Calcifications of Cerebral Medullary Veins
Associated with Carotid-JugularFistula
Wen C. Yang, M.D.’
Jay Castle, M.D.2
Harvey Lozman, M.D.3
Moses Nussbaum, M.D.3
Departments of Radiology,’ Neurology,*
and Surgery,3 Beth Israel Medical Center,
New York, New York
Unusual calcification of the cerebral medullary veins is
noted in a 34-year-old mentally retarded female who had a
right carotid-jugular
anastomosis 30 years earlier. The
patient was hospitalized because of a right neck mass, proptosis, and the recent onset of left flaccid hemiplegia. Arch
aortography and CT of the head and neck were performed.
Surgery to oblate the fistula provided dramatic relief of
venous engorgement in the orbits and face. The finding of
medullary vein calcification is emphasized because it is a
hitherto unreported entity.
Calcifications
have always generated great interest in
radiology because they are easily demonstrable on
roentgenograms. In many disease processes calcifications exhibit characteristic features that are essential
in diagnostic analysis. This is particularly true in intracranial calcified lesions. In the past few decades,
numerous reports have been published to provide better understanding of almost every conceivable type of
intracranial calcification (1). However, with the advent of computed tomography (CT), a new perspective for further discovery has evolved.
Recently we had the opportunity to use CT to
study a patient with a long-standing carotid-jugular
fistula. A new entity-medullary
vein calcificationwas discovered and is detailed in this paper.

CASE REPORT

A 34-year-old female was admitted to the Beth Israel
Medical Center in October, 1980, for evaluation of a
large neck mass, facial disfiguration, exophthalmos,
and cerebrovascular accident. The patient had been
Address reprint requests to Wen C. Yang, M.D., Department
of Radiology, Beth Israel Medical Center, IO Nathan D. Perlman
Place, New York, NY 10003.

CT: The Journal
‘~2 Elsevier

of Computed

Science

52 Vanderbilt

Publishing

Tomography

6,35-39

(1962)

mentally retarded since birth. At the age of 4 years she
underwent a right common carotid artery-internal
jugular vein anastomosis, a procedure then believed
to improve the cerebral circulation in mentally retarded individuals. There was no apparent improvement of her mental status.
Six years prior to this admission swelling of the
right side of the neck and right eyelid developed and
progressed to a state of facial disfiguration and
exophthalmos. One month prior to admission, she
developed a left hemiplegia.
Examination on admission revealed a large pulsatile right neck mass with an audible bruit. Vascular
engorgement and a pulsatile thrill were noted in both
upper eyelids, the right more than the left. There was
entropion of the lower eyelids with trichiasis. The
right conjunctiva and sclera were congested. The retinae and maculae were normal. Tests for extraocular
muscles revealed possible right VIth cranial nerve
palsy. There was flaccid paralysis of the left extremities.
Arch aortography revealed a right common carotid artery-internal jugular vein fistula with marked
dilatation of the internal jugular vein, which accounted for the neck mass (Figure 1). There was
engorgement and early filling of the lateral, straight,
and cavernous sinuses as well as the orbital and internal cerebral veins.
CT of the head and neck demonstrated the enlarged venous channels. The orbital veins on the right
were more dilated than those on the left. There was no
CT evidence of infarct, but striking calcifications of
the deep medullary veins were noted, predominantly
in the right cerebral hemisphere (Figures 2, 3).
The patient underwent corrective surgery to
ablate the arteriovenous fistula. Postoperatively there
was dramatic relief of the venous engorgement of the
orbits and face.
DISCUSSION

In 1949 Beck et al. (2, 3) introduced a surgical procedure with which they hoped to improve the cerebral
Cerebral

Medullary

Vein Calcification

Co., Inc.. 1982

Ave., New York, NY 10017

0149-936x/82,01035-0~~*.~~

Note the marked enlargement
of the right internal jugular vein (large arrowheads)
Figure 1. Arch aortogram.
with a blind pouch inferiorly giving a large sausagelike
appearance.
The anastomotic
site is at the middle segment of the common carotid artery (arrows).
Yang et al

36

CT scans reveal
Figure 2 A, 6, 81 C. Precontrast
linear calcifications
of various lengths and widths in
the white matter of cerebral hemispheres
(more on
the right) converging
toward the lateral angles of the
lateral ventricles.

circulation for mentally retarded or epileptic patients
by establishing a fistula between the common carotid
artery and the internal jugular vein. Several years
later accumulated experience led to the conclusion
that the carotid-jugular anastomosis lacked any therapeutic value and, when remaining open, created
complications (4-8). The procedure was therefore
abandoned.
Aside from the venous engorgement in the neck
and orbit as presented clinically, a new finding of
unusual cerebral calcifications in the courses of the
medullary veins is noted. To our knowledge, intracranial calcification of this type has not been previously
reported.
Medullary veins are venous channels of white
matter in the cerebral hemispheres and constitute part
of the deep cerebral venous system. Detailed anatomic studies with angiographic correlation have been
reported by Kaplan (9), Huang and Wolf (lo), and
Hooshmand et al. (11). Depending on the destination
of drainage, these veins may be divided into superficial or deep medullary veins. Superficial medullary
veins begin 1 to 2 cm beneath the cortical gray matter
and stream toward and through the cerebral cortex to
empty into the superficial cortical veins. Deep medullary veins start from the depth of the white matter and
drain into the subependymal veins at various locations of the lateral ventricle (10).
Cerebral

