The mechanisms by which the anatomical variations of the circle of Wil
lis develop is considered to be related to haemodynamic factors, i.e.
the differential growth of the Various parts of the brain will continu
ously change the haemodynamic demands and consequently the flow patter
ns in the cerebral arteries. It is therefore to be expected that, if a
selected part of the brain does not develop, the change in the haemod
ynamic demand will affect the development of some cerebral arteries. C
onsequently the arteries at the base of 2 arhinencephalic and 8 holopr
osencephalic brains were studied in conjunction with the brain malform
ations. The defects of holoprosencephaly are believed to arise from a
failure of the prosencephalon to separate fully into the telencephalon
and diencephalon and become manifest at the time that the prosencepha
lon normally starts to separate into the hemispheres, i.e. 28-34 d p.c
. Arhinencephalic brains are fully diverticulated. There is only a par
tial or complete agenesis of the olfactory tracts and bulbs. The defec
t causing arhinencephaly starts at 43 d p.c. In the arhinencephalic br
ains no particular vascular abnormalities were found. However, at the
base of the holoprosencephalic brains no complete circle of Willis was
present; the anterior part was lacking and was replaced by anterior b
ranches which emerged unilaterally or bilaterally from the internal ca
rotid artery. The choroidal arteries were of very large calibre and ra
n to the highly vascularised wall of the dorsal cyst which is usually
present in holoprosencephalic brains. In contrast to the anterior part
, the posterior arterial pattern was almost identical to the posterior
part of the circle of Willis of normal brains. The basic vascular pat
terns found in the holoprosencephalic brains displayed the features of
Padget's developmental stages 2 and 3 of the cerebral vasculature, i.
e the pattern that has normally developed within 28-40 d p.c. The furt
her modification of this pattern could largely be understood from the
functional demand imposed on the circulation by the enlarged anterior
choroidal arteries. Because the development of the anterior part of th
e circle of Willis precedes the developmental derangement causing arhi
nencephaly, a complete circle was found in these brains.