Acta Radiologica

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Intraosseous arteriovenous malformations

mimicking malignant disease

S. J. Savader, B. L. Savader & R. R. Otero

To cite this article: S. J. Savader, B. L. Savader & R. R. Otero (1988) Intraosseous

arteriovenous malformations mimicking malignant disease, Acta Radiologica, 29:1, 109-114, DOI:
10.1080/02841858809171222

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Published online: 04 Jan 2010.

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 Acta Radiologica 29 (1988) Fasc. 1

FROM T H E DEPARTMENT O F RADIOLOGY, UNIVERSITY OF SOUTH FLORIDA COLLEGE O F MEDICINE, TAMPA,

FLORIDA 33612, USA.

INTRAOSSEOUS ARTERIOVENOUS MALFORMATIONS MIMICKING

MALIGNANT DISEASE

S. J. SAVADER.
B. L. SAVADER
and R. R. OTERO

Abstract 1920’s. In 1932, DETAKAS(1) clearly laid grounds for their

Primary (congenital) intraosseous arteriovenous malformations origin in the abnormal development of the primitive vas-
are rare, accounting for less than one per cent of all primary cular system, with occasional involvement of anomalous
intraosseous lesions. They are quite variable in their gross and nerve and lymphatic elements. The vascular tree first
microscopic presentation, yet all can be traced to anomalous appears embryologically as an interlacing system of blood
development of the primitive vascular system. They may be
totally asymptomatic, cosmetically disfiguring, painful, or on rare spaces in the primitive mesenchyme. Separate venous and
occasions, cause high-output cardiac failure. Surgical treatment arterial vessels are not evident and mixed blood circulates
is often unrewarding with recurrence not uncommon. Intra-arte- within. As the arterial system differentiates, the blood
rial embolization has shown promising results. We present three Iakes are partially resorbed and coalesce, forming distinct
cases of primary intraosseous arteriovenous malformations
which on initial work-up mimicked malignant disease. Arteriogra-
arterial and venous structures on either side of a capillary
phy proved diagnostic in all three cases. network. A small portion of the primitive blood channel
remains as the capillary bed (8). The cardiovascular sys-
Key words: Arteriovenous malformations, extremities; -,
intraosseous. tem is the first functional embryonic system. Arrest or
misdirection of the normal developmental pattern occur-
ring at any stage can give rise to anomalous circulatory
Peripheral symptomatic arteriovenous malformations structures which can be closely correlated clinically and
are uncommon lesions. In an extensive study by SZILAGYI pathologically to the time of the insult.
et coll. (81, they accounted for approximately one in More than one-half of the reported cases of congenital
loo00 hospital admissions. Those arising primarily within arteriovenous malformations involve the extremities (10).
and involving bone are even less common. In general, Approximately one-third of these occur in the upper ex-
arteriovenous malformations have a wide variety of clini- tremity versus two-thirds in the lower extremity. While
cal manifestations ranging from totally asymptomatic, both soft tissue and intraosseous arteriovenous malforma-
barely perceptible strawberry birthmarks, to massive tions have identical origins, their clinical presentation and
clusters of varicose vascular channels that may cause true radiographic findings are quite different. We present three
limb deformity. A long list of descriptive names have been cases of primary intraosseous arteriovenous malforma-
given to their more impressive examples, such as heman- tions which mimicked malignant disease on initial conven-
gioma simplex, angioma telangiectaticum, angioma caver- tional radiographs and scintigrams.
nosum, strawberry birthmark, port wine stain, serpentine
aneurysm, cirsoid aneurysm, and angioma arteriale race-
m o w n (9). These lesions can occur as isolated phenom- Case reports
ena or may be multiple and diffuse as is seen in Osler- Case 1. A 28-year-old white female presented to the emergency
room with a six-week history of post-traumatic edema, erythema
Weber-Rendu syndrome, Parkers-Weber syndrome, and
and pain of the right upper extremity following an assault. Physi-
Klippel-Trenaunay syndrome.
Regardless of the name or syndrome all are unitary in
origin as suggested by REINHOFF (6) and REID (5) in the Accepted for publication 20 July 1987.

