Tag: vascular malformation

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Idiopathic cerebellar hemorrhage in a patient with isolated developmental venous anomaly: A case report

Surg Neurol Int. 2025;16:82.

A 33-year-old female with severe headaches and vertigo was diagnosed with cerebellar hemorrhage due to an isolated developmental venous anomaly (DVA). Conservative treatment led to full recovery, highlighting the importance of considering DVA in cerebellar hemorrhage etiology and recommending non-surgical management to avoid complications.

• Case Report: Idiopathic cerebellar hemorrhage with isolated developmental venous anomaly in a 33-year-old female.

• Symptoms: Severe headaches, vertigo, left oculomotor nerve palsy, left-sided hemidysmetria.

• Initial Misdiagnosis: Meniere’s disease.

• Imaging Findings: Acute hemorrhage in cerebellar vermis, isolated DVA with “caput medusae” sign.

• Management: Conservative treatment with steroids and analgesics, full recovery.

• DVA Characteristics: Benign anatomical variation, low-flow malformation, rarely causes hemorrhage.

• Hemorrhage Risk Factors: Coexisting cavernous malformation or arteriovenous malformation increase risk.

• Study Findings: Hemorrhage risk 0.22–0.68%/year, higher in infratentorial DVAs.

• Surgical Intervention: Reserved for life-threatening mass effects or raised intracranial pressure.

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Long-Term Risks of Hemorrhage and Adverse Radiation Effects of Stereotactic Radiosurgery for Brain Arteriovenous Malformations

Neurosurgery 90:784–792, 2022

The information about long-term risks of hemorrhage and late adverse radiation effects (AREs) after stereotactic radiosurgery for brain arteriovenous malformations (AVMs) is lacking.

OBJECTIVE: To evaluate the long-term risks of hemorrhage and late ARE rates in patients with AVM treated with Gamma Knife surgery (GKS). METHODS: We examined 1249 patients with AVM treated with GKS. The Spetzler–Martin grade was I in 313 patients (25%), II in 394 (32%), III in 458 (37%), and IV/V in 84 (7%). The median treatment volume was 2.5 cm3, and the median marginal dose was 20 Gy.

RESULTS: The median follow-up period was 61 months. The 5- and 10-year nidus obliteration rates were 63% and 82%, respectively. The 5- and 10-year cumulative hemorrhage rates were 7% and 10%, respectively. The annual hemorrhage rate was 1.5% for the first 5 years post-GKS, which decreased to 0.5% thereafter. During the follow-up period, 42 symptomatic cyst formations/ chronic encapsulated hematomas ([CFs/CEHs], 3%) and 3 radiation-induced tumors (0.2%) were observed. The 10- and 15-year cumulative CF/CEH rates were 3.7% and 9.4%, respectively.

CONCLUSION: GKS is associated with reduced hemorrhage risk and high nidus obliteration rates in patients with AVM. The incidence of late AREs tended to increase over time. The most common ARE was CF/CEH, which can be safely removed; however, careful attention should be paid to the long-term development of fatal radiation-induced tumors.

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Cavernous angiomas: deconstructing a neurosurgical disease

J Neurosurg 131:1–13, 2019

Cavernous angioma (CA) is also known as cavernoma, cavernous hemangioma, and cerebral cavernous malformation (CCM) (National Library of Medicine Medical Subject heading unique ID D006392). In its sporadic form, CA occurs as a solitary hemorrhagic vascular lesion or as clustered lesions associated with a developmental venous anomaly. In its autosomal dominant familial form (Online Mendelian Inheritance in Man #116860), CA is caused by a heterozygous germ-line loss-of-function mutation in one of three genes—CCM1/KRIT1, CCM2/Malcavernin, and CCM3/PDCD10—causing multifocal lesions throughout the brain and spinal cord.

In this paper, the authors review the cardinal features of CA’s disease pathology and clinical radiological features. They summarize key aspects of CA’s natural history and broad elements of evidence-based management guidelines, including surgery. The authors also discuss evidence of similar genetic defects in sporadic and familial lesions, consequences of CCM gene loss in different tissues at various stages of development, and implications regarding the pathobiology of CAs.

The concept of CA with symptomatic hemorrhage (CASH) is presented as well as its relevance to clinical care and research in the field. Pathobiological mechanisms related to CA include inflammation and immune-mediated processes, angiogenesis and vascular permeability, microbiome driven factors, and lesional anticoagulant domains. These mechanisms have motivated the development of imaging and plasma biomarkers of relevant disease behavior and promising therapeutic targets.

The spectrum of discoveries about CA and their implications endorse CA as a paradigm for deconstructing a neurosurgical disease.

 

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Pure arterial malformations

J Neurosurg 129:91–99, 2018

Over the last half century, there have been isolated case reports of purely arterial malformations. In this study, the authors report a consecutive series of patients with pure arterial malformations, emphasizing the clinical and radiological features of these lesions.

