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

Abstract

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.

• Authors: S G Weig, P C Marshall, I F Abroms, N S Gauthier
• Journal: Pediatric neurology (Pediatr Neurol) Vol. 13 Issue 4 Pg. 279-85 (Nov 1995) ISSN: 0887-8994 [Print] United States
• PMID: 8771162 (Publication Type: Journal Article, Twin Study)

Topics

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