Hydrocephalus resulting from superior vena cava thrombosis in an infant. Case report

Area of Science:

• Neurology
• Pediatrics
• Vascular Medicine

Background:

• Superior vena cava (SVC) thrombosis is a rare condition in infants.
• SVC thrombosis can lead to increased intracranial venous pressure and neurological complications.
• Dural sinus hypertension is a potential consequence, with varied clinical presentations.

Purpose of the Study:

• To report an unusual case of SVC thrombosis with subsequent communicating hydrocephalus in an infant.
• To review and categorize previously reported cases of dural sinus hypertension secondary to SVC thrombosis.
• To elucidate the pathophysiology linking SVC thrombosis to hydrocephalus versus pseudotumor-like syndromes.

Main Methods:

• Case report of an infant with SVC thrombosis and hydrocephalus.
• Systematic review of existing literature on dural sinus hypertension in this context.
• Classification of patients into two groups based on clinical outcomes (hydrocephalus vs. pseudotumor-like syndrome).
• Analysis of intracranial venous pressure and patient demographics.

Main Results:

• The case presented with SVC thrombosis, communicating hydrocephalus, and elevated intracranial venous pressure.
• Reviewed cases were divided into hydrocephalus and pseudotumor-like syndrome groups.
• Patients developing hydrocephalus typically had generalized increased intracranial venous pressure.
• Patients with pseudotumor-like syndrome often had normal pressure in specific major intracranial venous structures.
• Absence of venous cushioning of the choroid plexus pulse wave is hypothesized as a cause for ventricular enlargement in the hydrocephalus group.
• Patients with larger ventricles were younger than those with smaller ventricles.

Conclusions:

• SVC thrombosis in infants can precipitate hydrocephalus, particularly when associated with generalized increased intracranial venous pressure.
• The proposed mechanism of impaired venous cushioning of the choroid plexus pulse wave may explain ventricular enlargement.
• Age appears to be a factor differentiating patient groups within the spectrum of dural sinus hypertension secondary to SVC thrombosis.