Tag: cavernous malformation

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Surgery of brainstem cavernous malformations

Brainstem cavernoma

Acta Neurochir (2013) 155:2079–2083

Cavernomas are vascular hamartomas made up of thin-walled, grossly dilated blood vessels lined with endothelium. Between 4 and 35 % (mean 15 %) of cerebral cavernomas are located in the brainstem making resection of these lesions one of the most challenging tasks in neurosurgery.

Methods Patients with cavernomas within the brainstem or deep supratentorial structures were chosen from our prospectively collected database of operated patients with brain cavernomas. The timespan of treatment was between January 1998 and June 2012. Primary outcome was defined as percentage of patients with favourable outcome (Glasgow Outcome Scale (GOS) 4 or 5) at 1 year. Secondary outcome was defined as operation-related morbidity and mortality (drop at least 1 point on GOS at 1 year).

Results A total of 37 patients underwent surgery. The mean age was 34.7±11.7 years. The male to female ratio was 19:16. Thirty-two patients had a solitary lesion and 12 patients harboured multiple lesions. The Glasgow outcome score 4 or 5 was achieved after 34 operations (89.5 %). The mean follow-up was 39 months.We experienced two early post-operative deaths (5.3 %) and decrease in the Glasgow outcome scale postoperatively in 4 patients (10.5 %).

Conclusions • Favourable outcome was achieved in 89.5 % of cases. • Although M&M appears to be relatively high, surgery is method of choice for surgically accessible lesion which has bled for the first time due to reported high rebleed rate and high probability of poor outcome after cavernoma rebleed. • Radiosurgery should be reserved for those lesions which are deemed unresectable and where surgical intervention is considered favourable to observation alone.

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Use of microscope-integrated near-infrared indocyanine green videoangiography in the surgical treatment of intramedullary cavernous malformations

ICG video intram. cavernomas

J Neurosurg Spine 18:443–449, 2013

The characteristics and efficacy of indocyanine green (ICG) videoangiography in cavernous malformation (CM) have not been fully elucidated. The purpose of this paper is to examine the potential utility of ICG videoangiography in the surgical treatment of intramedullary CMs.

METHODS
The authors conducted a retrospective review of 8 cases involving 5 men and 3 women who had undergone surgery for intramedullary CM between January 2008 and July 2011. All patients were evaluated by means of MRI. The MRI findings and clinical history in all cases suggested intramedullary CM as a preoperative diagnosis. In 2 of 8 cases, dilated venous structures associated with CMs were demonstrated. In one of these cases, there were coexisting extramedullary CMs. Intraoperatively, ICG fluorescence was observed for 5 minutes using microscope-integrated videoangiography.

RESULTS
In all 8 cases, intra- and extramedullary CMs were seen as avascular areas on ICG videoangiography. Indocyanine green videoangiography helped surgeons to localize and predict margins of the lesions before performing myelotomy. Importantly, in the cases with associated venous anomalies, ICG videoangiography was useful in delineating and preserving the venous structures. In extramedullary CMs located dorsal to the spinal cord, gradual ICG infiltration was seen, starting at 110 seconds and maximal at 210 seconds after injection. Postoperative MRI confirmed total removal of the lesions in all cases, and subsequent recovery of all patients was uneventful.

CONCLUSIONS
Indocyanine green videoangiography provided useful information with regard to the detection of lesion margins by demonstrating intramedullary CMs as avascular areas. In cases associated with venous anomalies, ICG contributed to safe and complete removal of the CMs by visualizing the venous structure. In extramedullary CMs, ICG videoangiography demonstrated the characteristic of slow blood flow within CMs.

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The Contralateral Transcingulate Approach

Neurosurgery 71[ONS Suppl 1]:ons4–ons14, 2012

The contralateral transcingulate approach is a variation of the contralateral transcallosal approach for lesions located in the cingulate gyrus and deep white matter of the medial frontal lobe.

OBJECTIVE: To more thoroughly describe the contralateral transcingulate approach by reporting our operative technique and results.

METHODS: This approach positions the head with the midline horizontally, lesion on the upside, allowing gravity retraction of the dependent frontal lobe. Bifrontal craniotomy and splitting of the interhemispheric fissure create a crossing trajectory from the contralateral fissure to the ipsilateral cingulate gyrus that maximizes lateral exposure.

RESULTS: Eleven patients with vascular lesions were treated with the contralateral transcingulate approach (9 patients with cavernous malformations and 2 patients with arteriovenous malformations). Eight lesions were located on the left side, 5 in the cingulate gyrus, and 6 in the deep frontal lobe. The falx was cut in 5 patients to extend the crossing trajectory. All lesions were removed completely, with neurological morbidity in 1 patient caused by venous infarction.