109
 S . 1. SAVADER, 0 . L. SAVADER AND R. R. OTERO

Fig. 1. Case 1. a) Radiograph of the right shoulder demonstrates a merial phase there are multiple serpiginous intra- and extraos-
large irregular lytic lesion involving the humeral head and proxi- SeOUS small and medium sized arteries arising from the circum-
ma1 shaft. No penosteal reaction is seen and the cortex is intact. flex humera1 artery, early draining veins and residual venous
b) The bone scan demonstrates diffuse increased activity in the pooling within the arteriovenous malformation.
entire right humerus. At angiography in c) early and d) late
 INTRAOSSEOUS ARTERIOVENOUS MALFORMATIONS 111

a b C
Fig. 2. Case 2. a) Radiograph of right forearm demonstrates a arterial phase demonstrates a prominent interosseous and radial
large irregular lytic lesion of the distal radial shaft involving artery with innumerable permeating interosseous branches, and a
the medullary canal and cortex. Intact cortex and absence of small extraosseous component overlying the distal radial shaft
periosteal reaction. Angiography in b) early and c) late and scaphoid bone.

cal examination demonstrated a swollen, tender, right upper arm cal examination demonstrated multiple varicosities and a ‘boggy’
with a blanching telangiectatic rash sparing only the palm. Exten- feel to the soft tissue of the right forearm. There was no pain on
sive ecchymosis was present over the upper arm and shoulder palpation. Radiography showed multiple serpiginous lytic ap-
and a subclavian thrill was palpated. Initial radiography showed a pearing lesions of the distal radius (Fig. 2a). The cortex was
large irregular lytic lesion involving the humeral head and proxi- intact and periosteal reaction was absent. Arteriography demon-
mal humeral shaft (Fig. 1 a). strated multiple medium and small caliber corkscrew vessels
Prominent intraosseous channels demonstrated dense ‘cortical’ arising from the radial and interosseous artery which permeated
margins. Periosteal reaction, pathologic fracture, and a soft tissue the distal radius to the articular surface (Fig. 2 b, c). The radial
mass were all absent. A bone scan showed mild increased tracer artery overlying the lesion was also enlarged and quite tortuous.
uptake in the right humeral head and shaft (Fig. 1 b). Arteriogra- Draining veins were apparent but were not significantly tortuous
phy demonstrated a large intraosseous arteriovenous malforma- or distended.
tion with innumerable small and medium caliber ‘corkscrew’ The patient’s blood blister was not assumed to represent a
arteries arising principally from the circumflex humeral artery superficial manifestation of the arteriovenous malformation and,
and large dilated vessels draining into the subclavian vein (Fig. therefore, conservative physical therapy and orthopedic follow-
1 c, d). Generalized hypertrophy of the involved vessels, both up was recommended. At this time, the patient reports that she is
arterial and venous, was evident. A small component of the doing well and her forearm pain is controlled with mild analge-
arteriovenous malformation was present in the soft tissues of the sics.
elbow. A rapid venous phase due to a high flow state was noted. Case 3. A 14-year-old white male presented to the emergency
The patient had been asymptomatic prior to her injury, there- room five days following a football injury which resulted in
fore, conservative treatment was recommended. At this time, she increasing pain and swelling over the right tibia and fibula. Physi-
is again asymptomatic and scheduled for routine orthopedic fol- cal examination demonstrated a one centimeter leg length dis-
low-up. crepancy (right longer than left), noticeable swelling over the
Case 2. A 22-year-old white female saw her physician after a right tibia, fibula, and ankle, tenderness, decreased range of
painless blood blister on the ventral surface of her wrist demon- motion at the knee and ankle, and multiple prominent varicosities
strated increasingly recurrent spontaneous bleeding over the past on the anterior and posterior surface of the lower leg. Past
three months. She stated that she had noticed varicose veins on medical history included right lower leg pain since childhood and
her right forearm for the past year, and gradually increasing increasing over the past two years, varicose veins which were
forearm pain, unrelated to activity, over the past 10 years. Physi- diagnosed at age five, and an outside hospital diagnosis of multi-
112 S. J. SAVADER, B. L. SAVADER AND R. R. OTERO

