Cerebellar Arteriovenous Malformation Research Articles

Thoracic spinal cord injury after surgical removal of a ruptured cerebellar arteriovenous malformation in a patient in the Concorde position: illustrative case.

Thoracic spinal cord injury after posterior cranial fossa surgery in younger patients is a rare complication. There have been reports of this complication in tumor and spine fields but not in vascular surgery. A 22-year-old-man experienced cerebellar arteriovenous malformation rupture, and the malformation was surgically removed with the man in the Concorde position. After surgery, the man had severe paraplegia, and a thoracic spinal cord injury was diagnosed. In younger patients, cervical hyperflexion in the Concorde position can cause thoracic spinal cord injury even in surgery for cerebrovascular disease.

Management and outcome predictors of patients with ruptured deep-seated brain arteriovenous malformations.

Decision-making for the management of ruptured deep-seated brain arteriovenous malformations (bAVMs) is controversial. This study aimed to shed light on the treatment outcomes of patients with ruptured deep-seated bAVMs. Data on bAVM patients were retrieved from the authors' institutional database, spanning 1990-2021. The outcomes were annual hemorrhage risk (before and after intervention), number of follow-up hemorrhages, bAVM obliteration, poor modified Rankin Scale (mRS) score (i.e., mRS score > 2), worsened mRS score, and mortality. Multivariable Cox and logistic regression analyses were conducted to determine predictors of time-to-event and categorical outcomes, respectively. Of the 1066 patients in the database with brain bAVM, 177 patients harboring ruptured deep-seated bAVMs were included. The pretreatment annual hemorrhage risk was 8.24%, and the posttreatment risk was lowered to 1.65%. In multivariable Cox regression analysis, a prenidal aneurysm (HR 2.388, 95% CI 1.057-5.398; p = 0.036) was associated with a higher risk of follow-up hemorrhage, while definitive treatment (i.e., either surgery or radiosurgery vs endovascular embolization or conservative management) (HR 0.267, 95% CI 0.118-0.602; p = 0.001) was associated with a lower risk of follow-up hemorrhage. In multivariable logistic regression analysis, Spetzler-Martin grades IV and V (OR 0.404, 95% CI 0.171-0.917; p = 0.033) and brainstem arteriovenous malformation (AVM) (OR 0.325, 95% CI 0.128-0.778; p = 0.014) were associated with lower odds of obliteration, while definitive treatment (OR 8.864, 95% CI 3.604-25.399; p = 0.008) was associated with higher obliteration odds. Controlling for baseline mRS score, cerebellar AVM (OR 0.286, 95% CI 0.098-0.731; p = 0.013) and definitive treatment (OR 0.361, 95% CI 0.160-0.807; p = 0.013) were associated with lower odds of a poor mRS score, and definitive treatment (OR 0.208, 95% CI 0.076-0.553; p = 0.001) was associated with lower odds of a worsened mRS score. Furthermore, smoking (OR 6.068, 95% CI 1.531-25.581; p = 0.01) and definitive treatment (OR 0.101, 95% CI 0.024-0.361; p = 0.007) were associated with higher and lower mortality odds, respectively. A definitive treatment strategy seems to be beneficial in achieving higher obliteration and lower hemorrhage rates while decreasing the odds of a poor mRS score, worsened mRS score, and mortality. In this category of patients, prenidal aneurysms warrant treatment, and smoking cessation should be encouraged.

