Ruptured intracranial aneurysms in the first two decades of life

A study of 58 patients

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✓ An analysis of 3000 ruptured intracranial aneurysms revealed 58 cases in patients under the age of 19 years. There was a striking incidence of aneurysms of the carotid termination and anterior cerebral complex, accounting for 43 of 58 cases, and of these 20 involved the terminal portion of the carotid artery. Vasospasm occurred slightly less often than in adults and infarction was only seen in one postmortem examination. The surgical mortality in alert patients was 7% whereas in a comparable bedrest group it was 38%. This good tolerance to surgery was evident whether intracranial operation or carotid ligation was used, but the surgical method was not randomly allocated.

Abstract

✓ An analysis of 3000 ruptured intracranial aneurysms revealed 58 cases in patients under the age of 19 years. There was a striking incidence of aneurysms of the carotid termination and anterior cerebral complex, accounting for 43 of 58 cases, and of these 20 involved the terminal portion of the carotid artery. Vasospasm occurred slightly less often than in adults and infarction was only seen in one postmortem examination. The surgical mortality in alert patients was 7% whereas in a comparable bedrest group it was 38%. This good tolerance to surgery was evident whether intracranial operation or carotid ligation was used, but the surgical method was not randomly allocated.

Intracranial congenital berry aneurysms rupture mostly during adulthood. The rarity of their rupture in the young is attested by the fact that of the 6368 cases of intracranial aneurysms compiled by the Cooperative Study4 over a 7-year period, only 41 had ruptured in the age group of 1–19 years.

The cases of ruptured intracranial aneurysms reported in early life have been mostly isolated instances,7 aside from the series by Matson,3 and hence the natural history of ruptured aneurysms in the young is sketchy. We therefore undertook to review all cases of subarachnoid hemorrhage proven by lumbar puncture which were due to ruptured intracranial aneurysms (confirmed by angiography, surgery, or necropsy) in patients under 19 years of age who were admitted to the National Hospital, Queen Square, under the care of Sir Wylie McKissock, and the Atkinson Morley's Hospital. From 1944–1968, 58 cases were found of a total of about 3000 cases of intracranial aneurysms. The age and sex of the 58 patients are given in Table 1.

TABLE 1

Age and sex of 58 patients with ruptured intracranial aneurysms

Age (yrs)MaleFemaleTotal
15–19 251742
11–14 4812
8–10 314
0–7 000

Clinical Findings
Past History

Eleven of the 58 had a previous history of headaches; only one of these suffered from classical migraine. Previous intracranial hemorrhage was suspected in 11, of which eight occurred in the preceding 8 weeks. Subarachnoid hemorrhage proven at lumbar puncture had previously occurred in three, of which two had the episode in the week prior to admission; one had had a subarachnoid hemorrhage 4 years previously. None of the cases had a family history of intracranial aneurysms.

Significant Associated Clinical Features

Blood pressure was recorded on all patients immediately on admission. Fourteen had a blood pressure of 117/80 or less, whereas six had a blood pressure of 160/90 or above. In two moribund patients, the blood pressure reading was unreliable. The remaining 36 patients had a blood pressure between 117/80 and 160/90. In view of the difficulty in evaluating the true significance of blood pressure recording in subarachnoid hemorrhage as a reflection of longstanding hypertension, a blood pressure of 160/90 or above was arbitrarily taken as signifying hypertension for the purpose of this study. Of six patients in this group, five had coarctation of the aorta. In the remaining one, no cause for the hypertension could be found despite intravenous pyelography and abdominal aortography.

None of these 58 patients had any clinical evidence of atherosclerosis, inflammatory angiopathy, subacute bacterial endocarditis, syphilis, blood dyscrasia, or a collagen disorder.

The circumstances during which the aneurysm ruptured were known in 39 patients and are tabulated (Table 2).

