Coexisting Hemorrhagic Cerebral Cavernous Malformation and Developmental Venous Anomaly Resulting in Frontal Lobe Seizures: A Case Report

Abstract

Cerebral cavernous malformations (CCMs) are vascular malformations that can cause seizures and hemorrhages. Managing CCMs associated with developmental venous anomalies (DVAs) becomes more complex due to the risk of venous infarction and hemorrhage if the DVA is disrupted. This case report describes a 55-year-old man presenting with intractable seizures who was found to have a hemorrhagic CCM and an adjacent DVA in the frontal lobe. Surgical management involved complete removal of the CCM while preserving the DVA. Postoperatively, the patient had no further seizures, highlighting the importance of careful surgical planning and execution in managing coexisting CCMs and DVAs.

Keywords

hemangioma - cavernous - central nervous system - hemorrhage - seizures

Introduction

Cerebral cavernous malformations (CCMs) are vascular anomalies characterized by abnormally dilated capillaries that lack intervening brain parenchyma,[1] with an overall prevalence of 0.46%.[2] Although often asymptomatic, CCMs located in the supratentorial areas may present with intractable seizures.[3] Developmental venous anomalies (DVAs) are the most common congenital cerebral vascular malformations, having an incidence rate of 0.05 to 2.56%, and are typically asymptomatic unless associated with other vascular lesions, such as CCMs or thrombosis of the collecting vein.[4] [5] [6] The coexistence of CCMs and DVAs is the most common form of mixed intracranial vascular malformation.[7] However, there has been little information about the management of symptomatic CCMs coexisting with DVAs.[8] [9] [10] This case contributes to the limited literature on managing a rare condition, hemorrhagic CCM, associated with DVA, and highlights the surgical strategy.

Case Report

A 55-year-old man with no significant past medical history presented to our hospital after two consecutive convulsive episodes, followed by confusion and agitation. A brain computed tomography (CT) revealed a right frontal lobe hemorrhage. His cerebral CT angiography did not reveal any aneurysm or arteriovenous malformation. One week later, the patient experienced a cluster of five seizures accompanied by cognitive impairment, including two generalized seizures and three left-sided tonic seizures. Brain magnetic resonance imaging (MRI) findings were suggestive of a right frontal subacute hemorrhagic cavernoma and an adjacent intact DVA ([Fig. 1]). His electroencephalogram showed intermittent delta activity and spikes in the right frontal lobe. A diagnosis of right frontal epilepsy associated with cerebral cavernoma was made. The patient was discharged on oral valproate (1000 mg) and levetiracetam (500 mg) twice daily. Shortly after discharge, he had a 5-minute episode of confusion, followed by uncontrollable aggressive behavior, including an attempt to climb out of a 32nd-floor window. Because of dangerous behavior during the seizures, he decided to undergo microsurgical resection of the right frontal cavernoma. Intraoperatively, a DVA adjacent to the cavernoma was confirmed, but there is no direct connection between the DVA and the CCM. A resection of the cavernoma and the surrounding hemosiderin was performed while preserving the DVA ([Fig. 2]). The postoperative course was uncomplicated. Pathological examination confirmed the presence of a 1.2 × 1.0 × 0.8 cm type IB cavernous malformation ([Fig. 3]). The patient was seizure-free at the last follow-up, 9 months after the surgery. The status of the DVA after the surgery could not be evaluated because he refused to obtain a brain MRI control.

Fig. 1 Contrast-enhanced brain magnetic resonance imaging (MRI) demonstrates a well-defined lesion in the cortico-subcortical region of the right middle frontal gyrus (red arrow). The lesion exhibits a hypointense core with a peripheral rim of marked hypointensity on coronal T2-weighted imaging (A), a hyperintense rim on FLAIR (B), and a hyperintense signal on T1-weighted imaging (C). On SWI, (D1)shows a pronounced blooming artifact (red arrow) corresponding to the hemosiderin rim of the cavernoma, while (D2) highlights both the cavernoma (red arrow) and an adjacent venous anomaly (blue arrow). No enhancement is seen post-contrast (E1, E2). The venous anomaly (blue arrow) is evident on both SWI (C2) and post-contrast T1-weighted images (E1, E2). These findings are consistent with a subacute hemorrhagic cavernoma associated with a developmental venous anomaly.

