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Ruptured Intracranial Aneurysm during Pregnancy: Diagnosis and Treatment
RUPTURED
INTRACRANIAL DIAGNOSIS
ANEURYSM DURING AND TREATMENT
PREGNANCY:
ROBERT 1~. FELDMAN, M.D., SIDNEY W. GROSS, M.D., AND SEYMOUR WIMPFHEIMER, M.D., NEW YORK, N. Y. (From
the
Obstetricaland Neuroswgical
Services
of
the
Mount
Sinai
Hospital)
HEN the alarming signs and symptoms of subarachnoid hemorrhage occur in a pregnant patient, a cerebral complication of toxemia is usually suspected. Most nonobstetrical reports claim the rupture of an intracranial aneurysm to be the commonest cause of subarachnoid hemorrhage.ll 2l 3 Schreiber,4 reporting from the Harper Hospital in Detroit, claims that, in hospitals where this condition is carefully sought for, the incidence of one case per month is not unusual. Contrarily, it is considered to be a rare complication of pregnancy and is neglected completely by most textbooks of obstetrics. Hamby in his monograph, “Intracranial Aneurysms,” makes no mention of it as a complication of pregnancy. A review of the literature reveals only two cases proved by carotid angiography.6, 7 In the remaining reports the diagnosis is only assumed on the basis of the symptoms and cerebrospinal fluid or is made at necropsy. It is the purpose of this paper to clarify the differential diagnosis, review the literature, and discuss the treatment according to physiological principles.
W
Etiology There are three types of intracranial aneurysms generally described: (1) congenital, (2) mycotic, and (3) arteriosclerotic. Chief interest is centered on the congenital, which is saccular and occurs in the region of the angles formed by the bifurcation of the arteries of the circle of Willis or their branches. Common synonyms used are congenital, developmental, bifurcation, and berry aneurysms. Bremer* showed embryologically that the defects in the media at the bifurcations of the circle of Willis are true congenital aneurysms, an origin different from those previously postulated in the theories of GlynnQ and Richardson and Hyland.’ Signs and
Symptoms
Congenital aneurysms of the circle of Willis usually show few signs or symptoms until they rupture. Sudden headache is the most common primary symptom, usually occipital, then generalized. This most frequently occurs during ordinary activity. Other early signs are nausea, vomiting, and nuchal rigidity, followed in different cases by hemiplegia, weakness, convulsions, numbness, vertigo, unconsciousness, and visual disturbances. The temperature may be elevated and bradycardia is often present due to increased intracranial pressure. The symptoms vary with the degree of hemorrhage. Death The ocular signs in aneurysms of the circle often occurs without warning. 289
290
PELD~IAN,
GROSS, Am
~VIMPE~JIEIM:B
.\m. .1.Obst.& G:).mr Allgust, 195;
of Willis have received a great deal of attention.‘” Eye pain associated with ptosis and dilatation of the ipsilateral pupil should lead to :I suspicion of a11 aneurysm of the anterior portion of the circle of Willis. Grossly bloody spinal fluid is diagnostic of a hemorrhage which has either originated in the meninges or extended from the brain into the ventricles 01’ subarachnoid space. A spinal puncture during the acute stage usually shows an elevated pressure. Other conditions t,o be ruled out are hypertensive cardiovascular disease, epilepsy, brain tumors, meningitis, multiple sclerosis, t,raumatic intracranial hemorrhage, cerebral embolism from foci such as rhollmatic carditis and cerebral thrombophlebitis, Jeukemia, hemophilia, and other blood dyscrasias. The history of a previous aneurysm or similar symptoms Toxemia is ruled out by the must make one suspicious of another rupture. absence of its classical triad. The diagnosis was formerly made, before the advent of carotid angiography, on the basis of a history, physical findings, and examination of the spinal fluid. This is not enough t,o establish a positive diagnosis, however, as exemplified by the case reported by Lerer,ll whose pat,ient, in the fifth month of pregnancy, suddenly experienced all the signs and symptoms associated with rupture of an aneurysm of t,he circle of Willis, but, at postmortem no evidence of a congenital aneurysm was found. Most of the early reports of intracranial hemorrhage during pregnancy have no angiography or postmortem results, 12-loleaving the ultimate lesion in doubt. Cerebral angiography introduced by Moniz? in 1927 and f~llly reported in 1934, stimulated an interest in intracranial aneurysms and vascular malformations. GrosP in 1939 introduced the use of Diodrast for the ilemotlstration of the cerebral circulation radiologically.
Prognosis Subarachnoid hemorrhage due to t,he rupture of an intracranial aneurysm is associated with a high mortality, even without the added factors introduced by pregnancy and labor. According to Rhoads, about 50 per cent of the cases of ruptured intracranial aneurysm end fatally. Of those who recover on conservative therapy, a. large number die (luring the ensuing year as a result of a second hemorrhage. SchreibeP claims that one-third of his patients died during the first admission, and, of those surviving the first week, one-half hat1 recurrences.
