C A R O T I D - C A V E R N 0 U S F I S T U L A
B E R N A R D J . A I / P E K S *
The clinical features of cerebral aneurysm have become more clearly
defined in recent years, due largely to advances made possible by
arterio-graphy and neurosurgery. Among the aneurysm syndromes which have
benefited by the newer methods of diagnosis and treatment, is that of
communicating aneurysm of the internal carotid artery and the cavernous
sinus. Though the clinical features of aneurysm involving these structures
is well-known, it is desirable to review them in the light of newer knowledge,
in order to define their diagnosis and to suggest treatment.
R E P O R T O F C A S E S
Case 1 — W. 8. (Jeff. Hosp. No. JH 13595) — A 57 year old white gardener with sudden loss of consciousness about a year before admission to the hospital. Eleven months later sudden almost complete closure of left eye. A feiv days throbbing headache in the left frontal region associated with a "hammering noise" in his head. A week later right eye involved. Examination revealed, bilateral ptosis, more marked on the left, complete ophthalmoplegia on the left and almost complete on the right except for slight lateral motion. Corneal reflex absent on the left and diminished on the right. Definite impairment of sensation in the distribution of the left ophthalmic nerve. Distinct bruit over both eyes, in both temples and over both mastoids. A diagnosis of arteriovenous aneurysm in the cavernous sinus was made and the left common carotid artery ligated. The patient improved markedly. Later arteriography confirmed the diagnosis of left internal carotid-cavernous sinus fistula.
History — T h e patient w a s a d m i t t e d on M a y 2 7 , 1944 complaining of headache of one month's duration. O n e y e a r b e f o r e he had lost consciousness in his room a n d w a s taken to a hospital where he was f o u n d to b e c o n f u s e d and irrational.
H e recovered completely and w a s well until one m o n t h b e f o r e admission when he awakened in the m o r n i n g to find that his left eye w a s a l m o s t entirely closed.
S e v e r a l d a y s later he developed a t h r o b b i n g left f r o n t a l headache associated with a "hammeringlike noise" in his head. A b o u t one week later this condition extended
t o his right eye. A b o u t t w o weeks b e f o r e admission he had three episodes of nausea a n d vomiting. O n M a y 15, 1944 he w a s a d m i t t e d to the W i l l s E y e H o s p i t a l
w h e r e he w a s f o u n d to have a c o m p l e t e ophthalmoplegia of the left eye and a l m o s t complete paralysis of the right eye. A loud bruit w a s heard over the left
g l o b e which w a s t r a n s m i t t e d widely all o v e r the skull. T h e intraocular tension w a s elevated to 31 in the right eye a n d 41 in the left eye. T h e visual fields
showed concentric contraction. T h e corneal reflex w a s absent on the left and
a p p e a r e d to be diminished on the right. T h e r e w a s definite i m p a i r m e n t of sensa tion in the distribution of the left ophthalmic nerve. O n M a y 2 4 , 1944 a ligation
of the left c o m m o n carotid a r t e r y w a s p e r f o r m e d with a m a r k e d i m p r o v e m e n t in the patient's condition. H e w a s then t r a n s f e r r e d to J e f f e r s o n H o s p i t a l f o r
further study. O n admission he stated he had an aching sensation which extended f r o m the occipital area down the neck to the shoulder. H e w a s unable to see out
of the left eye a n d vision in the right eye w a s poor. T h e past history was negative except f o r occasional episodes of precordial pain associated with dyspnea. Physical
examination was negative except f o r the eye findings.
Examination — N e u r o l o g i c a l examination revealed the following: the left e y e w a s m o r e p r o m i n e n t than the r i g h t ; the pupils were small, regular and reacted o n l y slightly to light and • not at all to a c c o m m o d a t i o n ; the left corneal reflex
w a s d e c r e a s e d ; there w a s m a r k e d weakness of all the eye muscles, w o r s e on the left, with bilateral p t o s i s ; there was a soft distant bruit heard over the left eye
ball which w a s synchronous with the p u l s e ; diplopia w a s present in all fields; the visual acuity w a s 2 0 / 5 0 on the right and 2 0 / 1 0 0 on the l e f t ; a definite left
sided facial hypesthesia involving all divisions of the trigeminal nerve w a s p r e s e n t ; the deep tendon reflexes were all overactive and equal a n d he had g r e a t difficulty
walking heeltotoe. E l e c t r o c a r d i o g r a m showed a left axis deviation. X r a y s of the skull were negative. B l o o d and urine studies were normal.
