VoL. 95, No.

CIRSOID ANEURYSM OF THE UTERUS: SPECIFIC

ARTERIOGRAPHIC
REPORT OF A CASE

By V. A. M. FRENCKEN and G. H. M. LANDMAN

NIJMEGEN, THE NETHERLAND5

A CIRSOID aneurysm consists of multi- Apart

and pronounced
from
menstrual
the above-mentioned
blood loss, the his-
symptoms
ple arteriovenous aneurysms forming
a mass of dilated arteries and veins. This tory was negative. The blood pressure was first
condition is, as a rule, encountered in the read as i8o/oo mm. Hg but fellto io/8o mm.
Hg during clinical observation. The pulse was
cranium and in the extremities.
regular (76/mm.). On physical examination,
A cirsoid aneurysm of the uterus is a
percussion disclosed enlargement of the heart to
rarity; the usual textbooks of pathology
the left.
make no mention of this localization. In An early systolic murmur (Grade II) was
this condition the uterine wall is completely found in the left second intercostal space next to
or partly made up of numerous arteriove- the sternum. Auscultation of the abdomen dis-
American Journal of Roentgenology 1965.95:775-781.

nous fistulae, while the para-uterine arteries closed a high-frequency continous murmur
and veins show pronounced dilatation. (Grade iv) immediately below the umbilicus, in
Only 7 cases of uterine cirsoid aneurysm which area a thrill was palpable. No abnormal
have been described in the literature. In resistance was felt. The consulting cardiologist
addition, there are reports on a few cases in (J. Th. Ch. Vonk, M.D.) diagnosed an intra-
abdominal arteriovenous aneurysm. There were
which the abnormality was more localized,
no signs of hypercirculation. The circulation
and without dilatation of the uterine yes-
time was normal (I4 sec.). Digital vaginal ex-
sels. In nearly all cases, the patient was amination disclosed a moderately enlarged, soft
first examined because of severe vaginal uterus. The electrocardiogram was normal.
hemorrhages. In some cases a diagnostic Laboratory findings were normal. In view of the
curettage was carried out, but in nearly all menorrhagia, the gynecologist (J. C. Seelen,
cases the associated severe hemorrhages M.D., Department of Gynaecology; Head:
made it necessary to perform a hysterec- Prof. L. A. M. Stolte, M.D.) was consulted.
tomy immediately afterwards. Gynecologic The patient
Findings. had had io
We believe that the case presented in this children, the youngest of whom was I year old.
All pregnancies and parturitions had been nor-
paper is the first in which a definite pre-
mal. In 1948, the patient had been curetted
operative diagnosis was made by arteriog-
because of a molar pregnancy. Menstruation
raphy.
had been regular. The blood loss, previously
normal, had increased after the last parturition.
REPORT OF A CASE
A normal menstruation occurred during the
In of i 964, a 42 year old woman
May re- period of investigation. Gynecologic examina-
ported to the ophthalmologic out-patient clinic tion disclosed a soft uterus, the size of a 3 month
(Prof. J. E. A. van den Heuvel, M.D.) with pregnancy. Pronounced arterial pulsations were
symptoms of dyslexia. In addition, she corn- palpable on either side of the uterus. A pregnos-
plained of fatigue, headaches and dizziness. A ticon reaction (pregnancy test based on the
small hemorrhage was found in the right ocular immunologic demonstration of chorionic go-
fundus. The blood pressure was i8o/ioo mm. nadotropins) was carried out and repeatedly
Hg, and, for further investigation of the hyper- found to be negative.
tension, the patient was referred to the Depart- Roentgenographic Findings. The chest roent-
ment of Internal Medicine (Prof. C. L. H. genogram showed a moderately enlarged left
Majoor, M.D.). ventricle. Intravenous pyelography was per-

* From the Department of Radiology (Head: W.H.A.M. Penn), St. Radboud Hospital, University of Nijmegen, The Netherlands.

775
 776 V. A. M. Frencken and G. H. M. Landman NOVEMBER, 1965

the pathologic anatomic findings obtained in

the few cases of cirsoid uterine aneurysm de-
scribed in the literature. On this basis we be-
lieved ourselves justified in making this diagno-
sis. There was no precedent of an arteriographic
examination in such a case.
It was decided to perform an abdominal
hysterectomy, which for personal reasons had
to be postponed for some time.
In August, 1964 the patient (at home) no-
ticed a vaginal discharge, and she was admitted
to the gynecologic department. An I I week
pregnancy was diagnosed, which ended in a
spontaneous abortion.
Abdominal hysterectomy was performed in
December, 1964 (Prof. E. j. Moeys, M.D.,
surgeon and j. C. Seelen, M.D., gynecologist).
FIG. 1. The ovarian arteries are dilated and The uterus was found transformed into an in-
very tortuous.
tensively pulsating tumor. The uterine and
American Journal of Roentgenology 1965.95:775-781.

ovarian vessels were pencil to thumb-sized. The

formed in view of the previously observed hy- internal iliac arteries were twice as wide as the
pertension. The kidneys were of equal size and external iliac arteries. After ligation of the
excreted the contrast medium simultaneously. hypogastric and ovarian arteries, the uterus
The medial contour of, in particular, the distal greatly diminished in size. No peroperative or
portion of both ureters showed some constant postoperative complications occurred. A chest
semicircular filling defects suggestive of vascu- roentgenogram made 3 months after operation
tar impressions. The distal portion of the left showed that the heart had slightly diminished in
ureter, moreover, showed a fairly pronounced size.
lateral curve. Pathologic Findings. (Pathological Depart-
Since an arteriovenous fistula in the abdomen ment, Head Prof.
M. Schillings P.
M.D.). H.
was suspected, abdominal arteriography was The uterus measured I 2 X 8 X 5 cm. The myo-
carried out by the Seldinger technique. metrium showed pronounced hypertrophy. In
The ovarian arteries were dilated and tortu- the fundus, the myometrium contained convo-
ous (Fig. I.) Both common iliac arteries were lutions of dilated vessels and cavities of irregu-
wider than normal; the right common iliac lar shape, filled with blood. The two ligamenta
artery showed displacement in a craniad direc- lata also contained convolutions of dilated
tion. The internal iliac arteries showed pro- vessels (Fig. 3.)
nounced dilatation to a width about twice as Microscopic examination showed that the
large as that of the normal external iliac ar- vascular convolutions consisted partly of ar-
teries. The uterine arteries were likewise greatly teries and partly of veins. In both parametria,
dilated and tortuous. From here, numerous and in the ovarian hili as well, there were re-
larger and smaller tortuous vessels filled with markably large and dense convolutions of both
the contrast medium; these vessels formed an arteries and veins. The condition was diagnosed
approximately fist-sized mass in the true pelvis; as a cirsoid aneurysm of the uterus.
undoubtedly, this was an enlarged and highly
vascularized uterus (Fig. 2, A, B and C). DISCUSSION
Initially, a large number of tortuous vessels
Cirsoid aneurysm of the uterus-in
were visible immediately caudal to this mass in
which the uterine wall is completely or
the left parametrium; these vessels, however,
partly made up of numerous arteriovenous
emptied more rapidly than the uterine vessels.
The contrast medium drained off via greatly fistulae-gives rise to pronounced dilata-
dilated veins, including very wide ovarian veins tion of para-uterine arterial as well as
(Fig. 2D). The vascular pattern demonstrated venous vessels. The principal data on the
by arteriography completely corresponded with published cases are presented in Table .
 VOL. 95, No. 3 Cirsoid Aneurysm of the Uterus 777
American Journal of Roentgenology 1965.95:775-781.

FIG. 2. (A) Pronounced dilatation of the common and internal iliac arteries. The right common iliac artery
shows displacement in a craniad direction. The uterine arteries, too, show marked dilatation. (B and C)
Numerous tortuous vessels are filled which together constitute a vascular convolution, the approximate
size of a fist, in the uterus and parametria. (D) Greatly dilated and tortuous veins, e.g., the ovarian veins
are demonstrated.