Treatment The obstetrician or surgeon who is suddenly confrontecl with such a prob lem is often at a loss as to the best method of management. The treatment (11‘ subarachnoid bleeding due to rupture of an intracranial aneurysm is still a matter of controversy. Some physicians belong to the “do nothing” school and treat their patients with expectant optimism, hoping that the tear in the aneurysm will become sealed with a clot and that the bleeding will stop. Unfortunately, this is not usually the case. It seems to us that sound physical and physiological principles dictate that the first consideration is to control intracranial bleeding. It is now fairly well established that most aneurysms of the intracranial portion of the internal carotid artery and of the proximal branches of the circle of Willis can be successfully treated by ligation of the common or internal carotid artery in the neck.
Historical John Abernathy,z3 John Hunter’s immediate successor in London, ligated the common carotid artery for hemorrhage in 1798, but his patient died 30 hours later. Fleming in 1803,24a young surgeon in the Royal Navy, is credited with the first successful ligation of the common carotid artery. His patient attempted to commit suicide by cutting his throat while serving on
Volume70 Number
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INTRACRANIAL
ANEURYSM
2
IN
PREGNANCY
291
H.M.S. Tonnant. Fleming treated the neck wound and his patient was convalescing satisfactorily when eight days later, during a severe paroxysm of coughing, the carotid artery burst. Fleming cut down on the common carotid artery below the wound and tied it. The patient made an uneventful reDuring the first half of the nineteenth century, ligation of the carotid covery. artery was frequent, not only for hemorrhage and peripheral aneurysms but also for epilepsy, psychosis, and trigeminal neuralgia. In 1902, HorsleyZ5 ligated the right common carotid artery in the neck for an intracranial aneurysm discovered during a craniotomy for a suspected brain tumor. His pat,ient was followed for five years and was well at that time. Sporadic cases of carotid ligation for intracranial aneurysm appeared in the literature from t,hen until 1940 when Schorsteinz6 reported 60 cases of cafptid ligation for intracranial aneurysms of spontaneous origin. Rogers*’ oh 1947 reported ligation and discission of the common carotid artery in seven cases of spontaneous subarachnoid hemorrhage due presumably to leaking aneurysms. Wechsler and GrossZ8 in 1948 advocated arteriography and ligation of the common carotid artery not only in subarachnoid hemorrhage due to leaking aneurysms but also in some cases of cerebral vascular malformations. Wechsler, Gross, and Cohenzg in 1951 reported the results of common carotid ligation in the neck in 30 cases. There were 17 aneurysms of the circle of Willis with only one death.
Method The location of the aneurysm must first be accurately established by cerebral angiography, and the competency of the communicating arteries in providing an adequate blood supply to the brain must also be ascertained. Unilateral carotid puncture with compression of the opposite carotid artery during injection of the radiopaque medium with an anteroposterior x-ray exposure will usually provide the desired information. If satisfactory visualization of both sides of the anterior portion of the circle is not obtained by this method, then bilateral carotid angiography becomes essential. The treatment of the ruptured intracranial aneurysm during pregnancy does not differ materially from the treatment of such lesions where pregnancy is not involved. The principles are the same, hemorrhage must be stopped, recurrence of bleeding must be prevented, and an adequate blood supply to t,he brain must be provided if neurological complications are to be avoided. In most instances, bleeding from a ruptured intracranial aneurysm stops if the pressure within the aneurysmal sac is reduced. Ligation of the carotid artery in the neck reduces the systolic blood pressure above the point of ligation about 50 per cent, and the pulse pressure by about 75 per cent, thus clotting takes place in the aneurysm and further bleeding is prevented. ReOne most serious current hemorrhage rarely occurs after carotid ligation. objection to carotid ligation is the occurrence of neurological complications has varied greatly in the in some cases. The incidence of such complications Common carotid artery ligation is done only statistics of different operators. if the patient tolerates occlusion for at least 15 minutes.
Case Report The patient was a 2%year-old white primipara in the eighth month of pregnancy. She had always been in good health and her pregnancy up to the present illness had been Two weeks before admission to the hospital, while at home shaking a rug, uneventful. she felt a sudden pain in the top of her head and the back of her neck. During the next few days she improved, but five days later the head pain recurred and she was admitted to her local hospital. Spinal puncture showed a grossly bloody fluid. The patient improved for about ten days but then had a recurrence of severe head and neck pain. Spinal puncture at this time revealed old and fresh blood and the patient was admitted to the Mount
FELDMAN,
29”
GROSS,
9ND
Am. J. Obst. & Gynec. August, IQ<;
WIMPFHEIMER
The blood pressure was 136/l, She was confused and disoriented. Sinai Hospital. Temperature, pulse rate, respirations, and urine were normal. There was marked uuchal There was a slight right central farial rigidity. The fundi showed early papilledema. Thirty-six hours after admission thr right paresis and a positive right Habinski sign. On releasing the right common carotid arter!, pupil became slightly larger than the left. after a period of digital compression, the patient complained of a pounding pain in thl* head. Release after compression of tho left commnn carotid artery did not cause hear1
Fig.
l.-Aneurys~n
springing
from
bifurcation
eating
of
right
arteries.
