Importance of hypoxic/ischemic conditions in the development of cerebral lenticulostriate vasculopathy

Background We have observed many infants with lenticulostriate vasculopathy (LSV) on neurosonograms who do not have classic histories of prenatal infection, trisomy, or prenatal drug exposure. Objective. To investigate the underlying clinical conditions in patients with LSV. Materials and methods. Sixty-three cases of LSV were identified among approximately 2,400 neurosonograms performed over 42 months. All neurosonograms were reviewed. Medical records were reviewed for information regarding prenatal infection, chromosomal abnormality, respiratory and cardiac disease, and other pertinent diagnoses. Result. Hypoxic/ischemic conditions accounted for 33 cases: cardiac disease in 13, respiratory distress syndrome in 15, and perinatal asphyxia in 5. Twelve of these 33 had initially normal or minimal findings, with LSV developing or progressing on subsequent neurosonograms. The remaining 30 cases had varied clinical associations: congenital infection in 5, trisomies in 9, fetal substance exposure in 4, and unclear etiology in 12. Coexistent anomalies included posterior urethral valves, MCDK, myelomeningocele, placental abruption, and others. Thirty patients were premature. Fifteen patients died; histologic changes of LSV were found at autopsy in one patient. Conclusion. LSV has varied clinical associations. The common association with hypoxic/ischemic conditions and the progressive changes seen in 12 patients with cardiac and pulmonary disease suggest that postnatal hypoxia/ischemia is an important etiologic factor.