Intracranial venous anomaly

INTRACRANIAL

VENOUS ANOMALY *

DELBERT H.

WERDEN,

NeuroIogistand Neurosurgeon, SAN

T

HE

patient

reported of sixteen

whose

was

case

a white

San

DIEGO,

boy

years.

First Admission (March, 1930). His complaints were headache of seven years’ duration, convulsions foIlowing a blow on the head, rapid growth, awkwardness of gait, and mental deterioration. The patient was first observed whiIe convalescing from what was thought to be a head injury. WhiIe swimming he had been struck on the head by a wet Ieather water polo baI1. IHe came from the water shaking, weak and unabIe to stand, soon suffered severe headache, and vomited. He was brought home in a state of generaIized convuIsions which Iasted for two or three hours. The patient was then admitted to the hospita1 in coma as a skuI1 fracture suspect. The past history showed that at the age of six years he had faIIen on a piIe of bricks, striking the back of his head, and was brought home unconscious. At odd times for the next seven or eight years he had experienced much headache, and had had many speIIs of twitching of a11 extremities, and cIinching of the hands, but without Ioss of consciousness. Previous records reveaIed that the boy had been in the hospital at the age of nine years (1924) because of severe epistaxis occurring at intervals for five days foIIowing nasal trauma. At about this time he had aIso experienced spontaneous invoIuntary twitching of the Ieft foot for a period of five minutes. Examination upon admission in rg3o showed a sIender, poorly built, stupid appearing boy of sixteen years. His mouth n-as heId partially open; the teeth were irregular in size, shape and position, and the palatal arch was high. The right face was sIightIy weak. The pupiIs were diIated, the Ieft Iarger than the right, nith no light reaction in the right. The chest * From

NcuroIogicaI

and Neurosurgical

County

HospitaI

CALIF.

was of the “pigeon-breast” type. A systoIic murmur was present at the apex, and the bIood pressure was 106/56. AI1 deep reflexes were whiIe the Chaddock reflexes were absent, somewhat suggestive. Roentgenogram of the skuI1 showed thickening of the middIe tabIe, suggestive of CoIey’s anemia, but further examination faiIed to substantiate this suspicion. Course. The patient regained consciousness the next ciay, but continued to have headache and to vomit for four days, then improved and was discharged on the eighth day. The Ieft pupi remained diIated and fixed. Following discharge the patient continued to have convuIsions, the second occurring in one month, and the others at about four-month intervals. Second Admission (May, 1931). He was re-admitted because of frequent and intermittent convuIsions, aImost of a status epiIepticus type, which had been controIIed before admission onIy by hypodermic injections and ether inhalations. In addition to the headaches and ConvuIsions, the patient had deteriorated mentaIIy, had become awkward in his gait, and had grown 7 inches in the pre\%us tive months. The typica convulsion was preceded by four or five hours of headache, whereupon his mouth would open, draw and jerk to one side (thought to be to the left). There was frothing at the mouth and considerable cyanosis GeneraIized twitching of a11 extremities then occurred off and on for one or two hours. This was later foIIowed bv three or four hours of deep coma. On awakening and for the next twenty-four hours cerebration and mentaIity were much retarded; headache and vomiting continued for four or five days. Routine examination at this time (n’lay, 1931) reveaIed an unconscious patient with a temperature of 103.6~~. rectally, a pulse of 140 per minute, and a bIood pressure of 100,/70.

is herewith

American

Diego

M.D.

Service ‘IS

of Los AngeIes

County

General

Hospital.

116

American

Journal

of Surgery

Werden-IntracraniaI

The head was deviated to the Ieft. The pupils were contracted and fixed to Iight. AI1 deep reflexes were Iost, but there was a biIatera1

FIG. I. Posterior view of dura. EnIargement tortuosity of superfrcia1 veins, emmissary vein, double occipita1 sinus.

AnomaIy

JULY,193i

ties poorIy

but can run when commanded. Diagnosis: “This patient has an organic lesion, probabIy inff ammatory, affecting especiaIIy

and and

Babinski response. Dr. CyriI CourviIIe examined the patient on the same day and noted paIeness of the discs, fuIIness of the veins of both fundi and the scaIp, and maxihary His impression was, “ PossibIe prognathism. pituitary or supraseIIar neopIasm, chronic subdura1 hematoma (unIikeIy), or traumatic epiIepsy (to be considered).” Dr. Johannes NieIsen examined the patient Iive days Iater and recorded the foIIowing: “A tall and Ianky boy of sixteen with marked irregularity of the teeth and a high paIata1 arch. There is a stupid, adenoid, bIank, mask-like expression of the face. BiIateraI optic atrophy is present, more on the right. There is paralysis of upward gaze and of convergence, aIthough the patient is abIe to Iook toward the tip of his nose with either eye separateIy. The pupiIs are pecuIiar and interesting. When first seen the right was 4 mm., the Ieft 3 mm. Neither reacted very we11 to light. After about seven minutes the pupils were 3 mm. and equa1. After another seven minutes the Ieft was 4 mm., the right 3 mm. They stiI1 did not react “on conSome rigidity of the right arm is vergence.” present, aIso tremor and sIight incoordination of both hands in the finger-to-finger test. The deep reflexes of the upper extremities are diminished; those of the Iower extremities are hyperactive. The superficia1 rehexes are active and norma quaIitativeIy. The patient waIks with a staggering and uncertain gait. There seems to be an apraxia. He handIes his extreai-

FIG. Z. Anomalous

sinus from A to B. Latter is venous aneurysm. FIG. 4. DuraI sinuses as shown in Figure I. Paired superior longitudinal sinuses, paired occipital sinuses, with various other sinuses between each member of each pair. A. Junction of paired superior sinuses with anomaIous sinus. B. Lateral sinus. C. One of paired occipital sinuses.

the posterior IongitudinaI bundIe and the region of the colIicuIi giving optic atrophy, paraIysis of upward gaze and paraIysis of convergence.” Additiona note: “This patient is of poor materia1 to start, and has had many injuries. The cause of the seizures is compIicated, but it is highIy probabIe that the head injuries have much to do with them. In addition, the boy probabIy has an encephalitis. This may even be SchiIder’s disease or traumatic multipIe sclerosis.” Course. The patient remained unconscious for thirty-four hours after his second admission to the hospita1. The temperature and puIse became norma on the second day. In the next three days he vomited severa times, and had diffrcuIty swaIIowing. After the fifth day he couId eat and drink weI1. He was discharged on the eIeventh day feering weI1, but with an ataxic and stumbring gait, and poor cerebration. He was readmitted four days Iater because of a dark atrophic area on the sacrum, the cause of which was not clear, for he had been up and around for several days. He was discharged again after three weeks. The patient evidently did fairIy we11 for the next four

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Werden-Intracranial

months. He then began having severe headaches for two or three days, and suddenIy became unconscious, but without convuIsions.

AnomaIy

Amerkn

Journnl

of Surgery

1 1 ‘?

long, especiaIIy from the ears to the vertex, and was very narrow. The chest was narrow and flattened IateraIIy, the sternum protruding

Flc;. 3. Coronal section through splenium of corpus coIlosum showing space occupied by venous aneurysm. Surrounding veins stiI1 present. Interna hYdrocephaIus. Third Admission (November, 1931). He was again readmitted to the hospita1 folIowing three days of coma and fever. Examination by the writer was as foIloWs: “BiIateraI optic atrophy is present; the veins are distended and tortuous, but the discs are flat. The pupiIs are equal, but irreguIar and react poorIy to Iight. The right eye deviates to the right. The neck is not stiff. There is a bruit throughout the skuI1 and maIar eminences; the sharp initial sound is aImost a whistIe. A definite thriI1 is present over the Ieft interna jugular vein. The abdomina1 and cremasteric reAexes are lost. There is a biIatera1 Babinski reflex, but the deep reflexes are sIuggish. A Iumbar puncture reveaIs pink spina fluid under 330 mm. of water pressure. After the red ceIIs have settIed out the fIuid is cIear and of a deep yeIIow color. Diagnosis: Brain tumor, either pituitary or aneurysm, or hemangioma, right, with rupture into the subarachnoid space.” Treatment. The patient was given ice packs to the head and neck, IO per cent glucose intravenousIy, norma saIine hypodermicaIIy, and caffein and atropin every three hours. The temperature and puIse remained about the same. The patient died suddenIy twenty hours after admission. Postmortem Findings. The postmortem examination was done by Dr. John Schaefer of’ the coroner’s offIce. There was no externa1 evidence of injury. The head was abnormaIIy

FIG.

5. Grooves in occipita1 from each of paired

lobes made by occipita1 sinus.

pressure

forward from the thorax. On opening the head the bones of the skuI1 were found reinforced by thick ridges internaIIy, and the basa1 fossae were exceptionaIIy deep and narrow. The seIIa turcica was greatly ffattened from above downward. The petrous portions of the tempora1 bones were abnormaIIy high, and on opening were fount1 composed of numerous large ceIIs or cavities simiIar to and continuous with the mastoid ceIIs and the middIe ear. The contents of the skuI1 were of the utmost interest. The dura showed an enIargement and tortuosity of the superficia1 veins (Fig. I) draining into the superior IongitudinaI sinus. This sinus in turn was much Iarger than normal, having a diameter of 2.3 cm. just above the crista gaIIi. A most unusual finding, and one which had undoubtedly caused many of the patient’s symptoms and findings, was an oval, hoIIow, venous tumor measuring 2.5 X 3 X 4 cm., Iocated at the junction of the inferior Iongitudinai sinus and the straight sinuses (Fig. 2 B). The tumor was attached above and behind by a hoIIow pedicIe which formed a communication for the bIood from the tumor to the junction of the sinuses and

1x8

American Journal of Surgery

Werden-IntracraniaI

through these by the way of an anomalous sinus to join the superior Iongitudinal sinus 3 cm. above the torcuIa (Fig. 2 A). There was pro-