CONCLUSION: Although similar to the contralateral transcallosal approach, the contralateral transcingulate approach accesses lesions outside the ventricle and has a steeper crossing trajectory. This approach requires no disruption of brain tissue with lesions on the cingulate surface and only a small incision in cingulate gyrus with lesions in the deep frontal lobe. The ipsilateral pericallosal and callosomarginal arteries provide dependable landmarks for transcingulate dissection. The contralateral transcingulate approach offers an alternative medial approach to lesions near language and motor areas and avoids lateral transcortical approaches, awake speech mapping, and risk to eloquent cortex in the dominant hemisphere.

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Petrosal approaches to brainstem cavernous malformations

Neurosurg Focus 33 (2):E10, 2012

Although they provide excellent ventral and lateral exposure of the brainstem, petrosal approaches to brainstem cavernous malformations (CMs) are infrequently reported.

Methods. The authors reviewed their experience with petrosal approaches to brainstem CMs in combination with a comprehensive review of the literature to elucidate resection rates, complication rates, and outcomes.

Results. Including their own results, the authors found 65 cases in 20 reports of brainstem CMs treated with petrosal approaches. The specific approaches were posterior petrosal in 37 cases (57%), anterior petrosal in 17 (26%), extended posterior petrosal in 10 (15%), and a combined petrosal approach in 1 case (2%). For 50 cases in 16 reports with detailed outcome information, the overall complete resection rate was 90%, with early postoperative morbidity reported in 30% of cases and permanent morbidity in 14%. The rate of CSF leakage was 6%.

Conclusions. The versatile petrosal approaches to brainstem CMs are associated with good outcomes and an acceptable morbidity rate. More expansive lesions can be approached using a combination of the standard anterior and posterior petrosal approach, preserving hearing and avoiding the greater complication rates associated with extended posterior petrosal approaches.

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Supracerebellar transtentorial approach—resection of the tentorium instead of an opening—to provide broad exposure of the mediobasal temporal lobe: anatomical aspects and surgical applications

J Neurosurg 116:764–772, 2012.  http://thejns.org/doi/abs/10.3171/2011.12.JNS111256

The aim of this study was to describe the surgical anatomy of the mediobasal aspect of the temporal lobe and the supracerebellar transtentorial (SCTT) approach performed not with an opening, but with the resection of the tentorium, as an alternative route for the neurosurgical management of vascular and tumoral lesions arising from this region.

Methods. Cadaveric specimens were used to illustrate the surgical anatomy of the mediobasal region of the temporal lobe. Demographic aspects, characteristics of lesions, clinical presentation, surgical results, follow-up findings, and outcomes were retrospectively reviewed for patients referred to receive the SCTT approach with tentorial resection.

Results. Ten patients (83%) were female and 2 (17%) were male. Their ages ranged from 6 to 59 years (mean 34.5 ± 15.8 years). All lesions (3 posterior cerebral artery aneurysms, 3 arteriovenous malformations, 3 cavernous malformations, and 3 tumors) were completely excluded or resected. After a mean follow-up period of 143 months (range 10–240 months), the mean postoperative Glasgow Outcome Scale score was 4.9.

Conclusions. Knowledge of the surgical anatomy provides improvement for microsurgical approaches. The evolution from a small opening to a resection of the tentorium absolutely changed the exposure of the mediobasal aspect of the temporal lobe. The SCTT approach with tentorial resection is an excellent alternative route to the posterior part of mediobasal aspect of the temporal lobe, and it was enough to achieve the best neurosurgical management of tumoral and vascular lesions located in this area.

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Transsylvian-Transinsular Approaches to the Insula and Basal Ganglia

 

 

 

 

Neurosurgery 70:824–834, 2012 DOI: 10.1227/NEU.0b013e318236760d 

Lesions in the insula and basal ganglia can be risky to resect because of their depth and proximity to critical structures, particularly in the dominant hemi- sphere. Transsylvian approaches shorten the surgical distance to these lesions, preserve perisylvian temporal and frontal cortex, and minimize brain transgression. OBJECTIVE: To report our experience with transsylvian-transinsular approaches to vascular lesions.

METHODS: The anterior approach opened the sphenoidal and insular portions of the sylvian fissure and exposed the limen insulae and short gyri, whereas the posterior approach opened the insular and opercular portions of the sylvian fissure and exposed the circular sulcus and long gyri.

