89 results match your criteria Brain Imaging in Capillary Telangiectasia


Hereditary Hemorrhagic Telangiectasia - Early Childhood Presentation with Hepatic Failure.

J Coll Physicians Surg Pak 2018 Sep;28(9):S195-S197

Department of Pediatrics, Shifa College of Medicine, Islamabad.

Hereditary hemorrhagic telangiectasia (HHT) is a vascular dysplasia in which capillary bed is absent with direct draining of arterial blood into venous circulation. Due to increased pressure there is increased risk of bleeding. The classical triad consists of telangiectasias, epistaxis and a positive family history. Read More

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https://www.jcpsp.pk/data/view.php?id=364&type=pdf&j
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http://dx.doi.org/10.29271/jcpsp.2018.09.S195DOI Listing
September 2018
16 Reads

Pulmonary arteriovenous malformations: diagnosis.

Cardiovasc Diagn Ther 2018 Jun;8(3):325-337

Department of Radiology, UT Southwestern Medical Center, Dallas, Texas, USA.

Pulmonary arteriovenous malformations (PAVMs) are rare, abnormal low resistance vascular structures that connect a pulmonary artery to a pulmonary vein, thereby bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt. The spectrum of PAVMs extends from microscopic lesions causing profound hypoxemia and ground glass appearance on computed tomography (CT) but with normal catheter angiographic findings to classic pulmonary aneurysmal connections that abnormally connect pulmonary veins and arteries. These malformations most commonly are seen in hereditary hemorrhagic telangiectasia (HHT). Read More

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http://dx.doi.org/10.21037/cdt.2018.06.01DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6039795PMC
June 2018
5 Reads

Metabolic Changes of Brain Developmental Venous Anomalies on F-FDG-PET.

Acad Radiol 2018 Jun 27. Epub 2018 Jun 27.

Department of Neuroradiology, Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104. Electronic address:

Rationale And Objectives: To determine the metabolic effects of developmental venous anomalies (DVAs) and to correlate those effects with conventional magnetic resonance imaging (MRI) findings.

Materials And Methods: We conducted a retrospective review of MRI and brain 18F-fluorodeoxyglucose positron emission tomography (F-FDG-PET) examinations in subjects with DVAs. Conventional MRI was used to determine DVA number, location, size, and associated parenchymal findings such as atrophy, hemorrhage, cavernoma, capillary telangiectasia, cortical dysplasia/polymicrogyria, and white matter signal abnormality. Read More

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http://dx.doi.org/10.1016/j.acra.2018.05.021DOI Listing
June 2018
1 Read

Eight-and-a-Half Syndrome: A Combination of Intracranial Capillary Telangiectasia and Hematencephalon.

J Stroke Cerebrovasc Dis 2018 Jul 12;27(7):e135-e137. Epub 2018 Apr 12.

Department of Nerve medical center, The First Hospital of Changsha, Changsha, China.

Eight-and-a-half syndrome, a combination of one-and-a-half syndrome and ipsilateral facial palsy, was first described by Eggenberger in 1998. Intracranial capillary telangiectasia (ICT) is a rare type of latent cerebral vascular malformation characterized by a number of small, dilated, and thin-walled blood capillaries with normal brain tissues between them. Susceptibility weighted imaging is the recommended diagnostic method to detect ICT. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2018.02.020DOI Listing
July 2018
1 Read

Optical coherence tomography angiography: A comprehensive review of current methods and clinical applications.

Prog Retin Eye Res 2017 09 29;60:66-100. Epub 2017 Jul 29.

Department of Biomedical Engineering, University of Washington Seattle, Seattle, WA 98195, United States.

OCT has revolutionized the practice of ophthalmology over the past 10-20 years. Advances in OCT technology have allowed for the creation of novel OCT-based methods. OCT-Angiography (OCTA) is one such method that has rapidly gained clinical acceptance since it was approved by the FDA in late 2016. Read More

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http://dx.doi.org/10.1016/j.preteyeres.2017.07.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5600872PMC
September 2017
12 Reads

Pial Arteriovenous Fistula and Capillary Malformation-Arteriovenous Malformation Associated with RASA1 Mutation: 2 Pediatric Cases with Successful Surgical Management.

Pediatr Neurosurg 2017 31;52(4):261-267. Epub 2017 May 31.

Division of Pediatric Epilepsy, Department of Pediatric Neurology, Rainbow Babies and Children's Hospital, and Department of Neurology and Neurosurgery, The Neurological Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.

