Patterns of cerebral injury and clinical presentation in the vascular disruptive syndrome of monozygotic twins

Pediatr Neurol. 1995 Nov;13(4):279-85. doi: 10.1016/0887-8994(95)00219-7.


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.

Publication types

  • Twin Study

MeSH terms

  • Brain Diseases / diagnosis
  • Brain Diseases / etiology*
  • Female
  • Fetal Death*
  • Humans
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Pregnancy
  • Seizures / diagnosis
  • Seizures / etiology*
  • Syndrome
  • Tomography, X-Ray Computed
  • Twins, Monozygotic*
  • Vascular Diseases / diagnosis
  • Vascular Diseases / etiology*