Ruptured cerebral aneurysm in pregnancy and puerperium

Ruptured cerebral aneurysm in pregnancy and puerperium

RUPTURED CEREBRAL ANEURYSM IN PREGNANCY AND PUERPERIUM Report of 2 Cases MAXWELL N_ WACKER, M.D., CHICAGO, ILL. (From the Departments of Obstetri...

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RUPTURED CEREBRAL ANEURYSM IN PREGNANCY AND PUERPERIUM Report of 2 Cases MAXWELL

N_

WACKER,

M.D.,

CHICAGO, ILL.

(From the Departments of Obstetrics and Gynecology, The Chicago Medical School, Mount Sinai Hospital, and Edgewater Hospital)

T

HE maternal mortality is continuously being reduced below the so-called irreducible minimum. Death due to the common causes-hemorrhage, toxemia, and infection-is usually preventable. Subarachnoid hemorrhage due to ruptured cerebral aneurysm is one example of a nonpreventable cause of maternal death. The dramatic fashion of the onset of symptoms plus the high mortality of the mother and baby make this rerebrovascular accident a challenge to the diagnostician. The purpose of this paper is to review the literature briefly, to emphasize the diagnostie characteristics of subarachnoid hemorrhage, and to report 2 eases-one probable and one confirmed. The term, ''spontaneous subarachnoid hemorrhage,'' is descriptive of the clinical condition characterized by the sudden onset of severe headache followed shortly by nuchal rigidity, coma, and bloody spinal fluid. External physical trauma is excluded as an etiological factor. In the great majority of cases, the cause of the hemorrhage is rupture of an arterial aneurysm of the circle of "\Villis at the base of the brain. 2 ~ 7 , 11 This paper will consider only ruptured cerebral aneurysm followed by subarachnoid hemorrhage; the 2 terms will be used iilterchangeably. I shall not discuss unruptured cerebral aneurysm or intracerebral hemorrhage which breaks into the subarachnoid space. Many excellent monographs have been 'vritten on the subject of cerebral aneurysm 2 • 7 • 11 ; howevcl', it is not very oftC'n considered in obstetric publications. Pedowitz and PerelP 0 recently reviewed the literature; 32 proved cases and 47 probable cases of cerebral aneurysm occurring during pregnancy and puerperium were included in the review. Since their paper was published there have been other case reports.'· 5 CASE 1.-A 34-year-old white woman, gravida ii, para i, was seen on Dec. 24, 1949, with a history of amenorrhea since Oct. 30, 1949. Her past medical, surgical, and family history was unimportant. Her previous pregnancy and delivery were normal. The physical examination revealed no abnormal findings; the uterus was enlarged to the size of a 10 weeks' gestation. The blood pressure was 104/70. The laboratory findings were all within normal limits.

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& Gynec. December, 1951)

The subsequent prenatal course was uneventful and included 11 office visits; the blood pressure varied between 110/70 and 120/80. Labor began spontaneously one week prior to term on July 16, 1950, at 2:30 P.i\1. The patient was admitted to Edgewater Hospital and at 3:30 P.M. she was delivered spontaneously of a living male baby weighing 5 pounds. The puerperium was uneventful until the seventh day post parturn. At 11:30 P.M. ~lw complained of a sudden, severe headache. One·half hour later examination revealed the patient to be semicomatose. Nuchal rigidity was present. 'l'he blood pressure was 190/98. At 3:00 A.M. generalized tremors developed. Treatment included the administration of oxygen, 2 c.c. of 50 per cent solution of magnesium sulfate, antl 500 c.c. of 25 per cent solution of dextrose intravenously. Phenobarbital, ;~ grains, was given intramuscularly. Neurologic consultation was obtained. Spinal puncture revealed bloody spinal fluid under increased pressure. The patieu1 remained in deep coma. A second spinal tap done 2·1 hours after the first was also bloody. At this time urinalysis showed 4 plus proteinuria and 2 plus glycosuria. The blood pressure was now 120/70. The eondition of the patient became steadily worse; the temperature rose to 104 o F'. rectally, the blood pressure dropped to 80/0, and death occurred at 10:35 P.M., July 25, 1950, 9 days post partum and approximately 48 hours after the onset of headache. Permission for autopsy could not be obtained. The diagnosis was spontaneous subarachnoid hemorrhage due to rupture of cerebral aneurysm. CASE 2.-A 24-year-old white woman, gravida i, para 0, was admitted to Mount Sinai Hospital at 9:00 P.M., July 24, 1957, complaining of severe headache and blurred vision. The last normal menses occurred Nov. 16, 1956; the duration of gestation was 36 weeks. At the age of 18 the patient had been found to have a blood pressure of 200/100. Since then she had been under continuous medical supervision. In 1954, at the agf' of 21, she had been hospitalized because of blurring of vision in the left eye. Slight choking of the disc and retinal hemorrhage were found in the left eye. The results of complete blood chemistry studies and urinalysis were within normal limits. The blood pressure was 150/100. The patient was referred to me by the internist as soon as he discovered she wa~ pregnant. On Jan. 19, 1957, the blood pres8ure was 170/90. Physical examination did not reveal any abnormalities. Vaginal examination confirmed the presence of an 8 to 10 weeks' pregnancy. The pelvic measurements were normal. 'l'he routine laboratory tests and chemi· cal studies of the blood revealed normal values. The prenatal course for the following 7 months was uneventful. The blood pressure ranged between 180 and 160 systolic and between 100 and 90 diastolic. 'l'he urine remained normal; the patient's "-eight in<'reased by 8 pounds. Except for occasional headaches she had no complaints.

