Cerebral cavernous malformation treatment

Cerebral cavernous malformation treatment

see Intracranial cavernous malformation surgery.


There have been few comparative studys of microsurgical excision vs conservative treatment of cerebral cavernous malformations (CCM) and none of them has reliably demonstrated a statistically and clinically significant difference.

A prospective, population-based study to identify and independently validate definite cerebral cavernous malformation diagnoses first made in 1999-2003 in Scottish adult residents, used multiple sources of prospective follow-up to assess adults’ dependence and to identify and independently validate outcome events.

Moultrie et al., used univariate and multivariable survival analyses to test the influence of CCM excision on outcome, adjusted for prognostic factors and baseline imbalances.

Of 134 adults, 25 underwent CCM excision; these adults were younger (34 vs 43 years at diagnosis, p = 0.004) and more likely to present with symptomatic intracranial hemorrhage or focal neurological deficit than adults managed conservatively (48% vs 26%; odds ratio 2.7, 95% confidence interval [CI] 1.1-6.5). During 5 years of follow-up, CCM excision was associated with a deterioration to an Oxford Handicap Scale score 2-6 sustained over at least 2 successive years (adjusted hazard ratio [HR] 2.2, 95% CI 1.1-4.3) and the occurrence of symptomatic intracranial hemorrhage or new focal neurologic deficit (adjusted HR 3.6, 95% CI 1.3-10.0).

CCM excision was associated with worse outcomes over 5 years compared to conservative management. Long-term follow-up will determine whether this difference is sustained over patients’ lifetimes. Meanwhile, a randomized controlled trial appears justified.

CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that CCM excision worsens short-term disability scores and increases the risk of symptomatic intracranial hemorrhage and new focal neurologic deficits 1).


Antithrombotic therapy use is associated with a lower risk of intracranial haemorrhage or focal neurological deficit from cerebral cavernous malformations than avoidance of antithrombotic therapy. These findings provide reassurance about safety for clinical practice and require further investigation in a randomised controlled trial 2).

References

1)

Moultrie F, Horne MA, Josephson CB, Hall JM, Counsell CE, Bhattacharya JJ, Papanastassiou V, Sellar RJ, Warlow CP, Murray GD, Al-Shahi Salman R; Scottish Audit of Intracranial Vascular Malformations (SAIVMs) steering committee and collaborators. Outcome after surgical or conservative management of cerebral cavernous malformations. Neurology. 2014 Aug 12;83(7):582-9. doi: 10.1212/WNL.0000000000000684. Epub 2014 Jul 3. PubMed PMID: 24994841.
2)

Zuurbier SM, Hickman CR, Tolias CS, Rinkel LA, Leyrer R, Flemming KD, Bervini D, Lanzino G, Wityk RJ, Schneble HM, Sure U, Al-Shahi Salman R; Scottish Audit of Intracranial Vascular Malformations Steering Committee. Long-term antithrombotic therapy and risk of intracranial haemorrhage from cerebral cavernous malformations: a population-based cohort study, systematic review, and meta-analysis. Lancet Neurol. 2019 Aug 6. pii: S1474-4422(19)30231-5. doi: 10.1016/S1474-4422(19)30231-5. [Epub ahead of print] PubMed PMID: 31401075.

Insular Cavernous Malformation

Insular Cavernous Malformation

Surgical management of cavernous malformation (CM) of the insula consists of total resection of the lesion and the surrounding gliosis to avoid or reduce seizures. When located in the dominant hemisphere, an awake craniotomy with intraoperative mapping reduces the risk of functional damage. The insula is covered by the operculum and has a relationship with the middle cerebral artery and its branches that run along its lateral cortical surface. Therefore high expertise is required to manage the exposure of the insula and its complex anatomy.