We present case reports of 2 pediatric patients who were both found to have pial arteriovenous fistulas (AVFs) with subsequent genetic analysis revealing mutations in the RASA1 gene. Considering their family history of distinct cutaneous lesions, these mutations were likely inherited as opposed to de novo mutations. Patient 1 had large capillary malformations on the left side of the face and neck, associated with macrocephaly, and presented at the age of 32 months with speech delay, right-sided weakness, and focal seizures involving the right side of the body. Read More

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http://dx.doi.org/10.1159/000474942DOI Listing
May 2018
18 Reads

Cerebral Abscess Associated With Odontogenic Bacteremias, Hypoxemia, and Iron Loading in Immunocompetent Patients With Right-to-Left Shunting Through Pulmonary Arteriovenous Malformations.

Clin Infect Dis 2017 08;65(4):595-603

NHLI Cardiovascular Sciences, Imperial College London, London, UK.

Background: Cerebral abscess is a recognized complication of pulmonary arteriovenous malformations (PAVMs) that allow systemic venous blood to bypass the pulmonary capillary bed through anatomic right-to-left shunts. Broader implications and mechanisms remain poorly explored.

Methods: Between June 2005 and December 2016, at a single institution, 445 consecutive adult patients with computed tomography-confirmed PAVMs (including 403 [90. Read More

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http://dx.doi.org/10.1093/cid/cix373DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5849101PMC
August 2017
11 Reads

Symptomatic capillary telangiectasia of the pons: three pediatric cases diagnosed by suspectibility-weighted imaging.

Childs Nerv Syst 2016 Nov 19;32(11):2261-2264. Epub 2016 May 19.

Department of Pediatric Neurology, Faculty of Medicine, Gazi University, Besevler, 06510, Ankara, Turkey.

Introduction: Brain capillary telangiectasias (BCT) are small, clinically benign, usually asymptomatic and rarely seen lesions during childhood. Large symptomatic BCT during childhood are extremely rare, with only a few reports in the literature. They usually cannot be detected easily on conventional magnetic resonance imaging and angiography. Read More

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http://dx.doi.org/10.1007/s00381-016-3110-6DOI Listing
November 2016
3 Reads

Vascular Disorders of the Cerebellum in Children.

Neuroimaging Clin N Am 2016 Aug;26(3):435-58

Department of Radiology, Mount Sinai Medical Center, Box 1234, One Gustave Levy Place, New York, NY 10029, USA; Department of Neurosurgery, Mount Sinai Medical Center, One Gustave Levy Place, New York, NY 10029, USA; Department of Pediatrics, Mount Sinai Medical Center, One Gustave Levy Place, New York, NY 10029, USA.

Key differences exist in the epidemiology, pathophysiology, and clinical presentation of vascular lesions of the cerebellum in children versus adults. An understanding of these differences and an appreciation of the distinct imaging features of these lesions aid in distinguishing normal vascular variations from pathology, in predicting lesion etiology, and in directing effective treatment strategies. This paper reviews the embryogenesis of the normal vascular system of the cerebellum and brainstem and then discusses the clinical and imaging features of the common vascular lesions affecting these structures in the pediatric population. Read More

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http://dx.doi.org/10.1016/j.nic.2016.03.009DOI Listing
August 2016
12 Reads

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing-like attacks in a pediatric patient found to have a pontine capillary telangiectasia and developmental venous anomaly: A case report exploring the root of the problem.

Cephalalgia 2017 Oct 24;37(11):1093-1097. Epub 2016 Jun 24.

1 Department of Pediatric Neurology, University of Alberta, Edmonton, AB, Canada.

Introduction Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)-like attacks are rarely reported in the pediatric population and may remain undiagnosed and under-investigated as a result. Case presentation We present a case of a 15-year-old male with intermittent, episodic, right-sided brief headaches most in keeping with SUNCT, initially diagnosed as paroxysmal hemicrania, but with no response to indomethacin. The pain was likewise not responsive to typical migraine treatments or steroids. Read More

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http://dx.doi.org/10.1177/0333102416657148DOI Listing
October 2017
71 Reads

Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome.

J Neurosurg Pediatr 2016 Jul 1;18(1):79-82. Epub 2016 Apr 1.

Departments of 1 Neurology.

The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. Read More

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http://dx.doi.org/10.3171/2016.1.PEDS15482DOI Listing
July 2016
10 Reads

Neurovascular Manifestations of Hereditary Hemorrhagic Telangiectasia: A Consecutive Series of 376 Patients during 15 Years.