a b C d
Fig. 3. Case 3. a) Radiography of the right lower leg demonstrates nous system and superficial varicosities. Incompetent perforators
multiple lengthy subcortical and medullary lytic lesions of the were appreciated on close inspection. d) Selective angiography
tibia without periosteal reaction or a soft tissue component. b) shows multiple anomalous arterial vessels (+) arising from the
The bone scan shows diffuse homogeneous tracer uptake in the posterior tibial artery perforating the tibial cortex (nutrient arte-
proximal two-thirds of the right tibia. The remaining skeleton is ries) and forming arterial tufts within the medullary cavity (++).
normal. c) Phlebography demonstrates a large dilated deep ve-

ple venous angiomas without arterial involvement of the right arteriovenous malformations by SZILAGYI et coll. (8, 9),
lower leg. Radiography of the right tibia and fibula (Fig. 3a) revealed nearly equal male and female incidences. The
demonstrated multiple linear subcortical and intramedullary lytic
majority of these patients presented at birth. A second
lesions of the tibial midshaft without periosteal reaction or a soft
tissue component. The fibula was uninvolved. A bone scan (Fig. peak was present in the 20 to 40 year range. The average
3 b) demonstrated increased homogeneous tracer uptake in the age in our patient group was 21.7 years. This may suggest
proximal two-thirds of the right tibia. Phlebography was per- a later presentation due to the lack of a grossly visible
formed to rule out deep venous thrombosis (Fig. 3 c). There was lesion other than varicosities. These varicosities may be a
no evidence of deep venous thrombosis but the venous system
was greatly distended, with multiple varicosities and incompetent later occurring secondary development which are not
perforators. Selective arteriography showed multiple anomalous present at birth. In a study by SZILAGYI et coll. (9), disfigu-
arteries arising from the posterior tibial artery. These vessels ration was the dominant presenting complaint (21 %), fol-
perforated the tibial cortex forming arteriovenous tufts within the lowed by swelling (19%), pain (15%), and varicose veins
medullary cavity (Fig. 3d). Large draining veins, vessels with (1 1 %). In our group, all three patients had pain and two of
tumor encasement, and 'puddling' were absent. Primary intraos-
seous arteriovenous malformation was confirmed. three had noticeable varicose veins. Soft tissue arteriove-
Due to the extensive nature of the lesion and lack of a single nous malformations of the extremities most often involve
large feeding artery, conservative treatment consisting of physi- the femoral or iliac artery as the vessel of origin (62%),
cal therapy and unilateral Jobst stocking fitting was recommend- whereas the axillary, circumflex humeral, radial and pos-
ed with orthopedic follow-up. The patient has been lost to follow-
terior tibial arteries (the vessels of origin in our group)
UP.
accounted for less than 9 per cent (9). On physical exami-
nation, 85 per cent of the patients in the series of SZILAGYI
Discussion et coll. demonstrated swelling, 61 per cent varicose veins
Studies on intraosseous arteriovenous malformations either alone or in combination with other findings, and 43
are unavailable in the literature. However, there are nu- per cent had a cutaneous hemangioma. Both swelling and
merous studies on soft tissue arteriovenous malformations varicose veins were a common finding in our group, but
of the extremities. Two extensive studies of soft tissue none had a hemangioma.
 INTRAOSSEOUS ARTERIOVENOUS MALFORMATIONS 113

