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CC BY-NC-ND 4.0 · Asian J Neurosurg 2023; 18(02): 272-292
DOI: 10.1055/s-0043-1768577
Original Article

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

Krishna Shroff 
1   Department of Neurosurgical Oncology, Tata Memorial Centre, Parel, Mumbai, Maharashtra, India
,
Chandrashekhar Deopujari 
2   Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
,
Vikram Karmarkar 
2   Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
,
Chandan Mohanty 
2   Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India
› Author Affiliations Funding None.
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Abstract

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.

Keywords

cystic cavernomas - cystic cerebral cavernous malformations - cystic vascular malformations

Abbreviations

CCM – cerebral cavernous malformation


cCCM – cystic cerebral cavernous malformation


FND – focal neurological deficit


MRI – magnetic resonance imaging


CT – computed tomography


ICP – intracranial pressure


CPA – cerebellopontine angle


CSF – cerebrospinal fluid


GRE – gradient echo


SWI – susceptibility-weighted imaging


Informed Consent

Patients/parents of the patients included in the study were informed that their/their child's clinical data and imaging photographs may be used for educational purposes such as presentation in conferences/journals, and consent was obtained. No personal identifying information has been submitted in this manuscript or in [Figs. 1] to [5].




Publication History

Article published online:
12 June 2023

© 2023. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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