Spontaneous Regression Of An Unruptured Arteriovenous Malformation Due To Drainer Vein Thrombosis In A Patient With Protein S Deficiency: A Case Report And Literature Review
Noda, R., Akabane, A., Kawashima, M., Segawa, M., Tsunoda, S., & Inoue, T. (2023). Spontaneous Regression of an Unruptured Arteriovenous Malformation Due to Drainer Vein Thrombosis in a Patient with Protein S Deficiency: A Case Report and Literature Review. NMC case report journal, 10, 221–226. https://doi.org/10.2176/jns-nmc.2023-0056
View Original Source →Abstract
Spontaneous regression of an arteriovenous malformation (AVM) is a rare condition observed in 0.3%-1.3% of patients with AVMs and is most likely caused by hemorrhagic events. The regression of an unruptured AVM is rarer than that of a ruptured AVM. Moreover, due to its low frequency of occurrence, the etiology and natural course of spontaneous regression of an AVM is still unclear. This is the first report presenting a case of a spontaneous regression of an unruptured AVM caused by a gradual drainer vein thrombosis that was suspected to result from hypercoagulability due to protein S deficiency.
Case Details
Disease Location
Brain blood vessels
Personal Characteristics
33-year-old woman
Clinical Characteristics
Diagnosed with an unruptured avm on the fronto-parietal lobe with a nidus size of 43 mm on both internal carotid and vertebral angiograms. A flow-related aneurysm was located at the bifurcation of the right pericallosal artery. One year after the first MRI, a follow-up MRI revealed perifocal edema of the nidus with an intravenous thrombosis of the draining vein. The feeding artery, which is the enlarged anterior cerebral artery, and the dilated vein of galen showed reduced diameters. Three years later, signs of another thrombosis were detected with increased brain edema. The nidus, feeding artery, and draining vein also showed a regression in size. Five years later, MRI revealed signs of an additional thrombotic event without an in- crease in brain edema. At this time, the feeding artery and draining vein had normalized in size. Eight years after the initial presentation, the patient developed an epileptic seizure evaluation for the hypercoagulable state revealed pro- tein s deficiency
Remission Characteristics
The right internal carotid angiogram and both ver- tebral angiograms revealed complete obliteration of the avm, while the left internal carotid angiogram showed a small residual nidus in the capillary and venous phases and a decrease in the size of the flow-related aneurysm
Treatment & Mechanisms
Proposed Remission Mechanisms
Protein s deficiency could have contributed to avm regres- sion by causing thrombosis of the draining vein
Clinical Treatment
None reported
Non-Clinical Treatment
None reported