cavernous_malformation

Cavernous malformation

usually angiographically occult. May show up on MRI (open channels → flow void on T2WI, previous hemorrhage →“popcorn” pattern especially on T2* gradient echo) or contrast CT

● low-flow. No intervening neural parenchyma, no arteries. Associated with venous anomaly (represents venous outflow and should be preserved)

XRT is a risk factor for developing cavernous malformation

● presentation: usually seizures:

The 5-year risk of first-time seizure was 6% among CM patients presenting with symptoms vs. 4% for those with incidental CMs

● Hemorrhage: rare, the risk is difficult to predict (see Cavernous malformation hemorrhage)

● treatment:

a) surgery is the treatment of choice for symptomatic accessible lesions

b) radiosurgery should not be considered as a treatment option


Cavernous angioma (CA) is also known as cavernoma, cavernous hemangioma, and cerebral cavernous malformation (CCM) (National Library of Medicine Medical Subject heading unique ID D006392).

It is classified as a group of vascular malformations, where a collection of dilated blood vessels form a tumor.

Hubert von Luschka was the first to describe a cerebral (Cavernöse Blutgeschwulst) (cavernous malformation) in 1854.

The terms cavernoma, cavernous hemangioma, cavernous angioma, and cavernous malformation are used interchangeably and appear equally frequent in the literature. The terms cavernoma, cavernous angioma, and cavernous hemangioma suggest that it is a neoplastic lesion, which is inaccurate as cavernous malformations are true vascular malformations.

The term cavernous hemangioma is also confusing as cavernous hemangiomas in the cavernous sinus or in the orbit; both are slow-growing neoplastic diseases unrelated to vascular malformation

see Cavernous sinus hemangioma

Cavernous Malformation Classification.

see Cavernous malformation etiology.

Cavernous malformation (CCM)s are benign, low-flow vascular lesions with thin elastic endothelial walls that lack adventitial smooth muscle and frequently present with little intervening brain parenchyma.

Microscopically, cavernous angiomas consist of sclerotic, variably calcified compact vessels in a honeycomb pattern.

Unlike telangiectasis and arteriovenous malformation these lesions do not contain interstitial parenchyma.

The cells that form the vessels do not form the necessary junctions with surrounding cells and the structural support from the smooth muscle is hindered causing leakage into the surrounding tissue.

It is the leakage of blood, known as a hemorrhage from these vessels that causes a variety of symptoms known to be associated with this disease.

Cavernous malformation diagnosis.

see Cavernous malformation treatment.

When cavernous malformations can be completely removed, the risk of further growth or hemorrhage is essentially permanently eliminated 1) (however, recurrence of symptoms has been reported after partial and even seemingly-complete removal 2) 3). For CMs treated surgically, patients need to be aware that post-op neurologic worsening is very common, especially with brainstem CMs 4) Worsening may be transient, 5) but may take months to resolve.


Herten et al. performed a cross-sectional observational study on patients with cavernous malformation of the CNS (CCM). admitted to the University Hospital Essen. from 01.11.2017-10.01.2020 using standardized interviews (short-form -36 questionnaire, hospital anxiety and depression score (HADS-A/D), CCM-perception questionnaire). Including criteria was diagnosis of an untreated CCM and information about the diagnosis in a specialized CCM consultation. HRQOL data was analyzed and compared to German normal population. Uni- and multivariate analyses were carried out to identify variables with impact on outcome.

219 (93%) of 229 eligible patients were included. Mean age was 46.3 ±14.7 (18-86) years, 136 (62%) were female. 98 (45%) patients presented with symptomatic hemorrhage and 17 (8%) with repetitive SH. 92 (42%) patients were asymptomatic. 37 (17%) suffered from cavernoma-related epilepsy. 28 patients (13%) suffered from familial CCM. Patients showed significantly decreased component scores and subdomain scores compared to normal population with effects ranging from small to large. This accounted largely also for asymptomatic patients (except for physical component score and main physical subdomains). Multivariate regression analysis confirmed impact of functional impairment on physical component score. HADS-A was significantly increased. HADS-A/D strongly correlated with mental component score and individual perception of the CCM.

Patients with the diagnosis of a CCM showed decreased HRQOL compared to normal population even when not suffering functional impairment or neurological symptoms. This data may function as benchmark in evaluation of different (future) management strategies 6).


1)
Wascher TM, Spetzler RF, Carter LP, Spetzler RF, Hamilton MG. In: Cavernous malformations of the brain stem. Neurovascular Surgery. New York: McGraw -Hill; 1995:541–555
2)
Zimmerman RS, Spetzler RF, Lee KS, Zabramski JM, et al. Cavernous Malformations of the Brain Stem. J Neurosurg. 1991; 75:32–39
3)
Bertalanffy H, Gilsbach JM, Eggert HR, et al. Microsurgery of deep-seated cavernous angiomas: report of 26 cases. Acta Neurochir. 1991; 108:91– 99
4)
Weil SM, Tew JM,Jr. Surgical management of brain stem vascular malformations. Acta Neurochir (Wien). 1990; 105:14–23
5)
Bartolomei J, Wecht DA, Chaloupka J, Fayad P, Awad IA. Occipital lobe vascular malformations: prevalence of visual field deficits and prognosis after therapeutic intervention. Neurosurgery. 1998; 43:415–21; discussion 421-3
6)
Herten A, Chen B, Saban D, Santos A, Wrede K, Jabbarli R, Zhu Y, Schmidt B, Kleinschnitz C, Forsting M, Sure U, Dammann P. Health Related Quality of Life in Patients with Untreated Cavernous Malformations of the Central Nervous System- Quality of Life in patients with untreated CCM. Eur J Neurol. 2020 Sep 22. doi: 10.1111/ene.14546. Epub ahead of print. PMID: 32961598.
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