Videos

https://www.neurosurgicalatlas.com/cases/insular-cavernous-malformation


A video of Burkhardt et al. demonstrated the microsurgical resection of a de novo CM adjacent to a previously treated high-grade AVM and clipping of a middle cerebral artery (MCA) aneurysm. A 70-yr-old male with history of radiosurgery for AVM presented with aphasia and confusion. Preoperative angiography showed complete occlusion of the AVM. MRI showed multiple cystic lesions suspicious for radiation-induced necrosis and CM. IRB approval and patient consent was obtained. A pterional craniotomy was performed with transsylvian exposure of the insula. The radiated feeding arteries were followed to the occluded AVM nidus. A CM was noted deep to this candelabra of the MCA vessels, which were mobilized to access and resect the CM. A small incision was made in this insular cortex underneath the malformation circumferentially freeing it of adhesions. The sclerotic AVM nidus was circumferentially dissected and removed en bloc. Thorough exploration of the resection cavity revealed no residual CM or AVM nidus. Attention was then turned to the M2-MCA bifurcation aneurysm, which was occluded with a straight clip. Postoperative imaging confirmed complete CM resection. The patient recovered from his aphasia. This case demonstrates the management of a radiation-induced de novo CM following treatment of a high-grade AVM. Radiographic follow-up for radiosurgically treated AVM is needed to rule out long-term complications. Bleeding from a de novo CM mimics bleeding from residual AVM nidus, requiring careful angiographic evaluation 1).


A video of Norat et al. illustrated the use of a trans-Sylvian, trans-sulcal approach to resect a deep insular/basal ganglia cavernous malformation in a young patient. The use of the neuronavigation is essential for success in these types of operation as this tool limits the surgeon’s footprint in eloquent brain. Unlike superficial lesions where the removal of hemosiderin stained brain is possible and often safe, resection of deep-seated lesions requires the surgeon to distinguish between hemosiderin-stained brain and residual cavernous malformation. This task is not simple, and residual cavernous malformation is the most common reason for re-bleed in patients who have undergone surgery. Resection of symptomatic cavernous malformations in deep locations can be performed safely, but outcomes are heavily influenced by proper patient selection and surgeon experience. In patients with multiple cerebral cavernous malformations, such as the one in this case, genetic testing should be performed 2).


A video of Vigo et al. demonstrated the surgical management of a large left insular CM. A 29-year-old female with multiple CM and 7 years of partial seizures and recent onset of short memory loss. Neuroimaging showed a large left insular and planum polare CM with important mass effect and hemorrhage signs. The patient consented to surgery, and an awake pretemporal craniotomy was carried out with continuous motor evoked potential monitoring. No language function was localized in the superior temporal gyrus; therefore corticectomy of the middle portion was performed to expand the operative corridor. The vessel manipulation during wide opening of the sylvian fissure increased the risk of postoperative vasospasm and blood drain into the surgical field. The CM was exposed and completely removed without functional damage. The patient recovered from surgery without complications, and no seizures occurred at 2 months’ follow-up. Postoperative imaging showed complete removal of the CM 3).

Case reports

A 25-yr-old female presented with an acute-onset right homonymous hemianopsia. Neuroimaging revealed a large left insular CM, adjacent to the posterior limb of IC. After obtaining IRB approval and patient consent, a left pterional craniotomy with a wide distal Sylvian fissure split was completed. Using neuronavigation, an insular entry point was chosen for corticectomy. The CM was opened with subsequent hematoma evacuation and intracapsular resection technique. Inspection of the cavity revealed remnants anteromedially near the IC, which were removed meticulously, mobilizing the CM away from the IC. Postoperative MRI demonstrated gross total resection of the CM. The patient was discharged home on postoperative day 5 with persistent homonymous hemianopia.This case describes the use of a transsylvian-transinsular approach to access deep lesions with the shortest surgical distance and minimal cortical transgression. A wide Sylvian fissure split exposes the M2 MCA and accesses a safe insular zone, keeping the most eloquent structures deep to the lesion in the surgical corridor. This approach can safely expose vascular pathologies in the insular region without the risk of injury to overlying eloquent frontal and temporal lobes, even in the dominant hemisphere 4).