O n M a y 3 1 , 1944 D r . R u d o l p h J a e g e r i n j e c t e d 15 cc. of diodrast into the
left carotid a r t e r y . T h e vessel seemed t o be p a r t i a l l y thrombosed. R o e n t g e n o l o g i c studies w e r e m a d e immediately and indicated an arteriovenous communication be
tween the internal carotid a r t e r y and cavernous sinus on the left side.
N e u r o l o g i c a l examination 15 d a y s later revealed the f o l l o w i n g : the left eye
was m o r e p r o m i n e n t than the right; the pupils and discs were n o r m a l ; the right ocular m o v e m e n t s w e r e full but there w a s distinct limitation of m o v e m e n t of the
left eye o u t w a r d , slightly i n w a r d and u p w a r d ; convergence w a s about n o r m a l but there m a y have been slight limitation of the left e y e ; the corneal reflexes were
a b s e n t ; the cranial nerves were otherwise n o r m a l except for loss of pain over the entire f a c e a n d head and extending down the anterior aspect of both a r m s ;
there w a s slight weakness of the right hand g r i p , m a r k e d weakness of both legs, a n d g r e a t d i f f i c u l t y in w a l k i n g heeltotoe; the biceps, triceps, patellar and Achilles
reflexes w e r e overactive and equal.
O n June 17, 1944 D r . R u d o l p h J a e g e r injected 5 cc. of thorotrast into the
right internal carotid a r t e r y . D u r i n g the procedure the a r t e r y w a s t e m p o r a r i l y closed f o r a b o u t 10 seconds, during which the patient b e c a m e unconscious f o r
not m o r e than 5 seconds. N o ligation Avas p e r f o r m e d .
T h e patient was discharged one week later on J u n e 2 2 , 1944 g r e a t l y i m p r o v e d
in the m o v e m e n t of his eyeballs, the recession of his exophthalmos and his general
feeling of well being.
Summary — T h i s patient illustrates the spontaneous onset of arteriovenous aneurysm of the cavernous sinus, not an unusual m e t h o d of development, but the
clinical picture is interesting because of the development of bilateral signs. T h e patient responded well to ligation of the left c o m m o n carotid a r t e r y but a t t e m p t e d
occlusion of the right internal carotid a r t e r y resulted in t e m p o r a r y loss of con sciousness and no further occlusion w a s a t t e m p t e d . D e s p i t e his relatively advanced
a g e ( 5 7 y e a r s ) he withstood ligation of one c o m m o n carotid artery v e r y well and i m p r o v e d g r e a t l y in his s y m p t o m s despite only unilateral ligation.
hemi-anopsia, and a market bruit over the left eye and in the left temporal region, clearly transmitted to the right temporal region. X-ray examination of the skull revealed intracranial calcification of the left internal carotid artery. Partial liga-tion of the left common carotid artery.
History — T h e patient w a s a d m i t t e d on S e p t e m b e r 2 , 1943 with the chief complaint of headache and swelling of the left lower eyelid, of f o u r weeks' duration. She stated that while attending to her household duties she felt a s u d
den severe excruciating pain in the left parietal region of her skull, which forced her to go to bed where she remained for three days. D u r i n g this time the pain
remained very severe and had a throbbing quality. I t g r a d u a l l y decreased in intensity and on admission w a s felt as a constant ache with s o m e throbbing quality,
considerably relieved by an ice b a g a p p l i e d over the left eye. O n e week following the pain she n o t e d a sudden swelling a n d eversion of the left lower eyelid which
occurred overnight. I t w a s tender to touch, and w a s associated with profuse lachrymation. T h e p a s t history revealed that she had had "rheumatism" f o r the
past 2 to 3 years involving the right elbow, right knee a n d the right ankle. She had had headaches as a child but none during adult life.