TABLE 2

Circumstances during which aneurysm ruptured

Activity During OnsetNo. of Cases
walking, standing, or sitting11
swimming6
cycling6
asleep or lying in bed5
pregnancy (two 6 mos pregnant, one 5 mos,
 one 2 mos)4
riding a motorbike2
immediately after a head injury (no loss of
 consciousness)2
defecation1
arm-raising exercises1
while working (coalman)1

Neurological Features

The neurological status of the 58 patients at the time of admission is shown in Table 3. Thirteen of the 58 had hemiparesis or hemiplegia at the time of admission. Of these 13 patients, seven had an aneurysm of the opposite internal carotid artery termination, three of the internal carotid-posterior communicating region, two of the anterior cerebral-anterior communicating artery complex, and one of the middle cerebral artery.

TABLE 3

Relationship of death to the level of consciousness and the presence of focal neurological signs at the time of admission

No. of PatientsLevel of ConsciousnessFocal Neurological SignsDead
40alert117
12stuporous75
6comatose66

Congenital Abnormalities

Intracranial congenital vascular abnormalities were noted in four patients by bilateral carotid angiography. Of two patients with anterior cerebral-anterior communicating artery aneurysms, one had a congenital absence of the left anterior cerebral artery, whereas the other had hypoplasia of the main proximal left anterior cerebral artery. Of two patients with terminal internal carotid artery aneurysms, one had congenital septation of the anterior cerebral artery while the other had a hypoplastic anterior cerebral artery with early bifurcation of the middle cerebral artery on the same side. There was thus no increased incidence in comparison with adults.

Extracranial congenital abnormalities were observed in nine patients, seven with coarctation of the aorta and two with bilateral polycystic kidneys (Table 4).

TABLE 4

Coarctation of aorta

Case No.Sex, Age (yrs)B.P.Site of AneurysmTherapyCoarctation Operated LaterFollow-up (yrs)Status at Follow-up
1M15 230/120terminal int.com. carotidyes13 normal
 carotidligature 
2M17.5 160/90ant. cerebral—bedrestyes9 normal
 ant. comm. art. 
3F13 155/110ant. cerebral—clipping ant.yes3 hemiplegic
 ant. comm. art.cerebl. art. 
4F15 180/130ant. cerebral—bedrestnodied 4 wks 
 ant. comm. art.after bleeding 
5M17 170/100port. comm.com. carotidyes4 normal
 art.ligature 
6F11 160/65middle cereb.clipping aneurysmyes8 normal
 art.neck 
7M8 not recordableterminal basilarbedrestnodied before 
 art.angiography 

Size and Site of Aneurysm

The size of the ruptured aneurysm varied from 3 mm to 1.5 cm in its maximum diameter, as determined from angiographic films. Twenty-three patients had an aneurysm of the anterior cerebral-anterior communicating artery, 20 of the terminal internal carotid artery, six of the internal carotid-posterior communicating artery, three of the middle cerebral artery and three of the vertebrobasilar complex. Three had multiple intracranial aneurysms.

Angiographic Findings

Bilateral carotid angiography was performed on 54 of the patients. In only three of these cases did angiography fail to demonstrate an aneurysm. Of these, one had a normal bilateral carotid and vertebral angiogram 1 day after the subarachnoid hemorrhage; the second had a normal bilateral carotid angiogram 2 days following the first subarachnoid hemorrhage. Both of these patients were ultimately shown to harbor an anterior cerebral-anterior communicating artery aneurysm. The third patient had marked intracranial hypertension with poor filling of the intracranial blood vessels; necropsy revealed an aneurysm of the posterior communicating artery.

Thirteen patients showed vessel displacement indicative of an intracerebral hematoma, seven showed vasospasm (of which three were severe), and four showed intracranial vascular abnormalities. Twenty-nine of the 54 showed no evidence of spasm, displacement, or developmental anomalies of the intracranial blood vessels.

Vertebral angiography was performed in 11, of which two showed an aneurysm of the posterior circulation, while nine were normal. This procedure was performed only when the carotid angiograms were normal or the clinical state or radiological appearances indicated that the demonstrated supratentorial aneurysm might not be the responsible lesion. It has since been the routine at this hospital to perform four-vessel angiography.

Complications of Angiography

There were two post-angiographic deaths. One stuporous patient developed right hemiplegia after left vertebral angiography and died soon after. The second patient who was previously alert developed a contralateral hemiplegia, progressive stupor, coma, and death 6 hours after carotid angiography. In both of these patients, postmortem examination showed a recently ruptured aneurysm with intraventricular and subarachnoid hemorrhage.