Medullary

Vein Calcification
37

On a normal cerebral angiogram deep medullary
veins are more easily identified and can be observed in
more than 50% of cases in magnified angiograms
(12), whereas the superficial medullary veins are
usually obscured and difficult to distinguish from the
superficial cortical veins. In pathologic conditions of
the white matter such as infiltrating glioma or arteriovenous malformation the deep medullary veins are
usually dilated and may appear as relatively straight,
crowded, and converging vessels giving a conical
“spray” appearance (10). Dilatation of the medullary
veins has also been observed in reticulum cell sarcoma, cerebral hemorrhage, inflammatory disease,
and superficial venous thrombosis. No calcification
of medullary veins has ever been mentioned (12).
High-resolution CT makes possible the visualization and identification of the intracranial vessels. The
dilated vascular channels that characteristically converge toward the lateral angles of the lateral ventricles
are dilated deep medullary veins (Figure 2E, F). On
CT scans, the multiple linear calcifications with
“wedge” or conical “spray” appearance (Figures 2,
3) converging toward the lateral angles of the lateral
ventricles are therefore in the courses of the medullary
veins. It is not surprising that plain skull roentgenography failed to show these calcifications, since CT is 5
to 15 times more sensitive than plain skull films in the
detection of intracranial calcifications (13).
Yang et al.
38

Figure 2 D, E, 81 F. Postcontrast CT scans at corresponding
levels. In D a few dilated subependymal
veins (arrows) are noted along the wall of the left frontal horn. In E dilated deep medullary veins (arrows) are
enhanced
in the white matter of the left cerebral
hemisphere.

REFERENCES

1.

Figure 3. Direct coronal postcontrast CT scan. The
cluster of linear calcifications
has the same “wedge”
or conical “spray” appearance as that exhibited by
deep medullary veins collectively.

The pathogenesis of these calcifications is not
clear. There might exist some degree of venous stasis
at the levels of the medullary veins secondary to retrograde filling in the deep venous system as a result of
arteriovenous fistula. This postulation is substantiated by the finding that a few dilated deep medullary
veins were still noted in the white matter of the left
cerebral hemisphere (Figures 2E, F). A 30-year time
interval in our patient may have contributed to the occurrence of these venous calcifications.

Ozonoff MB, Burrows EH: Intracranial calcifications. In Newton, Potts (eds): Radiology of the Skull
and Brain, Vol I, Book 2. C. V. Mosby Co., St. Louis,
1971, pp 823-872
2. Beck CS, McKhann CF, Belnap DW: Revascularization of the brain through establishment of a cervical
arteriovenous fistula: Effects in children with mental
retardation and convulsive disorders. J Pediatr 35:
317-329, 1949
3. McKhann CF, Belnap DW, Beck CS: Cervical arteriovenous anastomosis in treatment of mental retardation, convulsive disorders and cerebral spasticity. Ann
Surg 132:162-175, 1950
4. Hammer JJ, Heersma SH, Macgregor JR, Dew RS,
Osius EA: Complications and effects observed in
thirty-three patients with cervical arteriovenous fistula. Arch Surg 631477, 1957
5. Holmes TW Jr: Circulatory dynamics in the presence
of carotid-jugular fistula. Effects of repair after 12
years of patency. Circulation 29905-910, 1964
6. Mortez WH, Bray PF: Observations on the effect of
carotid-jugular anastomosis. Am Surg 29:765-771,
1953
7. Jervis GA, McAllister FF, Hoff BM, Deterling RA:
Revascularization of the brain in mental defectives.
Neurology 3:871-878, 1953
8. Adam UG, Goetz RH: Surgically produced carotidjugular fistula: 18 year follow-up. Ann Surg 171:9397, 1970
9. Kaplan HA: The transcerebral venous system: An
anatomical study. Arch Neurol Psychiatry 1: 148- 152,
1959
10. Huang YP, Wolf BS: Veins of the white matter of the
cerebral hemispheres (the medullary veins): Diagnostic importance in carotid angiography. Am J Roentgenol 92:739-755, 1964
11. Hooshmand I, Rosenbaum AE, Stein RL: Radiologic
anatomy of normal cerebral deep medullary veins, criteria for distinguishing them from their abnormal
counterparts. Neuroradiology 7:75-84, 1974
12. Stein RL, Rosenbaum AE: Normal deep cerebral
venous system and abnormal supratentorial
deep
venous system. In Newton, Potts (eds): Radiology of
the Skull and Brain, Vo12, Book 3. C. V. Mosby Co.,
St. Louis, 1974, pp 1904-2110
13. Norman D, Diamond C, Boyd D: Relative detectability of intracranial calcifications on computed tomography and skull radiography.
J Comput Assist
Tomogr 2:61-64, 1978

CONTINUING MEDICAL EDUCATION QUESTIONS (CALCIFICATIONS
OF CEREBRAL MEDULLARY VEINS ASSOCIATED WITH CAROTID-JUGULAR

FISTULA)

True or false:
1.
2.
3.

Cerebral medullary veins are venous channels of white matter in the cerebral hemispheres.
Dilated cerebral medullary veins can be seen in infiltrating glioma, reticulum cell sarcoma, inflammatory disease and arteriovenous malformation.
Carotid-jugular anastomosis was a surgical procedure intended to improve cerebral circulation for the mentally retarded and has been proven to have some therapeutic value.

Cerebral

Medullary

Vein Calcification
39