Lenticulostriate vasculopathy in neonates: An imaging marker with heterogeneous clinical significance

Lenticulostriate vasculopathy (LSV) is a relatively common cranial ultrasound finding in neonates, characterized by linear or branching hyperechogenic striations within the basal ganglia and/or thalamus along the lenticulostriate arteries. Although initially associated with congenital infections, particularly cytomegalovirus (CMV), LSV is now recognized in a broad range of clinical settings, and its prognostic significance remains controversial. We report 3 neonatal cases illustrating the heterogeneous contexts in which LSV may occur. The first case was a term male neonate with transient respiratory distress and normal neurological examination; cranial ultrasound on day 3 showed unilateral right-sided fine linear striations in the basal ganglia without parenchymal abnormalities. Infectious workup was negative and outcome was favorable. The second case involved a 36-week male neonate born after acute fetal distress, with severe respiratory distress requiring resuscitation. Cranial ultrasound demonstrated unilateral left-sided LSV without hemorrhagic or hypoxic-ischemic lesions; subsequent brain MRI was normal, with favorable follow-up and slight regression of the ultrasound abnormalities. The third case was a term female neonate with prolonged jaundice, hypotonia, hepatosplenomegaly, thrombocytopenia, and abnormal liver function tests. Cranial ultrasound revealed bilateral branching striations, and congenital CMV infection was confirmed by urine PCR. These cases highlight the nonspecific nature of LSV, which may occur as an isolated finding, with perinatal hypoxic or hemodynamic disturbances, or with congenital infection. Recognition of LSV should be interpreted in the clinical context, with targeted investigation when abnormalities are present, while isolated forms may have a favorable outcome.