AJR 2005; 185:330-332
© American Roentgen Ray Society
CT Angiography of Pulmonary Artery Aneurysms in Hughes-Stovin Syndrome
Eric S. Ketchum1,
Roham T. Zamanian2 and
Dominik Fleischmann1
1 Department of Radiology, Thoracic and Cardiovascular Imaging Sections,
Stanford University Medical Center, 300 Pasteur Dr., Rm. S-072, Stanford, CA
94305-5105.
2 Department of Medicine, Vera Moulton Wall Center for Pulmonary Vascular
Disease, Division of Pulmonary and Critical Care Medicine, Stanford University
Medical Center, Stanford, CA.
Received August 26, 2004;
accepted after revision September 16, 2004.
Address correspondence to D. Fleischmann
(d.fleischmann{at}stanford.edu).
Introduction
Hughes-Stovin syndrome is a rare disorder of unknown cause that is
characterized by the combination of multiple pulmonary artery aneurysms and
deep venous thrombosis. We present a case of a middle-aged man with a 2-year
history of deep venous thrombosis who developed multiple pulmonary artery
aneurysms at sites of earlier endoluminal thrombi. We briefly consider the
pathogenesis of Hughes-Stovin syndrome and show the additional insights
offered by 3D volume rendering to visualize the presence of an increased
number of morphologically abnormal bronchial arteries even before aneurysm
formation.
Case Report
A 49-year-old Afghan man was first admitted to our hospital in August 2003
after presentation to our hospital's emergency department with acute pleuritic
chest pain and shortness of breath on exertion. His medical history was
significant for several treatments at another institution during the past 2
years, including a May 2001 bypass surgery for a spontaneous left posterior
tibial artery pseudoaneurysm, a December 2002 diagnosis of right thigh and
calf deep venous thrombosis, a June 2003 admission for fever and hemoptysis,
and a late July 2003 diagnosis by CT angiography of bilateral pulmonary emboli
during an admission for high fever and aching chest pain. His deep venous
thrombosis was being medicated with warfarin sodium.
At the time of admission to our institution, the patient reported recent
night sweats and chills, a dry cough, and 25-lb (11.4-kg) weight loss. He was
found to have a low-grade fever. Laboratory values included an erythrocyte
sedimentation rate of 111 mm/hr, an international normalized ratio of 2.8,
hematocrit of 28.2%, and hemoglobin of 9.3 g/dL. Bronchoscopy did not reveal
the cause of the hemoptysis, and bronchoalveolar lavage samples sent for
culture returned negative findings.
A pulmonary CT angiogram and lower extremity CT venogram were obtained with
an 8-MDCT scanner after IV administration of 150 mL of contrast medium (350 mg
I/mL). Images showed filling defects in the pulmonary arterial tree (Figs.
1A and
1B) and a nonocclusive filling
defect in the right popliteal vein that was consistent with pulmonary
embolism. A 25-mm-diameter pseudoaneurysm of the right superficial femoral
artery with narrowing of the common femoral vein was incidentally detected. A
Greenfield filter was placed in the inferior vena cava the next day. The right
superficial femoral artery aneurysm was treated electively with resection and
placement of a polytetrafluoroethylene graft at the beginning of October.
Pathology samples of the resected arterial pseudoaneurysm revealed fibrosis
and acute inflammation, but no microorganisms. The preoperative abdominal and
lower extremity CT angiograms revealed interval extension of the venous
thrombosis into the pelvis and inferior vena cava, which was treated with
percutaneous mechanical catheter-directed thrombectomy and stenting.
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Fig. 1A —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Pulmonary CT angiographic
images obtained in August 2003 show wall-adherent soft-tissue-density filling
defects in right lower lobe pulmonary artery (arrow, A) and in
left pulmonary artery (arrowhead, B). Note subtle rim of
enhancement at periphery of presumed endoluminal thrombus.
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Fig. 1B —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Pulmonary CT angiographic
images obtained in August 2003 show wall-adherent soft-tissue-density filling
defects in right lower lobe pulmonary artery (arrow, A) and in
left pulmonary artery (arrowhead, B). Note subtle rim of
enhancement at periphery of presumed endoluminal thrombus.
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The patient was readmitted to our institution in December 2003 after
experiencing morning episodes of hemoptysis and chest discomfort. A pulmonary
CT angiogram identified three saccular pulmonary aneurysms, measuring
15–27 mm in diameter, at the sites of the previously seen intraluminal
thrombi (Figs. 1C and
1D). Three-dimensional analysis
of the data set revealed prominent and tortuous bronchial arteries and
numerous small vessels surrounding the pulmonary artery aneurysms (Figs.
