eMedicine Specialties > Radiology > Brain/Spine
Brain, Capillary Telangiectasia
Updated: Apr 21, 2009
Introduction
Background
Capillary telangiectasias (CTSs) are small areas of abnormally dilated capillaries within otherwise normal brain tissue. Although CTSs most commonly occur in the pons, they have been described throughout the brain.
CTSs constitute one of the 4 types of vascular malformations of the brain, along with arteriovenous malformation, cavernous angioma, and venous angioma. CTSs are occasionally found in conjunction with these other vascular malformations. Typically, such cases involve CTSs and one other type of malformation, but the triad of CTSs, cavernous malformation, and developmental venous anomaly has been reported.1,2,3,4,5,6
Axial contrast-enhanced T1-weighted MRI demonstrates a subtle area of enhancement in the right parietal subcortical white matter in a patient with capillary telangiectasia (arrow) (same patient as in Image below).
Coronal contrast-enhanced T1-weighted MRI reveals enhancement without mass effect in a patient with capillary telangiectasia (arrow) (same patient as Images above and below).
Fluid-attenuated inversion recovery MRI obtained at the same level as in Image above shows no abnormal signal intensity in this area; this finding confirms the diagnosis of capillary telangiectasia. No abnormalities were present on the T2-weighted or nonenhanced T1-weighted images (same patient as in Image above).
Although the MRI appearance of CTSs is fairly specific, distinguishing them from cavernous angiomas without hemorrhage is often impossible. With both types of lesions, angiographic findings are typically negative. These features have led to the adoption of the term occult cerebrovascular malformations (OCVMs).
Most CTSs are clinically insignificant and are discovered as incidental findings on MRI. Rarely, however, CTSs are associated with hemorrhage.
Frequency
United States
CTSs are estimated to account for 16-20% of all brain vascular malformations. From autopsy studies, the prevalence is estimated to be 0.4%, although many of these CTSs are not visible on imaging studies.
Mortality/Morbidity
- One death in a pediatric patient reportedly resulted from a locally aggressive CTS.7
- Two cases of major hemorrhages from CTSs have been reported.8,9
- Diffuse, slowly progressive CTSs associated with neurologic degeneration in an adult patient has been reported.10
Race
No known race predilection exists.
Sex
No known sex predilection exists.
Age
CTSs can occur in patients of any age.
Presentation
Anatomy and Pathology
CTSs are formed by a network of aneurysmally dilated capillaries that are usually located in a section of normal brain tissue, although adjacent areas of gliosis and small amounts of hemorrhage have been described. The presence of normal brain tissue between the capillaries is a pathologic characteristic that distinguishes CTSs from cavernous angiomas, although they may resemble each other on imaging studies.
CTSs are typically small, ranging from a few millimeters to several centimeters in size. The pons is the most common site. McCormick et al described 30 CTSs in the posterior fossa, most of which involved the pons, and 22 in the supratentorial brain.11 CTSs can occur anywhere in the brain or spinal cord, however.
CTSs are usually solitary, but they may also be found in association with other brain vascular malformations, such as cavernous angiomas and venous angiomas (see Image 5). The association of CTSs with cavernomas is such that the 2 anomalies have been suggested to represent 2 points on the spectrum of a single disease process.
Signs and symptoms- Almost all patients with CTS are asymptomatic; CTSs are almost always an incidental radiologic finding.
- CTSs have, however, been associated with minor symptoms such as vertigo, headache, and dizziness, as well as weakness and seizures.
- No distinguishing clinical features are associated with CTS.
- CTSs rarely hemorrhage, but 2 cases of major hemorrhage have been reported in the literature, as well as 1 case of degenerative disease with slowly progressive, diffuse CTSs and 1 death due to local invasion.7,8,9,10
Preferred Examination
Although CTSs are occasionally visible on CT scans, the ideal means of detecting and imaging the lesions is contrast-enhanced MRI, which should include a gradient-echo sequence (eg, fast low-angle shot, gradient-recalled echo [GRE]). Angiography of any sort (ie, magnetic resonance angiography, computed tomographic angiography, conventional angiography) is not indicated because the lesions are typically occult on angiograms.
Limitations of Techniques
- MRI with contrast medium and fast low-angle shot imaging is necessary to detect most CTSs.
- Despite the high sensitivity of current MRI, many CTSs are not detectable and are found only at autopsy.