Hemangiomata in the uterus are more forming a cirsoid aneurysm. In the case
common. Ratzenhofer1t and Salm1’ pre- of a hemangioma, moreover, the pelvic
sent a good review of the cases described. vessels are not quite so greatly dilated. In
Generally, hemangiomata are more local- spite of these differences, the impression is
ized than the cirsoid aneurysm, and the that it is sometimes exceedingly difficult to
vessels which constitute the hemangioma differentiate these two conditions. Perhaps
show a uniform picture, unlike the vessels transi tional forms between hem angiom a
 778 V. A. M. Frencken and G. H. M. Landman NOVEMBER, 1965

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American Journal of Roentgenology 1965.95:775-781.

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 VOL. 95, No. 3 Cirsoid Aneurysm of the Uterus 779
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 780 V. A. M. Frencken and G. H. M. Landman NOVEMBER, 5965

Some authors consider it likely that a

cirsoid aneurysm forms in connection with
arteriosclerosis of the uterine vessels. Uter-
me vascular sclerosis is, in fact, mentioned
in a few case reports; there was no question
of it in our case. If arteriosclerosis were
the principal etiologic factor, then the ab-
normality should be much more frequently
seen. The average age of the patients at the
time of diagnosis was o years. The young-
est patient was 34 and the oldest 62. Four
patients were in the menopause. Two pa-
tients were nulligravidae; the remaining 6
patients had a total of 30 children. No
mention is made of abnormal blood loss
during parturition. A striking feature is
that 2 patients each had 3 abortions. Six of
the 8 patients first reported for treatment
American Journal of Roentgenology 1965.95:775-781.

with menorrhagia or metrorrhagia. In pa-

tients with a short history (2 weeks to 2
months) of severe vaginal blood loss or
menopausal blood loss, the menstruation is
described as having been normal.
Gynecologic exami nation disclosed an
enlarged uterus in 6 cases; also in 6 cases,
intensive arterial pulsations were felt in the
11G. 3. Section through the resected specimen. Par.. fornices. In only 3 cases was a murmur
ticularly in the fundus of the uterus and the heard and/or a thrill felt in the abdomen.
parametria, there are numerous cut surfaces of We consider it likely, however, that these
vessels, with smaller and larger blood spaces.
symptoms of arteriovenous fistulae must
have existed in the majority of cases. A
and cirsoid aneurysm exist. Some of the striking feature is that the difference be-
cases reviewed by Salm as instances of tween the systolic and diastolic pressure
hemangioma show features strongly sug- was never abnormally great; enlargement of
gestive of a cirsoid aneurysm. the heart is mentioned in 2 cases.
There are a few reports on arteriovenous In the cases described by Dubreuil and
fistulae, usually limited to one vessel, in the Loubat,5 Williams,15 and Gardner,7 the
uterus or in the region of the uterine yes- existence of an intra-abdominal arteriove-
sels1’2’4’14”6 which were not tabulated in nous fistula was suspected. Our case, in
view of their localized nature or because which pelvic arteriography was carried out,
the abnormality was largely extrauterine. is the only case in which the cirsoid aneu-
The pathogenesis of cirsoid aneurysm is rysm of the uterus was diagnosed preopera-
obscure. Tile anomaly is probably congeni- tively with certainty. In cases a diagnos-
tal. Particularly in the localized forms, tic curettage was performed. Because of the
some relationship with a preceding curet- associ ated severe hemorrhages, immedi ate
tage has been suggested. The cases de- laparotomy was required in these cases;
scribed in the literature and reviewed in treatment consisted of hysterectomy. In all
our table include only 2 patients with a his- cases, greatly dilated and tortuous vessels
tory of curettage (13 and i6 years before were found in the parametria and on the
diagnosis of the condition, respectively). surface of the uterus. The uterus often
 VOL. 95, No. Cirsoid Aneurysm of the Uterus 78!

showed pronounced pulsations and was of a lished by abdominal arteriography. There

sponge-like consistency. Flemostasis as a has been no precedent of an arteriograph-
rule offered difficulties. ic examination in a case of this type.

ROENTGENOLOGIC COMMENT V.A.M. Frencken and G.H.M. Landman

Afd. Radiologie
We believe that the arteriographic fea- St. Radboud Ziekenhuis
tures of a uterine cirsoid aneurysm have St. Annastraat 315

not been described before. In the arterial Nijmegen, Nederland

phase the characteristic features are pro- REFERENCES
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American Journal of Roentgenology 1965.95:775-781.

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