1G and
1H). Retrospective 3D analysis
of the August 2003 study revealed increased vascularity surrounding the
pulmonary arteries before the development of the pulmonary aneurysms (Figs.
1E and
1F). The warfarin sodium
therapy was deemed a failure and was replaced by low-molecular-weight heparin
injections.
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Fig. 1C —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Follow-up pulmonary CT
angiographic images obtained in December 2003 show interval development of
pulmonary artery aneurysms at sites of prior thrombus in right lower lobe
segmental branch (arrow, C) and in superior segment of left
lower lobe artery (arrowhead, D).
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Fig. 1D —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Follow-up pulmonary CT
angiographic images obtained in December 2003 show interval development of
pulmonary artery aneurysms at sites of prior thrombus in right lower lobe
segmental branch (arrow, C) and in superior segment of left
lower lobe artery (arrowhead, D).
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Fig. 1G —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Volume-rendered images
(posterior views) of pulmonary arteries from December 2003 that correspond to
pulmonary angiograms obtained at same time (C and D) show
pulmonary artery aneurysms (arrowhead, G; arrow,
H) in same anatomic locations as in other December 2003 images
(C and D). Note fine web of bronchial artery vessels surrounding
aneurysms.
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Fig. 1H —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Volume-rendered images
(posterior views) of pulmonary arteries from December 2003 that correspond to
pulmonary angiograms obtained at same time (C and D) show
pulmonary artery aneurysms (arrowhead, G; arrow,
H) in same anatomic locations as in other December 2003 images
(C and D). Note fine web of bronchial artery vessels surrounding
aneurysms.
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Fig. 1E —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Volume-rendered images
(posterior views) of pulmonary arteries from August 2003 that correspond to
pulmonary angiograms obtained at same time (A and B) show
increased vascularity surrounding left pulmonary artery at origin of superior
segment of left lower lobe branch (arrowhead, E) and in right
lower lobe (arrow, F).
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Fig. 1F —49-year-old man who presented to emergency department with
acute pleuritic chest pain and shortness of breath on exertion in August 2003.
Medical history was significant for May 2001 bypass surgery for spontaneous
left posterior tibial artery pseudoaneurysm, December 2002 diagnosis of right
thigh and calf deep venous thrombosis, June 2003 admission for fever and
hemoptysis, and late July 2003 diagnosis of bilateral pulmonary emboli during
admission for high fever and aching chest pain. Volume-rendered images
(posterior views) of pulmonary arteries from August 2003 that correspond to
pulmonary angiograms obtained at same time (A and B) show
increased vascularity surrounding left pulmonary artery at origin of superior
segment of left lower lobe branch (arrowhead, E) and in right
lower lobe (arrow, F).
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The patient has been followed closely since discharge from the hospital in
December 2003. A follow-up April 2004 CT angiography study revealed reduction
in the size of all three pulmonary aneurysms. Continued deep venous thrombosis
was present. As of August 2004, the patient has not reported additional
hemoptysis and has remained in generally good health.
Discussion
Cases of deep venous thrombosis coupled with pulmonary artery mural thrombi
and multiple aneurysms of the pulmonary arteries were first presented as a
discrete pathologic entity by Hughes and Stovin
[1] in 1959. The eponym
"Hughes-Stovin syndrome" was applied by Kopp and Green
[2] in 1962. The typical
clinical presentation of Hughes-Stovin syndrome consists of three phases: a
first stage involving symptoms of thrombophlebitis, a second stage consisting
of formation and enlargement of pulmonary aneurysms, and a third stage of
aneurysmal rupture that triggers massive hemoptysis and death
[3].
The cause of this entity is currently unknown, and a systemic vasculitis
has been suggested. All of our findings—spontaneous posterior tibial
artery aneurysm, acute inflammation within the resected superficial femoral
artery aneurysm, and the history of deep venous thrombosis and pulmonary
emboli—support systemic arterial involvement. Immunosuppression is a
frequent treatment, although its effectiveness has not been proven
[4]. Infectious causes have
generally been considered less likely because of the ineffectiveness of
antibiotic therapy and the lack of microbiologic findings in blood cultures,
although the possibility of undetected organisms of low-grade virulence has
been raised.