Recurrent Hemorrhage

Recurrent hemorrhage was proven by lumbar puncture in eight cases and was clinically certain in a further two patients. In three it occurred within 3 days of the first subarachnoid hemorrhage, in two at 21 days, in one at 43 days, and in two after a lapse of 5 years. Two patients had a suspected second hemorrhage 4 and 8 weeks later, and both died. Of the eight patients with proven second hemorrhages, six died.

Mortality

Of the 58 patients, 18 died (Table 3). Six were comatose and died within 48 hours of admission, whereas five were stuporous at the time of admission. Of the 40 alert patients, seven died during an interval varying from 3 days to 5 years, of which 5 deaths were due to proven rebleeding.

Medical Cases

Of 21 patients treated with regulated bedrest, 12 died, eight immediately and four delayed. Of the eight patients who died immediately, two were stuporous and five comatose on admission (Table 5). The remaining alert patient died 6 hours following carotid angiography. Follow-up of 1 to 15 years shows that, of nine patients alive, eight are normal and one has a minimal left-sided hemiparesis.

TABLE 5

Relationship of treatment to mortality

Level of ConsciousnessBedrestSurgeryTotal Cases
AliveDeadAliveDead  
alert8525240
stuporous126312
comatose05016
total91231658

Surgical Cases

The length of time between hemorrhage and surgery is shown in Table 6. Of 37 patients treated with various surgical procedures, 19 had common carotid artery ligation; 15 had intracranial surgery with definite procedures; one had both ligation and intracranial surgery; and two had exploratory burr holes with drainage of blood. Six of these surgical patients died: three with intracranial clipping of the aneurysm, one from rebleeding 3 months later, one from hemorrhagic infarction in the distribution of both anterior cerebral arteries 5 days after operation (shown at necropsy), and one from intraventricular hemorrhage 2 days after operation. Two were stuporous on admission. One patient who underwent carotid artery ligation in the neck died 5 days later of suspected hemorrhagic cerebral infarct, he being on postoperative Heparin. Two who had exploratory burr holes were moribund at the time of surgery and died immediately after the draining procedure. Of the 31 postoperative cases who are alive, 18 had common carotid ligation, 12 had intracranial surgery (clipping and/or wrapping with muslin), and one had a combined procedure. Of 18 patients with common carotid ligation, 13 are normal with a follow-up of 3 to 22 years, three have a mild hemiparesis with a follow-up of 4 to 7 years, and one is a psychopath. One who was normal on discharge could not be followed. Of 12 living patients with intracranial surgery, eight are normal with a follow-up of 1 to 11 years, four have severe hemiplegia (three developing it postoperatively). The single patient with the combined surgical procedures is normal after 3 years of follow-up.

TABLE 6

Length of time between aneurysm rupture and surgery

Preoperative Level of ConsciousnessNo. of CasesDay of Surgery after Rupture
1st day 2nd day3rd day4th day5th day6th day7th day8th day10th day2 wks4 wksafter 6 wks  
alert 2701240316*11*35
stuporous 92*2*201*1000010
comatose 11*00000000000

Denotes one death.

Necropsies

Necropsies were performed on 15 of 18 who died. Aside from the aneurysms seen in all, eight showed fresh subarachnoid hemorrhage, five intracerebral hemorrhage, eight intraventricular hemorrhage, two cerebral edema, and one subdural hemorrhage. Cerebral infarction was seen in only one instance in which clipping of the terminal internal carotid artery had resulted in hemorrhagic infarct in the distribution of both anterior cerebral arteries. Four of these 15 patients had angiographic evidence of vasospasm, two showing it severely. However, none had any evidence of cerebral infarct.

Evidence of atherosclerosis was found in four of 15 necropsies. In three, there was minimal fatty streaking of coronaries and aorta only. In one 19-year-old girl with a bi-lobed anterior cerebral-anterior communicating artery aneurysm, the superior ruptured loculation was free of atheroma, whereas the inferior intact loculus had evidence of atherosclerosis.