Endoscopic endonasal transclival clipping of a cerebellar arteriovenous malformation feeding vessel and associated aneurysm; a 2D operative video

Positioned along the ventral surface of the pons, proximal superior cerebellar artery (SCA) aneurysms account for only 1.7% of all intracranial aneurysms [1]. Unlike more commonly encountered basilar artery aneurysms, patients often experience good outcomes when treated via endovascular coiling or surgical clipping [1,2]. These lesions frequently have a lateral projection and paucity of perforator arteries [2]. With further development of endoscopic endonasal techniques, access to this region is possible via a direct frontal exposure to the ventral brainstem, basilar artery and branching vessels. To date, there are only a limited number of reports describing an endoscopic endonasal transclival (EETC) approach for surgical clipping [3–5]. In this operative video, we detail the surgical clipping of a cerebellar arteriovenous malformation feeding vessel and an associated aneurysm using the EETC approach in a 59-year-old woman who presented with sudden onset of a severe headache. The feeding vessel and aneurysm’s midline location, just below the take-off of the SCA made it a good candidate for this surgery. Major steps included in this video include 1) transsphenoidal exposure of and subsequent drilling of the clivus, 2) dural opening into the pre-pontine cistern and dissection of the aneurysm, 3) clipping of the aneurysm, and 4) multi-layered closure of the skull base defect. Overall, the patient tolerated the procedure well and was found to have no residual filling of the aneurysm or the AVM feeding vessel at 2-year follow-up. EETC is a viable surgical option for the treatment of aneurysm located along the midline of the pre-pontine cistern.

Concomitant moyamoya syndrome and infratentorial arteriovenous malformation in a neurofibromatosis type 1 patient: a case report

Background: Neurofibromatosis type 1 (NF1) is a genetic disorder with diverse phenotypic manifestations. Cerebral vasculopathy is one of the multisystem involvements often overlooked unless symptomatic. Case Report: A 28-year-old male patient with prolonged NF1 complained of right-hand position-specific rhythmic tremor after surviving an ipsilateral cerebellar arteriovenous malformation (AVM) hemorrhagic transformation. Not only did he suffer rupture of the infratentorial vasculopathy but he also endured asymmetric supratentorial occlusive vessel changes in Moyamoya syndrome. Due to contralateral limb clumsiness, his right hemispheric vasculature was revascularized by encephaloduroarteriosynangiosis 13 years before the AVM rupture.Conclusion: This case report describes exceptional NF1 CNS involvement where the cerebral vasculature had concomitant Moyamoya syndrome and unilateral cerebellar AVM in a single patient. Cerebral vasculopathy should be surveyed and adequately addressed during the follow-up of chronic NF1, as it can cause irreversible sequelae or can be life-threatening.

Hemifacial Spasm Caused by Cerebellar Arteriovenous Malformation and Spasm-Relief after Nidus Removal

Secondary hemifacial spasm (HFS) occurs by a conflict between the facial nerve and intracranial pathology, such as cerebellopontine angle tumor or vascular abnormality like arteriovenous malformation (AVM) or aneurysm. HFS caused by AVM is rare, and optimal combination of treatment options has not been established. AVM treatment with or without microvascular decompression (MVD) of the facial nerve was provided in the previous studies, and the outcomes in terms of spasm relief have been successful. Here, we report a case of HFS caused by cerebellar AVM. HFS symptom disappeared after AVM nidus removal without MVD of the facial nerve.

New aneurysm formation and regrowth associated with rebleeding of residual pediatric ruptured arteriovenous malformation: patient series.

If complete obliteration of ruptured pediatric arteriovenous malformation (AVM) cannot be achieved, the appropriate follow-up duration and predictors of rebleeding remain unknown. Pediatric patients with ruptured AVMs admitted to the authors' hospital within the past 30 years were evaluated. Rebleeding was confirmed in two patients. The first patient was a 5-year-old boy who experienced right thalamic hemorrhage. AVM was found in the bilateral thalamus and treated with stereotactic radiosurgery (SRS). New aneurysm formation and residual AVM regrowth were confirmed 21 years after the SRS. Eight months later, rebleeding occurred. The second patient was a 5-year-old boy who underwent removal of a left cerebellar hemorrhage and AVM. The residual AVM was treated with SRS. Residual AVM regrowth was detected at 6 years 7 months after SRS. Five months later, new aneurysm formation was confirmed. Two additional days later, rebleeding occurred. New aneurysm formation and residual AVM regrowth may predict rebleeding and can occur >20 years after the initial rupture and treatment. If AVM obliteration is not achieved, long-term follow-up is needed, even in adulthood, with attention to new aneurysm formation and residual AVM regrowth. Further treatment is recommended if these findings are confirmed.