Some researchers have suggested that Hughes-Stovin syndrome may be
partially manifested Behçet's syndrome
[5]. Pathologic studies of
Hughes-Stovin syndrome have repeatedly shown mural thrombus and inflammation,
and many of the reports of pulmonary manifestations of Behçet's
syndrome have suggested that an intraluminal clot in the pulmonary arteries
might evolve in situ secondary to pulmonary artery wall inflammation rather
than to venous thromboembolism—notably, in cases without deep venous
thrombosis.
In our patient, the initial CT images clearly show a rim of enhancement at
the pulmonary artery wall adjacent to the intraluminal filling defects; this
finding is consistent with inflammation—a finding that is not usually
seen in acute or chronic pulmonary embolism. Even in the presence of deep
venous thrombosis in our patient, the pulmonary artery thrombi could have
originated in situ. However, most of the reported cases of Hughes-Stovin
syndrome, including ours, have not fulfilled the diagnostic criteria of
Behçet's syndrome—oral ulceration and recurrent genital
ulceration, eye lesions, skin lesions, or a positive pathergy test
[4].
The pathogenesis of pulmonary artery aneurysms in Hughes-Stovin syndrome
has been attributed to weakening of the vessel wall due to inflammation, which
corresponds well to our observation that the aneurysms developed at the
locations of prior thrombus and abnormal enhancement. In their original
description, Hughes and Stovin
[1] noted degenerative changes
in the bronchial arteries at autopsy including fibrosis, a loss of muscle and
elastic fibers, and a narrowing of the vessel lumen
[1]. Mahlo et al.
[6] and Herb et al.
[7] performed digital
subtraction angiography of the bronchial arteries and noted distorted and
dilated bronchial arteries with convoluted small branches. Mahlo et al.
speculated that the cause of death in many of the previously reported
Hughes-Stovin cases may have been rupture of angiodysplastic bronchial
arteries rather than rupture of aneurysmal pulmonary arteries. Bronchial
artery embolization was performed in both cases and noted as an effective
therapeutic approach.
Although pulmonary angiography can clearly depict pulmonary aneurysms in
Hughes-Stovin syndrome [8], CT
angiography allows visualization of the vessel lumen, mural thrombus, vessel
wall, and mediastinum. The volume-rendered images of our patient showed
prominent and tortuous bronchial artery branches that apparently supplied a
web of smaller vessels at the sites of pulmonary artery wall inflammation even
before the actual aneurysm formation. Without pathologic confirmation, we can
only speculate that this finding corresponds to Hughes and Stovin's original
theory [1] that the involvement
of degenerative bronchial arteries leads to changes in the vasa vasorum of the
pulmonary arteries and the development of a novel response—namely, the
formation of an aneurysm—to thromboemboli.
Regardless, prominent bronchial arteries and pathologic enhancement of
pulmonary thromboemboli seen on CT pulmonary angiograms should caution the
radiologist to question the simple diagnosis of pulmonary embolism and should
raise suspicion of pulmonary vasculitis. Earlier diagnosis could, in some
cases, allow the prevention of the development of potentially life-threatening
pulmonary aneurysms.
References
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peripheral venous thrombosis. Br J Dis Chest1959; 53:19
–27[CrossRef][Medline]
- Kopp WL, Green RA. Pulmonary artery aneurysms with recurrent
thrombophlebitis: the "Hughes-Stovin syndrome." Ann
Intern Med 1962; 56:105
–114
- Reimold WV, Emmrich J, Harmjanz D, Kochsiek K. Multiple aneurysms
of the pulmonary artery following recurrent septic pulmonary embolism
(Hughes-Stovin syndrome): report of 1 case [in German]. Arch Klin
Med 1968; 215:1
–18[Medline]
- Erkan F, Gul A, Tasali E. Pulmonary manifestations of Behcet's
disease. Thorax 2001;56
: 572–578[Free Full Text]
- Durieux P, Bletry O, Huchon G, Wechsler B, Chretien J, Godeau P.
Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin
syndrome. Am J Med 1981;71
: 736–741[CrossRef][Medline]
- Mahlo HR, Elsner K, Rieber A, Brambs HJ. New approach in the
diagnosis of and therapy for Hughes-Stovin syndrome.
AJR 1996; 167:817
–818[Medline]
- Herb S, Hetzel M, Hetzel J, Friedrich J, Weber J. An unusual case
of Hughes-Stovin syndrome. Eur Respir J1998; 11:1191
–1193[Abstract]
- Ammann ME, Karnel F, Olbert F, Mayer K. Radiologic findings in the
diagnosis of Hughes-Stovin syndrome. AJR1991; 157:1353
–1354[Medline]
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Introduction
Case Report
