Prospective Hemorrhage Rates of Cerebral Cavernous Malformations in Children and Adolescents Based on MRI Appearance

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Fig 1.
CCM types according to the Zabramski classification. Graphic illustration (left 3 rows) and corresponding MR images (right 3 rows) of CCMs according to the MR imaging classification of Zabramski et al.³ Type IV CCM: arrowhead indicates a small T2* lesion. Type V: arrows indicate parts of the actual CCMs that are visible in the center of the hemorrhage; however, the CCM is not fully distinguishable from hemorrhage.
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Fig 2.
Hemorrhage-free survival. Kaplan-Meier diagram illustrates hemorrhage-free survival depending on the Zabramski CCM type.
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Fig 3.
Cumulative hazard. Diagram illustrates the cumulative hazard for hemorrhage according to our proposed CCM classification, 1) CCM with signs of acute or subacute hemorrhage, 2) CCM without signs of acute or subacute hemorrhage, and 3) dot-sized CCMs.

Table 1:

Original MRI classification of CCMs according to Zabramski et al³

Lesion Type	MRI Signal Characteristics	Pathologic Characteristics
Type I	T1: hyperintense core	Subacute hemorrhage, surrounded by a rim of hemosiderin-stained macrophages and gliotic brain
Type I	T2: hyper- or hypointense core with surrounding hypointense rim
Type II	T1: reticulated mixed-signal core	Loculated areas of hemorrhage and thrombosis of varying ages, surrounded by gliotic, hemosiderin-stained brain; in large lesions, areas of calcification may be seen
Type II	T2: reticulated mixed-signal core with surrounding hypointense rim
Type III	T1: iso- or hypointense core	Chronic resolved hemorrhage, with hemosiderin staining within and around the lesion
	T2: hypointense with a hypointense rim that magnifies the size of the lesion
	GE: hypointense with greater magnification than T2
Type IV	T1: poorly seen or not visualized at all	Two lesions in the category were pathologically documented as telangiectasias
	T2: poorly seen or not visualized at all
	GE: punctate hypointense lesions

Table 2:

Hemorrhage-free survival^{^a}

CCM Type	Mean Hemorrhage-Free Survival
	Estimator	Standard Error	95% CI
	Estimator	Standard Error	Lower	Upper
I	18.82	1.52	15.84	21.80
II	24.92	2.91	19.21	30.63
III	27.88	0.38	27.13	28.63
IV	37.78	0.40	37.00	38.57
V	21.34	2.67	16.10	26.59
I, II, V	22.63	1.50	19.68	25.57
All	36.06	0.59	34.91	37.21

↵a Demonstrating mean hemorrhage-free survival in months depending on the extended CCM type of Zabramski et al.³

Table 3:

Prospective hemorrhage rates in the literature^{^a}

Study	No. of Patients	No. of Lesions	Hemorrhage Assessment	Hemorrhage Rate without Prior Hemorrhage	Hemorrhage Rate with Prior Hemorrhage
Al-Shahi Salman et al¹	134	NI	MRI/clinical	2.4%	29.5%
Kondziolka et al⁸	122	NI	MRI/clinical	0.6%	4.5%
Moriarity et al¹⁵	68	228	MRI/clinical	3.1%^{^b}	NI
Porter et al¹⁶	173	NI	MRI/clinical	1.6%^{^b}	NI
Robinson et al¹⁷	57	66	MRI/clinical	0.7%^{^b,^c}	NI

Note:—NI indicates not indicated.
↵a Hemorrhage rates of supratentorial and infratentorial CCMs found in prospective registry studies. Hemorrhage rates are in patient-years.
↵b Overall hemorrhage rate.
↵c Hemorrhage rates in lesion-years.