RESULTS: Forty-one patients with vascular lesions (24 arteriovenous malformations [AVMs] and 17 cavernous malformations) were treated surgically with a transsylvian- transinsular approach. Complete resection was obtained in 87.5% of AVMs and 95% of cavernous malformations. Permanent neurological morbidity related to surgery was observed in 2 AVM patients (5%), with the remaining 39 patients (95%) improved or unchanged postoperatively (modified Rankin Scale scores 0-2 in 83%). There were no new language deficits in patients with dominant hemisphere lesions.

CONCLUSION: Transsylvian-transinsular approaches safely expose vascular pathology in or deep to the insula while preserving overlying eloquent cortex in the frontal and temporal lobes. The anterior transsylvian-transinsular approach can be differentiated from the posterior approach based on technical differences in splitting the sylvian fis- sure and anatomic differences in final exposure. Discriminating patient selection and careful microsurgical technique are essential.

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Intramedullary spinal cord cavernous malformations

J Neurosurg Spine 16:308–314, 2012. DOI: 10.3171/2011.11.SPINE11536

Intramedullary spinal cord cavernous malformations (CMs), once thought to be extremely rare, have been diagnosed more frequently since the advent of MR imaging. In the literature, however, only a few studies include more than 10 cases. The aim of this study was to discuss the clinical presentation of intramedullary spinal cord CMs and the outcome of microsurgery for these histologically benign but clinically progressive lesions.

Methods. The authors retrospectively reviewed the records of 20 patients who underwent microsurgery for intramedullary spinal cord CMs. All patients had undergone pre- and postoperative MR imaging, and they were all treated using microsurgical resection. The diagnosis of spinal cord CMs was based on pathological criteria. The pre- and postoperative neurological states of the patients were classified according to the McCormick scale and Frankel scale. The microsurgical outcomes are presented and discussed.

Results. In most cases, CMs can be diagnosed on the basis of MR imaging findings, since these lesions have certain characteristic imaging patterns. Patients with intramedullary spinal cord CMs may present with either a rapid, acute onset of symptoms or slow, progressive neurological decline. The CMs in 19 of 20 patients in this series were totally resected, and most patients neurologically improved postoperatively. As previously reported, the authors confirm that the treatment of choice for symptomatic intramedullary CMs is total removal of the lesion to avoid recurrence and the possibility of further hemorrhage.

Conclusions. This study has defined the clinical features of symptomatic intramedullary spinal cord CMs. Surgery is the mainstay treatment. Surgical outcome is associated with low mortality with a high probability of functional recovery, especially when symptoms are not severe and are of relatively recent onset.

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The natural history of intracranial cavernous malformations

Neurosurg Focus 30 (6):E24, 2011, DOI: 10.3171/2011.3.FOCUS1165

Literature reports on the natural history of cerebral cavernous malformations (CMs) are numerous, with considerable variability in lesion epidemiology, hemorrhage rates, and risk factors for hemorrhage.

In this review, the authors performed a meta-analysis of 11 natural history studies. The overall male-to-female ratio was 1:1, and the mean age at presentation was 30.6 years. Overall, 37% of patients presented with seizures, 36% with hemorrhage, 23% with headaches, 22% with focal neurological deficits, and 10% were asymptomatic. Some patients had more than one symptom. Seizure presentation was most prevalent among supratentorial CMs, while focal neurological deficits were common in patients with infratentorial CMs. By location, CMs were in the cerebral hemispheres (66%), brainstem (18%), basal ganglia or thalamus (8%), cerebellum (6%), and other (2.5% [combined supra- and infratentorial, callosal or insular]). Overall, 19% of patients harbored multiple intracranial CMs, and 9% had radiographically apparent associated developmental venous anomalies. An overall annual hemorrhage rate of 2.4% per patient-year (range 1.6%–3.1%) was identified across 3 studies.

Prior hemorrhage and female sex were risk factors for bleeding, while CM size and multiplicity did not affect hemorrhage rates. Although not impacting the hemorrhage rate itself, deep location was a risk factor for increased clinical aggressiveness.

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Surgical approaches to brainstem cavernous malformations

Neurosurg Focus 29 (3):E8, 2010. DOI: 10.3171/2010.6.FOCUS10128

Brainstem cavernous malformations (CMs) are low-flow vascular lesions in eloquent locations. Their presentation is often marked with symptomatic hemorrhages that appear to occur more frequently than hemorrhage from supratentorial cavernomas.

Brainstem CMs can be removed using 1 of the 5 standard skull-base approaches: retrosigmoid, suboccipital (with or without telovelar approach), supracerebellar infratentorial, orbitozygomatic, and far lateral.

Patients being referred to a tertiary institution often have lesions that are aggressive with respect to bleeding rates. Nonetheless, the indications for surgery, in the authors’ opinion, are the same for all lesions: those that are symptomatic, those that cause mass effect, or those that abut a pial surface. Patients often have relapsing and remitting courses of symptoms, with each hemorrhage causing a progressive and stepwise decline.