METHODS Pure arterial malformations were defined as dilated, overlapping, and tortuous arteries with a coil-like appearance and/or a mass of arterial loops without any associated venous component. Demographic characteristics of the patients, cardiovascular risk factors, presentation, radiological characteristics, and follow-up data were collected. Primary outcomes were new neurological symptoms including disability, stroke, and hemorrhage.

RESULTS Twelve patients meeting the criteria were identified. Ten patients were female (83.3%) and 2 were male (16.6%). Their mean age at diagnosis was 26.2 ± 11.6 years. The most common imaging indication was headache (7 patients [58.3%]). In 3 cases the pure arterial malformation involved the anterior cerebral arteries (25.0%); in 4 cases the posterior communicating artery/posterior cerebral artery (33.3%); in 2 cases the middle cerebral artery (16.6%); and in 1 case each, the superior cerebellar artery, basilar artery/anterior inferior cerebellar artery, and posterior inferior cerebellar artery. The mean maximum diameter of the malformations was 7.2 ± 5.0 mm (range 3–16 mm). Four lesions had focal aneurysms associated with the pure arterial malformation, and 5 were partially calcified. In no cases was there associated intracranial hemorrhage or infarction. One patient underwent treatment for the pure arterial malformation. All 12 patients had follow-up (mean 29 months, median 19 months), and there were no cases of disability, stroke, or hemorrhage.

CONCLUSIONS Pure arterial malformations are rare lesions that are often detected incidentally and probably have a benign natural history. These lesions can affect any of the intracranial arteries and are likely best managed conservatively.

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Transdural indocyanine green video-angiography of vascular malformations

Transdural indocyanine green video-angiography of vascular malformations

Acta Neurochir (2014) 156:1761–1767

The role of indocyanine green videoangiography (ICG-VA) in the surgical resection of vascular malformations has been largely described; conversely, the utility of ICG-VA before dural opening (transdural ICG-VA) in this situation remains unclear. The aim of this study is to present the application of transdural ICG-VA in a consecutive series of patients in order to explore the potential provided by a transdural visualisation of vascular malformations.

Method We retrospectively analysed the application of intraoperative ICG-VA before dural opening in 15 consecutive patients who underwent surgical resection of vascular malformations. The cases included 12 cerebral arterio-venous malformations (AVMs), 2 cerebral dural arterio-venous fistulas (dAVFs) and 1 spinal arterio-venous fistula (AVF).

Results ICG-VA before dural opening allowed the visualisation of the site and extension of the malformation in 13 out of 15 cases, whilst arterial feeders and venous drainages were identified in 9 out of 15 cases. In two patients with dAVF, the point of fistula could be transdurally identified through ICGVA. In 14% of cases, the size of bone flap designed on neuronavigation data was then modified according to transdural ICG-VA findings.

Conclusions Transdural ICG-VA proved an efficient tool that allows optimising the exposure of the malformation, performing a safe dural opening and identifying dural vascular connections of the lesion.

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Capillary telangiectasias: clinical, radiographic, and histopathological features

J Neurosurg 113:709–714, 2010. DOI: 10.3171/2009.9.JNS09282

Brain capillary telangiectasias (BCTs) are small, clinically benign, angiographically occult lesions that are usually incidental findings. Large capillary telangiectasias have not been reported previously as most BCTs are very small. Symptomatic BCTs are also rare, with few reports in the literature. The authors review the clinical manifestations, imaging, and histopathological characteristics of BCTs to further elucidate the diagnostic and clinical features of these vascular malformations.

Methods. The authors completed a retrospective radiological review of all cases of BCTs in the neuroradiology database at the University of Utah involving patients treated between January 1993 and December 2007. The MR imaging scans were reviewed, and the BCT was measured in 2 dimensions. They arbitrarily chose > 1 cm to define a large BCT as a majority of these lesions were smaller than that. The medical chart and the electronic database were used to gather each patient’s clinical information.

Results. One hundred thirty patients were identified in the archived neuroradiology database of capillary telangiectasias. Cases involving 105 patients with definite capillary telangiectasias were reviewed, and from these, 7 patients were identified to have a large capillary telangiectasia measuring > 1 cm. Upon further review, 2 of these patients were identified as having symptoms likely related to their capillary telangiectasia. These 2 cases are reported in the article. No patients with smaller BCTs were found to have symptoms related to their lesion.

Conclusions. Brain capillary telangiectasias are small vascular malformations that rarely cause symptoms. They are often overlooked on imaging because of their clinically benign nature; however, they have been misdiagnosed as glial tumors in the past. Specific MR imaging sequences (T1-weighted postcontrast and gradient refocused echo) are valuable in aiding diagnosis, as histopathological diagnosis is often not possible. These cases highlight that BCTs can cause symptoms, a finding that may actually be related to the size of the lesion (28.6% of large BCTs in this series were symptomatic, whereas none of the small ones were).

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