AnomaIy

JULY,

1935

A). Each division continued downward making deep grooves in each occipita1 lobe, and again divided (Fig. 5). One channel continued

4

Frc. 6. FIG. 7. FIG. 6. Grooves in cerebkllar lobes from pressure from enlarged, paired occipita1 sinuses. FIG. 7. Hemorrhage iavading brain stem. Note diIated, thin-waIled basilar vessel.

trusion of the mass downward and forward so that pressure was exerted upon the tegmenta1 surface of the mesencephaIon, pushing the latter forward against the spIenium of the corpus coIIosum. It had aIso pushed into the temporal Iobes IateraIIy (Fig. 3). The waI1 of this mass consisted of a pIexus of veins pIaced on a thin fibrous membrane. It is thought that these veins drained the great

FIG. 9. FIG. 8. FIG. 8. Tortuous vein compressing cervica1 cord. FIG. 9. Tortuous vein eroding into cervica1 cord.

of GaIen. Within the mass was a singIe cavity MIed with postmortem cIot. The dura1 sinuses showed a further remarkable picture. About 5 cm. above the torcuIa the superior IongitudinaI sinus divided (Fig. vein

laterally as the Iateral sinus (Fig. 4 B) ; the other went downward as one of a pair of coursed occipita1 sinuses (Fig. 4 c). These diagonaIIy down and forward continuing across the posterior fossae making deep grooves in the inferior surfaces of the cerebeIIar Iobes. (Fig. 6). Returning to the site above mentioned 5 cm. above the torcula, there was found an anomaIous dura1 venous channeI running downward and forward (Fig. 2 from B to A) to continue through the pedicIe of the venous tumor. The venous channeIs over the pons and meduIIa on both ventra1 and dorsa1 surfaces were greatIy engorged, tortuous and enIarged. A Iarge basiIar artery Made a groove for itseIf on the basal surface of the pons. There was aIso an accompanying vein with this artery which emptied into the occipital sinus on that side. The brain stem, when sectioned revealed a hemorrhage at the peduncIes, against the Ieft side of the brain stem (Fig. 7), 1.8 X 0.8 cm. with a the cIot measuring hemorrhagic softening of the adjacent brain. This hemorrhage probabIy came from the rupture of one of the radicIes of the large vein accompanying the basiIar artery, causing the patient’s death. The third ventricle protruded anteriorIy and was exceedingly thin, with many distorted vesseIs in its walIs. The IateraI ventricles were

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SLHIFS VOL. XXIX.

tremendously

No.

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Werden-Intracranial

enlarged, especiaIIy the tempora1

horns. The veins of the spina cord in the upper cervical region were about 4 mm. in diameter and extremely tortuous (Fig. 8). A convoIution of the posterior median artery had eroded more than half through the cord in the cervica1 area (Fig. 9). Upon opening the body a thymus weighing about 25 gm. was found. The superior vena subcIavian and interna cava, innominate, jugmar veins were large, deIicate and of measuring paper-thinness, the innominate about 3.5 cm. in diameter. The heart showed nothing unusua1. The visceral pleura showed numerous, extensive tortuous venous varicosities which might well be termed hemangiomas. The spIeen was about twice norma size and for the greater portion showed nothing unusual, but in portions appeared extremeIy \-ascuIar, having the appearance of a fine sponge. The abdomina1 vena cava and the common iIiac veins were about norma in size but thin walled. No other gross lesions were found. DISCUSSION

This case appears to be one of generahzed congenita1 venous anomaIy with the most abnorma1 changes in the intracrania1 venous channeIs. These changes can adequateIy expIain the cIinica1 findings. The venous aneurysm, because of its size and location above the brain stem produced the folIowing: I. An audible bruit throughout the skuI1.

AnomaIy

American Journal of Surircry

rr9

2. Pressure on the mesencephaIon giving paraIysis of upward gaze, dilated and irregular pupiIs, and obstruction of the aqueduct of SyIvius giving hydrocephalus, menta1 deterioration and optrc atrophy. 3. Pressure on the meduIIa giving vomiting and dysphagia. The thinness of the veins caused spontaneous subarachnoid hemorrhage and hemorrhage on the sIightest crania1 trauma. These produced the attacks of headache, coma, nausea and vomiting, and convuIsions. Erosion of the cervica1 cord by the intraspina1 veins possibly caused, together with the pressure on the brain stem, the weakness, ataxia and incoordination of the Iower extremities. This is a case in which the diagnosis, though difhcuIt and uncertain, ~vas IinaIIy approximated, and one in which all of the cIinica1 findings, though varied, could be expIained by the Iesions found at postmortem examination. Such a case emphasizes the foIIowing: I. The occurrence of sudden attacks of headache, nausea, vomiting, coma and possibIy ConvuIsions, then fever and stiffness of the neck, foIIowed by improvement, shouId suggest an intracrania1 hemorrhage. When these findings are associated with minor crania1 trauma and red or yeIIow spina fluid, bleeding into the ventricuIar or subarchnoid fluid shouId be kept in mind.