AJNR Am J Neuroradiol 2016 08 24;37(8):1479-86. Epub 2016 Mar 24.

From the Departments of Radiology (W.B., H.J.C., K.R.T., C.P.W.).

Background And Purpose: Hereditary hemorrhagic telangiectasia is associated with a wide range of neurovascular abnormalities. The aim of this study was to characterize the spectrum of cerebrovascular lesions, including brain arteriovenous malformations, in patients with hereditary hemorrhagic telangiectasia and to study associations between brain arteriovenous malformations and demographic variables, genetic mutations, and the presence of AVMs in other organs.

Materials And Methods: Consecutive patients with definite hereditary hemorrhagic telangiectasia who underwent brain MR imaging/MRA, CTA, or DSA at our institution from 2001 to 2015 were included. Read More

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http://www.ajnr.org/cgi/doi/10.3174/ajnr.A4762
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http://dx.doi.org/10.3174/ajnr.A4762DOI Listing
August 2016
13 Reads

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients.

J Neurointerv Surg 2017 Jan 26;9(1):26-28. Epub 2016 Feb 26.

Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.

Background And Purpose: Brain capillary vascular malformations (CVMs) are known to occur with relatively high frequency in hereditary hemorrhagic telangiectasia (HHT) patients. These lesions are thought to have a benign natural history but this has not been systematically studied. The purpose of our study was to examine the natural history of CVMs in a consecutive series of HHT patients. Read More

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http://dx.doi.org/10.1136/neurintsurg-2015-012252DOI Listing
January 2017
9 Reads

Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases.

J Neurosurg 2016 07 13;125(1):160-6. Epub 2015 Nov 13.

Departments of 1 Neurosurgery and.

Brain capillary telangiectasias (BCTs) are usually small and benign with a predilection in the pons and basal ganglion. Reports of large and symptomatic BCTs are rare. Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological defects, and they can be fatal if left untreated. Read More

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http://dx.doi.org/10.3171/2015.5.JNS142805DOI Listing
July 2016
25 Reads

Pediatric central nervous system vascular malformations.

Pediatr Radiol 2015 Sep 7;45 Suppl 3:S463-72. Epub 2015 Sep 7.

Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA.

Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Read More

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http://dx.doi.org/10.1007/s00247-015-3356-2DOI Listing
September 2015
5 Reads

Giant symptomatic capillary telangiectasia of the brain.

Arq Neuropsiquiatr 2015 Jan 1;73(1):66-7. Epub 2015 Jan 1.

Departamento de Radiologia, Universidade de São Paulo, Sao Paulo, SP, Brazil.

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http://dx.doi.org/10.1590/0004-282X20140185DOI Listing
January 2015
23 Reads

Capillary telangiectasia of the brain: imaging with various magnetic resonance techniques.

JBR-BTR 2014 Jul-Aug;97(4):233-8

Department of Radiology, İzmir Kâtip Çelebi Üniversitesi Atatürk Eğitim ve Araştirma Hastanesi, Basin Sitesi, Izmir, Turkey.

Brain capillary telangiectasia is an incidental vascular malformation found usually in pons and sometimes in extra- pontine sites. Typical MRI features are enhancement on post contrast T1 weighted images and signal loss on gradient echo images. We evaluated 10 patients with various MR techniques. Read More

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February 2015
3 Reads

Neurovascular manifestations in hereditary hemorrhagic telangiectasia: imaging features and genotype-phenotype correlations.

AJNR Am J Neuroradiol 2015 May 8;36(5):863-70. Epub 2015 Jan 8.

From the Division of Neuroradiology (T.K., S.P., K.G.t.B.), Department of Medical Imaging.

Background And Purpose: Hereditary hemorrhagic telangiectasia is an autosomal dominant disease that presents in 10%-20% of patients with various brain vascular malformations. We aimed to report the radiologic features (phenotype) and the genotype-phenotype correlations of brain vascular malformations in hereditary hemorrhagic telangiectasia.

Materials And Methods: Demographic, clinical, genotypic, and imaging information of 75 patients with hereditary hemorrhagic telangiectasia with brain arteriovenous malformations enrolled in the Brain Vascular Malformation Consortium from 2010 to 2012 were reviewed. Read More

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http://www.ajnr.org/content/36/5/863.full.pdf
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http://www.ajnr.org/cgi/doi/10.3174/ajnr.A4210
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http://dx.doi.org/10.3174/ajnr.A4210DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4433843PMC
May 2015
3 Reads

Genetic markers and their influence on cerebrovascular malformations.