Fig. 2 (A) Intraoperative view of the cavernoma (*) with a cortical vein (**). (B) The resected cavernoma. (C) Intraoperative view after the removal of the cavernoma, with the cortical vein connecting to the developmental venous anomaly (DVA) (**) and DVA (*) left intact.

Fig. 3 Histopathology of the resected cavernoma. (A) At low magnification, section show abnormality dialed and hyalinized capillary (cavern) without intervening brain tissue with erythrocytes (**) in the lumen, thick (*) and thin (arrow) wall (Hex4). (B) At high magnification, old hemorrhage with hemosiderin deposits is seen along the vessel wall (arrow) and an absence of a smooth muscle wall layer (Hex40).

Discussion

CCMs are typically low-pressure vascular malformations and are mostly asymptomatic, but they can be prone to repeated microhemorrhages, which may lead to the gradual onset of neurological symptoms, and seizure is the most common one.[3] [11] These seizures may result from irritation of the surrounding cortex caused by microhemorrhages or hemosiderin deposition.[12] In the present case, the patient initially experienced a combination of generalized and focal tonic seizures, a common presentation of cavernous malformations located in the frontal lobe, a seizure-prone region.[13] The decision to surgically resect the CCM was based on the patient's recurrent seizures and hemorrhages. Surgical intervention is recommended for symptomatic CCMs, particularly in patients with intractable seizures. Studies indicate that CCMs in eloquent brain areas, especially those causing hemorrhages or seizures, benefit from surgical removal to prevent further neurological deterioration.[12] In the present case, however, the presence of a DVA complicated both the decision-making process and the procedure. DVAs are the most common type of cerebral vascular malformation and are considered benign congenital anomalies. DVAs consist of dilated medullary veins that drain normal brain tissue into a central venous trunk. Unlike other vascular malformations, DVAs usually do not cause significant clinical symptoms unless they coexist with other lesions, such as cavernous malformations or arteriovenous malformations.[14] DVAs are often discovered incidentally on imaging studies conducted for unrelated reasons or, in some cases, identified intraoperatively. The coexistence of DVAs and CCMs is relatively common, with studies suggesting that DVAs may predispose individuals to develop adjacent cavernous malformations. The underlying pathophysiology remains unclear; however, some hypotheses suggest that abnormal venous drainage associated with DVAs creates a low-pressure environment that is conducive to cavernoma formation. Hemorrhagic events are more likely in patients with both DVAs and CCMs than in those with isolated DVAs.[15] When DVAs and CCMs coexist, management decisions become more complex. Surgical resection of a symptomatic CCM is often necessary, but preserving the DVA is crucial to avoid venous complications. Surgical intervention is rarely required for isolated DVAs, as these structures play an essential role in draining surrounding brain tissue. Disrupting a DVA during surgery can result in venous infarction, edema, or hemorrhage.[15] Therefore, preoperative imaging for CCMs should aim to identify any adjacent or concomitant DVAs, delineate the anatomy of both lesions, and guide the surgical approach to minimize the risk of damaging the DVA.[12] In some cases, however, the DVA is only identified during the operation. This highlights the importance of meticulous dissection in neurovascular surgery, as unanticipated critical structures or lesions are uncommon. For seizure control, some authors supported extended lesionectomy for patients with CCMs to have better outcomes.[16] However, in the present case, management is more challenging. An extended lesionectomy of the CCM would require a DVA resection, which may result in the risk of cerebral infarction postoperatively. Therefore, we decided to remove the cavernoma with the surrounding hemosiderin and preserve the DVA. Intraoperatively, we found no direct connection between the DVA and the seizure-inducing CCM. This condition is different from previous reports, which showed a connection between the CCM and DVA.[10]

The short-term seizure outcome in this patient is good. However, long-term postoperative follow-up is essential, as recurrent hemorrhages or seizures may occur. Serial MRI monitoring and neurological assessments are recommended to ensure no new lesions develop and the existing DVA remains stable.[15] Given the complexity of this case, the patient's seizure-free postoperative course highlights the effectiveness of surgical intervention when a balance is struck between resecting the symptomatic lesion and preserving the critical venous drainage provided by DVAs. While there is little information about the surgical management of epileptic patients having CCMs coexisting with DVAs, the present case adds to the limited medical literature guiding the management of symptomatic coexisting CCM and DVA patients. It may be helpful for the readers. In our opinion, resection of CCM and surrounding hemosiderin while preserving the adjacent DVA might be an appropriate option.