Differential Diagnoses
| [Brain, MRI Appearance Of Hemorrhage] | Brain, Arteriovenous Malformation |
| [Multiple Sclerosis, Brain] | Brain, Cavernous Angiomas |
| Arachnoid Cyst | Brain, Lymphoma |
| Arachnoid Cyst | Multiple Sclerosis, Spine |
Computed Tomography
Findings
Nonenhanced CT studies typically do not depict CTS, and most lesions are not visible even after the administration of contrast medium. When visible, CTSs appear as a small area of subtle contrast enhancement. Rarely, a tiny calcification may be associated with the lesion.
Degree of Confidence
Negative CT findings do not exclude CTS because most lesions are occult. The appearance on contrast-enhanced CT scans is nonspecific.
Magnetic Resonance Imaging
Axial contrast-enhanced T1-weighted MRI obtained through the pons demonstrates an area of mild enhancement without mass effect in a patient with a capillary telangiectasia (same patient as in Image below).
Axial T2-weighted MRI demonstrates no obvious abnormality; this finding confirms the diagnosis of capillary telangiectasia (same patient as in Image above).
Pontine capillary telangiectasia in a 39-year-old woman with dizziness. Note the lacy enhancement characteristic of this lesion. No abnormality was present on the T2-weighted MRI.
Axial enhanced T1-weighted MRI demonstrates the typical lacy enhancement pattern of a capillary telangiectasia. Image courtesy of Dr. Robert Koenigsberg, Professor of Radiology, MCP Hahnemann University.
Findings
MRI findings in CTS are variable, but contrast enhancement is required for diagnosis or even detection in almost all cases. Lee et al evaluated 18 patients and found enhancement in all patients, with little or no abnormal signal intensity on T2-weighted images12 ; however, increased signal intensity may be seen occasionally. T1-weighted images may show isointensity. The enhancement pattern is described as lacelike (see Images above and Images 1, 3, 9 in Multimedia) and usually subtle.
Occasionally, an associated prominent draining vein is present (see Image below and Image 5 in Multimedia).
Axial fast low-angle shot gradient-recalled echo MRI obtained through the pons shows a linear area of decreased signal extending from the inferior edge of the malformation (arrows). This finding indicates that the lesion may be a combined capillary telangiectasia and developmental venous anomaly because it has characteristics of both.
Use of susceptibility-weighted MRI to diagnosis pontine CTS has been reported.13 The patient's lesion did not demonstrate signal loss on conventional gradient-echo MRI, but susceptibility-weighted MRI showed marked signal loss of the lesion.
Gradient-echo sequences can facilitate the detection and diagnosis of CTS (see Image below and Image 4 in Multimedia). Both Barr et al14 and Lee et al12 describe susceptibility dephasing in all CTS lesions that are imaged by using GRE sequences. The exact reason why this susceptibility occurs is not clear, because hemosiderin and calcifications are not typically found on pathologic analysis. However, Lee and colleagues surmise that the hemoglobin within may be only partly converted to deoxyhemoglobin because the blood is relatively stagnant due to capillary dilatation; therefore, it has only a mild paramagnetic effect. This theory explains the imaging differences between cavernous angiomas and CTSs.
Axial fast low-angle shot MRI demonstrates decreased signal intensity associated with the brain capillary telangiectasia. This finding is characteristic of capillary telangiectasia, but it is also seen in developmental venous anomalies. The decreased signal intensity is not a result of hemorrhage, but rather, it is from the deoxyhemoglobin in the blood flowing in the malformations.
Axial contrast-enhanced T1-weighted MRI demonstrates a subtle area of enhancement in the right parietal subcortical white matter in a patient with capillary telangiectasia (arrow) (same patient as in Image below).
Coronal contrast-enhanced T1-weighted MRI reveals enhancement without mass effect in a patient with capillary telangiectasia (arrow) (same patient as Images above and below).
Fluid-attenuated inversion recovery MRI obtained at the same level as in Image above shows no abnormal signal intensity in this area; this finding confirms the diagnosis of capillary telangiectasia. No abnormalities were present on the T2-weighted or nonenhanced T1-weighted images (same patient as in Image above).
Because cavernous angiomas demonstrate susceptibility dephasing on GRE images, as a result of the presence of hemosiderin and sometimes calcifications, they also have markedly decreased signal intensity on T2-weighted images. Conversely, T2-weighted images of CTSs typically show no abnormality because the deoxyhemoglobin should not cause decreased signal intensity (see Images below and Images 2, 6-8 in Multimedia).
