S. Imaizumi et al., Intracerebral haematomas with agenesis of the internal carotid artery and tetralogy of fallot, ACT NEUROCH, 143(6), 2001, pp. 625-629
We report a rare case with tetralogy of Fallot (TOF) and agenesis of the in
ternal carotid artery (ICA) who presented serious intracerebral haematomas.
In the literature. this is the first documented case having these complica
tions simultaneously. Extreme hypoxic insults followed by recovery were det
ected by O-2 saturation monitor before two bleeds. Chronic brain hypoxia co
uld make the vasculature weak, which was shown in the histological examinat
ion.
A 2-year-old girl was transferred to us with a general convulsion due to in
tracerebral haematoma. She had been showing general cyanosis from birth due
to TOF. Repeated intracerebral haemorrhages ended her life. Histological s
tudy showed dilated vascular channels in the subarachnoid space and necroti
zing vasculature obstructed by fibrinous thrombi adjacent to the haematoma.
Fibrosis of the vessel wall with infiltration of macrophages suggested sub
acute or chronic lesions rather than acute necrosis due to the multiple hae
morrhages. The intracerebral haematomas and agenesis of the ICA were observ
ed as unilateral hemispheric vascular complications of TOF. Chronic brain h
ypoxia could play an important role in weakening the vessel wall and erythr
ocytosis caused obstructing thrombi. We speculate these factors generated t
he intracerebral.