On July 24, 1957, at R6 weeks' gestation, the patient notified me that she had had a constant severe headache all day with some blurring of vision. She was admitted to the hospital at 9:00 P.M. of the same day. On admission she did not appear to be acutely ill. Her chief complaint was severe constant headache and blurred vision. The blood pressure was 170/110. Funduscopic examination revealed moderate choked discs and recent hemorrhages in the right retina. The uterus was enlarged to the size of a 36 weeks' gestation; the presentation was cephalic and the fetal heart sounds were normal. A diagnosis was made of pregnancy complicated by essential hypertension with superimposed toxemia. The i=ediate treatment was rest in bed; icecap applied to the head; Demerol, 75 mg., and phenobarbital, 100 mg., given intramuscularly. About 30 minutes after admission thr patient began to complain bitterly of pain in the left eye. She became very restless aml began to scream. Two hours later she became comatose. Her skin was cold, pale, and sweaty; the respirations were irregular and gasping. The blood pressure was 250/130.

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The patient was placed in an oxygen tent. An infusion of 50 per cent dextrose in water was started and 2 c.c. of a 50 per cent solution of magnesium sulfate was given intra· muscularly. Neurologic examination revealed nuchal rigidity and hyperactive reflexes in all extremities. Multiple retinal hemorrhages and choked discs were seen bilaterally. For the next 12 hours the patient remained more or less in a coma. There were short periods of consciousness during which she complained of headache. The blood pressure dropped to 140/ 110; the pulse became strong and slow, with a rate of 72 per minute. At 11:40 A.M. on July 25, 1957, about 14 hours after admission, the patient had a generalized co nvulsion, became deeply cyanotic, and died 20 minutes after the onset of the convulsion. Preparations had been made for cesarean section when it was apparent that the mother was in extremis. The moment her heart sounds ceased, the abdomen and uterus were incised a nd a living female baby was delivered. The infant was immediately taken to the premature nursery. The birth weight was 4 pounds, 11 ounces. The baby thrived and was discharged from the hospital at 4 weeks of age weighing 5 pounds, 11 ounces, in apparently normal condition. Autopsy findings of the mother included ruptured aneurysm of the circle of Willis at the bifurcation of the basilar artery. The left adrenal was replaced by a large pear-shaped mass whi ch was attached to and in\Wlved the paravertebral sympathetic ganglia from T-3 to 'r-10.

.l<'i g .

1.-Case 2.

Ruptured aneurysm at the bifurcation of the basilar artery.

The diagnosis was ruptured berry aneurysm (Fig. 1) of the circle of Willis, subarachnoid hemorrhage, ganglioneurofibromatosis of thoracic sympathetic chain, and ganglioneuroma of left adrenal.

Comment These 2 cases are representative of 2 major groups of patients with ruptured cerebral aneurysm. In Case 1, the patient was apparently normal in every respect; subarachnoid hemorrhage was like a bolt from the blue. Approximately 40 per cent of patients in this group have no antecedent disease or complaints. 7 In about 25 per cent of reported cases10 rupture of the cerebral aneurysm occurred during labor or shortly after delivery. In this case (Case 1) the cerebral aneurysm withstood the increased pressure of labor only to rupture on the seventh postpartum day. Case 2 is typical of a large group of patients comprising about 20 per cent of one series. 7 These patients were hypertensive prior to the rupture of the cerebral aneurysm. In the second case it is likely that a hypertensive crisis occurred causing the aneurysm to rupture its wall.

