Capillary Telangiectasia Research Articles

Cerebral vascular malformations in hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder characterized by mucocutaneous telangiectasias, frequent nosebleeds, and visceral arteriovenous malformations (AVMs). Few reports have outlined the prevalence of the various cerebral vascular malformations found in patients with HHT. The authors set out to define the prevalence of cerebral vascular malformations in a population of HHT patients who underwent imaging with 3-T imaging (MRI/MR angiography [MRA]) of the brain. A retrospective review of prospectively collected data was carried out using a database of 372 HHT patients who were seen and examined at the Georgia Regents University HHT Center and screened with 3-T MRI/MRA. Data were tabulated for numbers and types of vascular malformations in this population. Arteriovenous malformations were identified in 7.7%, developmental venous anomalies in 4.3%, and cerebral aneurysms in 2.4% of HHT patients. The HHT AVMs tended to be supratentorial, small, and cortical in this series, findings consistent with other recent studies in the literature. An arteriovenous fistula, cavernous malformation, and capillary telangiectasia were identified in 0.5%, 1%, and 1.9% of HHT patients, respectively. Few studies have investigated the prevalence of the various vascular malformations found in HHT patients screened with 3-T MRI/MRA of the brain. Hereditary hemorrhagic telangiectasia AVMs are more likely to be multiple and have a tendency toward small size and cortical location. As such, they are often treated using a single-modality therapy.

Aneurysms, Arteriovenous Malformations, and Dural Arteriovenous Fistulas: Diagnosis and Treatment

Despite the fact that intracranial aneurysms and vascular malformations account for a small overall percentage of hemorrhagic strokes, their rupture is potentially devastating and often fatal. Imaging plays a crucial role in the accurate identification and treatment planning of these lesions. This article provides a review of the imaging findings of cerebral aneurysms, arteriovenous malformations (AVMs), and dural arteriovenous fistulae (dAVF). For the sake of length, we have not discussed other cerebral vascular malformations such as cavernous malformations or capillary telangiectasias. Basic pathologic and clinical concepts for each lesion have been discussed, with a focus toward the diagnostic imagingworkup. We also briefly discuss treatment options for these lesions.

Psychiatric Disturbance and Mild Intellectual Disability in the Context of a Vascular Malformation (Capillary Telangiectasia) of the Posterior Hippocampus and Subiculum

Psychiatric Disturbance and Mild Intellectual Disability in the Context of a Vascular Malformation (Capillary Telangiectasia) of the Posterior Hippocampus and Subiculum

Large Capillary Telangiectasia and Developmental Venous Anomaly of the Basal Ganglia: An Unusual Finding

Brain capillary telangiectasias are small, benign, asymptomatic, angiographically occult lesions that are incidentally discovered either at autopsy or on MR imaging in most cases. They are commonly located in the pons and can be associated with other vascular malformations. We describe a case of an unusually large capillary telangiectasia associated with a developmental venous anomaly involving the basal ganglia in a young woman, in which MR serial imaging suggested the diagnosis and avoided an unnecessary stereotaxic biopsy.

Cerebral capillary telangiectasias: a meta-analysis and review of the literature

As a result of their presumed benign natural history, cerebral capillary telangiectasias (CCTs) are infrequently addressed in the neurosurgical literature. We performed a comprehensive review of CCTs via the PubMed database to synthesize overall epidemiological, radiographic, natural history, and treatment results. Across ten series with 203 patients, mean age was 47, and 45% were male [95% confidence interval (CI), 0.30-0.65]. Notably, 78% of CCTs were in the pons (95% CI, 0.58-1.0). Six percent of CCTs were symptomatic. Across five radiographic series, all lesions enhanced after gadolinium, and all were hypointense on gradient echo sequences. Thirty-three percent were hypointense on T1-weighted pre-contrast imaging (95% CI, 0.2-0.51), 49% were hyperintense on T2-weighted imaging (95% CI, 0.31-0.72), and 74% were hypointense on diffusion-weighted imaging (95% CI, 0.5-1.0). Notably, 37% were associated with a prominent draining vein (95% CI, 0.21-0.6), and 11% with a developmental venous anomaly (95% CI, 0.04-0.25). Across four observational studies with 47 patients, there was no observed change in lesion morphology or hemorrhage in 65.7 patient-years of follow-up. Although the vast majority of CCTs are managed conservatively, we found ten cases of patients treated with surgical excision. We confirm that CCTs are a benevolent entity with a predilection for the pons. They have distinctive radiographic features including their lack of mass effect, consistent enhancement on T1-weighted sequences and hypointensity on gradient echo sequences, and common isointensity on pre-contrast T1-weighted and T2-weighted images. Management for these lesions has been nonoperative in almost all cases.

