Very small intracranial aneurysm

Very small intracranial aneurysm

Very small intracranial aneurysm (VSIA) (< 3 mm).

Ruptured VSIA group has higher percentage of females and lower aspect ratio than ruptured non-VSIA group. Further studies regarding the characteristics of ruptured and unruptured VSIA patients is required for assistance in clinical decision related to treatment of VSIA group before the aneurysm sac rupture 1).

The most common site of rupture of very small aneurysm was the anterior communicating artery (ACoA). Rupture of small and very small aneurysms is unpredictable, and treatment may be considered in selected high-risk patients according to factors such as young age, ACoA location, and hypertension 2).

Treatment of very small unruptured intracranial aneurysms (VSUIAs, defined as ≤3 mm) can be indicated in selected circumstances. The feasibility and outcomes of endovascular therapy for VSUIAs have been recently published; however, the efficacy and complication rate of surgical clipping has not been reported in any large series to date.

In a study, 183 patients (128 women, mean age 51.3 years) were treated with 190 procedures for a total of 228 aneurysms. Most were anterior circulation aneurysms (n = 215). The majority were directly clipped (n = 222, 97.4%), with coagulation or wrapping in the remainder. After 1 reoperation for incomplete clipping, postoperative imaging of 225 aneurysms confirmed complete occlusion in 221 (98.2%), 1 neck remnant (0.44%), and 3 partial occlusions (1.3%). Mortality was 0%. Early postoperative neurological deficit developed in 12 patients (6.6%); posterior circulation location was a significant risk factor for early neurological deficit (P < .001). Middle cerebral artery aneurysms had the lowest rate of postoperative deficits at 1.5% (P = .023). After the initial 30-day perioperative period, all deficits related to treatment of posterior circulation aneurysms recovered; overall neurological morbidity decreased to 2.7% with no mortality.

VSUIA clipping is highly effective and is associated with a low morbidity rate. For VSUIAs selected for treatment, our data support surgical clipping as the modality of choice 3).


Aneurysms treated with a Pipeline Embolization Device in vessels less than 2.5 mm between June 2012 and August 2014 were included. They evaluated risk factors, family history of aneurysms, aneurysm characteristics, National Institute of Health Stroke Scale (NIHSS), and modified Rankin scale (mRS) on admission and angiography and clinical outcome at discharge, 6 months, and 1 year.

They included seven patients with a mean age of 65 years. The parent vessel size ranged from 1.5 to 2.3 mm; mean 1.9 mm. Location of the aneurysms was as follows: two aneurysms centered along the pericallosal artery (one left, one right), one on the right angular artery, one aneurysm at the anterior communicating artery (ACom), one at the ACom-right A2 anterior cerebral artery (ACA), one at the lenticulostriate artery, and one at the A1-A2 ACA artery. Aneurysms ranged from 1 to 12 mm in diameter. All aneurysms were treated with a single Pipeline™ Embolization Device (PED). No peri- or post-procedural complications or mortality occurred. The patients were discharged with no change in NIHSS or mRS score. Angiographic follow-up was available in six patients. Angiography showed complete aneurysm occlusion in all. NIHSS and mRS remained unchanged at follow-up.

The preliminary results show that flow diversion technology is an effective and safe therapy for aneurysms located on small cerebral arteries. Larger studies with long-term follow-up are needed to validate our promising results 4).


