Unusual Presentation of Cerebral Cavernous Malformation

Kim, Won-Hyung; Lim, Dong-Jun; Choi, Jong‐Il; Ha, Sung-Kon; Kim, Sang Dae; Kim, Se Hoon

doi:10.7461/jcen.2015.17.3.257

Cited by 2 publications

(10 citation statements)

References 24 publications

(47 reference statements)

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“…The appropriate timing of surgery is controversial and has been debated, 5 11 and it is commonly believed that attempting surgery in the subacute stage is fruitful as it allows time for the hematoma to liquefy and partially resolve that allows better intraoperative visualization and a better plane of dissection. 46…”

Section: Discussionmentioning

confidence: 99%

“…46 Because patients with cCCMs usually show some symptoms (more commonly due to local mass effect and/or raised intracranial pressure), surgical treatment is generally necessary. 4 The appropriate timing of surgery is controversial and has been debated, 5,11 and it is commonly believed that attempting surgery in the subacute stage is fruitful as it allows time for the hematoma to liquefy and partially resolve that allows better intraoperative visualization and a better plane of dissection. 46 Complete removal of the solid component or nodule is recommended in cCCMs; but the surgical management of the cyst wall has been debated.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3] They account for about 5 to 13% of intracranial vascular malformations and their population prevalence is 0.5%. [4][5][6][7][8] CCMs are the only vascular malformations known to form cysts. 9,10 The traditional classification systems (like Zabramski's) do not describe a cystic variety separately.…”

Section: Introductionmentioning

confidence: 99%

“…Cerebral cavernous malformations (CCMs) have been variously called cryptic angioma, cavernous angioma, cavernous hemangioma, cavernoma, and “angiographically occult vascular malformations.” 1 2 3 They account for about 5 to 13% of intracranial vascular malformations and their population prevalence is 0.5%. 4 5 6 7 8 CCMs are the only vascular malformations known to form cysts. 9 10 The traditional classification systems (like Zabramski's) do not describe a cystic variety separately.…”

Section: Introductionmentioning

confidence: 99%

“… 9 10 The traditional classification systems (like Zabramski's) do not describe a cystic variety separately. 5 Cystic CCMs (cCCMs) are rare 4 9 11 12 13 and their prevalence has not been reported in literature. They can cause considerable diagnostic and management dilemmas.…”

Section: Introductionmentioning

confidence: 99%

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Cystic Cerebral Cavernous Malformations: Report of Five Cases and a Review of Literature

et al. 2023

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Introduction Cerebral cavernous malformations (CCMs) account for about 5 to 13% of intracranial vascular malformations. Cystic cerebral cavernous malformations (cCCMs) are a rare morphological variant and can cause diagnostic and therapeutic dilemmas. We describe our five such cases and review the existing literature on this entity. Methods A search of the PubMed database for cCCMs was done, and all articles in English emphasizing the reporting of cCCMs were selected. A total of 42 publications describing 52 cases of cCCMs were selected for analysis. Epidemiological data, clinical presentation, imaging features, the extent of resection, and outcome were analyzed. Radiation-induced cCCMs were excluded. We have also described five of our cases of cCCMs and reported our experience. Results The median age at presentation was 29.5 years. Twenty-nine patients had supratentorial lesions, 21 had infratentorial lesions, and 2 had lesions in both compartments. Among our four patients, three had infratentorial lesions, whereas one had a supratentorial lesion. Multiple lesions were seen in four patients. A majority (39) had symptoms of mass effect (75%), and 34 (65.38%) had raised intracranial pressure (ICP), whereas only 11 (21.15%) had seizures. Among our four operated patients, all of them had symptoms of mass effect, and two of them also had features of raised ICP. The extent of resection was gross total in 36 (69.23%), subtotal in 2 (3.85%), and not reported in 14 (26.93%). All four of our operated patients underwent gross total resection, but two of them underwent a second surgery. Of the 48 patients in whom the surgical outcome was reported, 38 improved (73.08%). One showed a transient worsening followed by improvement, one developed a worsening of the pre-existing focal neurological deficit (FND), two developed a new FND, and 5 had no improvement in their FNDs. Death occurred in one patient. All four of our operated patients improved after surgery, although three of them showed a transient worsening of FNDs. One patient is under observation. Conclusion cCCMs are rare morphological variants and can cause considerable diagnostic and therapeutic dilemmas. They should be considered in the differential diagnosis of any atypical cystic intracranial mass lesion. Complete excision is curative, and the outcome is generally favorable; although transient deficits may be seen.

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Section: Discussionmentioning

confidence: 99%