Discussion

One of the purposes of this study was to determine the presence of special factors (if any) that might lead to rupture of intracranial aneurysms in certain children and teenagers. We have been unable to incriminate any specific factor. Thus, the study of sex distribution, presence or absence of previous hemorrhages, circumstances during the time of rupture, the size, site, or shape of the aneurysm, have all yielded negative results. However, what has emerged is that congenital intracranial aneurysms do rupture during the first two decades of life, in the absence of hypertension, atherosclerosis, or physical overexertion.

Concerning the site of the ruptured aneurysm, an interesting observation has been that of the 58 patients 20 had aneurysms of the terminal internal carotid artery, an incidence of almost 35%. This is in sharp contrast to its over-all incidence of only 3% to 5% in adults with subarachnoid hemorrhage.6 A clinical corollary to this is that of 13 patients with hemiplegia in this series, seven had aneurysms of the opposite internal carotid artery termination.

The seven cases of coarctation of the aorta in this series (12%) points to the need for appropriate additional diagnostic measure in all youngsters presenting with subarachnoid hemorrhage and hypertension.

Reifenstein, et al.,5 in reviewing 104 autopsied cases of coarctation of the aorta, found 11 deaths ascribed to intracranial lesions, with an average patient age of 28.8 years. Of these 11, nine had necropsies. Five of these showed a ruptured intracranial aneurysm. No ruptured intracranial aneurysm could be demonstrated in two additional patients with subarachnoid hemorrhage.

The presence of two patients with congenital bilateral polycystic kidneys in this series gives an incidence of 3.45%. Dalgaard,2 in a series of 284 patients with bilateral polycystic kidneys, estimated that intracranial aneurysms would occur in 16% of gene carriers.

Of 21 patients treated with regulated bedrest, seven of eight immediate deaths occurred in the stuporous and comatose cases. Of 13 alert patients, five died and eight are living and normal with a follow-up of 1 to 15 years.

Of 37 patients surgically treated, six died, of whom four were stuporous or comatose on admission. Of 12 survivors from intracranial surgery, three had severe postoperative hemiplegia. In sharp contrast, only one of 18 survivors with common carotid ligation had hemiparesis (mild).

These results suggest that common carotid ligation carries less risk than intracranial surgery in the management of ruptured congenital intracranial aneurysms in the young and that both these procedures are better tolerated by them than by adults.

The relatively poor prognosis for patients with ruptured intracranial aneurysms in this series (18 deaths in 58, or 30%) irrespective of the type of management is less gloomy than in adults, who have an average mortality of over 50%.

Of 10 patients who rebled, eight were treated with regulated bed rest. Of two surgically treated, one had common carotid ligation and the other had intracranial clipping and wrapping of the aneurysm.

Some of the noncomatose patients in this series were treated with regulated bedrest instead of surgery, because they were seen during a period when a controlled trial of treatment was in progress.

An analysis of results in this series tends to favor surgery over conservative therapy in the young with ruptured intracranial aneurysms and who are not comatose.

A striking feature of the necropsy studies in this series is the absence of any evidence of cerebral infarct (including patients with angiographic evidence of vasospasm) except for a solitary case which had intracranial surgery. This observation is in striking contrast to the high necropsy incidence (60%) of cerebral infarct in adults with ruptured intracranial aneurysms,1 and this, combined with the absence of cerebral atherosclerosis (only one in a 19-year-old), perhaps helps to explain the relatively low incidence of mortality and morbidity in young patients with ruptured intracranial aneurysms as compared to that in adults.

References

  • 1.

    Crompton MR: Cerebral infarction following the rupture of cerebral berry aneurysms. Brain 87:2632801964Crompton MR: Cerebral infarction following the rupture of cerebral berry aneurysms. Brain 87:263–280 1964

  • 2.

    Dalgaard OZ: Bilateral Polycystic Disease of the Kidneys: A Follow-Up of Two Hundred and Eighty-Four Patients and Their Families. CopenhagenMunksgaard1957Dalgaard OZ: Bilateral Polycystic Disease of the Kidneys: A Follow-Up of Two Hundred and Eighty-Four Patients and Their Families. Copenhagen Munksgaard 1957

  • 3.

