It was traditionally thought to result from the passage of thromboplastic material into the circulation of the surviving twin which causes ischemic structural defects in various organs (particularly the highly vascularised organs such as the central nervous system, gastrointestinal tract, and genitourinary system). Acute haemodynamic shift from live to dead fetus resulting in hypoperfusion is more recently thought to play a role 3.
There is usually an underlying twin-twin transfusion syndrome as a causative association.
The presence of a dead twin associated with a surviving twin with various anomalies may suggest the diagnosis. Such anomalies include :
Non CNS anomalies
Treatment and prognosis
While it carries a poor outcome, prenatal sonographic diagnosis may influence antenatal neonatal management of the surviving monozygous twin 1.
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