internal
carotid
and
uosterior
comnmni-
pain. At this time both common carotid arteries were exposed under Pentothal anesthesia after an endotrac’heal lube had been passed. T,ocal 1 per cent procaine was used as a supplementary anesthetic. Angiography was carried out with two injections each of I(1 C.C. of 35 per cent Diodrast into the right common carotid artery. During the second illjection of Diodrast an anteroposterior x-ray exposure w-as made while the left common carotid artery was occluded with a rubber tape. The lateral films disclosed a berry aneurysm springing from the junction of the internal carotid artery and the right posterior communicating artery (Fig. 1). The anteroposterior view showed an adequate passage of Diodrast from the right c,arotid system t,o the left. This indicated a competent anterior collateral circulation. The right common carotid artery was occluded with a rubber tape for fifteen minutes. The patient. was permitted to awaken. No untoward signs or symptoms developed. The right common carotid artery was then doubly ligated with braided silk.
F”llger
‘2”
RUPTURED
INTRACRANIAL
ANEURYSM
IN
The patient recovered rapidly following these procedures. deliver her by cesarean section at or about term three weeks following carotid artery ligation, the membranes ruptured. Delivery was by cesarean section. The patient and the charged three weeks later.
PREGNANCY The later. newborn
293 plan had been to However, ten days infant
were
dis-
Review of the Literature There have been only 5 reported cases of a ruptured circle of Willis during pregnancy or the early puerperium ography or necropsy. Strohscheim and Suzuki6 in July, intracranial aneurysm during pregnancy
aneurysm of the proved by angi-
1947, reported one case of ruptured proved at necropsy.
This patient was a 35.year-old, gravicla ii, para i, admitted to the hospital two weeks before term with persistent occipital and frontal headaches of thirty-one hours’ duration. On the fourth hospital day, the patient went into labor and a low cervical section was performed under ethylene-oxygen-ether anesthesia. Four days later, after a normal postpartum course, she lapsed into coma and died. Necropsy findings revealed a ruptured aneurysm on the posterior communicating artery measuring 4 mm. in size. Microscopically, there was fresh hemorrhage within the subarachnoid space.
RhoadP in September, 1947, reported 2 cases of congenital aneurysm of the circle of Willis associated with pregnancy, one proved at postmortem, the other a tentative diagnosis made by signs and symptoms. The first patient had had three episodes of frank cerebrovascular hemorrhage over a period of twelve years, Her symptoms on various admissions included dizziness, fainting, unconsciousness, left-sided numbness, nuchal rigidity, pin-point pupils, pain in the left eye, swelling of the globe of the left eye, and vomiting. On three separate admissions after the recurrence of neurological symptoms the spinal fluid was found to be grossly bloody and under increased tension. No angiograms were performed. The patient was next admitted three months pregnant for consideration of a therapeutic abortion. A consultation was held and it was decided not to perform a therapeutic abortion, but that the patient should be sterilized forty-eight hours after delivery. Two months later the patient died following a generalized convulsion after being admitted in a semicomatose condition. At postmortem, a berry aneurysm of the circle of Willis was found. The second case reported by Rhoads was of a 27-year-old patient who, on the ninth postpartum day, had sudden convulsions beginning on the right and becoming generalized, a positive Brudzinski sign on the left, and xanthochromic spinal fluid. There were no signs or symptoms of toxemia. The only symptoms before the convulsions were severe headaches. The spinal fluid pressure was 320 mm. of water. The patient recovered on repeated lumbar punctures and bed rest. The tentative diagnosis was rupture of a congenital aneurysm of the circle of Willis.
Herzig3”
in 1948 reported
the following
case.
A 34-year-old gravida ii, para i, was admitted in coma two months from term. She had a convulsion one hour prior to admission at which time a spinal tap revealed bloody fluid, and a diagnosis of subarachnoid hemorrhage was made. The patient died undelivered after being in coma for five days, with a few lucid intervals. Postmortem examination disclosed a ruptured berry aneurysm of the right anterior cerebral artery.
Willis
Brehm7 in 1951 reported the first case of an aneursym of the circle of in pregnancy, proved by angiography and treated by carotid ligation.
A 33-year-old primipara, in the last trimester of a normal pregnancy, developed a sudden excruciating headache and nuchal rigidity with grossly bloody cerebrospinal fluid. For the next 10 days she was treated with repeated spinal taps and bed rest. On the An angiogram was performed which fifteenth hospital day she became unconscious.
294
FELDMAN,
GROSS,
AND
WIMPFHEIMER
Am. J. Obst. & Ggnec. August. 105;
showed an aneurysm involving the region of the origin of the posterior communicat.ing artery. The right common carotid artery was then ligated. The patient improved rapidly and thirty-nine days later had a normal infant, delivered by cesarean section.