Neuroimaging Clin N Am 2015 Feb;25(1):69-82

UNC at Chapel Hill, Chapel Hill, NC 27516, USA. Electronic address:

Cerebrospinal vascular malformations are a group of anomalies affecting the arterial wall, the capillary arteriovenous interface, or the venous and lymphatic structures. Heritability and family studies allow identification of mutations in single genes associated with rare familial conditions causing cerebral or spinal vascular malformations, as is the case in hemorrhagic hereditary telangiectasia diseases. This article reviews the genetic and epigenetic influences increasingly reported in recent years as causal factors or triggers involved in the formation and growth of cerebromedullary vascular malformations. Read More

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http://dx.doi.org/10.1016/j.nic.2014.09.009DOI Listing
February 2015
3 Reads

Constitutively active Notch4 receptor elicits brain arteriovenous malformations through enlargement of capillary-like vessels.

Proc Natl Acad Sci U S A 2014 Dec 2;111(50):18007-12. Epub 2014 Dec 2.

Laboratory for Accelerated Vascular Research, Department of Surgery, Division of Vascular Surgery, University of California, San Francisco, CA 94143;

Arteriovenous (AV) malformation (AVM) is a devastating condition characterized by focal lesions of enlarged, tangled vessels that shunt blood from arteries directly to veins. AVMs can form anywhere in the body and can cause debilitating ischemia and life-threatening hemorrhagic stroke. The mechanisms that underlie AVM formation remain poorly understood. Read More

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http://dx.doi.org/10.1073/pnas.1415316111DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4273347PMC
December 2014
20 Reads

Pulmonary arteriovenous malformations.

Authors:
Claire L Shovlin

Am J Respir Crit Care Med 2014 Dec;190(11):1217-28

1 NHLI Cardiovascular Sciences, Imperial College, London; and.

Within the past decade, pulmonary arteriovenous malformations (PAVMs) have evolved from rare curiosities to not uncommon clinical states, with the latest estimates suggesting a prevalence of ~1 in 2,600. PAVMs provide anatomic right-to-left shunts, allowing systemic venous blood to bypass gas exchange and pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands result, although both are usually asymptomatic. Read More

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http://dx.doi.org/10.1164/rccm.201407-1254CIDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4315816PMC
December 2014
1 Read

Susceptibility-weighted MR imaging: a better technique in the detection of capillary telangiectasia compared with T2* gradient-echo.

AJNR Am J Neuroradiol 2014 Dec 21;35(12):2302-5. Epub 2014 Aug 21.

From the Department of Radiology (U.S.C., D.E.D., B.A.P.) Section of Neuroradiology (D.E.D., B.A.P.), University of Iowa Hospitals and Clinics, Iowa City, Iowa.

Background And Purpose: Enhancing lesions on brain MR imaging can present a diagnostic quandary as both benign lesions such as brain capillary telangiectasia and pathologic lesions such as demyelination may appear similar. Stagnation of blood in low-flow venous channels of brain capillary telangiectasias results in susceptibility effect secondary to the increased local deoxyhemoglobin. Both T2* gradient-echo imaging and SWI were demonstrated as valuable in the diagnosis of brain capillary telangiectasia. Read More

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http://www.ajnr.org/lookup/doi/10.3174/ajnr.A4082
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http://dx.doi.org/10.3174/ajnr.A4082DOI Listing
December 2014
3 Reads

Rates and characteristics of radiographically detected intracerebral cavernous malformations after cranial radiation therapy in pediatric cancer patients.

J Child Neurol 2015 Jun 13;30(7):842-849. Epub 2014 Aug 13.

Department of Neurology, University of California, San Francisco.

Rates and characteristics of intracerebral cavernous malformations after cranial radiation therapy remain poorly understood. Herein we report on intracerebral cavernous malformations detected on follow-up imaging in pediatric cancer patients who received cranial radiation therapy at age ≤18 years from 1980 to 2009. Through chart reviews (n = 362) and phone interviews (n = 104) of a retrospective cohort, we identified 10 patients with intracerebral cavernous malformations. Read More

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http://journals.sagepub.com/doi/10.1177/0883073814544364
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http://dx.doi.org/10.1177/0883073814544364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4326593PMC
June 2015
5 Reads

Myoclonic axial jerks for diagnosing atypical evolution of ataxia telangiectasia.

