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 infe ction, trisomy, or prenatal drug exposure. Objective. To investigate the underlying clinical conditions in patients wi th LSV. Materials and methods. Sixty-three cases of LSV were identified among appro ximately 2,400 neurosonograms performed over 42 months. All neurosonograms were reviewed. Medical records were reviewed for information regarding pren atal infection, chromosomal abnormality, respiratory and cardiac disease, a nd 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. T welve of these 33 had initially normal or minimal findings, with LSV develo ping or progressing on subsequent neurosonograms. The remaining 30 cases ha d varied clinical associations: congenital infection in 5, trisomies in 9, fetal substance exposure in 4, and unclear etiology in 12. Coexistent anoma lies included posterior urethral valves, MCDK, myelomeningocele, placental abruption, and others. Thirty patients were premature. Fifteen patients die d; histologic changes of LSV were found at autopsy in one patient. Conclusion. LSV has varied clinical associations. The common association wi th hypoxic/ischemic conditions and the progressive changes seen in 12 patie nts with cardiac and pulmonary disease suggest that postnatal hypoxia/ische mia is an important etiologic factor.