging George
Hermann,1
Marvin
Gilbert,2
S.
bleeding
in soft tissues.
CT is useful
in evaluating
pseudotumors. and regression
subtle
the joints leads to hemophilic arthropathy. Bleeding into musdes causes joint contractures, and bleeding into bone and adjacent soft tissues results in osseous and soft-tissue pseudotumors.
bony
Sonography of soft-tissue
Hemophilic
Arthropathy
Repeated decade Hemophilia is a defect in coagulation by a deficiency of clotting factor or clotting that is nonfunctional. Deficiencies of factor VIII (antihemophilic factor) lead to classic hemophilia (hemophilia A), and deficiencies offactor IX (plasma prothromboplastin component) lead to Christmas disease (hemophilia
B). These
genetic
disorders
Involvement
and I. Fikry Abdelwahab1
The purpose of this essay is to illustrate features of the musculoskeletal complications of hemophilia as shown by CT, sonography, and MR imaging. MR can be used to detect early synovial and cartilaginous changes that may not be evident on conventional radiography and to differentiate between acute and chronic erosion and intra- and extraosseous is valuable in following progression hematomas.
Essay
are X-Iinked
recessive.
They
primarily affect males, but are transmitted by females. The prevalence of hemophilia in the United States is 1 :1 0,000 for factor VIII deficiency and 1 :50,000 to 1 :1 00,000 for factor IX deficiency [1 ]. The clinical and radiologic features of patients with classic hemophilia and Christmas disease are virtually identical. Hemorrhage most often occurs in the synovial joints. In descending order, the knee, ankle, elbow, shoulder, and hip are involved [11. Repetitive bleeding into the musculoskeletal system is the most common complication of both conditions. Bleeding into
oflife.
early treatment
bleeding
into the joints often occurs
Joint degeneration
is progressive,
can slow the process,
destruction
(SE) MR images
(e.g.,
500/20
[TR/TE])
and intermediate
signal intensity on 12-weighted images (e.g., 1600/80) (Fig. 1). Cartilage has low signal intensity on both Ti - and 12weighted images [3] (Fig. 2). Hemorrhage first causes thickening of the synovia and later leads to thinning of the cartilage. As long as the subchondral
requests to G. Hermann, Box 1234. 2 Department of Orthopaedics, The Mount Sinai Medical Center, City University of New York, New York, NY 10029-6574, January
1992 0361 -803x/92/1
581 -01 19 © American
Roentgen
of the joint
is unavoidable. Sonography, CT, and MR imaging each have specific advantages as diagnostic tools. Although they all can be used to evaluate soft-tissue masses, intraarticular processes are best assessed by using MR. MR is capable of showing synovial abnormalities, ligamentous tears, periarticular bleeding, cartilaginous and osseous abrasions, and joint space narrowing [2]. Hypertrophied synovia resulting from repeated intraarticular bleeding has low signal intensity on Ti -weighted spin-echo
Received May 20, 1991 : accepted after revision August 26, 1991. I Department of Radiology, The Mount Sinai Medical Center, City University of New York, One Gustave L. Levy Pt., New York, NY 10029-6574.
AJR 158:119-123,
in the first
and although
Ray Society
Address reprint
Downloaded from www.ajronline.org by NYU Langone Med Ctr-Sch of Med on 04/21/15 from IP address 128.122.253.212. Copyright ARRS. For personal use only; all rights reserved
120
HERMANN
hemoshows extremely hypertrophied synovial tissue (arrowheads) with intermediate signal intensity and eroslons of patella. Posterior cruciate ligament (arrow) is stretched but Intact.
Fig. 2.-32-year-old
Fig. 1.-22-year-old hemophiliac with philic arthropathy of right knee. MR image
Fig. 3.-13-year-old hemophiliac with mild knee Plain radiograph was normal. MR image shows thinning of femoral cartilage with irregular pain.
outline.
A and B, Proton
show decreased
El
AL.
AJR:158,
January
1992
hemophiliac with hemophilic arthropathy.
density-weighted
(SE 1600/30)(A)
and T2-weighted
signal intensity, which represents
arrows), and fibrosis. Femoral condyle in B (solid arrows) indicates effusion.
is thinned
subarticular
and eroded.
