Dilated capillary blood vessels, seperated by normal neural tissue; rarely, symptomatic (i.e., hemorrhage) Hereditary form with multiple cutaneous and hereditary lesions is Osler-Weber-Rendu, which is often symptomatic.

Location: most pons; cerebral cortex, subcortical white matter. Angiogram usually negative. CT: cerebellar atrophy. Lesion usually image-occult.

1: AJNR Am J Neuroradiol. 2001 Sep;22(8):1553-5.

MR imaging and histologic features of capillary telangiectasia of the basal
ganglia.

Castillo M, Morrison T, Shaw JA, Bouldin TW.

Department of Radiology, University of North Carolina School of Medicine, Chapel
Hill, NC 27599-7510, USA.

Capillary telangiectasias are being recognized with increasing frequency on MR
imaging studies. Most are located in the brain stem and show slightly increased
signal intensity on T2-weighted images, low signal intensity on T2*-weighted
images (reflecting the presence of deoxyhemoglobin), and contrast enhancement.
These findings are considered fairly typical for capillary telangiectasia, and
pathologic correlation is not generally pursued. We present a case of a proven
capillary telangiectasia in the basal ganglia. The imaging features of the
lesion were identical to those described for capillary telangiectasias in the
brain stem.

Publication Types:
Case Reports

[PubMed REF] [PubMed - indexed for MEDLINE]



2: AJNR Am J Neuroradiol. 2000 Sep;21(8):1483-5.

Abnormal white matter signal in ataxia telangiectasia.

Ciemins JJ, Horowitz AL.

Department of Radiology, Resurrection Medical Center, Chicago, IL 60631, USA.

We report herein the MR finding of multiple, small, hypointense white matter
foci on T1- and T2-weighted images obtained from a patient with ataxia
telangiectasia. To our knowledge, this finding has not been previously reported
in association with a patient with this disease entity.

Publication Types:
Case Reports

[PubMed REF] [PubMed - indexed for MEDLINE]



3: Neurosurg Clin N Am. 1999 Jul;10(3):419-32.

Diagnostic imaging of angiographically occult vascular malformations.

Rabinov JD.

Department of Neuroradiology, Harvard Medical School, Massachusetts General
Hospital, Boston, Massachusetts 02114, USA.

Occult vascular malformations of the central nervous system include cavernous
malformations, capillary telangiectasis, and developmental venous anomalies
(DVAs). Cavernous malformations are prone to multiple small hemorrhages and may
enlarge with time. They have a heterogeneous appearance on CT and MR imaging
with areas of hemorrhage in various states of evolution and sometimes
calcifications. Developmental venous anomalies and capillary telangiectasis are
usually incidental findings on imaging studies. They usually are not the cause
of pathology by themselves, although DVAs can be associated with cavernous
malformations in 8% to 33% of cases.

Publication Types:
Review
Review Literature

[PubMed REF] [PubMed - indexed for MEDLINE]



4: Neurol Med Chir (Tokyo). 1998;38 Suppl:250-4.

Surgery of angiomas in the brainstem with a stress on the presence of
telangiectasia.

Fukui M, Matsushima T, Ikezaki K, Natori Y, Inamura T, Ohara S, Kawamura T.

Department of Neurosurgery, Neurological Institute, Kyushu University Faculty of
Medicine, Fukuoka.

This report deals with the surgery of angiomas other than arteriovenous
malformation in the brainstem. The surgical cases were three cavernomas, two
telangiectasias, and two venous malformations. We performed surgery when an
angioma bled and the resulting hematoma was situated near the surface of the
brainstem or the fourth ventricle. The cases were operated on at the subacute or
chronic stages after hemorrhage. Although a magnetic resonance (MR) image showed
a subacute or chronic localized hematoma with a low intensity rim, the case was
not always a cavernoma, but a telangiectasia. Cavernomas could be totally
removed, but telangiectasia could not. In the cases of medullary venous
malformation the diagnosis was obtained radiologically, and when the hematoma
was large, only hematoma evacuation was performed. In all cases the
postoperative Karnofsky scores were improved or unchanged. Postoperative
rebleeding in the hematoma cavity continued insidiously in a case of
telangiectasia. The abnormal vessels of telangiectasia in the brainstem were
preoperatively not visualized by cerebral angiography or MR imaging, but became
visualized by enhanced MR imaging after evacuation of hematoma in two cases. It
is stressed that an angioma with a hematoma intensity core surrounded by a low
intensity rim on MR images is not always a cavernoma, but possibly is a
telangiectasia.