    Matson DD: Intracranial arterial aneurysms in childhood. J Neurosurg 23:5785831965Matson DD: Intracranial arterial aneurysms in childhood. J Neurosurg 23:578–583 1965

  • 4.

    Nishioka H: Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Section VII. Part 1. Evaluation of the conservative management of ruptured intracranial aneurysms. J Neurosurg 25:5745921966Nishioka H: Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Section VII. Part 1. Evaluation of the conservative management of ruptured intracranial aneurysms. J Neurosurg 25:574–592 1966

  • 5.

    Reifenstein GHLevine SAGross RE: Coarctation of the aorta: a review of 104 autopsied cases of “adult type,” 2 years of age or older. Amer Heart J 33:1461681947Reifenstein GH Levine SA Gross RE: Coarctation of the aorta: a review of 104 autopsied cases of “adult type” 2 years of age or older. Amer Heart J 33:146–168 1947

  • 6.

    Richardson A: Subarachnoid haemorrhage. Brit Med J 4:98921969Richardson A: Subarachnoid haemorrhage. Brit Med J 4:98–92 1969

  • 7.

    Thompson RAPribram HFW: Infantile cerebral aneurysm associated with ophthalmoplegia and quadriparesis. Neurology (Minneap) 19:7857891969Thompson RA Pribram HFW: Infantile cerebral aneurysm associated with ophthalmoplegia and quadriparesis. Neurology (Minneap) 19:785–789 1969

  • 8.

    Watson EHLowrey GH (eds): Growth and Development of Children. ChicagoYear Book Medical Publishers1962Watson EH Lowrey GH (eds): Growth and Development of Children. Chicago Year Book Medical Publishers 1962

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Article Information

Address reprint requests to: Aneel N. Patel, M.D., Division of Neurology, Hahnemann Medical College, 230 North Broad Street, Philadelphia, Pennsylvania 19102.

© AANS, except where prohibited by US copyright law.

Headings

References

1.

Crompton MR: Cerebral infarction following the rupture of cerebral berry aneurysms. Brain 87:2632801964Crompton MR: Cerebral infarction following the rupture of cerebral berry aneurysms. Brain 87:263–280 1964

2.

Dalgaard OZ: Bilateral Polycystic Disease of the Kidneys: A Follow-Up of Two Hundred and Eighty-Four Patients and Their Families. CopenhagenMunksgaard1957Dalgaard OZ: Bilateral Polycystic Disease of the Kidneys: A Follow-Up of Two Hundred and Eighty-Four Patients and Their Families. Copenhagen Munksgaard 1957

3.

Matson DD: Intracranial arterial aneurysms in childhood. J Neurosurg 23:5785831965Matson DD: Intracranial arterial aneurysms in childhood. J Neurosurg 23:578–583 1965

4.

Nishioka H: Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Section VII. Part 1. Evaluation of the conservative management of ruptured intracranial aneurysms. J Neurosurg 25:5745921966Nishioka H: Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Section VII. Part 1. Evaluation of the conservative management of ruptured intracranial aneurysms. J Neurosurg 25:574–592 1966

5.

Reifenstein GHLevine SAGross RE: Coarctation of the aorta: a review of 104 autopsied cases of “adult type,” 2 years of age or older. Amer Heart J 33:1461681947Reifenstein GH Levine SA Gross RE: Coarctation of the aorta: a review of 104 autopsied cases of “adult type” 2 years of age or older. Amer Heart J 33:146–168 1947

6.

Richardson A: Subarachnoid haemorrhage. Brit Med J 4:98921969Richardson A: Subarachnoid haemorrhage. Brit Med J 4:98–92 1969

7.

Thompson RAPribram HFW: Infantile cerebral aneurysm associated with ophthalmoplegia and quadriparesis. Neurology (Minneap) 19:7857891969Thompson RA Pribram HFW: Infantile cerebral aneurysm associated with ophthalmoplegia and quadriparesis. Neurology (Minneap) 19:785–789 1969

8.

Watson EHLowrey GH (eds): Growth and Development of Children. ChicagoYear Book Medical Publishers1962Watson EH Lowrey GH (eds): Growth and Development of Children. Chicago Year Book Medical Publishers 1962

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