Examination revealed swelling and eversion of the left eyelid. T h e blood pressure was 1 5 0 / 9 0 . T h e r e was a m a r k e d bruit audible u p o n auscultation over the left eye and in the left t e m p o r a l region, clearly t r a n s m i t t e d to the right
temporal region. T h e remainder of the physical examination w a s normal except
for the presence of a blowing systolic m u r m u r at the apex.
N e u r o l o g i c a l examination revealed the left p u p i l to be smaller than the r i g h t ;
it failed to react to light and consensual responses. T h e right pupil reacted p r o m p t l y to light and convergence. T h e left eyeball p r o t r u d e d a n d the left optic
nerve w a s obscured in outline. T h e r e w a s only i n w a r d and slight d o w n w a r d m o v e m e n t of the left eyeball. T h e left corneal reflex w a s absent. T h e remainder of
the examination was normal.
B l o o d studies were n o r m a l and the W a s s e r m a n n a n d K a h n reactions of the blood w e r e negative. L u m b a r puncture revealed an initial pressure of 120 m m .
of spinal f l u i d ; the fluid w a s clear and colorless; there w a s 1 cell p e r cu. m m . ; the protein was 3 6 m g m s . and the W a s s e r m a n n reaction w a s negative. E x o p h
thalmometer reading w a s 2 0 m m . Ü . D . and 2 9 m m . O . S . X r a y s of the skull showed a calcified shadow near the sella turcica consistent with an aneurysm
o f the internal carotid artery.
O n S e p t e m b e r 2 3 , 1943, a partial ligation of the left c o m m o n carotid artery
w a s p e r f o r m e d by D r . R u d o l p h J a e g e r . U n d e r local anesthesia the common carotid artery w a s elevated. B y direct pressure it w a s c o m p l e t e l y occluded and
the patient lost consciousness after a b o u t 60 seconds. She quickly regained con sciousness on release of the pressure. I t was thought best to p a r t i a l l y occlude
the a r t e r y b y an aluminum band. O n O c t o b e r 1, 1943 a second a t t e m p t w a s m a d e to occlude the artery. W h e n the aluminum b a n d w a s tightened so as to
permit a b o u t 1 / 4 as much blood as n o r m a l to pass t h r o u g h , the patient developed loss of consciouness a n d flaccid paralysis of the right side. T h e a r t e r y w a s left
c l a m p e d f o r a p p r o x i m a t e l y 10 minutes and since her condition did n o t i m p r o v e the b a n d w a s loosened to p e r m i t a b o u t 1 / 2 the n o r m a l circulation to p a s s through.
T h e patient then returned to normal. A loud bruit could be heard o v e r the left eyeball, however, when the clip w a s opened to this extent. O n O c t o b e r 1 1 , 1943
the clip w a s tightened to a point where it p e r m i t t e d not m o r e than 1 / 1 0 the n o r m a l amount of circulation. T h e patient remained conscious throughout this
procedure. T h e next d a y she left the hospital against advice.
Summary — T h i s patient represents an e x a m p l e of internal carotidcavernous sinus aneurysm which developed spontaneously without previous evidence of i n j u r y . T h e r e w e r e no unusual features of the clinical picture, b u t the demonstration of
encountered on ligation of the c o m m o n carotid a r t e r y illustrate the f a c t that
ligation m u s t be a p p r o a c h e d cautiously in instances in which the circulation a p
p e a r s to be inadequate.
Case 3 — P. K. (Jeff. Hosp. No. KH 2607) — A J/5 year old white miner fell off a scaffold striking the right side of his head. A bout one week later the left eye became swollen and red, and he noticed a continuous thumping in the
frontal portion of his head, synchronous with his heart rate. Examination %l
/2
months later revealed a proptosed and inflamed left eye. The extraocular move-ments in his eye were limited especially outward and upward in which directions he experienced diplopia. There was a clearly audible murmur over the eyeball and, above it. The left common carotid artery was ligated in several stages. Three weeks later he was moderately improved with a lessening of the proptosis, and a complete absence of the bruit.