The earlier literature which we had originally intended to include in our statistical analysis, on closer evaluation can only properly be reported as subarachnoid hemorrhage of undiagnosed origin associated with pregnancy. possibly due to the rupture of a congenital aneurysm (Mondy,lZ Wagner,“‘ Apajalahti, I6 Pancot and Gelle,l? Guttrnacherl”). .ln 1928, EastmanI writing on puerperal hemiplegias, commented that. since 1904 when Rudolph von Hosslin,‘” a German neurologist, published a monumental study of the various puerperal paralyses in which he reviewed, among other types, 146 cases of cerebral palsy! scant attention has been paid to puerperal hemiplegia in either the obstetrical or neurological literature. When mentioned in textbooks it is usually discussed briefly as one of the rare complications of eclampsia. At that time Eastman felt that. the number of cases von Hosslin was able to collectZ as well as the 7 cases of his own, rated careful consideration, particularly in the matters of etiology and prognosis. Eastman’s survey of the literature showed the same divergence of opinion as to the incidence of puerperal hemiplegia as is found today. Villa observed only 2 examples in 27 years among 10,000 deliveries, while Immelman, in the l’rauenklinik of Berlin, saw 8 ca.ses within two years. In none of these four, reports is an aneurysm of the circle of Willis contemplated. Comment Ruptured congenital aneurysms of the circle of Willis end fatally in from 35 per cent to 50 per cent of cases without the added hemodynamic and vascular changes t,hat occur during pregnancy and labor. There are significant factors in pregnancy that must be presented in the sequence of events preceding death in many of these patients. In any pregnancy there is an increased volume strain on the vascular system. With each hemorrhage the damage done to the vessel makes it more vulnerable to subsequent bleeding. SlemonP demonstrated considerable increase in pulse pressure in normal pregnancy, especially marked during the latter part. The increased blood volume during pregnancy, added to the fluctuation in the blood pressure inherent in the stress and strain of labor, superimposed on an already potentially disastrous aneurysm under even nonpregnant conditions, seems t,o make elective cesarean section the most logical method of delivery. A regimen of anesthesia which meticulously avoids the production of a hypertensive state and minimizes the danger of postoperative vomiting is most important. On the other hand, delivery per vaginam with terminal caudal anesthesia is satisfact,ory in those known to have rapid labors. The anesthetic of choice in cesarean section appears then to be a field block in a well-sedated patient. Ergotrate, with its possible hypertensive side effect, is strongly contraindicated. With the advent of toxemia of pregnancy, the hazards are greatly increased and heroic treatment is mandatory. We believe that the high mortality associated with this disease warrants t.ubal ligation in those with conclusive proof of a previous subarachnoid hemorrhage caused by a ruptured aneurysm. By the same token, when this accident occurs in early pregnancy, therapeutic termination, if desired, seems justified.
Lolume 70 Number 2
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ANEURYSM
IN
PREGNANCY
295
The possibility of a concomitant aneurysm as a cause of intracranial bleeding in patients with hypertension is an interesting conjecture. Since it is not feasible to perform angiography during eclampsia, the high mortality following complicating cerebrovascular accidents should certaJnly be followed at necropsy by close inspection of the internal carotid artery and the circle of Willis for evidence of a ruptured aneurysm. Where aut.opsy permission cannot be obtained, we suggest the possibility of shedding further light on this subject by injecting contrast media into the carotid vessels of the cadaver, i.e., morbid angiography.
Summary 1. The history and literature of ruptured intracranial aneurysm during pregnancy are briefly reviewed. 2. The etiology, symptomatology, and prognosis are presented. 3. A case of ruptured intracranial aneurysm of the circle of Willis during pregnancy, with recovery, is reported. 4. The diagnosis by angiography and treatment by carotid ligation are stressed. 5. The added gravity pregnancy imposes on such vascular lesions has been shown by case reports. 6. Cases of obscure obstetrical death may represent undiagnosed ruptured intracranial aneurysms. References 1. 2. 3. 4. 5. G. 7. :: 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30.
