Objective:

We aim to better understand the clinical course of neonates with deep medullary venous thrombosis, an increasingly recognized entity in neonatal neurology.

Background:

Deep medullary venous thrombosis (DMVT) is characterized by the formation of blood clots within the deep medullary veins of the brain, often in neonates. Advances in MRI technology have improved detection of DMVT, but there is controversy about their etiology, clinical significance, and management.

Design/Methods:

We identified children with medullary venous thrombosis or congestion identified on MRI between 2008-2023 at Stanford Children’s Health. Charts were reviewed for pregnancy and delivery history, clinical presentation, MRI findings, management, and neurodevelopmental outcomes.

Results:

Our analysis included 64 infants with DMVT. Maternal, delivery, and neonatal comorbidities were common, including advanced maternal age (30%), pre-eclampsia/eclampsia (11%), C-section delivery (42%), hypoxic ischemic encephalopathy (22%), and congenital heart disease (22%). Most infants were neonates (median age at presentation 3 days, IQR 1-8 days). Infants presented with seizure (45%), hypotonia (20%), encephalopathy (38%), and respiratory distress (31%). Most DMVT were bilateral (69%) and often associated with parenchymal ischemic (50%) or hemorrhagic injury (36%). 15/64 patients received intervention with hyperhydration (8/15), anticoagulation (3/15), or both (4/15). 81% of patients survived to discharge, but neurologic morbidity at first follow-up visit was common, including seizures (49%), weakness (15%), spasticity (21%), hypotonia (38%), and developmental delay (55%).

Conclusions:

Symptomatic DVMT presents early in the neonatal period, commonly with seizures, encephalopathy, respiratory distress, and abnormal tone. Symptomatic DVMT is often bilateral and associated with concurrent parenchymal injury. The majority of infants in our cohort had associated comorbidities. Notably, our cohort had high rates of neurodevelopmental morbidity on follow up, underscoring the need for continued research into DMVT causes and treatments.