Degree of Confidence
The finding of a small area of enhancement without an abnormality or mass effect on a T2-weighted image and the finding of susceptibility dephasing on GRE images is strongly suggestive of a CTS, particularly if it the lesions is in the pons. If doubt exists, short-term follow-up studies can be performed to document stability of the lesion.
Although capillary telangiectasia is usually not visible on T2-weighted and nonenhanced T1-weighted images, abnormalities on T2-weighted images can be associated with capillary telangiectasias. However, the presence of such signal intensity should prompt consideration of alternative diagnoses.
Angiography
Findings
Angiography of any sort (ie, magnetic resonance angiography, computed tomographic angiography, conventional angiography) is not indicated because CTSs are typically angiographically occult. However, tiny capillary vessels may be seen on the venous phase.
Intervention
Medicolegal Pitfalls
- Suggesting that a CTS represents a more clinically important lesion, such as a glioma, may result in unnecessary intervention and associated morbidity and mortality. This pitfall can be avoided by recognizing the possibility of CTS, and in case of doubt, by following up the lesion with serial MRIs.
- Conversely, a CTS can be misdiagnosed as another lesion. The use of GRE sequences at MRI assists in the diagnosis, and serial MRI helps in confusing cases.
Multimedia
Media file 1: Axial contrast-enhanced T1-weighted MRI obtained through the pons demonstrates an area of mild enhancement without mass effect in a patient with a capillary telangiectasia (same patient as in Image below).
Media file 2: Axial T2-weighted MRI demonstrates no obvious abnormality; this finding confirms the diagnosis of capillary telangiectasia (same patient as in Image above).
Media file 3: Pontine capillary telangiectasia in a 39-year-old woman with dizziness. Note the lacy enhancement characteristic of this lesion. No abnormality was present on the T2-weighted MRI.
Media file 4: Axial fast low-angle shot MRI demonstrates decreased signal intensity associated with the brain capillary telangiectasia. This finding is characteristic of capillary telangiectasia, but it is also seen in developmental venous anomalies. The decreased signal intensity is not a result of hemorrhage, but rather, it is from the deoxyhemoglobin in the blood flowing in the malformations.
Media file 5: Axial fast low-angle shot gradient-recalled echo MRI obtained through the pons shows a linear area of decreased signal extending from the inferior edge of the malformation (arrows). This finding indicates that the lesion may be a combined capillary telangiectasia and developmental venous anomaly because it has characteristics of both.
Media file 6: Axial contrast-enhanced T1-weighted MRI demonstrates a subtle area of enhancement in the right parietal subcortical white matter in a patient with capillary telangiectasia (arrow) (same patient as in Image below).
Media file 7: Coronal contrast-enhanced T1-weighted MRI reveals enhancement without mass effect in a patient with capillary telangiectasia (arrow) (same patient as Images above and below).
Media file 8: Fluid-attenuated inversion recovery MRI obtained at the same level as in Image above shows no abnormal signal intensity in this area; this finding confirms the diagnosis of capillary telangiectasia. No abnormalities were present on the T2-weighted or nonenhanced T1-weighted images (same patient as in Image above).
Media file 9: Axial enhanced T1-weighted MRI demonstrates the typical lacy enhancement pattern of a capillary telangiectasia. Image courtesy of Dr. Robert Koenigsberg, Professor of Radiology, MCP Hahnemann University.
References
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Keywords
capillary telangiectasia, brain capillary telangiectasia, capillary angioma, CTSs, abnormally dilated capillaries, brain vascular malformation, occult cerebrovascular malformations, OCVMs, cavernous angiomas, cavernomas, venous angiomas
Contributor Information and Disclosures
Author
Andrew L Wagner, MD, Assistant Professor of Radiology, Instructional Faculty, University of Virginia School of Medicine; Director of Neuroradiology, Department of Radiology, Rockingham Memorial Hospital
Andrew L Wagner, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, and Radiological Society of North America
Disclosure: Nothing to disclose.
Medical Editor
Robert A Koenigsberg, DO, MSc, FAOCR, Professor, Director of Neuroradiology, Program Director, Diagnostic Radiology and Neuroradiology Training Programs, Department of Radiology, Hahnemann University Hospital, Drexel University College of Medicine
Robert A Koenigsberg, DO, MSc, FAOCR is a member of the following medical societies: American Osteopathic Association, American Society of Neuroradiology, Radiological Society of North America, and Society of NeuroInterventional Surgery
Disclosure: Nothing to disclose.
Pharmacy Editor
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
CME Editor
Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Chief Editor
James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.
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