Susceptibility-weighted angiography for the detection of high-flow intracranial vascular lesions: preliminary study

Susceptibility-weighted magnetic resonance imaging (MRI) sequences may demonstrate various signal intensities of draining veins in cases of high-flow vascular malformation (HFVM), including arteriovenous malformation (AVM) and dural arteriovenous fistula (dAVF). Our objective was to evaluate susceptibility-weighted angiography (SWAN) for the detection of HFVM. Fifty-eight consecutive patients with a suspected intracranial vascular malformation were explored with SWAN and post-contrast MRI sequences at 3 T. The diagnosis of slow-flow vascular malformation (SFVM), including developmental venous anomaly (DVA) or brain capillary telangiectasia (BCT), was based on MRI. Patients with suspected HFVM underwent digital subtraction angiography (DSA). SWAN images were analysed by three blinded readers according to a three-point scale of the venous signal. Thirty-one patients presented 35 SFVM (26 DVA and 9 BCT) that systematically appeared hypointense on SWAN images. In patients with atypical MRI findings, DSA revealed one patient with an atypical DVA and 26 patients with HFVM (22 AVM and 4 dAVF). SWAN revealed at least one venous hyperintensity in all patients with HFVM. Agreement between readers was excellent. SWAN appears reliable for characterising blood flow dynamics in brain veins. In clinical practice, SWAN can routinely rule out HFVM in patients with atypical brain veins.

Blepharospasm in a Patient With Pontine Capillary Telangiectasia

Blepharospasm is rarely due to an identifiable etiology. In the majority of cases, imaging fails to reveal any structural lesion. Here we describe an otherwise healthy patient with blepharospasm who was found to have pontine capillary telangiectasia. We propose a potential association between blepharospasm and pontine capillary telangiectasia.

A patient with short term memory problems and ataxia

A patient with short term memory problems and ataxia

A patient with short term memory problems and ataxia

The patient had a shunt placed for obstructive hydrocephalus. An endoscopic biopsy of the intraventricular and septal mass was performed which showed findings consistent with a cavernous angioma (Fig. 1). Intracranial vascular malformations are divided into four groups: (i) capillary telangiectasia, (ii) cavernous angioma, (iii) arteriovenous malformation, and (iv) venous malformation. Cavernous angiomas comprise 5% to 13% of all vascular malformations and occur in about 0.5% of the general population. Cerebral cavernous angiomas occur sporadically and in a familial form. Familial cavernous angiomas account for up to 54% of cerebral cavernous angiomas. The most frequent symptoms are seizures, gross intracranial hemorrhages, focal neurological deficits, and migraine-like headaches. The prospective annualized hemorrhage rate calculated on a per-patient basis is 1.6% to 3.1%. Intracranial cavernomas are commonly located in the supratentorial region, brain stem, basal ganglia, and cerebellar hemisphere. On the basis of MRI, four

Spinale Gefäßmalformationen

Spinal vascular malformations are a group of rare diseases with different clinical presentations ranging from incidental asymptomatic findings to progressive tetraplegia. This article provides an overview about imaging features as well as clinical and therapeutic aspects of spinal arteriovenous malformations, cavernomas and capillary telangiectasia.

Abstract 2421: Neurologic Phenotype Associated With Hereditary Haemorrhagic Telangiectasia In A Monocentric Cohort Of 154 Patients