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Pathogenesis.-The aneurysm develops because of a congenital weakness of the wall of the vessels of the circle of ·willis. True congenital cerebral aneurysms have been reported in several newborns." Usually the aneurysm occurs at a bifurcation of the vessel. The size of the aneurysn1 is often snla1L varying from 4 to 10 rum. At autopsy skillful disseetion is usuall.v nt'cessary to locate the site of the blowout. '"That causes the aneurysm to bllrst ~ The rupture of a cerebral aneur~ys1n is contingent on such vagaries as its age, size, and location. It is logical to assume that physical stresses and strains which tend to raise the pressure of thP blood in the cerebral vessels should predispose to rupture. Ho,Never~ rnptnr(•s occur in the second trimester, with its rnidgestational drop in blood pressur!', almost as often as they occur during labor, when presumably the stress is greater. Most ruptures occur in the third trimester. 10 About 80 per cent of diagnosed unruptured aneurysms eventually rupture.' Approximately 50 per cent of patients with ruptured aneurysm die shortl)' after the first attack 11 ; the second rupture mmally occurs 2 to 4 weeks after the first and kills 75 per cent of the remaining patients. The operative mortalit)· of brain surgery for cerebral aneurysm is 50 per cent. Treatment.-It is oln·ious on the basis of mortalitv statistics that not much can be done for the mother after subarachnoid h~morrhage has occurred. Symptomatic treatment including spinal puncture is generally advocated. If the patient survives the first attack, angiograms and arteriograms may he made to localize tl1e lesion in the brain and to determine the operability. 1 • ., When the fetus is viable the best method of delivery is often dehatablP. Spontaneous delivery6 is advocated as often as cesarean seetion. 3 It iR regrrttahle that often the mother dies undelivered. The fetus deserving the most consideration is the one at or near term. Rarely are the circumstances propitious for a rapid forceps delivet~y prior to the death of the mother. Reliance for the rescue of the baby from the> dying mother must be placed on cesarean section. This is usually performed immediately after the death of the mother. Faiiure of the baby to survive is probably due to the progressive anoxemia. Continuous administration of oxygen to the dying mother may help to maintain adequate oxygen tension. ir1 the circulation. In addition to the Inaturity· of the fetus, the prognosis for the baby seems also dependent on how quickly it can be> removed from the uterus after the death of the mother. Poshnortern, Cesarean Section.-The pros and eons of this operation have been outlined adequately in the literature, 8 • 12 including the legal and moral issues. The survival rate of babies delivered by postmortem cesarean section is extremely low. At l\'Iount Sinai Hospital the records of the last 42 ~'ears inelude 9 postmortem cesarean sections; none of the 9 babies survived. The following factors may have contributed to the survival of the baby in Case 2: (1) the. advanced gestational age of the fetus-36 weeks (most of th(' neonatal deaths have been associated with prematurity); (2) tlw continuous administration of oxygen to the mother; (3) the rapid course of the illness: ( 4) the prompt removal of the fetus from the utPrns bPfore severe h~rpoxia caused irreversible brain damage. Summary and Conclusion Spontaneous subarachnoid hemorrhage is nearly always due to rupture of a cerebral aneurysm. The clinical and pathologic characteristics were discussed. Pregnancy does not seem to alter these characteristics. Prompt diagnosis may prolong the life of the mother and the fetus though the mortality is very high.

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Two cases were discussed. In one, a postmortem cesarean section resulted in a normal baby which survived. I lvish to express my appreciation for the assistance of Dr. H. T. Grinvalsky, ment of Pathology, Mount Sinai Hospital, Chicago, Ill.

Depart~

References 1. Bleakney, R.: South. M. J. 50: 1168, 1957. 2. Brain, W. R.: Diseases of the Nervous System, ed. 4, New York, 1951, Oxford University Press. 3. Conley, J. W., and Rand, C. W.: A. M. A. Arch. Neurol. & Psychiat. 66: 443, 1951. 4. Feldman, R. L., Gross, S. W., and Wimpfheimer, S.: AM. J. 0BST. & GYNEC. 70: 389, 1955. 5. Finola, G. C.: AM. J. 0BST. & GYNEC. 74: 1342, 1957. 6. Fleming, S. P. 1 and Mauzy, C. H.: AM. J. 0BST.' & GYNEC. 70: 1133 1 1955. 7. Hamby, W. B.: Intracranial Aneurysm, Springfield, Ill., 1952, Charles C Thomas, Publisher. 8. Lattuada, H. P.: Am. J. Surg. 84: 212, 1952. 9. Newcomb, A. L., and Munns, G. F.: Pediatrics 3: 769, 1949. 10. Pedowitz, P., and Perell, A.: AM. J. 0BST. & GYNEC. 73: 736, 1957. 11. Richardson, J. C., and Hyland, H. H.: Medicine 20: 1, 1941. 12. Weil, A. M., and Graber, V. R.: AM. J. OBST. & GYNEC. 73: 754, 1957.