Conclusion

The coexistence of a CCM and DVA presents significant management challenges, particularly in epileptic patients. In the present case, successful management was achieved through careful surgical resection of the CCM and the hemosiderin ring while preserving the DVA. This highlights the importance of preserving venous drainage during such procedures to prevent complications, such as venous infarction.

Conflict of Interest

None declared.

• References

• 1 Wong JH, Awad IA, Kim JH. Ultrastructural pathological features of cerebrovascular malformations: a preliminary report. Neurosurgery 2000; 46 (06) 1454-1459
• 2 Flemming KD, Graff-Radford J, Aakre J. et al. Population-based prevalence of cerebral cavernous malformations in older adults: Mayo Clinic study of aging. JAMA Neurol 2017; 74 (07) 801-805
• 3 Ganz JC. Cavernous malformations. In: Progress in Brain Research. Vol. 268 Netherlands: Elsevier; 2022: 115-132
• 4 Lasjaunias P, Burrows P, Planet C. Developmental venous anomalies (DVA): the so-called venous angioma. Neurosurg Rev 1986; 9 (03) 233-242
• 5 Hon JML, Bhattacharya JJ, Counsell CE. et al; SIVMS Collaborators. The presentation and clinical course of intracranial developmental venous anomalies in adults: a systematic review and prospective, population-based study. Stroke 2009; 40 (06) 1980-1985
• 6 Ostertun B, Solymosi L. Magnetic resonance angiography of cerebral developmental venous anomalies: its role in differential diagnosis. Neuroradiology 1993; 35 (02) 97-104
• 7 San Millán Ruíz D, Delavelle J, Yilmaz H. et al. Parenchymal abnormalities associated with developmental venous anomalies. Neuroradiology 2007; 49 (12) 987-995
• 8 Das KK, Rangari K, Singh S, Bhaisora KS, Jaiswal AK, Behari S. Coexistent cerebral cavernous malformation and developmental venous anomaly: does an aggressive natural history always call for surgical intervention?. Asian J Neurosurg 2019; 14 (01) 318-321
• 9 Nagao S, Hayashi N, Hori S, Nagai S, Kurimoto M, Endo S. Surgical approach for patients combined with cavernoma and developmental venous anomaly. Nosotchu No Geka 2011; 39 (01) 19-23
• 10 Irsyad MA, Fitra F, Sanjaya FA, Suroto NS, Al Fauzi A. Cerebellar cavernoma excision with a preserved venous anomaly: a case report in girl 28-year-old. Int J Surg Case Rep 2023; 107: 108332
• 11 Smith ER. Cavernous malformations of the central nervous system. N Engl J Med 2024; 390 (11) 1022-1028
• 12 Gross BA, Du R. Cerebral cavernous malformations: natural history and clinical management. Expert Rev Neurother 2015; 15 (07) 771-777
• 13 Zabramski JM, Wascher TM, Spetzler RF. et al. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg 1994; 80 (03) 422-432
• 14 Pereira VM, Geibprasert S, Krings T. et al. Pathomechanisms of symptomatic developmental venous anomalies. Stroke 2008; 39 (12) 3201-3215
• 15 San Millán Ruíz D, Gailloud P, Rüfenacht DA, Delavelle J, Henry F, Fasel JHD. The craniocervical venous system in relation to cerebral venous drainage. AJNR Am J Neuroradiol 2002; 23 (09) 1500-1508
• 16 Yeon JY, Kim JS, Choi SJ, Seo DW, Hong SB, Hong SC. Supratentorial cavernous angiomas presenting with seizures: surgical outcomes in 60 consecutive patients. Seizure 2009; 18 (01) 14-20

Publication History

Article published online:
08 September 2025