Introduction Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that leads to the formation of vascular malformations in mucocutaneous tissues, visceral organs, and central nervous system. The most frequently reported neurologic injuries are cerebral vascular malformations (CVM), especially cerebral arteriovenous malformations (CAVM). Neurologic manifestations secondary to a right-to-left shunt through pulmonary arteriovenous malformations (PAVM) are less known. Methods All patients with HHT collected between 1991 and 2010 in a prospective registry in a tertiary teaching hospital were included in this study. Neurologic data and brain imaging were assessed by 2 neurologists and 1 neuroradiologist. Results In our cohort of 154 patients with HHT, 27 (17.5%) had a symptomatic neurologic injury, while symptomatic neurologic injury was present in 27 (29%) patients among the 93 patients with a PAVM. Prevalence of stroke or TIA was 14.2% in the overall group and 23.6% in the subgroup with PAVM. At 3 months, patients with stroke had good recovery (modified Rankin Score ≤ 2). Aetiological work-up for stroke or TIA revealed a right-to-left shunt through PAVM in 94.6% cases, including 13.6% of cases occurring during PAVM embolization. Ischemic events occurred in 4.5% of cases during cerebral AVM embolization. No cerebral ischemic recurrence occurred in patients with complete PAVM embolization. Four patients (0.03%) had cerebral abscesses. One patient had a symptomatic cerebral haemorrhage caused by a brain cavernoma. Various and asymptomatic CVMs were found on MRI in our study population: CAVM with small niduses (10 in 6 patients), capillary telangiectasias (8 in 6 patients), venous developpmental anomalies (7 in 4 patients), and cavernomas (3 patients). Conclusion Neurologic symptoms related to HHT affect more than 1 patient in 6. Our results underline the high prevalence of stroke or TIA in HHT, especially in those with a PAVM. Cerebral vascular anomalies are common and atypical in HHT patients.

Susceptibility-Weighted MR Imaging for Diagnosis of Capillary Telangiectasia of the Brain

BCT is a benign entity, whose appearance on conventional MR imaging makes its differentiation from neoplastic, inflammatory, or subacute ischemic disease challenging. SWI is sensitive to susceptibility effects from deoxyhemoglobin with excellent spatial resolution. Only scarce case reports have described the utility of SWI in cases of BCT. Our aim was to show the diagnostic value of SWI applied to a larger series of cases. This was an observational retrospective study of 33 BCTs in 27 consecutive patients examined from August 2009 to January 2011 with MR imaging, including SWI. Morphology, signal intensity characteristics, and additional vascular malformations were analyzed. Preceding or follow-up examinations were available in 18 patients with a median time interval of 14.5 months (range, 2-115 months). Twenty-five pontine and 8 supratentorial BCTs demonstrated distinct signal-intensity loss on SWI in combination with postcontrast enhancement. Mean lesion diameter was 4.9 mm (range, 1.5-17 mm). Thirty-nine percent showed slight signal-intensity changes on T1 and/or T2; the remainder were isointense to normal brain. In 30%, a prominent draining vessel was observed. Additional cerebral vascular malformations were found in 5 patients. SWI represents a valuable tool for confirmation of presumed BCT. Demonstration of signal-intensity loss on SWI in an enhancing focal brain lesion, otherwise unremarkable on conventional MR images, is highly specific for BCT, thus excluding serious pathology and reassuring the patient and referring physician. This is particularly helpful for BCT in less typical locations.

Role of magnetic resonance venography in evaluation of cerebral veins and sinuses occlusion

IntroductionCerebral venous occlusion (CVO) means intraluminal obstruction by cerebral venous thrombosis (CVT) or external compression. The differential diagnosis is broad including stroke, brain capillary telangiectasia, contusion, hypertensive hemorrhage, metastases and venous vascular malformations. MRI in conjunction with MRV is considered the modality of choice in diagnosis of CVO. ObjectiveTo assess the role of magnetic resonance venography in the evaluation of cerebral veins and sinuses occlusion. MethodsThe study was conducted on 15 patients with cerebral venous occlusion and subjected to MRI and MRV. ResultsThe study included 13 patients with cerebral venous occlusion due to thrombosis and two patients with occlusion by tumors (meningioma). ConclusionMRI in conjunction with MRV is considered an accurate, safe, non-invasive, non-ionizing diagnostic modality in assessing CVO. It can assess parenchymal lesions and detect intraluminal small thrombi and its extension.

Symptomatic intracranial capillary telangiectasia in a patient with contralateral common carotid artery dissection

Intracranial capillary telangiectasia (ICT) is a rare benign disease with few clinical reports, as most capillary telangiectasias locate in the brainstem, small, asymptomatic and angiographically invisibale. We present a case of intracranial capillary telangiectasia with seizure and hemiplegiahemiparesia. Cranial magnetic resonance imaging (MRI) studies revealed right frontal ischemic lesion. Digital subtraction angiography (DSA) demonstrated right hemispheric capillary telangiectasia of ipsilateral frontal ischemic lesion and left common carotid artery dissection. Key words: Capillary telangiectasia, acute ischemic stroke, digital subtraction angiography.