References

1)

Burkhardt JK, Gandhi S, Tabani H, Benet A, Lawton MT. Left Transsylvian-Transinsular Approach for Radiation-Induced Cavernous Malformation: 3-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Aug 1;17(2):E62-E63. doi: 10.1093/ons/opy357. PubMed PMID: 30418603.
2)

Norat P, Yagmurlu K, Park MS, Kalani MYS. Keyhole, Trans-Sylvian, Trans-Sulcal Resection of an Insular Cerebral Cavernous Malformation: 2-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Jul 1;17(1):E18. doi: 10.1093/ons/opy326. PubMed PMID: 30496497.
3)

Vigo V, Zanabria Ortiz R, Paganelli SL, da Costa MDS, Campos Filho JM, Chaddad-Neto F. Awake Craniotomy for Removal of Left Insular Cavernous Malformation. World Neurosurg. 2019 Feb;122:209. doi: 10.1016/j.wneu.2018.10.220. Epub 2018 Nov 9. PubMed PMID: 30415050.
4)

Mascitelli J, Gandhi S, Wright E, Lawton MT. Transsylvian-Transinsular Approach for an Insular Cavernous Malformation Resection: 3-Dimensional Operative Video. Oper Neurosurg (Hagerstown). 2019 Feb 1;16(2):50. doi: 10.1093/ons/opy155. PubMed PMID: 29905877.

Intracranial cavernous malformation surgery

Intracranial cavernous malformation surgery

Indications

Indications for surgery for intracranial cavernous malformation:

1. accessible lesions with

a) Focal neurologic signs

b) or symptomatic hemorrhage

c) or seizures:

● new onset seizures: there is a suggestion that removing CMs before “kindling” occurs may have a better chance of preventing future seizures.

● difficult to manage seizures

2. less accessible lesions that repeatedly bleed with progressive neurologic deterioration may be considered for excision, even in delicate regions such as the brainstem 1) 2) 3).

Surgical technique

Goal of surgery: complete removal of the malformation. Since CMs are not particularly bloody, piecemeal excision is an option; especially important in brainstem lesions.

Stereotactic localization or intraoperative ultrasound may be particularly helpful in localizing. When operating on CMs that have bled, one usually encounters a cavity containing the CM and blood degradation products 4).

Initial dissection is directed at separating the lesion from the adjacent brain. Although bleeding is usually not a problem, it occasionally may be brisk if the CM is entered before the dissection and devascularization is complete. Once the dissection is complete, the contents of the CM capsule may be removed piecemeal to minimize the parenchymal opening (especially important in the brainstem). For supratentorial CMs presenting with seizures, it is desirable to also remove the hemosiderin-stained brain immediately surrounding the CM. Keep in mind the relatively common association of CMs with venous angiomas, which if encountered should not be removed as they represent the venous drainage of the area.


Eichberg et al., reviewed a single institution’s transcortical-transtubular intracranial cavernous malformation resections using either BrainPath endoport system (NICO, Indianapolis, Indiana) or ViewSite Brain Access System (Vycor Medical, Boca Raton, Florida) tubular retractors performed from 2013 to 2018 (n = 20).

Gross total resection was achieved in all patients. When a developmental venous anomaly (DVA) was present, avoidance of DVA resection was achieved in all cases (n = 4). All patients had a supratentorial cavernoma with mean depth below cortical surface of 44.1 mm. Average postoperative clinical follow-up was 20.4 wk. Early neurologic deficit rate was 10% (n = 2); permanent neurologic deficit rate was 0%. One patient (5%) experienced early postoperative seizures (< 1 wk postop). No patients experienced late seizures (> 1 wk follow-up). Engel class 1 seizure control at final clinical follow-up was achieved in 87.5% of patients presenting with preoperative epilepsy.

Tubular retractors provide a low-profile, minimally invasive operative corridor for resection of subcortical cavernomas. There were no permanent neurologic complications in the series of 20 cases, and long-term seizure control was achieved in all patients. Thus, tubular retractors appear to be a safe and efficacious tool for resection of subcortical cavernomas 5).