Publication Types:
Case Reports

[PubMed REF] [PubMed - indexed for MEDLINE]



5: Radiology. 1997 Dec;205(3):797-805.

Brain capillary telangiectasia: MR imaging appearance and clinicohistopathologic
findings.

Lee RR, Becher MW, Benson ML, Rigamonti D.

Department of Radiology and Radiological Science, Johns Hopkins University
School of Medicine, Baltimore, Md., USA.

PURPOSE: To demonstrate the clinical and magnetic resonance (MR) imaging
findings of brain capillary telangiectasia and compare them with postmortem
specimens. MATERIALS AND METHODS: MR images obtained in and clinical histories
of 18 adult patients with a presumed diagnosis of capillary telangiectasia
examined within 3 years were retrospectively reviewed. All patients had
undergone MR imaging with conventional T1- and T2-weighted spin-echo sequences
and gadolinium-enhanced T1-weighted and susceptibility-sensitive gradient-echo
(GRE) sequences. No biopsies had been performed. Fourteen patients had undergone
clinical and MR imaging follow-up (median, 11 months). Postmortem tissues from
three cases of histopathologically confirmed capillary telangiectasia were
imaged. RESULTS: All lesions were small, homogeneously enhancing, and hypo- to
isointense on T1-weighted images and iso- to slightly hyperintense on
proton-density- and T2-weighted images. None was hypointense on proton-density-
or T2-weighted images. All lesions showed marked GRE signal loss. None had
changed at follow-up. Two patients had multiple classic cerebral cavernous
angiomas. The three specimens showed no abnormal susceptibility and contained no
hemosiderin at tissue analysis. CONCLUSION: Capillary telangiectasia has mild
contrast material enhancement but is otherwise undetectable on conventional MR
images. It lacks the "hemosiderin rim" of cavernous angioma and demonstrates
increased susceptibility only on GRE images, likely owing to blood
oxygen-level-dependent contrast. GRE is essential in diagnosing brain capillary
telangiectasia, which could otherwise be misdiagnosed as neoplasia, subacute
infarction, or demyelination.

[PubMed REF] [PubMed - indexed for MEDLINE]



6: AJNR Am J Neuroradiol. 1996 Jan;17(1):71-8.

Comment in:
AJNR Am J Neuroradiol. 1996 Oct;17(9):1798-9; author reply 1799-800.

Slow-flow vascular malformations of the pons: capillary telangiectasias?

Barr RM, Dillon WP, Wilson CB.

Department of Radiology, University of California San Francisco, USA.

PURPOSE: To report clinical and MR features that suggest telangiectatic vascular
malformations of the pons: METHODS: The MR scans and clinical data of 12
patients demonstrating an enhancing pontine lesion with minimal or no signal
abnormality on T2-weighted images were reviewed. None of the patients underwent
angiography or biopsy. Follow-up scans, available for all patients between 3
weeks and 40 months (range, 11.5 months), were reviewed. RESULTS: The patients
presented with a variety of symptoms including headache (n = 4), vertigo (n =
3), gait abnormality (n = 3), and hearing loss (n = 2). Two were referred for
biopsy or treatment of presumed pontine glioma. On precontrast MR, 3 of 12
lesions were isointense on both T1- and T2-weighted images. Three of 12 lesions
were slightly hypointense on T1-weighted images and 8 of 12 were slightly
hyperintense on T2-weighted images. Postgadolinium images showed a discrete
focus of enhancement with irregular or brushlike borders. Eight of 12 had an
anomalous draining vessel from the lesion to the surface of the pons. None
demonstrated mass effect or hemorrhage. Gradient-echo sequences in 7 patients
all showed marked T2 shortening, despite the absence of hemorrhage on either T1-
or T2-weighted images. None of the follow-up scans showed radiographic or
clinical progression. CONCLUSION: The benign clinical course, lack of mass
effect, and minimal or no T2 prolongation argue against neoplasm and instead
indicate a vascular cause. We suspect the decreased signal on gradient-echo
sequences represents elevated intravascular deoxyhemoglobin from stagnant blood
flow. The findings are atypical for cavernous angioma or classic venous
malformation. Although pathologic confirmation is lacking, the radiographic
features are most consistent with capillary telangiectasia or a transitional
capillary-venous malformation. Despite the absence of progression or hemorrhage
in any of the patients to date, the long-term prognosis currently is unknown. We
emphasize the importance of recognizing the nonneoplastic nature of these
lesions.

[PubMed REF] [PubMed - indexed for MEDLINE]