RUPTURE
OF
ANEURYSM
OF
THE
CIRCLE
OF
WILLIS
IN
THE
NEWBORN
By ALVAH L. NEWCOMB, M.D., AND GEORGE F. MUNNS, M.D.
W’innetka, Ill.
CUTE spontaneous cerebral vascular accidents in infants are extremely rare. Despite
voluminous literature on cerebral aneurysm, no case in the newborn period has
been found.
The earliest recorded instance’ was a patient with congenital polycystic disease of the
kidneys who died at 1 3 weeks of age. The aneurysm was an evagination from the ventral
wall of the basilar artery, one mm. in diameter, at the level of the bifurcation into the
two posterior cerebral arteries.
A history of mild trauma may precede the rupture of an aneurysm. A 23 day old male
infant was admitted to Childrens’ Memorial Hospital after an accidental blow on the
head from the elbow of his mother. He became cyanotic rapidly, developed rapid
breathing and died four hours after admission. Postmortem subdural tap and lumbar
puncture yielded bloody fluid. Autopsy revealed a ruptured aneurysm arising from the
circle of Willis. The exact location was not determined. The aneurysmal sac was divided
into four fairly well demarcated layers. There was no smooth muscle in the wall. Other
in-stances of mild trauma producing rupture were the 41/a year old boy, reported by
Herman,’ who “headed” a light rubber toy ball, and the 6 year old boy with
pneumo-coccal meningitis, reported by Sullivan,’ who died from a ruptured basilar artery. Talbot,t
in 1919, reported the fatal rupture in a
9#{189}
year old boy of an aneurysm of thebegin-ning of the left cerebral artery. (This patient had violent spasms of coughing from
pertussis.)
Severe exercise or injury was apparently responsible for two other ruptures. A 12
year old boy reported by Schreiber,’ collapsed after rounding the bases in a ball game
and died five days later of a fatal subarachnoidal hemorrhage. Dial and Maurer’ record
the rupture of an aneurysm of the right vertebral artery in a 2 year old who had
appar-ently recovered from a skull fracture. After discharge from the hospital on the 7th day,
he became irritable and developed convulsions, dyspnoea and cyanosis. Death occurred
36 days after the accident.
Many excellent articles have appeared in the literature since Fearnsides’7 classical
paper in 1916. McDonald and Karb8 have reviewed the literature and charted the location
of 1,125 verified cases of ruptured intracranial aneurysms.
Martland#{176} charted his cases and reported that 2% of all sudden and unexplained
deaths encountered by medical examiners were due to spontaneous subarachnoid
hemor-rhage; that 70% of these were due to ruptured “berry” aneursyms, and that associated
congenital defects such as polycystic disease of the kidneys and liver support the theory
of congenital origin. Bremer,’#{176} from a thorough embryologic study, showed that “berry”
Ant.
Mid
Ant.
c&otid
Right
Lcft
Fig.
I
770 ALVAH L. NEWCOMB AND GEORGE F. MUNNS
rysmal pouches. Other aneurysms may develop at the fork of the vessels. If these forks lack the media, the rapid spreading and widening may produce local aneurysms. Both
types
may be true congenital aneurysms.Hodes, Perryman and Chamberlain,” in a report on cerebral angiography, accept Bremer’s thesis that most “berry” aneurysms (80%
)
are congenital. These arise at thesite of primitive embryologic vessels which have completely absorbed, leaving “nubbins”
on the arterial trunk which later enlarge and become aneursysmal.
Richardson and Hyland,” referring to Forbus’ work1’ in 1930, believe that the medial
defects are probably developmental. They have described the syndrome well.
Forbus demonstrated congenital defects in the cerebral, coronary, mesenteric and
pul-monary arteries. He recently stated14 that an aneurysm does not date from embryonic life,
but develops after birth and is therefore acquired.
The characteristic syndrome as described in the adult consists of sudden headache,
fol-lowed quickly by an apopleptic seizure and sudden loss of consciousness and deep coma.
The spinal fluid is invariably bloody. Localized findings, such as pressure symptoms
pro-duced by aneurysm affecting the intracranial nerves, are well described by Hodes, Perryman
and Chamberlain.”
CASE REPORT
These observations are on twins, born in Evanston Hospital, who died in the first few days of
life. The smaller and younger, a female weighing 2400 gm., died 31 hrs. after birth. Unfortunately,
a complete autopsy was not done. Atelectasis of both lungs was found, but the brain was not examined.
The male, weighing 2850 gm., died 64 hrs. after birth. The infants presented identical symptoms,
except that those of the smaller female were found more severe at birth and were more rapidly
progressive.