Controversies

An online survey composed of 61 items was sent to 26 centers to establish a multicenter international retrospective cohort of adult patients who underwent a surgical resection as the first-line treatment of a supratentorial cavernous angioma located within or close to eloquent brain area.

272 patients from 19 centers (mean 13.6 ± 16.7 per center) from eight countries were included. The pre-operative management varied significantly between centers and countries regarding the pre-operative functional assessment, the pre-operative epileptological assessment, the first given antiepileptic drug, and the time to surgery. The intra-operative environment varied significantly between centers and countries regarding the use of imaging systems, the use of functional mapping with direct electrostimulations, the extent of resection of the hemosiderin rim, the realization of a post-operative functional assessment, and the time to post-operative functional assessment. The present survey found a post-operative improvement, as compared to pre-operative evaluations, of the functional status, the ability to work, and the seizure control.

They observed a variety of practice between centers and countries regarding the management of cavernous angioma located within eloquentregions. Multicentric prospective studies are required to solve relevant questions regarding the management of cavernous angioma-related seizures, the timing of surgery, and the optimal extent of hemosiderin rim resection 6).


Meta-analysis and subgroup analyses were conducted to compare extended lesionectomy with lesionectomy. Pooled analysis demonstrated that seizure outcome was not statistically significantly improved in patients who underwent extended lesionectomy compared with lesionectomy (OR 0.77; 95% CI [0.39-1.51]; P=0.44; I2=15%).

Extended lesionectomy cannot contribute to better seizure control for CCMs with epilepsy. Resection of lesion and surrounding hemosiderin is sufficient for CCMs presenting with epilepsy 7).

References

1)

Bicknell JM. Familial Cavernous Angioma of the Brain Stem Dominantly Inherited in Hispanics. Neurosurgery. 1989; 24:102–105
2)

Ondra SL, Doty JR, Mahla ME, et al. Surgical Excision of a Cavernous Hemangioma of the Rostral Brain Stem: Case Report. Neurosurgery. 1988; 23:490–493
3)

Zimmerman RS, Spetzler RF, Lee KS, Zabramski JM, et al. Cavernous Malformations of the Brain Stem. J Neurosurg. 1991; 75:32–39
4)

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
5)

Eichberg DG, Di L, Shah AH, Ivan ME, Komotar RJ, Starke RM. Use of Tubular Retractors for Minimally Invasive Resection of Deep-Seated Cavernomas. Oper Neurosurg (Hagerstown). 2019 Jul 13. pii: opz184. doi: 10.1093/ons/opz184. [Epub ahead of print] PubMed PMID: 31301143.
6)

Zanello M, Meyer B, Still M, Goodden JR, Colle H, Schichor C, Bello L, Wager M, Smits A, Rydenhag B, Tate M, Metellus P, Hamer PW, Spena G, Capelle L, Mandonnet E, Robles SG, Sarubbo S, Martino González J, Fontaine D, Reyns N, Krieg SM, Huberfeld G, Wostrack M, Colle D, Robert E, Noens B, Muller P, Yusupov N, Rossi M, Conti Nibali M, Papagno C, Visser V, Baaijen H, Galbarritu L, Chioffi F, Bucheli C, Roux A, Dezamis E, Duffau H, Pallud J. Surgical resection of cavernous angioma located within eloquent brain areas: International survey of the practical management among 19 specialized centers. Seizure. 2019 Mar 28;69:31-40. doi: 10.1016/j.seizure.2019.03.022. [Epub ahead of print] PubMed PMID: 30959423.
7)

Shang-Guan HC, Wu ZY, Yao PS, Chen GR, Zheng SF, Kang DZ. Does extended lesionectomy need to cerebral cavernous malformations presenting with epilepsy? A meta-analysis. World Neurosurg. 2018 Sep 6. pii: S1878-8750(18)31994-6. doi: 10.1016/j.wneu.2018.08.208. [Epub ahead of print] PubMed PMID: 30196170.
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