Martland, H. S.: Am. J. Surg. 43: 10, 1939. Richardson, J. C., and Hyland, H. H.: Medicine 20: 1, 1941. Sahs, .A. L., and Keil, P. G.: Am. Heart J. 26: 645, 1943. Harper Hosp. Bull. 3: 68, 1945. Schreiber, F.: Intracranial Aneurysms, Springfield, Ill., 1952, Charles C Thomas. Hamby, W. B.: Grace Hospital Bull. 25: 71, 1947. Strohscheim, D. F., and Suzuki, M.: Ohio State M. J. 47: 12, 1951. Brehm, W.: Arch. Path. 35: 819, 1943. Bremer, J. L.: Glynn, L. E.: J. Path. & Bact. 61: 213, 1940. Walsh, F. B., and King, A. B.: Arch. Ophth. 27: 1, 1942. Brit. Emp. 54: 659, 1947. Lerer, 8.: J. Obst. & Gynaec. Glasgow M. J. 59: 339, 1903. Mondy, L. L.: Arch. psychiat. u. Nervenkr. 38: 730, 1904. Von Hosslin, R.: AM. J. OBST. & GYNEC. 15: 758, 1928. Eastman, N. J.: Wagner: Quoted by Lerer.11 Acta obst. et pynec. scandinav. 18: 31, 1938. Anaialahti. A.: P&cot and Gelle: Bull. Sot. &&. et d’obst. 27: 366,‘1938. Johns Hopkins Hosp. Bull. 18: 448, 1907. Slemons, J. M.: Guttmacher, A.: Personal communication. Egas Moniz: L’angiographie &6brale, Paris, 1934, Masson & Cie. Gross, 8. W.: Proc. Sot. Exper. Biol. & Med. 42: 258, 1939. Rhoads, E. E.: AM. J. OBST. & GYNEC. 54: 533, 1947. Garrison, F. H.: History of Medicine, Philadelphia, 1913, W. B. Saunders Company, D. 278. Keevii, J. J.: Brit. J. Surg. 37: 92, 1949. Horsley, V.: Quoted by Beadles, C. F.: Brain 30: 285, 1907. Schorstein, J.: Brit. J. Surg. 28: 50, 1940. In Dandy, W. E.: Intracranial Arterial Aneurysms, Ithaca, N. Y., 1947, Rogers, L.: Comstock Publishing Co., Inc., p. 67. Wechsler, I. S., and Gross, S. W.: J. A. M. A. 136: 517, 1948. Wechsler, I. S., Gross, 8. W., and Cohen, I.: J. Neurol., Neurosurg. & Psychiat. 14: 25. 1951. Herzig,’ N. : Harlem Hospital Bull. 1: 80, 1948.
INTRACRANIAL DIAGNOSIS
ANEURYSM DURING AND TREATMENT
PREGNANCY:
ROBERT 1~. FELDMAN, M.D., SIDNEY W. GROSS, M.D., AND SEYMOUR WIMPFHEIMER, M.D., NEW YORK, N. Y. (From
the
Obstetricaland Neuroswgical
Services
of
the
Mount
Sinai
Hospital)
HEN the alarming signs and symptoms of subarachnoid hemorrhage occur in a pregnant patient, a cerebral complication of toxemia is usually suspected. Most nonobstetrical reports claim the rupture of an intracranial aneurysm to be the commonest cause of subarachnoid hemorrhage.ll 2l 3 Schreiber,4 reporting from the Harper Hospital in Detroit, claims that, in hospitals where this condition is carefully sought for, the incidence of one case per month is not unusual. Contrarily, it is considered to be a rare complication of pregnancy and is neglected completely by most textbooks of obstetrics. Hamby in his monograph, “Intracranial Aneurysms,” makes no mention of it as a complication of pregnancy. A review of the literature reveals only two cases proved by carotid angiography.6, 7 In the remaining reports the diagnosis is only assumed on the basis of the symptoms and cerebrospinal fluid or is made at necropsy. It is the purpose of this paper to clarify the differential diagnosis, review the literature, and discuss the treatment according to physiological principles.
W
Etiology There are three types of intracranial aneurysms generally described: (1) congenital, (2) mycotic, and (3) arteriosclerotic. Chief interest is centered on the congenital, which is saccular and occurs in the region of the angles formed by the bifurcation of the arteries of the circle of Willis or their branches. Common synonyms used are congenital, developmental, bifurcation, and berry aneurysms. Bremer* showed embryologically that the defects in the media at the bifurcations of the circle of Willis are true congenital aneurysms, an origin different from those previously postulated in the theories of GlynnQ and Richardson and Hyland.’ Signs and
Symptoms
Congenital aneurysms of the circle of Willis usually show few signs or symptoms until they rupture. Sudden headache is the most common primary symptom, usually occipital, then generalized. This most frequently occurs during ordinary activity. Other early signs are nausea, vomiting, and nuchal rigidity, followed in different cases by hemiplegia, weakness, convulsions, numbness, vertigo, unconsciousness, and visual disturbances. The temperature may be elevated and bradycardia is often present due to increased intracranial pressure. The symptoms vary with the degree of hemorrhage. Death The ocular signs in aneurysms of the circle often occurs without warning. 289
290
PELD~IAN,
GROSS, Am
~VIMPE~JIEIM:B
.\m. .1.Obst.& G:).mr Allgust, 195;
of Willis have received a great deal of attention.‘” Eye pain associated with ptosis and dilatation of the ipsilateral pupil should lead to :I suspicion of a11 aneurysm of the anterior portion of the circle of Willis. Grossly bloody spinal fluid is diagnostic of a hemorrhage which has either originated in the meninges or extended from the brain into the ventricles 01’ subarachnoid space. A spinal puncture during the acute stage usually shows an elevated pressure. Other conditions t,o be ruled out are hypertensive cardiovascular disease, epilepsy, brain tumors, meningitis, multiple sclerosis, t,raumatic intracranial hemorrhage, cerebral embolism from foci such as rhollmatic carditis and cerebral thrombophlebitis, Jeukemia, hemophilia, and other blood dyscrasias. The history of a previous aneurysm or similar symptoms Toxemia is ruled out by the must make one suspicious of another rupture. absence of its classical triad. The diagnosis was formerly made, before the advent of carotid angiography, on the basis of a history, physical findings, and examination of the spinal fluid. This is not enough t,o establish a positive diagnosis, however, as exemplified by the case reported by Lerer,ll whose pat,ient, in the fifth month of pregnancy, suddenly experienced all the signs and symptoms associated with rupture of an aneurysm of t,he circle of Willis, but, at postmortem no evidence of a congenital aneurysm was found. Most of the early reports of intracranial hemorrhage during pregnancy have no angiography or postmortem results, 12-loleaving the ultimate lesion in doubt. Cerebral angiography introduced by Moniz? in 1927 and f~llly reported in 1934, stimulated an interest in intracranial aneurysms and vascular malformations. GrosP in 1939 introduced the use of Diodrast for the ilemotlstration of the cerebral circulation radiologically.