Cerebral arterio-venous malformations hemodynamics changes in hereditary hemorrhagic telangiectasia – case report

BackgroundSpontaneous obliteration of cerebral arteriovenous malformations (AVMs) is a rare phenomenon. Hereditary Hemorrhagic Telangiectasia (HHT) is a predisposal genetic condition for AVMs development in all organs. Case illustrationWe report the case of a 34 years old woman with HHT family history. After radiosurgical treatment of a symptomatic evolving cerebellar AVM, late control subtracted digital angiography (DSA) demonstrated the complete obliteration of this AVM but also spontaneous obliteration of 3 fronto-parietal AVMs without any hemorrhagic sign on MRI. ConclusionTo our knowledge, this is the first report of spontaneous obliteration of multiple and unruptured AVMs in a HTT case.

The influence of hemorrhage presentation on clinical outcomes of curative embolisation in 125 cerebellar arteriovenous malformations

Background This study investigated the influence of periprocedural hemorrhage and clinical outcomes with an endovascular therapeutic strategy for cerebellar arteriovenous malformations (cAVMs). Methods From December 2006 to January 2018, 125 cAVMs were classified as types I–IV and received endovascular embolization via Onyx or Glubran 2. The risk factors of hemorrhage were analyzed using univariate and multivariate logistic analyses. A modified Rankin Scale (mRS) score was used to evaluate the neurological function before and 1 year after the operation. Results Of 125 patients, 63 had type I cAVMs, 2 type II cAVMs, 48 type III cAVMs, and 12 type IV cAVMs. A total of 88 (70.4%) patients had clinical manifestations of intracranial hemorrhage. Multivariate logistic regression analysis showed that age (OR, 2.276; 95% CI, 1.132 − 5.663), flow-related aneurysm (OR, 2.845; 95% CI, 1.265 − 6.248), lesion size (OR, 3.005; 95% CI, 1.119 − 5.936), and the number of feeding arteries (OR, 0.105; 95% CI, 0.081 − 0.312) were still the significant independent risk factors of intracranial hemorrhage. During a 1-year follow-up, 109 patients (87.2%) had good outcomes (mRS 0 − 2), 12 patients (9.6%) had poor outcomes (mRS 3 − 4), 4 patients (3.2%) died, and 3 patients had intracranial hemorrhage due to the incomplete embolization of cAVMs. Conclusions Endovascular embolization is a feasible treatment for cAVMs. Age, flow-related aneurysm, lesion size, and the number of feeding arteries are the significant risk factors of periprocedural hemorrhage. Moreover, the lesion characteristics must be given full consideration when using Onyx or Glubran 2 before cAVMs embolization.

Endovascular treatment of posterior inferior cerebellar artery trunk aneurysm.

The posterior inferior cerebellar artery (PICA) generally originates from the vertebral artery (VA) and is the most complex cerebellar artery. Aneurysms can occur at any site along the PICA trunk. Although most PICA aneurysms are located at the VA-PICA junction, a small proportion is located at the PICA trunk. Endovascular treatment (EVT) of aneurysms in the PICA trunk may be difficult and complex. We performed a review to expound upon EVT of PICA trunk aneurysms. The PICA can be divided into five segments (p1-5); the p1-3 segments are proximal segments, and the p4-5 segments are distal segments. Most PICA trunk aneurysms are dissecting aneurysms. Sometimes, the PICA can give rise to flow-related aneurysms in association with cerebellar arteriovenous malformations. Most aneurysms of the PICA trunk require aggressive treatment, especially those that have ruptured. Currently, the EVT mainly includes selective coiling with/without stent assistance and parent artery occlusion. Recently, some new devices, such as flow diversion and Barricade and Kaneka ED coils, can be used to treat PICA trunk aneurysms. The risk of complications with EVT seems to be higher; however, most complications are only transient or mild, and some are even clinically silent. In addition, open surgery is still an important option. For PICA trunk aneurysms, the treatment choice should be assessed on a case-by-case basis. The rate of permanent morbidity of the EVT is low. EVT is an effective method for treating PICA trunk aneurysms.

