====== Cavernous malformation ====== {{rss>https://pubmed.ncbi.nlm.nih.gov/rss/search/18qVEVbjJjoy2kA2lFU0eJMmRqMYS9vCQG83n83vzbPqxL050B/?limit=15&utm_campaign=pubmed-2&fc=20250206032805}} Cavernous [[malformation]]s are [[cluster]]s of abnormally formed [[blood vessel]]s with thin walls, resembling a small raspberry. They can cause bleeding in the brain, leading to neurological symptoms, seizures, or headaches. ---- In [[1928]], Cushing and Bailey classified [[cavernous malformation]]s as a solid subtype of [[hemangioblastoma]]s ((Cushing H, Bailey P (1928) Tumors arising from the blood-vessels of the brain. Charles C. Thomas, Springfield)) ===== General information ===== ==== Key concepts ==== usually [[angiographically occult]]. May show up on [[MRI]] (open channels → [[flow void]] on [[T2 weighted image]], 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 [[seizure]]s: 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 malformation]]s, 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]]. ===== Synonyms ===== 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]] ===== Classification ===== [[Cavernous Malformation Classification]]. ===== Etiology ===== see [[Cavernous malformation etiology]]. ===== Pathology ===== 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. ===== Clinical features ===== 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. ===== Diagnosis ===== [[Cavernous malformation diagnosis]]. ===== Differential Diagnosis ===== [[Cavernous malformation differential diagnosis]]. ===== Treatment ===== see [[Cavernous malformation treatment]]. ===== Complications ===== [[Cavernous malformation hemorrhage]] ===== Outcome ===== When [[cavernous malformation]]s can be completely removed, the risk of further growth or hemorrhage is essentially permanently eliminated ((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)) (however, recurrence of symptoms has been reported after partial and even seemingly-complete removal ((Zimmerman RS, Spetzler RF, Lee KS, Zabramski JM, et al. Cavernous Malformations of the Brain Stem. J Neurosurg. 1991; 75:32–39)) ((Bertalanffy H, Gilsbach JM, Eggert HR, et al. Microsurgery of deep-seated cavernous angiomas: report of 26 cases. Acta Neurochir. 1991; 108:91– 99)). For CMs treated surgically, patients need to be aware that post-op neurologic worsening is very common, especially with brainstem CMs ((Weil SM, Tew JM,Jr. Surgical management of brain stem vascular malformations. Acta Neurochir (Wien). 1990; 105:14–23)) Worsening may be transient, ((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)) 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 ((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.)). ===== References =====