From the Research
Lenticulostriate vasculopathy (LSV) in children is a condition that requires a thorough evaluation to identify any underlying condition, and the management approach depends on the underlying cause and associated symptoms. LSV is primarily a radiological finding rather than a specific disease and often requires no specific treatment when found incidentally. The condition can be either congenital or acquired, with congenital causes including intrauterine infections (TORCH infections - toxoplasmosis, rubella, cytomegalovirus, herpes simplex), while acquired causes include hypoxic-ischemic injury, metabolic disorders, and genetic syndromes 1, 2.
When LSV is detected, a thorough evaluation should be conducted including:
- Detailed history
- Physical examination
- Appropriate laboratory tests to identify any underlying condition Neuroimaging follow-up may be necessary to monitor progression 3, 4.
The clinical significance of isolated LSV without symptoms remains controversial, but children with LSV should be monitored for neurodevelopmental outcomes, particularly if other risk factors are present 5. The mineralization process affecting these deep penetrating arteries may represent a response to vascular injury, though the exact pathophysiological mechanism remains incompletely understood. Prognosis varies widely depending on the underlying cause, with isolated LSV generally having a better outcome than LSV associated with congenital infections or other significant conditions.
It is essential to note that LSV can be a sign of central nervous system (CNS) involvement in infants with congenital cytomegalovirus (CMV) infection, and these infants are at high risk for sensorineural hearing loss 5. Therefore, infants with LSV should be screened for congenital CMV infection and monitored for hearing loss, and antiviral therapy should be considered in these cases. Large, prospective observational studies are needed to determine the incidence of LSV and the relative contribution of infectious and non-infectious conditions to LSV in the neonate 2.