History — P. K . "was a d m i t t e d on J u l y 2 8 , 1947 complaining of thumping in his head a n d swelling of his left eye of 2 y3 months' duration. O n M a y 1 4;
1 9 4 7 he fell o f f a s c a f f o l d 7 or 8 feet to a concrete floor, landing on the right
side of his head. H e w a s dazed but not unconscious and was able to return to
w o r k the next day. A b o u t one week a f t e r the fall the left eye became i n f e c t e d ;
t w o d a y s l a t t e r it w a s swollen a n d almost closed b u t this d i s a p p e a r e d in several hours. A t the s a m e time he became a w a r e of a persistent t h u m p i n g on both
sides of his head in the f r o n t o t e m p o r a l region, synchronous with his heart beat.
T h e swelling of the periorbital region present several d a y s a f t e r his fall returned
a n d persisted, being greatest on awakening and receding during the day. In 1941 he had been struck on the vertex of the skull b y a 6 lb. l u m p of coal during
mining a n d sustained a scalp laceration. Physical examination w a s n o r m a l t x c e p t
f o r edema of the left eyelids a n d periorbital tissues. A bruit w a s heard over
the left eye, obliterated by compression of the left internal carotid artery.
Examination — N e u r o l o g i c a l examination revealed that the left pupil was slightly l a r g e r than the right but reacted well to light, a c c o m m o d a t i o n and con vergence. T h e r e w a s obscuring of the nasal m a r g i n s of the left f u n d u s , with
grayish discoloration of the t e m p o r a l side a n d the veins were full. T h e eyeball p r o t r u d e d but w a s nonpulsating. T h e r e w a s 7 / 8 ptosis of the left eyelid and
limitation of i n w a r d a n d o u t w a r d m o v e m e n t of the left eyeball. D i p l o p i a w a s demonstrable on o u t w a r d , u p w a r d a n d d o w n w a r d gaze. T h e remainder of the
neurological examination was normal. V i s i o n w a s 2 0 / 3 0 in the right eye and 2 0 / 5 0 in the left eye. E x o p h t h a l m o m e t e r readings showed the left eye to be pushed
to 10 to 12 m m . f o r w a r d as c o m p a r e d t o the right eye. T h e visual fields were normal. X r a y s of the skull a n d routine blood and urine studies w e r e normal.
O n A u g u s t 15, 1947 the left carotid arteries were exposed and a clip placed
on the internal carotid a r t e r y b y D r . R u d o l p h Jaeger. F o u r hours later the p a t i e n t developed a rightsided hemiplegia and the clip had to be removed. On
A u g u s t 18, 1947 the left c o m m o n carotid artery w a s partially occluded b y an aluminum band w i t h o u t h a r m f u l effects. F o u r d a y s later it w a s completely closed
b y the clip. O n S e p t e m b e r 6, 1947 the patient was discharged f r o m the hospital at which time he w a s m o d e r a t e l y i m p r o v e d with a lessening of the proptosis of
the left eye, a complete d i s a p p e a r a n c e of the bruit, and absence of the thumping sensation which had caused him so much distress.
