Is lenticulostriated vasculopathy a sign of central nervous system insult in infants with congenital CMV infection?

Background: In previous studies, lenticulostriated vasculopathy (LSV) was detected in 0.4-5.8% of neonates who had undergone brain ultrasound studies during the neonatal period. Most infants were referred from neonatal intensive cAre units. Various clinical conditions were associated with LSV including intrauterine infections. Objective: To investigate whether LSV as a single abnormal finding in neonates with congenital cytomegalovirus (CMV) infection is a sign of central nervous system (CNS) involvement. Methods: Ultrasonographic and clinical data of all infants with congenital CMV infection, followed in our hospital, were collected. All infants with symptomatic congenital CMV infection and CNS involvement were treated with ganciclovir for 6 weeks, followed by valganciclovir until the age of 1 year. Infants with asymptomatic as well as symptomatic infections were followed up with brainstem evoked response and behavioural studies every 4 months until 4 years of age. Results: 92 infants diagnosed with congenital CMV infection were included in the study. In 50 (54.3%) infants, LSV was detected on initial brain ultrasound. Among these patients, 21 (42%) infants had other ultrasonographic findings consistent with congenital CMV infection; 11 (22%) had other symptoms of CNS involvement and in 18 (36%) cases the only abnormal finding was LSV. In 9 of the 18 infants with LSV as the only finding on initial examination, antiviral therapy was not started. Hearing deterioration developed in all nine infants between ages 4 and 34 months. Subsequent to these cases, the authors modified their therapy protocol and began treating congenital CMV infants with only LSV. 9 infants were treated and all maintained normal hearing after 8-27 months of follow-up (p<0.01). Conclusions: LSV is a common finding in infants with symptomatic congenital CMV infection and is a sign of CNS involvement. Moreover, LSV is a possible marker of high risk for sensorineural hearing loss in infants with congenital CMV infection.