Parent artery occlusion for flow-related aneurysms at the proximal segment of the main feeding arteries of cerebellar arteriovenous malformations

Background Flow-related aneurysms (FRAs) associated with cerebellar arteriovenous malformations (AVMs) are complicated, and their management is difficult. We performed a retrospective study to explore the efficacy and outcome of parent artery occlusion (PAO) for FRAs at the proximal segment of the main feeding arteries of cerebellar AVMs. Materials and methods A retrospective study was performed for patients admitted for a cerebellar AVM between January 2015 and December 2019. Patients were included if (a) they presented with a ruptured or symptomatic FRA at the proximal segment of the main feeding arteries of the cerebellar AVM, (b) the cerebellar AVM did not bleed before admission, (c) the FRA was secured via PAO of the parent feeding artery, and (d) the cerebellar AVM was not intentionally managed or embolized through other feeding arteries during hospitalization. Results Eight patients aged between 27 and 72 (48.6 ± 14.3) years were identified. Eight ruptured and symptomatic FRAs at the proximal segment of the main feeding arteries of the cerebellar AVMs underwent PAO with coils or Onyx while preserving the arteries distal to the aneurysms. All the patients achieved a favorable recovery after treatment in a follow-up ranged from 8 to 48 months. One patient experienced FRA recurrence and underwent a second PAO. Conclusions PAO for FRAs at the proximal segment of the main feeding arteries of cerebellar AVMs is a feasible option. This method is effective in preventing re-rupture of the FRAs and relieving the symptoms in the short term, allowing sufficient time for the patients to receive subsequent treatment.

Imaging characteristics and endovascular treatment strategy for cerebellar arteriovenous malformations.

Cerebellar arteriovenous malformations (CAVMs) have increased probabilities of rupture and bleeding compared with arteriovenous malformations (AVMs) in other locations of the brain. Endovascular treatment (EVT) for CAVMs is difficult; as the angioarchitecture of CAVMs is complex, EVT may be associated with complications, due to the involvement of crucial structures, such as the brainstem. The present study aimed to determine the efficacy of EVT for CAVMS. For this purpose, 33 cases of CAVMs treated with EVT from January, 2015 to January, 2020 were retrospectively analyzed. The 33 patients were aged 8 to 73 years (mean age, 40.4±17.8 years) and 21 were female (63%, 21/33). Rupture and bleeding occurred in 29 patients (87.9%, 29/33). Among the 33 CAVM cases, 15 (45.5%, 15/33) were fed by a single artery, and 18 (54.5%, 18/33) were fed by multiple arteries. In total, 27 patients (81.8%, 27/33) had superficial vein drainage alone. Among the 33 cases, 15 were complicated by 16 aneurysms, including 14 prenidal aneurysms and 2 intranidal aneurysms. Among the 33 cases, the nidus of the CAVM (87.9%, 29/33) was treated with Onyx™ casting in 29 patients: 8 cases (27.6%, 8/29) had an embolization volume of <1/3 of the nidus, 11 cases had a volume of 1/3-2/3 of the nidus (37.9%, 11/29) and 10 cases had a volume >2/3 of the nidus (34.5%, 10/29). Among the EVT complications, there were 3 cases (9.1%, 3/33) of intraoperative and post-operative bleeding, which resulted in two deaths (on the 1st and 7th days). The length of hospital stay was 10.7±5.4 days. In total, 27 patients (81.7%, 27/33) had a Glasgow Outcome Scale (GOS) score of 5 at discharge. On the whole, the present study demonstrates that overall, EVT is a feasible treatment for CAVM and may be used to obtain acceptable therapeutic effects.