Brain Dev 2015 Mar 18;37(3):362-5. Epub 2014 Jun 18.

Department of Pediatrics, Tohoku University School of Medicine, Aoba-ku, Sendai, Japan.

Background: Ataxia telangiectasia (A-T) is a common inherited cause of early childhood-onset ataxia, distinguished by progressive cerebellum malfunction, capillary vessel extension, and immunodeficiency. The diagnosis of A-T is sometimes difficult to establish in patients with atypical clinical evolution.

Case Report: We experienced a pediatric 12-years-old female patient, who was finally diagnosed with classic A-T, demonstrating progressive dystonic-myoclonic axial jerks with ataxia as a predominant clinical feature. Read More

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http://dx.doi.org/10.1016/j.braindev.2014.06.001DOI Listing
March 2015
14 Reads

Ischaemic strokes in patients with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia: associations with iron deficiency and platelets.

PLoS One 2014 19;9(2):e88812. Epub 2014 Feb 19.

Department of Imaging, Imperial College Healthcare NHS Trust, London, United Kingdom.

Background: Pulmonary first pass filtration of particles marginally exceeding ∼7 µm (the size of a red blood cell) is used routinely in diagnostics, and allows cellular aggregates forming or entering the circulation in the preceding cardiac cycle to lodge safely in pulmonary capillaries/arterioles. Pulmonary arteriovenous malformations compromise capillary bed filtration, and are commonly associated with ischaemic stroke. Cohorts with CT-scan evident malformations associated with the highest contrast echocardiographic shunt grades are known to be at higher stroke risk. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0088812PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3929507PMC
December 2014
9 Reads

Cerebral vascular malformations in hereditary hemorrhagic telangiectasia.

J Neurosurg 2014 Jan 15;120(1):87-92. Epub 2013 Nov 15.

Departments of Neurological Surgery.

Object: 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. Read More

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http://dx.doi.org/10.3171/2013.10.JNS122402DOI Listing
January 2014
2 Reads

Large capillary telangiectasia and developmental venous anomaly of the Basal Ganglia: an unusual finding.

Neuroradiol J 2012 Dec 20;25(6):744-9. Epub 2012 Dec 20.

Department of Biomorphological and Functional Sciences, Federico II University of Naples; Naples, Italy -

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. Read More

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http://journals.sagepub.com/doi/10.1177/197140091202500614
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http://dx.doi.org/10.1177/197140091202500614DOI Listing
December 2012
2 Reads

Pulmonary arteriovenous malformations.

Chest 2013 Sep;144(3):1033-1044

Department of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.

Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular structures that most often connect a pulmonary artery to a pulmonary vein, bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt. As a consequence, patients with PAVM can have hypoxemia and paradoxical embolization complications, including stroke and brain abscess. PAVMs may be single or multiple, unilateral or bilateral, and simple or complex. Read More

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http://dx.doi.org/10.1378/chest.12-0924DOI Listing
September 2013
4 Reads

Cerebral abnormalities in adults with ataxia-telangiectasia.

AJNR Am J Neuroradiol 2014 Jan 25;35(1):119-23. Epub 2013 Jul 25.

Departments of Radiology and Radiological Science.

Ataxia-telangiectasia, an autosomal recessive disorder caused by defect of the ataxia-telangiectasia mutated gene, is characterized by progressive neurologic impairment with cerebellar atrophy, ocular and cutaneous telangiectasia, immunodeficiency, heightened sensitivity to ionizing radiation and susceptibility to developing lymphoreticular malignancy. Supratentorial brain abnormalities have been reported only rarely. In this study, brain MRI was performed in 10 adults with ataxia-telangiectasia having stable neurologic impairment. Read More

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http://dx.doi.org/10.3174/ajnr.A3646DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4106125PMC
January 2014
1 Read

Vascular malformations of the brain.

Authors:
Eric S Nussbaum

Minn Med 2013 May;96(5):40-3

Minnesota Neurovascular and Skull Base Surgery, St. Louis Park, USA.

Vascular malformations of the brain are a heterogeneous group of disorders involving the blood vessels of the brain. These entities are relatively rare; therefore, most primary care physicians are somewhat unfamiliar with their clinical implications and management. Vascular malformations of the brain range from the completely benign capillary telangiectasia to the potentially fatal arterio-venous malformation. Read More

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http://cdn.agilitycms.com/jaocr/Images/Issues/Vol1Iss1RA2Smi
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http://stroke.ahajournals.org/content/40/12/e694.full.pdf
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May 2013
13 Reads

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

Neurosurg Rev 2013 Apr 29;36(2):187-93; discussion 194. Epub 2012 Nov 29.

