PEDIATRICS Vol. 78 No. 1 July 1986, pp. 85-87
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Congenital Microcephaly Due to Vascular Disruption: In Utero Documentation

Helen E. Hughes MB, BCh1 and Murray Miskin MD1

1 From the Departments of Pediatrics and Genetics, The Hospital for Sick Children, and Department of Radiology, University of Toronto, Toronto

Death in utero of one member of a monozygotic twin pair has been associated with vascular disruptive phenomena in the surviving twin. It has been hypothesized that this event initiates clot formation in the surviving twin with consequent necrosis of tissues distal to the occluded vessels. This case report describes onset in utero of multicystic encephalomalacia and microcephaly in a surviving twin whose brain appeared normal on ultrasound scanning before death of the cotwin at 21 weeks' gestation. The case provides further support for the hypothesized pathogenetic sequence and illustrates the importance of reviewing all prenatal ultrasound scans in infants with congenital microcephaly.

Key Words: microcephaly • vascular disruption • multicystic encephalomalacia • twins

Submitted on April 15, 1985
Accepted on August 20, 1985




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