(SE 1600/80)(B)
lesion, detached
Synovium
is thickened.
MR images (open Bright signal
cartilage
Fig. 4.-18-year-old
hemophiliac with severe repeated hemarthrosis. TI-weighted (SE 500/20) coronal MR image (A) of knee shows enlarged epiphyses. Articular surface is destroyed. Large subarticular cyst formation is present on medial side (A, arrowheads). Central area of low signal intensity in A (arrow) is surrounded by high-signalA and B, Relatively
intensity structure that remains bright on T2-weighted (SE 2300/80) image (B, short wide arrow). Arrowheads in B point to bloody fluid. Femoral condyle shows subarticular defect as well (B, long thin arrows).
bone is intact, findings on conventional radiographs are normal. MR is capable of detecting early erosion of the cartilage
Soft-Tissue
(Fig. 3). Subchondral cysts usually bearing area as a result of fissuring
Bleeding into the large muscles is common. The most commonly involved muscles are the iliopsoas quadriceps and gastrocnemius. A permanent contraction results if the hematoma is replaced by fibrous tissue. When bleeding in a large muscle fails to resolve, it becomes encapsulated and forms a pseudotumor. Because sonography is convenient, inexpensive, and in-
Fluid may penetrate
from
imaging
to differentiate
clear
can be used
fluid before
radiographs
the cyst
(Figs.
bone resorption
develop at the weightof the articular cartilage.
the joint
blood
becomes
4 and 5). Bone
are well visualized
into the cyst
cysts
[3].
in the cyst
obvious caused
MR from
on the plain by trabecular
on CT (Fig. 6).
Hematomas
AJA:i58,
January
MUSCULOSKELETAL
i992
INVOLVEMENT
IN HEMOPHILIA
121
with severe hemo(5E 800/20) cownal MR image shows depression of medial plateau. Large area of destruction laterally (arrow) was not
Downloaded from www.ajronline.org by NYU Langone Med Ctr-Sch of Med on 04/21/15 from IP address 128.122.253.212. Copyright ARRS. For personal use only; all rights reserved
Fig. 5.-42-year-old patient philia. Moderately Ti-weighted
seen on conventional
radiograph.
Fig. 6.-26-year-old throsis
of right
man with recurrent
hemar-
shoulder.
CT scan of shoulder shows periarticular erosion (arrow) and subarticular cystlike formation of glenoid (arrowheads).
Fig. 7.-30-year-old
hemophiliac
with left-sided
pelvic mass. A, Sonogram of pelvis shows echo-free lesion in left psoas muscle (arrow). B, Sonogram obtained 3 weeks after A shows lesion is markedly smaller (arrow). Echoes have increased.
A Fig. 8.-72-year-old philia and numerous
B
patient with severe hemopseudotumors. CT scan
shows huge mass in left iliac fossa bulging into abdomen. Smaller pseudotumor with dense calcification
is present
at right iliac fossa.
Fig. 9.-34-year-old
patient
with severe
hemo-
philia and pelvic mass. CT scan of pelvis shows large pseudotumor in right iliac fossa. Mass displaces contrast-filled bowel (solid arrows). Note low attenuation
at center
of mass
(open
arrow).
volves no radiation, it is the method of choice for following the progression and regression of soft-tissue hematomas [4] (Fig. 7). Hemophilic pseudotumor is a rare complication that occurs in 1 -2% of hemophiliacs. Most develop in the muscles of the pelvis and lower extremity, where the large muscles have a rich blood supply, or in bone following intraosseous bleeding.
Sonography, Cl, and MR imaging have important roles in detecting pseudotumors, especially when they are in the pelvis. Cl is useful in detecting both the extent of soft-tissue masses and the involvement of bone. Pseudotumors contain coagulated blood and are surrounded by a thick wall (Fig. 8). Contrast-enhanced Cl is useful in determining the thickness of the wall. In the acute
Downloaded from www.ajronline.org by NYU Langone Med Ctr-Sch of Med on 04/21/15 from IP address 128.122.253.212. Copyright ARRS. For personal use only; all rights reserved
122
HERMANN
El
AL.
AJA:158,
January
1992
C
A Fig. lO.-36-year-old patient with severe hemophilia and mass in thigh. A, CT scan of thigh shows thick-walled low-density mass. B, Transaxial upside-down sonogram shows central anechoic area (arrow) C, Longitudinal sonogram shows extent of mass. Irregular echoes (arrow)
with enhancement
(E) of echoes
behind
mass,
indicating
fluid content.
represent solid material.
Fig. 1 1.-56-year-old hemophiliac with numerous pseudotumors. A, Ti-weighted (SE 500/27) MR image of pelvis shows high-signal-intensity mass in left gluteal region (straight arrow). Large mass in left pelvic region shows inhomogeneous, mostly decreased signal intensity (arrowhead). B, T2-weighted (SE 2000/80)
image.
Mass
left gluteal region remained bright. Left-sided
in
pcI-
vic mass shows signal intensity
inhomogeneous, mostly increased (arrowhead). Hypointense rim of masses in both A and B (curved arrows) represents chronic pseudotumor.
Fig. 12.-22-year-old hemophiliac with pelvic pseudotumor. A, Moderately Ti-weighted MR image (SE 700/ 24) shows well-demarcated mass with bright signal intensity surrounded by low-signal-intensity mass (arrows). B, T2-weighted MR image (SE 2100/90) shows part of mass is bright (arrow). Other areas show decreased signal intensity, indicating presence of blood of different ages (arrowhead). A
B
Downloaded from www.ajronline.org by NYU Langone Med Ctr-Sch of Med on 04/21/15 from IP address 128.122.253.212. Copyright ARRS. For personal use only; all rights reserved
AJR:i58,
January
1992
MUSCULOSKELETAL
INVOLVEMENT
stage, the center of the pseudotumor is hypodense on CT, but the periphery is isodense and indistinguishable from surrounding muscle [5] (Fig. 9). In the acute and subacute stages, sonography shows a central anechoic area with increased echoes behind the lesion, caused by fluid in the pseudotumor. A distinct plane separates the mass from the surrounding muscles (Fig. 10). CT shows the thick wall more consistently than does sonography. Multiple irregular echoes represent solid material that cannot be documented on CT. Differential diagnosis from abscess may be difficult. The MR signal characteristics of soft-tissue hemorrhage vary with the age of the blood [2]. Hematomas in the acute stage (1 -6 days) have the same signal as muscle has on Ti weighted MR images and low signal intensity on 12-weighted images [6]. Subsequently, the Ti relaxation time shortens progressively and the signal intensity increases (Fig. i 1). After 7 days, the peripheral portion of the hematoma becomes hyperintense on Ti - and 12-weighted images (Fig. 1 2). The center gradually becomes hyperintense over the next several weeks, usually in a patchy distribution [7].
IN HEMOPHILIA
The hypointense rim of a pseudotumor 12-weighted images consists of fibrous hemosiderin [6, 7] (Fig. 1 1).
123
on both 11 - and tissue that contains
REFERENCES 1 . Aesnick D, Niwayama G. Bleeding disorders. In: Aesnick 0, Niwayama G. eds. Diagnosis of bone and joint disorders. Philadelphia: Saunders,
1989:2497-2522 2. Julish BS, Lieberman JM, Strandjord SE, Bryan PJ, Mulopulos MT. Hemophilic arthropathy: assessment with MR imaging.
GP, Modic Radiology
1987:164:759-762 3. Murphy WA. Magnetic resonance imaging. In: Resnick D. Bone and joint imaging. Philadelphia: Saunders, 1989:120-135 4. Aspelin P. Pettersson H, Sigurjonsson S, Nilsson IM. Ultrasonographic examination of muscle hematomas in hemophiliacs. Acta Radiol 1984; 25: 51 3-516 5. Hermann G, Yeh HC, Gilbert MS. Computed tomography and ultrasonography of the hemophilic pseudotumor and their use in surgical planning. Skeletal Radiol 1986:15:123-128 6. Wilson DA, Prince JR. Imaging of hemophilic pseudotumors. AJR
1988:150:349-350 7. Aubin JI, Gomori JM, Grossman Al, Gefter WB, Kressel HY. High-field imaging of extracranial hematomas. AJR 1987;148 :813-817
MR
