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

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Abstract

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.

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Authors and Affiliations

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

    Bradley A. Gross, A. John Popp & Rose Du

  2. Department of Radiology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, 02115, USA

    Ajit S. Puri

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Correspondence to Rose Du.

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Comments

Ernst Delwel, Rotterdam, The Netherlands

The authors have performed a meta-analysis and review of the literature on the infrequently addressed cerebral capillary telangiectasia (CCT). The resulting article is interesting and useful.

They have concluded that the lesion has a low prevalence with almost always a benign course and is located most often in the pons and basal ganglia. Symptoms rarely occur, and in many cases, one may argue whether described symptoms are indeed related to the CCT. When symptoms occur, they are explained by hypoperfusion of the perilesional parenchyma or by epileptic phenomena and are related to the larger CCTs. Hemorrhage from CCT is exceedingly rare contrary to a cavernous malformation (CM). Like a CM, CCT probably is an acquired angiographically occult vascular malformation, related to impaired venous outflow through a venous anomaly.

CCT has typical radiological features and can be distinguished from CM. The authors have demonstrated that CCT is a benign lesion which rarely causes symptoms besides the fact that the lesion is often less amenable for surgery as they have a predilection for pons and basal ganglia and consist of capillaries interspersed among neural parenchyma. Apart from rare exceptions, surgical treatment of CCT should be avoided.

Vini G. Khurana, Canberra, Australia

The authors have written an excellent article regarding a somewhat neglected area in the neurosurgery literature, namely, cerebral capillary telangiectasis (CCT). Their paper incorporates a meta-analysis of 203 CCT patients drawn from ten published series, and a review of the literature. Despite the expected limitations in the meta-analysis owing to methodological variations in the studies analyzed by the authors, the demographic, radiological, and natural history data are succinctly and intuitively summarized.

Two take-home messages of this paper are as follows: First, the differential diagnosis of a CCT, namely cavernous malformation (CM) and developmental venous anomaly (DVA), should be carefully considered by clinicians (see Table 3). This is in order to confirm if the lesion is a CCT and, if so, to determine if the CCT is isolated or, alternatively, anatomically colocalized with another lesion such as a CM or DVA. Second, the authors confirm that CCT is a truly benign entity with an unremarkable natural history, and only in exceedingly rare (i.e., “reportable”) circumstances would an operative intervention be considered. That is, “leave it alone” appears to be the rule of thumb as far as an isolated CCT is concerned!

Aki Laakso, Helsinki, Finland

Capillary telangiectasias of the brain are lesions often neglected by neurosurgeons—neglected for the very reasonable reason that they are mostly very benign lesions with no clinical consequence to the patient. The authors have performed a helpful systematic literature review for clinical reports concerning cerebral capillary telangiectasias. Their synthesis of the existing data confirms the idea that, from the neurosurgical point of view, these lesions are best left untouched. They are usually asymptomatic, and, despite being vascular anomalies, show almost nonexistent propensity to bleed. Moreover, their typical location in pons or basal ganglia makes them undesirable surgical targets even when suspected as culprits for certain symptoms. Their radiological appearance may also be rather variable, as pointed out by the authors, and once seen in MRI imaging, appropriate identification of these lesions obviously saves the patient from unnecessary concerns, follow-ups, and perhaps even from unindicated surgery. Having said that, it also seems that in very rare cases these lesions may indeed become symptomatic. Given the rarity of these instances, however, it is always a wise precaution to consider other possible etiologies for the patient’s symptoms, especially if the symptoms are not clearly localizing.

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Gross, B.A., Puri, A.S., Popp, A.J. et al. Cerebral capillary telangiectasias: a meta-analysis and review of the literature. Neurosurg Rev 36, 187–194 (2013). https://doi.org/10.1007/s10143-012-0435-9

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