Prognosis Subarachnoid hemorrhage due to t,he rupture of an intracranial aneurysm is associated with a high mortality, even without the added factors introduced by pregnancy and labor. According to Rhoads, about 50 per cent of the cases of ruptured intracranial aneurysm end fatally. Of those who recover on conservative therapy, a. large number die (luring the ensuing year as a result of a second hemorrhage. SchreibeP claims that one-third of his patients died during the first admission, and, of those surviving the first week, one-half hat1 recurrences.
Treatment The obstetrician or surgeon who is suddenly confrontecl with such a prob lem is often at a loss as to the best method of management. The treatment (11‘ subarachnoid bleeding due to rupture of an intracranial aneurysm is still a matter of controversy. Some physicians belong to the “do nothing” school and treat their patients with expectant optimism, hoping that the tear in the aneurysm will become sealed with a clot and that the bleeding will stop. Unfortunately, this is not usually the case. It seems to us that sound physical and physiological principles dictate that the first consideration is to control intracranial bleeding. It is now fairly well established that most aneurysms of the intracranial portion of the internal carotid artery and of the proximal branches of the circle of Willis can be successfully treated by ligation of the common or internal carotid artery in the neck.
Historical John Abernathy,z3 John Hunter’s immediate successor in London, ligated the common carotid artery for hemorrhage in 1798, but his patient died 30 hours later. Fleming in 1803,24a young surgeon in the Royal Navy, is credited with the first successful ligation of the common carotid artery. His patient attempted to commit suicide by cutting his throat while serving on
Volume70 Number
RUPTURED
INTRACRANIAL
ANEURYSM
2
IN
PREGNANCY
291
H.M.S. Tonnant. Fleming treated the neck wound and his patient was convalescing satisfactorily when eight days later, during a severe paroxysm of coughing, the carotid artery burst. Fleming cut down on the common carotid artery below the wound and tied it. The patient made an uneventful reDuring the first half of the nineteenth century, ligation of the carotid covery. artery was frequent, not only for hemorrhage and peripheral aneurysms but also for epilepsy, psychosis, and trigeminal neuralgia. In 1902, HorsleyZ5 ligated the right common carotid artery in the neck for an intracranial aneurysm discovered during a craniotomy for a suspected brain tumor. His pat,ient was followed for five years and was well at that time. Sporadic cases of carotid ligation for intracranial aneurysm appeared in the literature from t,hen until 1940 when Schorsteinz6 reported 60 cases of cafptid ligation for intracranial aneurysms of spontaneous origin. Rogers*’ oh 1947 reported ligation and discission of the common carotid artery in seven cases of spontaneous subarachnoid hemorrhage due presumably to leaking aneurysms. Wechsler and GrossZ8 in 1948 advocated arteriography and ligation of the common carotid artery not only in subarachnoid hemorrhage due to leaking aneurysms but also in some cases of cerebral vascular malformations. Wechsler, Gross, and Cohenzg in 1951 reported the results of common carotid ligation in the neck in 30 cases. There were 17 aneurysms of the circle of Willis with only one death.
Method The location of the aneurysm must first be accurately established by cerebral angiography, and the competency of the communicating arteries in providing an adequate blood supply to the brain must also be ascertained. Unilateral carotid puncture with compression of the opposite carotid artery during injection of the radiopaque medium with an anteroposterior x-ray exposure will usually provide the desired information. If satisfactory visualization of both sides of the anterior portion of the circle is not obtained by this method, then bilateral carotid angiography becomes essential. The treatment of the ruptured intracranial aneurysm during pregnancy does not differ materially from the treatment of such lesions where pregnancy is not involved. The principles are the same, hemorrhage must be stopped, recurrence of bleeding must be prevented, and an adequate blood supply to t,he brain must be provided if neurological complications are to be avoided. In most instances, bleeding from a ruptured intracranial aneurysm stops if the pressure within the aneurysmal sac is reduced. Ligation of the carotid artery in the neck reduces the systolic blood pressure above the point of ligation about 50 per cent, and the pulse pressure by about 75 per cent, thus clotting takes place in the aneurysm and further bleeding is prevented. ReOne most serious current hemorrhage rarely occurs after carotid ligation. objection to carotid ligation is the occurrence of neurological complications has varied greatly in the in some cases. The incidence of such complications Common carotid artery ligation is done only statistics of different operators. if the patient tolerates occlusion for at least 15 minutes.