Combined strategies on the treatment of cerebellar arteriovenous malformation.

Cerebellar arteriovenous malformations (AVMs) comprise 10%–15% of all intracranial AVMs and have a higher risk for morbidity and mortality than supratentorial AVMs. Patients with cerebellar AVMs present with hemorrhage more often than patients with cerebral AVMs, justifying an interventional treatment. Patient outcome can be predicted with specific grade systems, guiding vascular neurosurgeons in decision-making. The authors present the case of a 42-year-old man incidentally diagnosed with an unruptured cerebellar inferior vermian AVM, which was managed through a combined strategy of preoperative endovascular embolization of the main arterial feeders followed by microsurgical resection via midline suboccipital craniotomy, with a favorable outcome.The video can be found here: https://youtu.be/3WESejZbk90

Microsurgical resection of unruptured cerebellar arteriovenous malformation presenting with trigeminal neuralgia.

Cerebellar arteriovenous malformations (AVMs) represent 10%–15% of all intracranial AVMs and are associated with a greater risk for hemorrhagic presentation compared with supratentorial AVMs. When they reach the cerebellopontine angle cistern, neurovascular compression syndromes, including trigeminal neuralgia and hemifacial spasm, can occur. Due to the aggressive natural history of cerebellar AVM, an effective treatment strategy is required. In this video, the authors demonstrate the technical nuances of microsurgical resection of an unruptured cerebellar AVM in a 24-year-old female presenting with trigeminal neuralgia. The patient underwent right retrosigmoid craniotomy and complete resection of the AVM with resolution of trigeminal neuralgia.The video can be found here: https://youtu.be/6GmNjgFQwx8

Gamma Knife Radiosurgery for Cerebellar Arteriovenous Malformation: Long-term Outcome and Safety Profile

Gamma Knife Radiosurgery for Cerebellar Arteriovenous Malformation: Long-term Outcome and Safety Profile

Intraoperative application of a new-generation 3D IV-DSA technology in resection of a hemorrhagic cerebellar AVM.

Although intravenous digital subtraction angiography (IV-DSA), cone-beam CT, and rotational angiography are well-established technologies, using them in a single system in the hybrid operating room to acquire high-quality noninvasive 3D images is a recent development. This video demonstrates microsurgical excision of a ruptured cerebellar arteriovenous malformation (AVM) in a 66-year-old male followed by intraoperative IV-DSA acquisition using a new-generation system (Artis Icono). IV-DSA confirmed in real time that no residual remained following excision without the need to reposition the patient. To the best of the authors’ knowledge, this is the first surgical video to demonstrate the simplified workflow and application of this technology in neurovascular surgery.The video can be found here: https://youtu.be/bo5ya9DQQPw

Microsurgical treatment of a posterior fossa arteriovenous malformation initially mistaken for a dural arteriovenous fistula: avoidance of near disaster.

Many brain arteriovenous malformations (AVMs) derive dural blood supply, while 10%–15% of dural arteriovenous fistulas (dAVFs) have pial arterial input. To differentiate between the two is critical, as treatment of these entities is diametrically opposed. To treat dAVFs, the draining vein(s) is disconnected from feeding arteries, which portends hemorrhagic complications for AVMs. The authors present an operative video of a subtle cerebellar AVM initially treated as a dAVF by attempted embolization through dural vessels. The lesion was subsequently microsurgically extirpated. The authors show a comparison case of an AVM mistaken for a dAVF and transvenous embolization that resulted in a fatal hemorrhage.The video can be found here: https://youtu.be/eDeiMrGoE0Q

Combined treatment for a hemispheric cerebellar AVM

Posterior fossa arteriovenous malformations (AVMs) can be a challenging disease, especially those large in size. AVMs can be treated with a combination of endovascular treatment and microsurgery. Here, the authors present the case of a 16-year-old female patient with progressive dizziness and episodic syncope. The workup of the patient showed a hemispheric cerebellar AVM, Spetzler-Martin grade IV. She underwent combined treatment (endovascular and microsurgery) with no complications and cure of the malformation.The video can be found here: https://youtu.be/rNw_Kyd76Mg

Endovascular treatment for the flow-related aneurysm originating from an anterior inferior cerebellar artery supplying the cerebellar arteriovenous malformation.