Many patients experience new postoperative deficits, most of which are transient and resolve fully. Despite the risks associated with operating in this highly eloquent tissue, most patients have had favorable outcomes in the authors’ experience. Surgical treatment of brainstem CMs protects patients from the potentially devastating effects of rehemorrhage, and the authors believe that the benefits of intervention outweigh the risks in patients with the appropriate indications.

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Stereotactic radiosurgery for symptomatic solitary cerebral cavernous malformations considered high risk for resection

J Neurosurg 113:23–29, 2010. DOI: 10.3171/2010.1.JNS081626

A retrospective study was conducted to reassess the benefit and safety of stereotactic radiosurgery (SRS) in patients with solitary cerebral cavernous malformations (CCMs) that bleed repeatedly and are poor candidates for surgical removal.

Methods. Between 1988 and 2005 at the University of Pittsburgh, the authors performed SRS in 103 evaluable patients (57 males and 46 females) with solitary symptomatic CCMs. The mean patient age was 39.3 years. Ninetyeight percent of these patients had experienced 2 or more hemorrhages associated with new neurological deficits. Seventeen patients (16.5%) had undergone attempted resection before radiosurgery. Ninety-three CCMs were located in deep brain structures and 10 were in subcortical lobar areas of functional brain importance. The median malformation volume was 1.31 ml, and the median tumor margin dose was 16 Gy.

Results. The follow-up ranged from 2 to 20 years. The annual hemorrhage rate—that is, a new neurological deficit associated with imaging evidence of a new hemorrhage—before SRS was 32.5%. After SRS 22 hemorrhages were observed within 2 years (10.8% annual hemorrhage rate) and 4 hemorrhages were observed after 2 years (1.06% annual hemorrhage rate). The risk of hemorrhage from a CCM was significantly reduced after radiosurgery (p < 0.0001). Overall, new neurological deficits due to adverse radiation effects following SRS developed in 14 patients (13.5%), with most occurring early in our experience. Modifications in technique (treatment volume within the T2- weighted MR imaging–defined margin, use of MR imaging, and dose reduction for CCM in critical brainstem locations) further reduced risks after SRS.

Conclusions. Data in this study provide further evidence that SRS is a relatively safe procedure that reduces the rebleeding rate for CCMs located in high-surgical-risk areas of the brain.

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Management of pediatric brainstem cavernous malformations: experience over 20 years at the Hospital for Sick Children

J Neurosurg Pediatrics 4:040580–400640, 2009.DOI: 10.3171/2009.6.PEDS0923

Because of their location and biological behavior, brainstem cavernous malformations (CMs) pose a formidable clinical challenge to the neurosurgeon. The optimal management of these lesions requires considerable neurosurgical judgment. Accordingly, the authors reviewed their experience with the management of pediatric brain- stem CMs at the Hospital for Sick Children.

Methods. The authors performed a retrospective chart review of pediatric patients who had received diagnoses of a brainstem CM at the Hospital for Sick Children over the past 20 years.

Results. Twenty patients were diagnosed with brainstem CMs. The mean age at diagnosis was 10.1 ± 5.4 years, and the patients included 13 boys and 7 girls. The mean maximal diameter of the CM was 14.3 ± 11.2 mm. The le- sions were evenly distributed on the right and left sides of the brainstem with 4 midbrain, 13 pontine, and 3 medullary lesions. Seven patients underwent surgery for the management of their CMs, with a mean age at presentation of 5.2 years, and a mean CM size of 21.0 mm. Of note from the surgical group, 2 patients had a family history of CMs, 2 lesions were medullary, the CM reached a pial surface in 6 of 7 patients, and 6 of 7 lesions were located on the right side. The mean age at presentation among the 13 patients in the nonsurgical group was 12.7 years, and the mean CM size was 10.6 mm. Seven of these patients had a prior history of radiation for tumor, and only 3 had lesions that reached a pial surface.

Conclusions. The management of brainstem CMs in children is influenced by multiple factors. The majority of patients received conservative management and tended to be asymptomatic with smaller lesions. Patients with larger lesions and direct pial contact, in whom symptoms arose at a younger age were more likely to undergo surgical management. A history of familial CM was also a predictor for receiving surgical treatment. No patients with a prior history of radiation therapy underwent surgery for CMs. The presence of multiple lesions seemed to have no impact on the type of management chosen. Patients who underwent surgery did suffer morbidity related to the procedure, and tended to improve clinically over time. Conservative management was associated with new deficits arising in children, some of which improved with time. Consideration of many clinical and radiological parameters is thus prudent when managing the care of children with brainstem CMs.

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