Summary — T h e cavernous sinus a n e u r y s m in this patient developed about one w e e k a f t e r a head i n j u r y and w a s associated with exophthalmos, partial
ophthalmoplegia, an audible bruit over the eye and an awareness of a pulsation in the head b y the patient himself. T h o u g h the diagnosis of arteriovenous aneu
r y s m w a s not verified b y direct observation, the findings were such as to m a k e any other localization impossible. C o m p l e t e subjective relief followed common ca
Case 4 — C N. (Jeff. Hosp. No. LH 6ÕÍ20 M.E.) — A 50 year old truck helper who had been in a fight three weeks before admission to the hospital, was knocked down and struck his left forehead and temple against an automobile fender. The left eye became swollen and intermittent frontal headaches developed. Exam-ination revealed a bruit over the left eyeball, proptosis of the left eye and marked limitation of its motion except for slight downward movement. Arteriogram re-vealed, an internal carotid-cavernous sinus aneurysm. The left common carotid artery was ligated. Seven days later the bruit had disappeared, the proptosis had subsided and there was some improvement in the movement of the eye
History — C. N . w a s a d m i t t e d on O c t o b e r 2 2 , 1948 with the complaint of swelling and redness of the left eye a n d frontal headaches f o r the past three weeks. H e had been in a fight resulting in his, being knocked against a truck fender, striking the left forehead and t e m p l e , without loss of consciousness. T w o or three d a y s later he b e c a m e a w a r e that his left eye w a s becoming red and
swollen and seemed to be protruding. A t the same time h? developed supra orbital headache, located chiefly above the left eye and partially relieved by aspirin and heat. T h e headache w a s constantly present while lying down but in termittently while in the upright position. H e stated that on one occasion he
heard a throbbing noise in the left side of his head. O n l y occasionally w a s there double vision. T w o days b e f o r e admission he w a s seen at W i l l s E y e H o s p i t a l where examination revealed exophthalmos of the left eye, partial ptosis and m a r k e d limitation of the extraocular m o v e m e n t s of the left eye in all directions but most noticeably in o u t w a r d rotation. A loud bruit w a s heard over the left eye and
left t e m p o r a l region with transmission to the right t e m p o r a l region. T h e only significant f a c t in the past history was that he had been told 8 months previously that he had high blood pressure. H e w a s t r a n s f e r r e d to the Jefferson H o s p i t a l f o r further study.
Physical examination revealed a blood pressure of 1 1 0 / 7 0 , mild systolic aortic and p u l m o n a r y m u r m u r s , a left inguinal hernia, and bony prominences of his f i n f e r s suggestive of osteoarthritis. T h e remainder of the physical examination
w a s normal.
Neurological examination revealed visual fields which were full b y confronta tion. T h e left eyelid w a s ptosed and m o v e m e n t of the left eye w a s markedly restricted except for slight d o w n w a r d gaze. T h e r e w a s a bruit over the left eye
ball a n d over the left carotid artery in the neck. It was also heard faintly over the left t e m p o r a l region. D i g i t a l compression of the left carotid a r t e r y in the
neck resulted in d i s a p p e a r a n c e of the bruit. V i s i o n in the right eye was 2 0 / 2 0 and in the left eye 2 0 / 3 0 . E x o p h t h a l m o m e t e r reading of the right eye was 18 m m .
and of the left eye 2 9 m m . T h e left p a l p e b r a l fissure w a s n a r r o w e r than the right but the lid actions a p p e a r e d equal. T h e pupil of the l i f t eve was irregularly
oval c o m p a r e d to the right but the reactions were normal. O n ophthalmoscopic examination the right eye w a s normal. In the left eye the u p p e r and lower
disc m a r g i n s were blurred but no m e a s u r a b l e edema was noted. N o spontaneous venous pulsations were noted, but the retinal veins were e n g o r g e d , distended and
tortuous. T h e m a c u l a a p p e a r e d normal and no hemorrhages or exudates were seen. A p a r t from these findings the neurological examination was normal.
Laboratory studies revealed the following: T h e spinal fluid was under an initial pressure of 180 m m . of w a t e r ; the fluid w a s clear and colorless; there w a s less than one cell per cnim., the protein w a s 2 8 m g s . and the W a s s e r m a n n
reaction w a s negative. B l o o d studies were normal. U r i n a l y s i s revealed one to two plus albumin, 2 to 5 pus cells, and 2 3 R . B . C . per highpowered field. X r a y of
the chest w a s normal. T h e r e were m a r k e d degenerative changes in the vertebrae Calcification of the blood vessels along the lateral aspect of the right f e m u i
was seen.
A r t e r i o g r a p h y w a s p e r f o r m e d b y direct exposure of the carotid artery. The
sclerosed. A r t e r i o g r a m revealed a b r u p t cessation of the internal carotid artery in the region of the cavernous sinus, with a suggestion of a small aneurysm al
this point. B e c a u s e of the d i s a p p e a r a n c e of the bruit by digital compression oi the carotid a r t e r y as well as absence of neurological signs on t e m p o r a r y occlusion
f o r one hour b y silver clip, D r . R u d o l p h J a e g e r tied o f f the left common carotid a r t e r y in the neck.