The radiologic findings in patients with soft tissue and arterial embolization, conservative therapy, or a combina-
intraosseous arteriovenous malformations are different. tion of these modalities. Surgical therapy alone often
Soft tissue arteriovenous malformations (other than a pos- yields unrewarding results (2, 4, 8-10). When the primary
sible soft tissue mass) are usually not demonstrated at feeding vessel is surgically ligated, collateral circulation
conventional radiography or scintigraphy. In contradis- will often restore the vascular supply to the lesion. Usual-
tinction to this, intraosseous arteriovenous malformations ly, the arterial supply is so extensive that complete surgi-
demonstrate significant findings on both studies. cal ablation is impossible. In addition, intraosseous arte-
Our three patients presented with extremity pain and riovenous malformations would require extensive and mu-
abnormal findings in the area of interest. Initial evaluation tilating bone surgery in order to attempt cure. Surgery has
of each case demonstrated multiple lytic appearing bone proven to be most beneficial in very focal, well defined
lesions which raised strong suspicion for an osseous ma- lesions (8). FRY(2) states that surgery is indicated: I )
lignancy. Further examinations showed this not to be so when large arteriovenous malformations precipitate heart
in each case. Careful review of the films revealed multiple failure secondary to massive shunting of blood, 2) for
characteristic findings consistent with the final diagnosis relief of pain caused by encroachment on nerves or weight
of intraosseous arteriovenous malformation. These find- bearing surfaces, or 3) to eliminate the risk of hemorrhage
ings include: 1) multiple smooth lytic or lucent appearing caused by superficial lesions vulnerable to trauma.
serpiginous lesions, often with sclerotic borders, involv- Intra-arterial embolization has met with promising SUC-
ing the cortex and medullary canal, 2) smooth serpiginous cess as both an adjunct to surgery and as the primary
lytic or lucent lesions that extend from the medullary mode of therapy ( 3 , 4 , 7) as shown in a study by OLCORet
canal through the cortex without associated periosteal coll. (4).Intra-arterial embolization has proven to be rela-
abnormalities, 3) lack of a Codman’s triangle or other less tively safe, although transient post-procedure fever,
dramatic periosteal reaction, 4) expansion of the medul- chills, and non-fatal pulmonary embolus have been re-
lary canal, 5 ) absence of cortical expansion although the ported (7).
lytic lesions may extend to the cortical margin, and 6) lack Conservative therapy also has its place in the treatment
of an associated soft tissue mass. Evaluation of the radio- of arteriovenous malformations. SZILACYI et coll. (8) re-
graphs in our patient group demonstrated the presence of ported that 12 of 23 (52 %) patients treated conservatively
at least five of these six important characteristic findings experienced no progression of their symptoms over an
in each case. average follow-up period of 3 112 years. None of the
Bone scanning with 99Tcm-MDP, while non-specific, patients improved but only 3 of 23 (13%) experienced
demonstrates homogedeously increased tracer uptake in worsening of their symptoms. In our group two patients
the involved bone, while all other skeletal structures show have been followed for up to 15 months and neither has
normal tracer activity. experienced a progression of symptoms with conservative
Phlebography in both soft tissue and intraosseous arte- therapy.
riovenous malformations demonstrates similar character- In summary, congenital intraosseous arteriovenous
istics including venous dilatation, early draining veins and malformations are complex lesions. Clinical findings are
varicosities. Intraosseous arteriovenous malformations, non-specific and radiologic examination is a necessity.
however, may demonstrate intraosseous draining veins. Conventional radiography can be misleading in that the
While this study will not usually distinguish between the intraosseous arterial and venous channels can appear as
two types of arteriovenous malformations, it will lend irregular, lytic, and expansile lesions suggestive of a ma-
further support to the final diagnosis. lignancy. Careful scrutiny of the films for the characteris-
Arteriography is the final and confirmatory diagnostic tic smooth, often sclerotic, serpiginous channels, and the
test in both soft tissue and intraosseous arteriovenous lack of periosteal reaction, cortical destruction, or soft
malformations. Both can include arteriovenous communi- tissue mass, along with other characteristic findings can
cations, arterial dilatation, arterial tortuosity, and abnor- lead to an early diagnosis. If the lesion is highly suspicious
mal arterial branching. Only intraosseous arteriovenous for an intraosseous arteriovenous malformation, one may
malformations, however, will demonstrate arterial vessel wish to proceed directly to arteriography for confirma-
perforating adjacent bone cortex, intraosseous arterial tion. Treatment must be tailored for each patient based on
tufts and corkscrew vessels, and intraosseous early drain- the symptoms, the extent and location of the lesion as
ing veins. Hypervascular malignant tumors may show defined by arteriography , and the surgical risks.
some of the same findings; however, intraosseous arterio- Request for reprints: Dr Scott J. Savader, Department of Ra-
venous malformations will not demonstrate the tumor diology, University of South Florida College of Medicine, 12901
blush and encased and occluded vessels typical of a malig- N . 30th Street, Box 17, Tampa, Florida 33612, USA.
nant lesion. The absence of a uniform accumulation will
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