Symptomatic Infratentorial Thrombosed Developmental Venous Anomaly: Case Report and Review of the Literature

Abstract

Background: Developmental venous anomalies (DVAs) are variations of normal transmedullary veins draining white and gray matter. In the vast majority of cases, DVAs are diagnosed incidentally and should be considered as benign entities. In extremely rare circumstances, DVAs may become symptomatic due to mechanical or flow-related etiologies. Thrombosis of the collector vein of a DVA is a rare type of a flow-related complication with only 29 cases reported in the literature, the majority of which are supratentorial. Infratentorial thrombosed DVAs are thus extremely rare and the few cases reported have typically caused symptoms due to venous ischemic infarctions. Summary: We report a case of an infratentorial DVA with a thrombosed drainage vein in a patient with nonhemorrhagic, noninfarcted venous congestive edema, which was successfully treated with high-dose glucocorticoids and short-term anticoagulation. We review the pertinent venous anatomy of the posterior fossa as well as the literature of symptomatic infratentorial thrombosed DVAs. Key Message: The presented case of an infratentorial thrombosed DVA with cerebellar and pontine venous congestive edema is extremely rare. A working knowledge of posterior fossa venous anatomy and possible pathomechanisms responsible for the rarely symptomatic lesion will aid in the timely and efficacious treatment of such lesions.

Cerebral Cavernous Malformations, Developmental Venous Anomaly, and Its Coexistence: A Review

“…DVAs that are asymptomatic or found incidentally are not treated. Though no large-scale controlled studies exist to support use of anticoagulants, it has been recommended in symptomatic, thrombosed venous angioma after extensive evaluation of affected patients for additional hemorrhagic risks [48]. Surgical excision of a DVA can result in venous congestion, leading to severe venous infarction thus favoring conservative man- agement.…”

“…DVAs have an incidence rate of >2% based on both autopsy and imaging studies [47]. The hemorrhagic risk of DVA is considered <0.7% and has been seen more in patients having lesions in the posterior fossa or in pregnant patients [48].…”

Case report: Successful thromboprophylaxis with enoxaparin in a pregnant woman with internal jugular vein agenesis

“…From a hemodynamic point of view, it is associated with low-flow and low-resistance veins, and it therefore confers a low risk of complications such as rupture or microthrombosis ( 9 ). With the increased use of MRI, microthromboses are more frequently being diagnosed ( 10 , 11 ), as is reflected in the literature ( 12 – 14 ). However, when these abnormalities are associated with other prothrombotic conditions such as hormone intake, pregnancy, and acute medical illness, the potential risk for thrombotic complications in the cerebral vessels increases, as was recently demonstrated by the association between cerebral venous thrombosis and anti–SARS-CoV-2 vaccines ( 15 , 16 ).…”

Hemifacial spasm caused by the brainstem developmental venous anomaly: A case report and review of the literature

“…Pathophysiological mechanisms underlying the clinical manifestations of symptomatic DVAs are divided into idiopathic, flow-related (misbalance of the in-and outflow of blood), and mechanical causes (direct compression of neural structures). [2,3,7,9,11,[14][15][16]18,19,22,24,26,32] Rare cases of DVA causing mechanical compression of the aqueduct with obstructive hydrocephalus were described and treated with ventriculoperitoneal shunting, aqueductal stenting, and endoscopic third ventriculoscopy. [12] e large venous collector of a DVA can compress the cranial nerves and cause different clinical presentations.…”