Management of incidentally discovered intracranial vascular abnormalities

With the widespread use of brain imaging studies, neurosurgeons have seen a marked increase in the number of incidental intracranial lesions, including vascular abnormalities. Specifically, the detection of incidentally discovered aneurysms, arteriovenous malformations, cavernous angiomas, developmental venous anomalies, and capillary telangiectasias has increased. The best management strategy for most of these lesions is controversial. Treatment options include observation, open surgery, endovascular procedures, and radiosurgery. Multiple factors should be taken into account when discussing treatment indications, including the natural history of the disease and the risk of the treatment. In this article, the authors focus on the natural history of these lesions and the risk of the treatment, and they give recommendations regarding the most appropriate management strategy based on the current evidence in the literature and their experience with intracranial vascular abnormalities.

Cerebral cavernous malformations: from genes to proteins to disease

Over the past half century molecular biology has led to great advances in our understanding of angio- and vasculogenesis and in the treatment of malformations resulting from these processes gone awry. Given their sporadic and familial distribution, their developmental and pathological link to capillary telangiectasias, and their observed chromosomal abnormalities, cerebral cavernous malformations (CCMs) are regarded as akin to cancerous growths. Although the exact pathological mechanisms involved in the formation of CCMs are still not well understood, the identification of 3 genetic loci has begun to shed light on key developmental pathways involved in CCM pathogenesis. Cavernous malformations can occur sporadically or in an autosomal dominant fashion. Familial forms of CCMs have been attributed to mutations at 3 different loci implicated in regulating important processes such as proliferation and differentiation of angiogenic precursors and members of the apoptotic machinery. These processes are important for the generation, maintenance, and pruning of every vessel in the body. In this review the authors highlight the latest discoveries pertaining to the molecular genetics of CCMs, highlighting potential new therapeutic targets for the treatment of these lesions.

The Fetal Cerebellum

The cerebellum undergoes a protracted development, making it particularly vulnerable to a broad spectrum of developmental events. Acquired destructive and hemorrhagic insults may also occur. The main steps of cerebellar development are reviewed. The normal imaging patterns of the cerebellum in prenatal ultrasound and magnetic resonance imaging (MRI) are described with emphasis on the limitations of these modalities. Because of confusion in the literature regarding the terminology used for cerebellar malformations, some terms (agenesis, hypoplasia, dysplasia, and atrophy) are clarified. Three main pathologic settings are considered and the main diagnoses that can be suggested are described: retrocerebellar fluid enlargement with normal or abnormal biometry (Dandy-Walker malformation, Blake pouch cyst, vermian agenesis), partially or globally decreased cerebellar biometry (cerebellar hypoplasia, agenesis, rhombencephalosynapsis, ischemic and/or hemorrhagic damage), partially or globally abnormal cerebellar echogenicity (ischemic and/or hemorrhagic damage, cerebellar dysplasia, capillary telangiectasia). The appropriate timing for performing MRI is also discussed.

Cavernous Malformations: A Paradigm for Progress

Cavernous Malformations: A Paradigm for Progress

Mri findings in giant pontine capillary telangiectasis asso - ciated with a developmental venous anomaly

We report a 32-year-old woman with an exceptionally large capillary telangiectasia in the brainstem which is associated with a developmental venous anomaly (DVA). Her major problems were nystagmus in both eyes, binocular diplopia, gait abnormalities, cerebellar ataxia, slightly disturbed finger-nose test, an instable Romberg test and obvious dysartria. The diagnosis was made on the basis of specific imaging findings, and the use of gradient echo-weighted images proved to be helpful in making a differential diagnostic decision.

Telangiectasia capilar y anomalía del desarrollo venoso: una rara asociación

We review the relevant literature for a case of capillary telangiectasia associated with a developmental venous anomaly in the pons discovered incidentally. The most common established associations among vascular malformations are capillary telangiectasias with cavernous malformations and developmental venous anomalies with cavernous malformations. The association of a capillary telangiectasia with a developmental venous anomaly is very rare and only two cases affecting the posterior fossa have been reported. An analysis of the associations among vascular malformations leads to a discussion of the role of obstructed flow in their pathogenesis and whether these malformations can be considered a spectrum of manifestations of a single disease.