Department of Neurological Surgery, Brigham and Women's Hospital and Harvard Medical School, 75 Francis St, Boston, MA 02115, USA.

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. Read More

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http://link.springer.com/content/pdf/10.1007/s10143-012-0435
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http://link.springer.com/10.1007/s10143-012-0435-9
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http://dx.doi.org/10.1007/s10143-012-0435-9DOI Listing
April 2013
6 Reads
2 Citations
1.861 Impact Factor

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

Eur Radiol 2013 Apr 31;23(4):1122-30. Epub 2012 Oct 31.

Department of Radiology, Hôpital Saint Joseph, Paris, France.

Objectives: 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.

Methods: Fifty-eight consecutive patients with a suspected intracranial vascular malformation were explored with SWAN and post-contrast MRI sequences at 3 T. Read More

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http://link.springer.com/10.1007/s00330-012-2690-0
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http://dx.doi.org/10.1007/s00330-012-2690-0DOI Listing
April 2013
5 Reads

[Macrocephaly-capillary malformation. A neonatal case].

Arch Pediatr 2012 Sep 9;19(9):917-20. Epub 2012 Aug 9.

Service de néonatologie, pôle de pédiatrie, CHU Clermont-Ferrand, CHU Estaing, 1, place Lucie-Aubrac, 63001 Clermont-Ferrand, France.

Unlabelled: Macrocephaly-cutis marmorata telangiectatica congenita is a multiple congenital anomaly syndrome first described in 1997 in children with macrocephaly, cutis marmorata telangiectatica congenita, and several other abnormalities. Since 2007, this syndrome has been renamed macrocephaly-capillary malformation.

Case Report: The pregnancy was marked by polyhydramnios associated with fetal macrosomia and macrocephaly. Read More

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http://dx.doi.org/10.1016/j.arcped.2012.06.017DOI Listing
September 2012
5 Reads

Macrocephaly-capillary malformation: a report of four Chinese patients and literature review.

Clin Dysmorphol 2012 Apr;21(2):64-8

Clinical Genetic Service, Department of Health, Hong Kong, China.

We report a series of four patients with macrocephaly-capillary malformation (M-CM) who are the first ever reported M-CM patients among Chinese individuals. The salient clinical features and recent diagnostic criteria are discussed. M-CM is a multisystem disease characterized by macrocephaly and cutaneous vascular malformation. Read More

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http://dx.doi.org/10.1097/MCD.0b013e328350509bDOI Listing
April 2012
21 Reads

Pial arteriovenous fistulae in pediatric patients: associated syndromes and treatment outcome.

J Neurointerv Surg 2013 Jan 2;5(1):10-4. Epub 2012 Jan 2.

Department of Neurosurgery, Massachusetts General Hospital, Boston, Massachusetts, USA.

Objective: Pediatric pial arteriovenous fistulae (pAVF) are rare vascular lesions of the CNS, reported to have up to a 25% association with hereditary hemorrhagic telangiectasia. The presentation, treatment and syndromes associated with pAVF in children are reported here.

Design: A pediatric database for pAVF was retrospectively reviewed. Read More

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http://dx.doi.org/10.1136/neurintsurg-2011-010168DOI Listing
January 2013
3 Reads

Susceptibility-weighted MR imaging for diagnosis of capillary telangiectasia of the brain.

AJNR Am J Neuroradiol 2012 Apr 22;33(4):715-20. Epub 2011 Dec 22.

Institute of Diagnostic and Interventional Neuroradiology, University Bern, Inselspital, Bern, Switzerland.

Background And Purpose: 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. Read More

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http://dx.doi.org/10.3174/ajnr.A2893DOI Listing
April 2012
1 Read

MRI findings in giant pontine capillary telangiectasis associated with a developmental venous anomaly.

JBR-BTR 2011 Sep-Oct;94(5):293-4

Department of Radiology, AZ Sint Lucas Hospital, Gent, Belgium.

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. Read More

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January 2012
2 Reads

Stereotactic biopsy of a cerebral capillary telangiectasia after a misleading F-18-FET-PET.