1)

Park GT, Kim JH, Jung YJ, Chang CH. Characteristics of patients with ruptured very small intracranial aneurysm sized less than 3 mm. J Cerebrovasc Endovasc Neurosurg. 2020 Oct 22. doi: 10.7461/jcen.2020.E2020.07.001. Epub ahead of print. PMID: 33086456.
2)

Lee GJ, Eom KS, Lee C, Kim DW, Kang SD. Rupture of Very Small Intracranial Aneurysms: Incidence and Clinical Characteristics. J Cerebrovasc Endovasc Neurosurg. 2015 Sep;17(3):217-22. doi: 10.7461/jcen.2015.17.3.217. Epub 2015 Sep 30. PubMed PMID: 26526401; PubMed Central PMCID: PMC4626345.
3)

Bruneau M, Amin-Hanjani S, Koroknay-Pal P, Bijlenga P, Jahromi BR, Lehto H, Kivisaari R, Schaller K, Charbel F, Khan S, Mélot C, Niemela M, Hernesniemi J. Surgical Clipping of Very Small Unruptured Intracranial Aneurysms: A Multicenter International Study. Neurosurgery. 2016 Jan;78(1):47-52. doi: 10.1227/NEU.0000000000000991. PubMed PMID: 26317673.
4)

Puri AS, Massari F, Asai T, Marosfoi M, Kan P, Hou SY, Howk M, Perras M, Brooks C, Clarencon F, Gounis MJ, Wakhloo AK. Safety, efficacy, and short-term follow-up of the use of Pipeline™ Embolization Device in small (<2.5mm) cerebral vessels for aneurysm treatment: single institution experience. Neuroradiology. 2015 Dec 23. [Epub ahead of print] PubMed PMID: 26700827.

Spontaneous spinal subdural hematoma

Spontaneous spinal subdural hematoma

Spontaneous spinal subdural hematomas are extremely rare.

A nontraumatic acute spontaneous spinal subdural hematoma (sSDH) is a rare complication after spinal surgery. Although an sSDH is often associated with anticoagulation therapy, vascular malformations, or lumbar puncture, the pathogenesis of nontraumatic spontaneous sSDH remains unclear 1)

In the majority of cases, spontaneous hematomas are idiopathic. However, when attributed to anticoagulation therapy coumarins are more common than direct factor Xa inhibitors such as apixaban. Previous reports have linked direct factor Xa inhibitors with intracranial subdural hematomas much more frequently than spinal subdural hematomas. The manifestation of severe neurological deficits, such as sensorimotor disturbances and loss of sphincter control, is common and is considered a surgical emergency 2).


An 82-year-old patient with a history of ischemic heart disease and atrial fibrillation under acenocoumarol was admitted to emergency department with sudden onset of paraplegia and intense back pain associated with urinary incontinence and anal sphincter disorder. On examination his lower limb power was MRC grade 0 out of 5 in all ranges of movement bilaterally and a complete bilateral anesthesia reaching the T12 dermatome was noted. Biological test results showed an International Normalized Ratio at 10. Magnetic resonance imaging revealed a posteriorly located spinal hematoma at T12 level, measuring 36 mm with spinal cord compression. After correction of hemostasis disorders the patient was admitted to the operating room for a T11-L1 laminectomy with evacuation of the subdural hematoma. Muscle power showed a gradual improvement in the lower limbs estimated at 3/5 with regression of sphincter disorders but unfortunately a sequellar sensory impairment persisted.

SSH is a rare situation of acenocoumarol bleeding incident, it should be evoked in any patient treated by this molecule with signs of spinal cord compression 3).


A case of a patient with a spontaneous spinal thoracic subdural hematoma secondary to apixaban use with loss of sphincter control and paraplegia. After 6 months of follow-up, the patient recovered completely 4).

Spinal subdural hematoma (S-SDH) rarely occurs after aneurysmal subarachnoid hemorrhage (SAH). Little information is known regarding the management and prognosis of patients with both S-SDH and SAH. Here, we present an illustrative Case and provide a systematic review of S-SDH in the setting of SAH. METHODS:

A systematic literature review using PRISMA guidelines revealed 11 previous cases of concurrent intracranial SAH and spinal SDH, which are presented with our new reported Case. RESULTS:

Intracranial sources of spontaneous SAH included 8 aneurysms, 1 pseudoaneurysm, and 3 angiogram negative cases. Hunt Hess grade ranged from 1-4, mean time between SAH and S-SDH was 5.8 days, and S-SDH presented most frequently in the lumbar spine. 8 patients showed significant to complete clinical recovery, 2 had continued plegia of the lower extremities, and 2 expired. Modified Rankin score ranged from 0-6, with mRS > 2 for 4 out of 12 patients. Patients with a poor clinical outcome (mRS > 2) had an initially negative cerebral angiogram, earlier presentation with less time between SAH and S-SDH (0.8 vs 7.6 days), use of antithrombotic medication, no diversion of CSF, and cervical or thoracic S-SDH. CONCLUSION:

S-SDH is an uncommon occurrence in the setting of aneurysmal SAH with better outcomes associated with lumbar location, delayed presentation, CSF diversion, and lack of antithrombotic use. Conservative treatment may be sufficient in cases with delayed S-SDH and lack of significant neurological deficits. More reported cases will allow greater understanding of this clinical entity 5).

Surgical intervention is recommended in patients presenting with severe neurologic deficits. Conservative treatment is a reasonable option for asymptomatic patients 6).

Raymaekers et al. presented the case of an intradural hematoma after an extraforaminal surgery through the Wiltse approach for an extraforaminal disk herniation at L5/S1. This 58-year-old woman experienced hypoesthesia and progressive motor dysfunction in the left leg several hours postoperation. Urgent magnetic resonance imaging revealed an intradural hematoma at the L1/L2 to L2/L3 level in the ventral dural sac proximal to the surgical level. Surgical decompression was performed. There was no evidence of trauma, coagulopathy, or anticoagulation therapy. To our knowledge, this case is the first to report an acute sSDH proximal to the surgery level after an extraforaminal spinal surgery through the Wiltse approach for an extraforaminal disk herniation. It illustrates that attentive postoperative neurologic monitoring, even in the absence of intraoperative irregularities, remains important to diagnose and treat this complication at the early stage 7)


A 55-year-old woman without malignancy or coagulopathy history presented with progressive low back pain for the past 2 weeks. Progressive bilateral leg weakness happened 1 week ago. On the day she called for help, she presented with bilateral leg grade 2 muscle power and generalized back pain. There was no headache or meningeal sign. An absent bilateral knee reflex was found. Magnetic resonance imaging showed a space-occupying lesion at the T2-T6 and T12-L1 levels in the ventral and dorsal spinal canal, leading to cord compression. Due to rapid neurologic function deterioration, emergent T12-L1 laminectomy was performed. We found a T12-L1 tense dura sac with subdural hematoma ventral to the cord. Removal of the SDH was performed. T2-T6 levels were treated conservatively. She returned ambulant 1 week after operation. Magnetic resonance images at 3 months and 1 year later showed the SDH being absorbed and replaced by adhesive arachnoid cysts along the whole T and L spine. However, these lesions are asymptomatic for at least 2 years 8).


Sanchez et al. reported a case of Reverse Takotsubo Cardiomyopathy in an otherwise healthy 23-year-old man presenting with back pain, urinary retention, bradycardia, and hypertension. Troponin levels and brain natriuretic peptide (BNP) were elevated, and echocardiogram revealed an ejection fraction (EF) of less than 20%. In addition, MRI demonstrated a spinal subdural hematoma from T1-S1 with no cord compression. Repeated echocardiogram demonstrated an EF of 20-25% with a reverse Takotsubo pattern of cardiomyopathy. With supportive care, his clinical picture improved with normalization of cardiac enzyme and BNP values. This case represents a r-TTC presenting as heart failure in a young, apparently healthy male likely incited by a spinal subdural hematoma. To our knowledge, it is the first of its kind reported 9).


