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 |
More on Brain, Capillary Telangiectasia |
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| Imaging: Brain, Capillary Telangiectasia |
| Follow-up: Brain, Capillary Telangiectasia |
| Multimedia: Brain, Capillary Telangiectasia |
| References |
| Further Reading |
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References
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Acciarri N, Galassi E, Giulioni M, Pozzati E, Grasso V, Palandri G, et al. Cavernous malformations of the central nervous system in the pediatric age group. Pediatr Neurosurg. 2009;45(2):81-104. [Medline].
McInnes M, Fong K, Grin A, ter Brugge K, Blaser S, Halliday W, et al. Malformations of the fetal dural sinuses. Can J Neurol Sci. Jan 2009;36(1):72-7. [Medline].
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Huddle DC, Chaloupka JC, Sehgal V. Clinically aggressive diffuse capillary telangiectasia of the brain stem: a clinical radiologic-pathologic case study. AJNR Am J Neuroradiol. Oct 1999;20(9):1674-7. [Medline].
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McCormick PW, Spetzler RF, Johnson PC, Drayer BP. Cerebellar hemorrhage associated with capillary telangiectasia and venous angioma: a case report. Surg Neurol. Jun 1993;39(6):451-7. [Medline].
Tang SC, Jeng JS, Liu HM, Yip PK. Diffuse capillary telangiectasia of the brain manifested as a slowly progressive course. Cerebrovasc Dis. 2003;15(1-2):140-2. [Medline].
McCormick WF, Hardman JM, Boulter TR. Vascular malformations ("angiomas") of the brain, with special reference to those occurring in the posterior fossa. J Neurosurg. Mar 1968;28(3):241-51. [Medline].
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Auffray-Calvier E, Desal HA, Freund P, et al. [Capillary telangiectasis, angiographically occult vascular malformations. MRI symptomatology apropos of 7 cases]. J Neuroradiol. Dec 1999;26(4):257-61. [Medline].
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Guibaud L, Pelizzari M, Guibal AL, et al. Slow-flow vascular malformation of the pons: congenital or acquired capillary telangiectasia. AJNR Am J Neuroradiol. Oct 1996;17(9):1798-9; discussion 1799-800. [Medline].
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Further Reading
Related eMedicine topics
Intracranial Arteriovenous Malformation
Vascular Malformations of the Spinal Cord
Brain, Arteriovenous Malformation
Chiari I Malformation
Chiari II Malformation
Brain, Cavernous Angiomas
Clinical guidelines
Stereotactic radiosurgery for patients with intracranial arteriovenous malformations (AVM). IRSA - Professional Association. 2003 Sep. 10 pages. NGC:003285
ACR Appropriateness Criteria® cerebrovascular disease. American College of Radiology - Medical Specialty Society. 1996 (revised 2006). 20 pages. NGC:005545
ACR Appropriateness Criteria® headache - child. American College of Radiology - Medical Specialty Society. 1999 (revised 2005). 6 pages. [NGC Update Pending] NGC:004790
Clinical trials
Influence of MMP on Brain AVM Hemorrhage
Tetracycline-Derivatives for Treatment of Cerebral Arteriovenous Malformations and Aneurysms
Comparison of Abnormal Cortical Development in Brain Malformations on Postmortem Imaging With Autopsy
Genetic Analysis of the Chiari I Malformation
Duragen Versus Duraguard in Chiari Surgery
Keywords
capillary telangiectasia, brain capillary telangiectasia, capillary angioma, CTSs, abnormally dilated capillaries, brain vascular malformation, occult cerebrovascular malformations, OCVMs, cavernous angiomas, cavernomas, venous angiomas