T h e d a y following operation the bruit was gone from the left eye. F i v e days
p o s t o p e r a t i v e l y the patient had a brief episode of confusion but no other ill effects. Seven d a y s a f t e r the ligation the bruit was still absent. T h e conjunctival
a n d periorbital edema and cheilosis had subsided considerably. T h e r e w a s still weakness of the 3 r d and 4th cranial nerves but m o v e m e n t s of the eyeballs were
improved and the exophthalmos a p p e a r e d to be improved.
Summary — T h i s case of arteriovenous aneurysm of the left carotid sinus is of particular interest because of its rapid development. T h r e e weeks after a
head i n j u r y sustained as a result of a b r a w l , the patient developed headache and evidence of ocular paralysis. T h e r e a f t e r the march of events was rapid, with resulting exophthalmos, paralysis of the 3 r d , 4th and 6th cranial nerves, and a toandfro bruit over the left £ y e and t e m p o r a l region. B e c a u s e of disappearance
of the bruit by digital compression of the common carotid artery, ligation of this vessel w a s p e r f o r m e d in the neck, with resulting recession of the exophthalmos and i m p r o v e m e n t in ocular m o v e m e n t s .
Case 5 — M. L. (Jeff. Hosp. No. NH 8890) — Bilateral cavernous sinus aneurysms: carotid-cavernous fistula on the right and left saccular aneurysm.
History — T h e patient, a 4 5 year old w o m a n , w a s t r a n s f e r r e d to the Jef ferson H o s p i t a l from the service of D r . I. M . T a s s m a n n in the W i l l s H o s p i t a l
on 1 2 / 2 / 5 0 . She had been well until a b o u t 5 weeks before entrance ( 1 0 / 3 / 5 0 ) when she suddenly developed a dizzy spell while undressing for bed. She described this episode as a "sinking, s w i m m i n g sensation" from which she had suffered f r o m time to time in the past. A s a result of her dizzy spell she fell to the floor striking her vertex and left t e m p o r o p a r i e t a l region. She w a s dazed but
not rendered unconscious and upon arising from the floor she became aware for the first time of severe p o u n d i n g left f r o n t o t e m p o r a l headache. W i t h i n a few minutes thereafter her left eye became swollen and her eyelid c o m p l e t e l y closed. O n several occasions during this period she lifted her eyelid and found that she
was unable to m o v e her eyeball. H o t packs relieved her swelling but failed to improve the motility of the eyeball.
T h r e e d a y s a f t e r the onset of her trouble she experienced severe throbbing
pain in the right f r o n t o t e m p o r a l region associated with nausea and vomiting, while standing at a window gazing out the street. T h e following morning on arising she became a w a r e of a noise in her head and swelling and ptosis of the right eyelid and prominence of the right eyeball.
T h e past history disclosed recurrent headaches lasting 1 to 2 d a y s in the
premenstrual period a n d relieved by aspirin. In D e c e m b e r , 1949, about 11 months before her present s y m p t o m s , she developed weakness but not complete paralysis
of the left arm clearing u p in about 2 months. A b o u t 2 to 3 years before onset of her present illness she developed a noise in her head which persisted until 6
weeks before the onset of the trouble which brought her into the hospital. She described the noise as sounding like machinery, heard in all parts of her head
and m o r e pronounced when her blood pressure was elevated.
Neurological examination, revealed bilateral exophthalmos, m o r e pronounced in the right eye. O n neither side was there pulsation of the eyeball. T h e r e w a s
pronounced edema of the eyelids. T h e r e was complete ptosis of the left eyelid with complete i m m o b i l i t y of the left eyeball; a dilated left pupil with loss of
CAROTID-CA VEHXOUS FISTULA 209
pupil was small and failed to react to light and in a c c o m m o d a t i o n . T h e right eyeball was completely immobile and failed to m o v e in any direction. T h e right
corneal reflex was lost. N o changes were f o u n d in the fundi, the optic nerves being welldefined and of n o r m a l color. V i s u a l field studies p e r f o r m e d in the
W i l l s H o s p i t a l revealed normal fields for f o r m and color, with no scotomas and no enlargement of the blind spots. A p a r t f r o m the ocular findings there were
no changes to be observed in routine neurological examination. A loud bruit was heard over the entire right side of the skull, m o s t pronounced over the right eye
and face, but heard faintly over the vertex and over the vessels on the right side of the neck. I t was completely obliterated by compression of the carotid
vessels in the right side of the neck.