Delivery was normal. Duration of labor in the first stage was 5 hrs., 1 5 mm. Premedication was
demerol, 100 mg., and scopolamine, 0.4 mg. Nitrous oxide was administered for 30 mm. Delivery
was by low forceps.
Both infants were cyanotic at birth and were revived with difficulty. Cyanosis was partially relieved
by oxygen. The female was immediately placed in an incubator with a high content of oxygen. The male was given oxygen by mask.
At the time of the first examination, an expiratory grunt was present in both infants together with
suprasternal and diaphragmatic retraction. Breath sounds were clear anteriorly in the male, but suppressed or faint in the female and heard distinctly only in the left axillary region. In addition, the
male at times had manifested a high pitched cry. There was no bulging of the fontanelles or rigidity
of the neck.
The fatal course was more rapid in the female who became intensely cyanotic when out of oxygen.
The boy appeared to improve for 48 hrs. and then became progressively weaker, pale and dyspneic until death.
Complete autopsy on the male revealed:
772 ALVAH L. NEWCOMB AND GEORGE F. MUNNS
of the hemorrhage which was the greatest in the contiguous portions of the arachnoid
space. No other definite lesion was found.
Lungs-Lungs revealed primary atelectasis and bilateral bronchopneumonia.
SUMMARY
A case of spontaneous cerebral hemorrhage from an aneurysm of the circle of Willis
is reported in the male of newborn twins. The smaller female twin died earlier with
similar symptoms and signs, but no intracranial autopsy was performed.
REFERENCES
1. Forster, F. M., and Alpers, B. J., Aneurysm of circle of Willis associated with congenital poly-cystic disease of kidneys, Arch. Neurol. & Psychiat. 50:669, 1943.
2. Herman, K., Cerebral hemorrhage from rupture of congenital intracranial aneurysm in child, Brit.M.J. 1:323, 1943.
3. Sullivan, R. B., Case Report, Clinical Proceedings of the Children’s Hospital, Washington, D.C.,
2:97, 1946.
4. Talbot, F. B., in Contributions to Medical and Biological Research, New York, Paul B. Hoeber,
Inc., 1919, vol. 2, p. 1004.
5. Schreiber, F., Aneurysm of circle of Willis, Harper Hosp. Bull. 3:68, 1945.
6. Dial, D. L., and Maurer, G. B., Intracranial aneurysms, Am. J. Surg. 35: 1, 1937.
7. Fearnsides, E. G., Intracranial aneurysms, Brain 39:224, 1916
8. McDonald, C. A., and Karb, M., Intracranial aneurysms, Arch. Neurol. & Psychiat. 42:298, 1939.
9. Martland, H. S., Spontaneous subarachnoid hemorrhage and congenital “berry” aneurysms of
circleof Willis, Am. J. Surg. 43:10, 1939.
10. Bremer, J. L., Congenital aneurysms of cerebral arteries; embryologic study, Arch. Path. 35:819, 1943.
11. Hodes, P. J., Perryman, C. R., and Chamberlain, R. R., Cerebral angiography, Am. J. Roentgenol.
58:543, 1947.
12. Richardson, J. C., and Hyland, H. H., Intracranial aneurysms, Medicine 20:1, 1941.
13. Forbus, W. D., Origin of miliary aneurysms of superficial cerebral arteries, Bull. Johns Hopkins
Hosp. 47:239, 1930.
14. Forbus, W. D., Etiology and pathogenesis of cerebral aneurysms, Chicago M. Soc. Bull. 50:227, 1947.
SPANISH ABSTRACT
Ruptura de Aneurisma del Circulo de Willis en el Recien-nacido
Se presenta un caso de ruptura de aneurisma del cIrculo de Willis que ocurri#{243}en el recien-nacido.
Tales accidentes vasculares espontaneos son extremadamente raros; no se ha encontrado relato de un talfen#{243}meno.
Este caso ocurri#{243} en el varon de un par de gemelos, ambos de los cuales murieron dentro de 31
hora despu#{233}s del nacimiento, cada uno de los gemelos present#{243} smntomas id#{233}nticos. Desgraciadamente no se hizo Ia autopsia de Ia cabeza de Ia hembrita gemela. La que se Ic hizo al varoncito gemelo
revel#{243}un aneurisma roto en el punto de union de la arteria cerebral posterior y el ramo comunicante posterior a la derecha.
Este caso ayuda a coroborar Ia opinion de que Ia mayor parte de los aneurismas craneales “berry” son de origen cong#{233}nito.
