Unilateral megalencephaly associated with neonatal high output cardiac failure.
High-flow cerebral arteriovenous fistulae are well known to present in the neonatal period with macrocephaly, cranial bruits and high-output cardiac failure. This report describes a newborn infant with such a clinical presentation, who had unilateral megalencephaly without macroscopic arteriovenous shunts. Ultrasound Doppler examination of the carotid and cerebral vessels showed diastolic flow, or a decreased pulsatility index, consistent with decreased intracranial vascular resistance. The ipsilateral cerebral arteries and veins were markedly enlarged at angiography and at post-mortem examination, but there was no arteriovenous malformation. The infant expired from high-output cardiac failure and hypoglycemia. It is postulated that the high-output cardiac failure was due to increased blood flow through the enlarged, dysplastic cerebral hemisphere.
Walters
B C
BC
Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.
Burrows
P E
PE
Musewe
N
N
Chuang
S H
SH
Armstrong
D
D
D001921:Brain / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002303:Cardiac Output, Low / Q000150:complications*; D006332:Cardiomegaly / Q000150:complications; D002533:Cerebral Angiography; D002560:Cerebrovascular Circulation; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed