Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins.
The prenatal histories, clinical courses, and neuroradiographic studies of 8 infants who had survived the in utero demise of a homozygous co-twin were reviewed. Three distinct modes of clinical presentation were found: (1) severe neonatal encephalopathy with seizures; (2) a more benign neonatal course with onset of seizures and profound developmental disabilities within the first 6 months of age; (3) late infantile presentation with seizures. Only the third group had milder outcomes. Neuroradiographic studies demonstrated two pathologic patterns: varying degrees of periventricular white matter infarction with migrational abnormalities observed with earlier demise of the co-twin, and multicystic encephalomalacia observed when demise occurred at or near term. Pathophysiology is uncertain and most likely multifactorial. Exsanguination injury to the survivor can occur acutely following co-twin demise, so urgent delivery may be appropriate at or near term.
Weig
S G
SG
Department of Pediatrics, University of Massachusetts Medical School, Worcester 01655, USA.
Marshall
P C
PC
Abroms
I F
IF
Gauthier
N S
NS
D001927:Brain Diseases / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005313:Fetal Death; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D011247:Pregnancy; D012640:Seizures / Q000175:diagnosis / Q000209:etiology*; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014430:Twins, Monozygotic; D014652:Vascular Diseases / Q000175:diagnosis / Q000209:etiology*