Case Report The patient was a 2%year-old white primipara in the eighth month of pregnancy. She had always been in good health and her pregnancy up to the present illness had been Two weeks before admission to the hospital, while at home shaking a rug, uneventful. she felt a sudden pain in the top of her head and the back of her neck. During the next few days she improved, but five days later the head pain recurred and she was admitted to her local hospital. Spinal puncture showed a grossly bloody fluid. The patient improved for about ten days but then had a recurrence of severe head and neck pain. Spinal puncture at this time revealed old and fresh blood and the patient was admitted to the Mount
FELDMAN,
29”
GROSS,
9ND
Am. J. Obst. & Gynec. August, IQ<;
WIMPFHEIMER
The blood pressure was 136/l, She was confused and disoriented. Sinai Hospital. Temperature, pulse rate, respirations, and urine were normal. There was marked uuchal There was a slight right central farial rigidity. The fundi showed early papilledema. Thirty-six hours after admission thr right paresis and a positive right Habinski sign. On releasing the right common carotid arter!, pupil became slightly larger than the left. after a period of digital compression, the patient complained of a pounding pain in thl* head. Release after compression of tho left commnn carotid artery did not cause hear1
Fig.
l.-Aneurys~n
springing
from
bifurcation
eating
of
right
arteries.
internal
carotid
and
uosterior
comnmni-
pain. At this time both common carotid arteries were exposed under Pentothal anesthesia after an endotrac’heal lube had been passed. T,ocal 1 per cent procaine was used as a supplementary anesthetic. Angiography was carried out with two injections each of I(1 C.C. of 35 per cent Diodrast into the right common carotid artery. During the second illjection of Diodrast an anteroposterior x-ray exposure w-as made while the left common carotid artery was occluded with a rubber tape. The lateral films disclosed a berry aneurysm springing from the junction of the internal carotid artery and the right posterior communicating artery (Fig. 1). The anteroposterior view showed an adequate passage of Diodrast from the right c,arotid system t,o the left. This indicated a competent anterior collateral circulation. The right common carotid artery was occluded with a rubber tape for fifteen minutes. The patient. was permitted to awaken. No untoward signs or symptoms developed. The right common carotid artery was then doubly ligated with braided silk.
F”llger
‘2”
RUPTURED
INTRACRANIAL
ANEURYSM
IN
The patient recovered rapidly following these procedures. deliver her by cesarean section at or about term three weeks following carotid artery ligation, the membranes ruptured. Delivery was by cesarean section. The patient and the charged three weeks later.
PREGNANCY The later. newborn
293 plan had been to However, ten days infant
were
dis-
Review of the Literature There have been only 5 reported cases of a ruptured circle of Willis during pregnancy or the early puerperium ography or necropsy. Strohscheim and Suzuki6 in July, intracranial aneurysm during pregnancy
aneurysm of the proved by angi-
1947, reported one case of ruptured proved at necropsy.
This patient was a 35.year-old, gravicla ii, para i, admitted to the hospital two weeks before term with persistent occipital and frontal headaches of thirty-one hours’ duration. On the fourth hospital day, the patient went into labor and a low cervical section was performed under ethylene-oxygen-ether anesthesia. Four days later, after a normal postpartum course, she lapsed into coma and died. Necropsy findings revealed a ruptured aneurysm on the posterior communicating artery measuring 4 mm. in size. Microscopically, there was fresh hemorrhage within the subarachnoid space.
RhoadP in September, 1947, reported 2 cases of congenital aneurysm of the circle of Willis associated with pregnancy, one proved at postmortem, the other a tentative diagnosis made by signs and symptoms. The first patient had had three episodes of frank cerebrovascular hemorrhage over a period of twelve years, Her symptoms on various admissions included dizziness, fainting, unconsciousness, left-sided numbness, nuchal rigidity, pin-point pupils, pain in the left eye, swelling of the globe of the left eye, and vomiting. On three separate admissions after the recurrence of neurological symptoms the spinal fluid was found to be grossly bloody and under increased tension. No angiograms were performed. The patient was next admitted three months pregnant for consideration of a therapeutic abortion. A consultation was held and it was decided not to perform a therapeutic abortion, but that the patient should be sterilized forty-eight hours after delivery. Two months later the patient died following a generalized convulsion after being admitted in a semicomatose condition. At postmortem, a berry aneurysm of the circle of Willis was found. The second case reported by Rhoads was of a 27-year-old patient who, on the ninth postpartum day, had sudden convulsions beginning on the right and becoming generalized, a positive Brudzinski sign on the left, and xanthochromic spinal fluid. There were no signs or symptoms of toxemia. The only symptoms before the convulsions were severe headaches. The spinal fluid pressure was 320 mm. of water. The patient recovered on repeated lumbar punctures and bed rest. The tentative diagnosis was rupture of a congenital aneurysm of the circle of Willis.
Herzig3”
in 1948 reported
the following
case.
A 34-year-old gravida ii, para i, was admitted in coma two months from term. She had a convulsion one hour prior to admission at which time a spinal tap revealed bloody fluid, and a diagnosis of subarachnoid hemorrhage was made. The patient died undelivered after being in coma for five days, with a few lucid intervals. Postmortem examination disclosed a ruptured berry aneurysm of the right anterior cerebral artery.
Willis
Brehm7 in 1951 reported the first case of an aneursym of the circle of in pregnancy, proved by angiography and treated by carotid ligation.
A 33-year-old primipara, in the last trimester of a normal pregnancy, developed a sudden excruciating headache and nuchal rigidity with grossly bloody cerebrospinal fluid. For the next 10 days she was treated with repeated spinal taps and bed rest. On the An angiogram was performed which fifteenth hospital day she became unconscious.
294
FELDMAN,
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WIMPFHEIMER
Am. J. Obst. & Ggnec. August. 105;
showed an aneurysm involving the region of the origin of the posterior communicat.ing artery. The right common carotid artery was then ligated. The patient improved rapidly and thirty-nine days later had a normal infant, delivered by cesarean section.
The earlier literature which we had originally intended to include in our statistical analysis, on closer evaluation can only properly be reported as subarachnoid hemorrhage of undiagnosed origin associated with pregnancy. possibly due to the rupture of a congenital aneurysm (Mondy,lZ Wagner,“‘ Apajalahti, I6 Pancot and Gelle,l? Guttrnacherl”). .ln 1928, EastmanI writing on puerperal hemiplegias, commented that. since 1904 when Rudolph von Hosslin,‘” a German neurologist, published a monumental study of the various puerperal paralyses in which he reviewed, among other types, 146 cases of cerebral palsy! scant attention has been paid to puerperal hemiplegia in either the obstetrical or neurological literature. When mentioned in textbooks it is usually discussed briefly as one of the rare complications of eclampsia. At that time Eastman felt that. the number of cases von Hosslin was able to collectZ as well as the 7 cases of his own, rated careful consideration, particularly in the matters of etiology and prognosis. Eastman’s survey of the literature showed the same divergence of opinion as to the incidence of puerperal hemiplegia as is found today. Villa observed only 2 examples in 27 years among 10,000 deliveries, while Immelman, in the l’rauenklinik of Berlin, saw 8 ca.ses within two years. In none of these four, reports is an aneurysm of the circle of Willis contemplated. Comment Ruptured congenital aneurysms of the circle of Willis end fatally in from 35 per cent to 50 per cent of cases without the added hemodynamic and vascular changes t,hat occur during pregnancy and labor. There are significant factors in pregnancy that must be presented in the sequence of events preceding death in many of these patients. In any pregnancy there is an increased volume strain on the vascular system. With each hemorrhage the damage done to the vessel makes it more vulnerable to subsequent bleeding. SlemonP demonstrated considerable increase in pulse pressure in normal pregnancy, especially marked during the latter part. The increased blood volume during pregnancy, added to the fluctuation in the blood pressure inherent in the stress and strain of labor, superimposed on an already potentially disastrous aneurysm under even nonpregnant conditions, seems t,o make elective cesarean section the most logical method of delivery. A regimen of anesthesia which meticulously avoids the production of a hypertensive state and minimizes the danger of postoperative vomiting is most important. On the other hand, delivery per vaginam with terminal caudal anesthesia is satisfact,ory in those known to have rapid labors. The anesthetic of choice in cesarean section appears then to be a field block in a well-sedated patient. Ergotrate, with its possible hypertensive side effect, is strongly contraindicated. With the advent of toxemia of pregnancy, the hazards are greatly increased and heroic treatment is mandatory. We believe that the high mortality associated with this disease warrants t.ubal ligation in those with conclusive proof of a previous subarachnoid hemorrhage caused by a ruptured aneurysm. By the same token, when this accident occurs in early pregnancy, therapeutic termination, if desired, seems justified.
Lolume 70 Number 2
RUPTURZD
INTRACRANIAL
ANEURYSM
IN
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The possibility of a concomitant aneurysm as a cause of intracranial bleeding in patients with hypertension is an interesting conjecture. Since it is not feasible to perform angiography during eclampsia, the high mortality following complicating cerebrovascular accidents should certaJnly be followed at necropsy by close inspection of the internal carotid artery and the circle of Willis for evidence of a ruptured aneurysm. Where aut.opsy permission cannot be obtained, we suggest the possibility of shedding further light on this subject by injecting contrast media into the carotid vessels of the cadaver, i.e., morbid angiography.
Summary 1. The history and literature of ruptured intracranial aneurysm during pregnancy are briefly reviewed. 2. The etiology, symptomatology, and prognosis are presented. 3. A case of ruptured intracranial aneurysm of the circle of Willis during pregnancy, with recovery, is reported. 4. The diagnosis by angiography and treatment by carotid ligation are stressed. 5. The added gravity pregnancy imposes on such vascular lesions has been shown by case reports. 6. Cases of obscure obstetrical death may represent undiagnosed ruptured intracranial aneurysms. References 1. 2. 3. 4. 5. G. 7. :: 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30.
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