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Review
. 2016 May 24;86(21):1984-91.
doi: 10.1212/WNL.0000000000002701. Epub 2016 Apr 22.

Natural history of cavernous malformation: Systematic review and meta-analysis of 25 studies

Shervin Taslimi  1 , Amirhossein Modabbernia  1 , Sepideh Amin-Hanjani  1 , Fred G Barker 2nd  1 , R Loch Macdonald  2
Affiliations
Review

Natural history of cavernous malformation: Systematic review and meta-analysis of 25 studies

Shervin Taslimi et al. Neurology. .

Abstract

Objective: We pooled the results of studies on natural history of cavernous malformations (CM) to calculate point estimates and investigate main sources of heterogeneity.

Methods: We searched MEDLINE, EMBASE, and ISI Web of Science for relevant studies published before May 2015. We used fixed or random effects models and meta-regression to pool the data.

Results: Twenty-five studies were entered into the meta-analysis (90-1,295 patients depending on the analysis). Bleeding was defined as symptomatic hemorrhage plus radiologic evidence of hemorrhage. Sources of heterogeneity were identified as mixture of hemorrhage and rehemorrhage, mixture of rehemorrhage before and after 2 years of first bleeding, brainstem vs other locations, and calculation method. The rehemorrhage rate was higher than the hemorrhage rate (incidence rate ratio 16.5, p < 0.001, 95% confidence interval [CI] 9.7-28.0). Rehemorrhage within 2 years of the first hemorrhage was higher than after that (incidence rate ratio 1.8, p = 0.042, 95% CI 1.5-2.0). In two metaregression models, rough estimate of the annual incidence rate of hemorrhage was 0.3% (95% CI 0.1%-0.5%) and 2.8% (2.5%-3.3%) per person year in nonbrainstem and brainstem lesions and rough estimate of annual rehemorrhage rate per person year was 6.3% (3%-13.2%) and 32.3% (19.8%-52.7%) in nonbrainstem and brainstem lesions. Median time to rehemorrhage was 10.5 months. Posthemorrhage full recovery was 38.8%/person-year (28.7%-48.8%). Posthemorrhage full recovery or minimal disability was 79.5%/person-year (74.3%-84.8%). Mortality after bleeding was 2.2%.

Conclusions: The incidence of symptomatic hemorrhage or rehemorrhage is higher in brainstem lesions. First symptomatic hemorrhage increases the chance of symptomatic rehemorrhage, which decreases after 2 years.

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Figures

Figure 1
Figure 1. Rehemorrhage rate vs hemorrhage rate
Dashed vertical line represents pooled value. Analysis was done on logarithmic scale and the x-axis is shown with transformation to normal scale. REM = random effect model. On the x-axis, 1 represents null hypothesis.
Figure 2
Figure 2. Percentage of brainstem cavernous malformation lesions in each study and the overall reported incidence rate of rehemorrhage
Radius of each circle represents the precision of each study.
Figure 3
Figure 3. Percentage of brainstem cavernous malformation lesions in each study and the overall reported incidence rate of hemorrhage
Radius of each circle represents the precision of each study.
See this image and copyright information in PMC

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References

    1. Washington CW, McCoy KE, Zipfel GJ. Update on the natural history of cavernous malformations and factors predicting aggressive clinical presentation. Neurosurg Focus 2010;29:E7. - PubMed
    1. Dziedzic T, Kunert P, Matyja E, Ziora-Jakutowicz K, Sidoti A, Marchel A. Familial cerebral cavernous malformation. Folia Neuropathol 2012;50:152–158. - PubMed
    1. Wilczynski NL, Haynes RB. Optimal search strategies for detecting clinically sound prognostic studies in EMBASE: an analytic survey. J Am Med Inform Assoc 2005;12:481–485. - PMC - PubMed
    1. Wilczynski NL, Haynes RB. Developing optimal search strategies for detecting clinically sound prognostic studies in MEDLINE: an analytic survey. BMC Med 2004;2:23. - PMC - PubMed
    1. Barker FG, II, Amin-Hanjani S, Butler WE, et al. Temporal clustering of hemorrhages from untreated cavernous malformations of the central nervous system. Neurosurgery 2001;49:15–24; discussion 24–25. - PubMed

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