When a flow-related aneurysm originates from an anterior inferior cerebellar artery (AICA) supplying the cerebellar arteriovenous malformation (AVM), the management becomes very complicated. Endovascular treatment (EVT) was an option, but no consensus has been achieved.Methods and materials: A retrospective investigation was performed for patients with flow-related aneurysm originating from an AICA supplying the cerebellar AVM. Ten patients, harboring 13 aneurysms, were identified. Of the 5 a1 aneurysms, 2 underwent stent assisted coiling, 2 underwent parent artery occlusion (PAO), and 1 was intact. Of the 8 a2 aneurysms, 3 underwent coiling with preservation of the AICA, 3 underwent PAO with Onyx, 1 underwent PAO with coils, and 1 was intact. Seven patients underwent partial embolization of the cerebellar AVM, 3 were intact. One patient died 6 hours postoperatively for cerebellar AVM rebleeding. During a follow-up from 6 months to 6 years, 9 patients had favorable recovery. For the flow-related aneurysm originating from an AICA supplying the cerebellar AVM, the EVT depends on the specific circumstances. When the aneurysm is located at the a1 segment, coiling of the aneurysm with preservation of the parent AICA should be performed. PAO is the last resort. When the a2 aneurysm is proximal to the internal auditory artery, coiling of the aneurysm with preservation of the AICA is preferred. When the aneurysm is distal to the internal auditory artery, PAO can be safely performed.

Single-session Curative Embolization of Unruptured Cerebellar Arteriovenous Malformation with Precipitating Hydrophobic Injectable Liquid

AbstractMicrosurgery is the treatment of choice for cerebellar arteriovenous malformations (AVMs). Nevertheless, endovascular therapy aims to be an effective treatment since the introduction of new embolic agents. Cerebellar AVMs represent a subgroup of vascular lesions with a high rate of hemorrhagic presentation compared with their cerebral counterpart. Moreover, they have a higher incidence of flow-related aneurysms when diagnosed. With this in mind, prompt treatment must be performed to avoid rebleeding with devastating consequences. Herein, we present the case of an unruptured cerebellar AVM treated with precipitating hydrophobic injectable liquid (PHIL) with complete obliteration.

Mejora del equilibrio durante la marcha en paciente con hemiparesia secundaria a enfermedad vascular cerebral mediante el Concepto Bobath

Se plantea un tratamiento basado en el Concepto Bobath, para la rehabilitación de la Enfermedad Vascular Cerebral, principalmente para la mejora de control postural en el movimiento selectivo de extremidades y el desempeño de las actividades de la vida diaria. El reporte de caso correspondiente a una paciente femenina de 49 años de edad, sufrió un EVC con hemorragia intracraneal e intraventricular complicada con una meningitis a los de 38 años de edad, por una malformación arterio-venosa cerebelar diagnosticada desde los 4 años de edad. Se presentó al servicio de Terapia Física 10 años luego del evento y 6 años luego de su última rehabilitación, debido a que presentaba una marcha atáxica y pérdida de balance tanto en dinámico como estático. El abordaje consistió en rehabilitación basada en los principios del Concepto Bobath por dos semanas, con un total de 10 sesiones para realizar valoración, tratamiento y revaloración. La rehabilitación neurológica demuestra una mejora a corto plazo en sus actividades de la vida diaria y el control del movimiento para su desempeño.