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Postoperative dural arteriovenous fistula in a patient with Cowden disease: a case report
Hirokazu Sadahiro1, Hideyuki Ishihara1, Hisaharu Goto1
1Department of Neurosurgery and Clinical Neuroscience, Yamaguchi University School of Medicine, Yamaguchi, Japan.
Insights
Cowden disease patients may develop dural arteriovenous fistulas (dAVFs) due to postoperative angiogenesis. This case highlights a potential link between Cowden disease, brain surgery, and the formation of new abnormal blood vessels.
Area of Science:
- Neurology
- Neurosurgery
- Vascular Malformations
Background:
- Cowden disease is a rare genetic disorder associated with an increased risk of various tumors and vascular abnormalities.
- Brainstem cavernous hemangiomas can cause significant neurological deficits due to rupture and hemorrhage.
Observation:
- A 37-year-old male with Cowden disease presented with neurological deficits from a ruptured brainstem cavernous hemangioma.
- Post-operatively, imaging revealed abnormal cerebellar vessels, leading to the diagnosis of a dural arteriovenous fistula (dAVF).
Findings:
- The dAVF involved the meningeal artery shunting to the inferior vermian vein with cortical reflux.
- Surgical findings indicated neovascularization and adhesion between the dura mater and cerebellar surface, suggesting angiogenesis as the cause of the fistula.
Implications:
- This case suggests that postoperative angiogenesis is a potential mechanism for dAVF formation in patients with Cowden disease.
- Further research is warranted to understand the specific pathways of angiogenesis in Cowden disease and its impact on vascular complications.
Abstract:
We report the case of a 37-year-old male with Cowden disease that caused a gradual neurological deficit because of rupture of a brain stem cavernous hemangioma. Removal of the hemangioma and hematoma was performed with an infrafacial triangle approach. Nine months after the operation, magnetic resonance imaging showed abnormal vessels on the cerebellar surface. Digital subtraction angiography showed a dural arteriovenous fistula (dAVF) from part of the meningeal artery to the ectatic inferior vermian vein with cortical reflux. After embolization, surgical obliteration of the dAVF was performed. Surgical findings showed neovascularization in the thickened dura, in which dural vessels shunted to cerebellar vessels through adhesion between the dura mater and cerebellar surface. Therefore, the thickened dura was removed with the cerebellar surface. This case suggests that postoperative angiogenesis may cause arteriovenous fistula in patients with Cowden disease.