Laboratory studies revealed the following findings: U r i n a l y s i s w a s negative except for a specific g r a v i t y of 1.012, a trace of albumin and an occasional red
blood cell. B l o o d studies revealed: H g b . 11 g m . ; erythrocytes 3 , 6 2 0 , 0 0 0 ; leukocytes 4,500 with a n o r m a l differential count. T h e blood urea nitrogen w a s 9.2 m g .
and 4.80 m g . on two occasions. T h e W a s s e r m a n n and K a h n reactions of the blood w e r e negative. Spinal fluid studies were not m a d e . R o e n t g e n o g r a m s of
the skull revealed demineralization of the dorsum sellae and the floor of the sella turcica. T h e right anterior clinoid process was described as being shortened
a n d d i f f e r e n t from the left.
A r t e r i o g r a p h y with t h o r o t r a s t w a s p e r f o r m e d on 1 2 / 6 / 5 0 by D r . W i l l i a m
H . W h i t e l e y : on the right side there w a s noted a huge d i f f u s e dilatation of the internal carotid a r t e r y j u s t before its bifurcation into the anterior and m i d d l e
cerebral vessels ( f i g . 1 ) ; the left internal carotid a r t e r i o g r a m revealed a saccular aneurysm arising from the lateral aspect of the internal carotid a r t e r y about one
inch before its bifurcation into its anterior and m i d d l e branches ( f i g . 2 ) .
Fig. 1 ( c a s e 5 ) — Internal carotid a r t e r i o g r a m showing
2 1 0
rlRQUIVOS DE XEURO-PSIQUI ATRIA
Fig. 2 ( c a s e 5 ) — Internal carotid a r t e r i o g r a m showing saccular
aneurysm in the cavernous sinus on the left ( B ) ; also carotid
cavernous fistula on the right ( A ) .
Summary — T h i s patient, a 4 5 year old w o m a n , developed a bilateral cavernous sinus aneurysm against a b a c k g r o u n d of premenstrual headache extending over a number of years. She w a s known to have hypertension, and about 11 months
before the onset of her present illness she developed weakness of the left arm lasting a b o u t 2 months. A b o u t 2 to 3 years previously she developed a noise
in her head which persisted until 6 weeks before her present trouble. A b o u t 5 weeks before entrance into the hospital she developed severe leftsided headache
a n d complete ophthalmoplegia followed in 3 d a y s b y rightsided headache and right ophthalmoplegia. E x a m i n a t i o n revealed bilateral exophthalmos, greater on
the right, with c o m p l e t e ophthalmoplegia bilaterally. A loud bruit w a s heard over the right side of the face and head. A r t e r i o g r a p h y revealed a right carotid
cavernous fistula and a left saccular aneurysm. D u r i n g the course of her hospital s t a y the patient recovered partial use of the right eye. She was discharged with
out operation. R e v i e w of her p r o b l e m 5 weeks a f t e r discharge revealed complete return of m o v e m e n t of the right eyeball except for residual external rectus
paralysis. T h e ophthalmoplegia of the left side remained unchanged. T h e bruit w a s still heard over the entire head but was loudest on the right side.
D I S C U S S I O N
The subject of carotid-cavernous fistula or pulsating exophthalmos has
been completely reviewed on several occasions. Sattler
1reviewed 588 cases to 1924. The subject has been reviewed more recently
(1943) by Martin and Mabon \ The problem of communicating aneurysm
within the cavernous sinus has been extensively studied therefore, and its
fundamental features clearly defined. The five cases reported illustrate
the main features of aneurysm in this location, and contribute in addition
an unusual instance of bilateral aneurysms within the cavernous sinus, a
carotid-cavernous fistula of one side and a saccular aneurysm of the other
(case 5 ) .
The symptoms of carotid-cavernous fistula develop dramatically in the
majority of instances. The presenting symptoms in most cases is a bruit
of which the patient is aware, described as synchronous with the pulse.