J Neurol Surg A Cent Eur Neurosurg 2012 Nov 1;73(6):407-9. Epub 2012 Aug 1.

University of Rostock, Neurosurgery, Rostock, Germany.

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https://www.thieme-connect.com/products/ejournals/pdf/10.105
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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0032-1313640
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http://dx.doi.org/10.1055/s-0032-1313640DOI Listing
November 2012
2 Reads

Symptomatic capillary telangiectasia of the pons and intracerebral developmental venous anomaly - A rare association.

Coll Antropol 2011 Jan;35 Suppl 1:333-8

University of Zagreb, School of Medicine, Zagreb University of Hospital Centre, Department of Neurology and Croatian Institute for Brain Research, Neuron Diagnostic Centre, Zagreb, Croatia.

Various combinations of vascular malformations of the brain in one lesion have been reported, while others seem to be very rare. In this report, the authors discuss the case of a coexistence of an capillary telangiectasia of the pons and intracerebral venous anomaly. To our knowledge, this is the first report of coexistence of a capillary telangiectasia of the pons and intracerebral venous anomaly apparted from each other. Read More

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January 2011
10 Reads

[Diagnostic imaging of hemangiomas in the brain].

Brain Nerve 2011 Jan;63(1):5-15

Department of Radiology, Teikyo University School of Medicine, Tokyo, Japan.

In this study, we reviewed the findings of diagnostic imaging of cerebral vascular anomalies classified as cerebral cavernous malformation (CCM)/hemangioma, developmental venous anomalies (DVAs)/venous malformation (VM), and capillary telangiectasia. In addition, we described the imaging findings of cavernous sinus hemangioma and orbital hemangioma/vascular anomaly. CCM has 2 forms: a sporadic form characterized by isolated lesions and a familial form characterized by multiple lesions with an autosomal dominant mode of inheritance. Read More

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January 2011
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The potential of capillary birthmarks as a significant marker for capillary malformation-arteriovenous malformation syndrome in children who had nontraumatic cerebral hemorrhage.

J Pediatr Surg 2010 Dec;45(12):2419-22

Douglas Stephens Surgical Research Unit, Murdoch Children's Research Institute, Melbourne, Australia.

Background/purpose: Capillary malformation-arteriovenous malformation (CM-AVM) is a new autosomal dominant disorder with cutaneous capillary malformations (CM) and high-flow cerebral arteriovenous malformations (AVM). Patients may have Parkes-Weber syndrome. This study determined if cutaneous CM are a significant indicator of CM-AVM in children with cerebral bleeds. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00223468100071
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http://dx.doi.org/10.1016/j.jpedsurg.2010.08.043DOI Listing
December 2010
7 Reads

Macrocephaly-capillary malformation: Analysis of 13 patients and review of the diagnostic criteria.

Am J Med Genet A 2010 Dec;152A(12):3101-6

Instituto de Genética Médica y Molecular (INGEMM), IdiPAZ, Hospital Universitario La Paz, Madrid, Spain.

Macrocephaly-capillary malformation (M-CM) is a genetic syndrome of unknown etiology characterized by an enlarged head circumference and patchy, reticular capillary malformation. We describe the clinical features of 13 cases, report on the genome-wide Copy Number Variation characterization of these patients, analyze the main clinical features of this syndrome and propose a modification of the current diagnostic criteria: the inclusion of both overgrowth/asymmetry and neuroimaging alterations as major criteria. Read More

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http://dx.doi.org/10.1002/ajmg.a.33514DOI Listing
December 2010
48 Reads

Symptomatic brain capillary telangiectasia.

JBR-BTR 2010 May-Jun;93(3):138-9

Department of Radiology, UZ Gasthuisberg, KUL, Leuven, Belgium.

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September 2010
3 Reads

The putative role of the venous system in the genesis of vascular malformations.

Neurosurg Focus 2009 Nov;27(5):E9

Mayo Clinic College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Recent clinical and experimental evidence has challenged the traditional concept of the venous system as a "passive" element in the genesis and evolution of intracranial vascular malformations. The authors review the clinical and experimental evidence linking the venous system and its anomalies to the genesis of various intracranial vascular malformations, including dural arteriovenous fistulas, cavernous malformations, parenchymal arteriovenous malformations, and capillary telangiectasia. They also describe the potential significance of different associations of these vascular anomalies. Read More

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http://dx.doi.org/10.3171/2009.8.FOCUS09161DOI Listing
November 2009
2 Reads

Capillary telangiectasias: clinical, radiographic, and histopathological features. Clinical article.

