Cerebral Venous Sinus Thrombosis in a Newborn: A Unique Case of Sinus Confluence Thrombosis with Cranial Nerve and Ophthalmological Involvement and Literature Review.
Elliot Gabriel Gama Reyes1, Javier Andrés Galnares Olalde1, Ma. Fernanda Castillo Vaca2, Sydney Abisag Sandoval Nava3
1Headache Clinic, 2Radio-neurosurgery, National Institute of Neurology and Neurosurgery, Mexico, 3Medical Unit, National System for the Integral Development of the Family (DIF), Mexico
Objective:
To analyze the unique clinical presentation, ophthalmological manifestations, diagnostic challenges, and management of neonatal CVST with cranial nerve involvement through a case report and literature review.
Background:
Cerebral venous sinus thrombosis (CVST) affects 6.4 in 100,000 neonates and is associated with Virchow's triad: endothelial damage, blood stasis, and hypercoagulability. Prothrombotic conditions, such as inherited thrombophilias (e.g., factor V Leiden, protein C/S deficiency, and antithrombin III deficiency), increase the risk. CVST typically presents as neonatal encephalopathy with altered alertness, seizures, respiratory distress, and decreased reflexes. Non-specific signs include weight loss, dehydration, irritability, and bulging fontanelles. Diagnosis requires a comprehensive neurological examination with MRI preferred over CT because of the exposure to ionizing radiation. Anticoagulation in the acute phase is considered reasonable due to the lack of post-treatment hemorrhage, while the absence of anticoagulation may lead to thrombus propagation. Long-term complications include epilepsy, motor deficits, and neurodevelopmental disorders.
Results:
A term male newborn (9 days old) without significant perinatal risk factors was initially admitted for hyperbilirubinemia and later developed septic shock with focal neurological signs. Examination revealed fontanelle collapse, poor reflexes, dry mucous membranes, right ptosis, abnormal pupillary reflexes, exotropia, and corneal reflex loss. Additional signs later appeared, including facial asymmetry, mydriasis, limited eye movements, and nystagmus. Despite negative CSF and blood cultures, a CT scan showed occipital edema, white matter changes, and sinus confluence thrombosis. Laboratory tests for inherited thrombophilias were negative and the patient was treated with enoxaparin before transitioning to rivaroxaban for discharge. Severe visual impairment was later diagnosed, and the patient remains under observation for further neurological sequelae.
Conclusions:
This case highlights the critical importance of recognizing CVST with focal neuro-ophthalmological signs. Early anticoagulation and close monitoring are essential to prevent complications and improve outcomes in neonatal CVST.
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