Associated with it is protrusion of one eyeball which may be present at the
onset or may be delayed in its development. These, the most prominent
symptoms, may be associated with headache, diplopia, and complaints of
failing vision in the affected eye. The symptoms are ordinarily of brief
duration and help is quickly sought. Examination reveals unilateral
exoph-talmos with swelling and chemosis; an audible bruit heard over the side
of the communication, but audible over the entire head; limitation of
movement of the eyeball varying from involvement of a single muscle to
complete ophthalmoplegia; often loss of the corneal reflex, and loss of
sensation over the forehead in the 1st division of the trigeminal nerve.
Trauma is responsible for the development of the fistula in about
7 5 % of cases, but spontaneously developing instances are not uncommon.
Of the five cases recorded, three were spontaneous and two developed
fol-lowing trauma. In none of the five cases was pulsation of the eyeball
found either by inspection or palpation. Lack of pulsation is said to be
a feature at times of the spontaneous cases of carotid-cavernous fistula
(Martin and M a b o n ) , but it was absent in the two traumatic cases as well
in the present series.
As illustrated by case 1, the exophthalmos is at times bilateral.
Sal-tier has found that bilateral exophthalmos developed in about one-third
of the cases, and was distinctly more frequent in the traumatic cases.
Though it occurs simultaneously with the development of exophthalmos on
the affected side, its occurrence in the opposite eye may be delayed for
weeks or even months.
Treatment of carotid-cavernous fistula is usually surgical. Since most
patients survive the rupture of the artery into the cavernous sinus,
opera-tion has been directed against the bruit and exophthalmos which constitute
the problem of greatest importance to the patient. The procedures advocated
for the treatment of carotid-cavernous fistula include: ( 1 ) ligation of the
common carotid artery; ( 2 ) ligation of the internal carotid artery in
the neck; ( 3 ) ligation of arteries and veins in the neck; ( 4 )
intra-cranial trapping; ( 5 ) non-operative treatment. The operation of choice
appears to be common carotid ligation, since this is less hazardous than
ligation of the internal carotid artery. Of the five cases recorded, four
had common carotid artery ligation and one (case 5 ) recovered
spontane-ously. Of the four cases which received ligation the bruit disappeared in
three instances, while in the other (case 2 ) the patient left the hospital
before control observations could be made. In the other instances (cases
1, 3 and 4 ) there was improvement in the exophthalmos and in movement
of the eyeball subsequent to the ligation. The spontaneous improvement
recorded in case 5, though unusual, has been observed in other series and
was described by Sattler who reported spontaneous cure in 16 of 322 cases
of carotid-cavernous fistula.
Though improvement developed with ligation of the common carotid
artery in the present series of cases, the preferred mode of treatment for
carotid-cavernous fistula has not yet been determined, and there remain
many instances in which ligation o f other vessels are necessary, such as
the internal or external carotid arteries. In rare instances trapping of the
aneurysm becomes desirable when improvement cannot be accomplished by
other means. Elimination of the bruit is of greatest importance in those
suffering from carotid-cavernous fistula because of its demoralizing effect.
If, in addition, the exophthalmos can be made to recede, much has been
accomplished. Improvement in movement in the eyeball may take place
over an extended period and may result in partial or complete return of
movement.
Department of Neurology, Jefferson Medical College (1025, Walnut Street), Philadelphia, Pa., U.S.A.
B I B L I O G R A P H Y
1 . S a t t l e r , C . H . — P u l s i e r e n d e r E x o p h t h a l m u s . H a n d . d. G e s . A u g e n h e i l . V o l . 9 , A b t . 1, P t . 2 , C a p . 1 3 . E d . v o n G r a e f e , B e r l i n , 1 9 2 0 .
2 . L o c k e , C . E . ( J r . ) — I n t r a c r a n i a l a r t e r i o - v e n o u s a n e u r y s m o r p u l s a t i n g
e x o p h t a l m o s . A n n . S u r g . , 8 0 : 1 , 1 9 2 4 .
3 . M a r t i n , J . D . a n d M a b o n , R . F . — P u l s a t i n g e x o p h t h a l m o s . J . A . M . A . , 1 2 1 :