J Neurosurg 2010 Oct;113(4):709-14

Department of Neurosurgery, University of Utah, Salt Lake City, Utah 84132, USA.

Object: 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. Read More

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http://dx.doi.org/10.3171/2009.9.JNS09282DOI Listing
October 2010
3 Reads

Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia.

J Clin Invest 2009 Nov 1;119(11):3487-96. Epub 2009 Oct 1.

Department of Physiology and Functional Genomics, College of Medicine, University of Florida, 1376 Mowry Road, Room 456, Gainesville, Florida 32610, USA.

Arteriovenous malformations (AVMs) are vascular anomalies where arteries and veins are directly connected through a complex, tangled web of abnormal arteries and veins instead of a normal capillary network. AVMs in the brain, lung, and visceral organs, including the liver and gastrointestinal tract, result in considerable morbidity and mortality. AVMs are the underlying cause of three major clinical symptoms of a genetic vascular dysplasia termed hereditary hemorrhagic telangiectasia (HHT), which is characterized by recurrent nosebleeds, mucocutaneous telangiectases, and visceral AVMs and caused by mutations in one of several genes, including activin receptor-like kinase 1 (ALK1). Read More

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http://dx.doi.org/10.1172/JCI39482DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2769195PMC
November 2009
10 Reads

Pontine capillary telangiectasia as visualized on MR imaging causing a clinical picture resembling basilar-type migraine: a case report.

J Neurol 2009 Oct 17;256(10):1775-7. Epub 2009 Jun 17.

A case of presumed pontine capillary telangiectasia in an 18-year-old woman with a clinical diagnosis of basilar-type migraine is reported. Since both are very rare diagnoses, this case provides some evidence to suggest that pontine capillary telangiectasia might cause a clinical picture resembling basilar-type migraine. Read More

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http://dx.doi.org/10.1007/s00415-009-5204-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2758207PMC
October 2009
3 Reads

Increased tissue perfusion promotes capillary dysplasia in the ALK1-deficient mouse brain following VEGF stimulation.

Am J Physiol Heart Circ Physiol 2008 Dec 3;295(6):H2250-6. Epub 2008 Oct 3.

University of California, San Francisco, Department of Anesthesia and Perioperative Care, 1001 Potrero Ave., Rm. 3C-38, San Francisco, CA 94110, USA.

Loss-of-function activin receptor-like kinase 1 gene mutation (ALK1+/-) is associated with brain arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia type 2. Other determinants of the lesional phenotype are unknown. In the present study, we investigated the influence of high vascular flow rates on ALK1+/- mice by manipulating cerebral blood flow (CBF) using vasodilators. Read More

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http://dx.doi.org/10.1152/ajpheart.00083.2008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2614529PMC
December 2008
7 Reads

Pulmonary arteriovenous malformations: how do I embolize?

Authors:
Robert I White

Tech Vasc Interv Radiol 2007 Dec;10(4):283-90

Yale Vascular Malformation Center, Yale University School of Medicine, New Haven, CT 06520, USA.

Pulmonary arteriovenous malformations (PAVM) are high-flow, low-pressure shunts, consisting of a single feeding artery connecting via an aneurysmal sac to a draining vein. The aneurysmal connection is referred to as an aneurysmal sac. The "filter capacity" of the pulmonary capillaries is lost and results in predisposition to brain abscess, stroke, and transient ischemic attack and, when multiple, dyspnea, because of right-to-left shunting and hypoxemia. Read More

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http://dx.doi.org/10.1053/j.tvir.2008.03.007DOI Listing
December 2007
2 Reads

The neurovascular triad: mixed cavernous, capillary, and venous malformations of the brainstem.

J Neurosurg 2007 Dec;107(6):1113-9

Department of Neurosurgery, Sections of Neuroradiology and Pathology, Bellaria Hospital, Bologna, Italy.

Object: The four types of cerebrovascular malformations may sometimes be combined and more often occur in pairs; triads are exceptional. The authors present six patients with the clinicoradiographic profile of mixed vascular malformations of the brainstem, including cavernous malformation (CM), capillary telangiectasia, and developmental venous anomaly (DVA).

Methods: Five patients (one of whom was a child) suffered from hemorrhage, suggesting that this complex association has a high bleeding potential. Read More

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http://dx.doi.org/10.3171/JNS-07/12/1113DOI Listing
December 2007
2 Reads