A 7-yr old girl presented to Neurology Department, Mofid Hospital, ShahidBeheshti University of Medical Sciences, Tehran, Iran with limping and pain in lower extremities and acute paraplegia without history of direct trauma. The patient had muscle weakness in lower limbs and was unable to bear weight. Deep Tendon Reflexes (DTR) in lower extremities had increased. Her MRI showed spinal subdural hematoma we reextended from T2 to T6. We performed laminectomy from T2 to T5 and about 70 cc of subdural hematoma was evacuated. One month after the surgery, the patient’s neurological deficit resolved completely. The results showed the pivotal role of attention to clinical manifestation in acute spinal subdural hematoma and early diagnosis to prevent irreversible neurologic complication 10)


Spinal subdural hematoma in pediatric nonaccidental trauma 11)


A case of spontaneous, atraumatic subdural hematoma involving the thoracic region in an 80-year-old woman on warfarin is reported. The patient presented with gross motor and sensory loss, delayed onset of incontinence, and no other symptoms. An MRI suggested an epidural hematoma concentrated around the T4-T9 levels. She was taken emergently to the OR approximately 30 hours after the initial onset of symptoms for a T3-T11 laminectomy. No epidural hematoma was noted. However, discoloration and bulging of the thecal sac were noted, and the dura was incised longitudinally from T2 to T10 revealing an expansive jelly-like blood clot which was evacuated. Postoperatively, the patient had regained 1/2 sensory function in the bilateral lower extremities. At the 2-week mark, the patient was still incontinent and showed 2/2 sensory and 2/5 motor functions in select muscle groups in her bilateral lower extremities. Completely nontraumatic, spontaneous subdural hematomas of the spine are very rare, and early surgical decompression within 24 hours from symptom onset may allow neurological recovery. Large extensive laminectomies up to 10 thoracic levels have been shown to be safe and effective in a few cases, including our case 12).


Acute lumbar spinal subdural hematoma inducing paraplegia after lumbar spinal manipulation 13).


Cases of non-traumatic spinal subdural hematoma accompanied by intracranial hemorrhage are even more rare. There are a few reports of spontaneous spinal subdural hematoma with concomitant intracranial subdural or subarachnoid hemorrhage, but not with intracerebral hemorrhage. Especially in a case of Lee et al., the evaluation and diagnosis were delayed because the spontaneous intracerebral hemorrhage accompanying the unilateral spinal subdural and subarachnoid hemorrhages caused hemiplegia. They reported a case of spinal subdural and subarachnoid hemorrhage with concomitant intracerebral hemorrhage, for the first time, with a relevant literature review 14).


A 76-year-old woman with a spinal subdural hematoma (SDH) was presented with severe back pain without headache. Magnetic resonance imaging (MRI) performed 4 days after onset showed SDH extending from Th2 to L3. She was diagnosed with spontaneous SDH without neurological manifestation, and conservative treatment was selected. Transient disturbance of orientation appeared 7 days after onset. Small subarachnoid hemorrhage (SAH) was detected on head CT, and strict antihypertensive therapy was started. Symptoms changed for the better. Back pain disappeared 4 weeks after onset. On follow-up MRI at 6 months after onset, the SDH had been resolved spontaneously. Although adhesive arachnoiditis was observed at Th4-6, the recurrence of clinical symptoms was not observed at one year and a half after onset. Spinal subdural space is almost avascular; a hematoma in a subdural space is considered to come from a subarachnoid space when it is a lot. A hemorrhage in subarachnoid space was flushed by cerebral spinal fluid; hematoma or arachnoiditis was not formed in general. In this case, hemorrhage was a lot and expansion of SDH was large enough to cause cranial SAH and arachnoiditis. But longitudinally expanded SDH did not show neurological manifestation and resolved spontaneously 15).


A 38-year-old male patient presented with sudden lower back and bilateral leg pain.

A magnetic resonance imaging (MRI) scan on the third day after the onset of symptoms revealed a subdural hematoma from L1 to S1, presenting as hyperintensities on T1 weighted sequences and hypointensities to isointensities on T2 weighted sequences.

Laminectomy and subdural evacuation were performed immediately.

An abnormal ligamentum flavum was observed intraoperatively. A histological examination revealed extravasation of blood in the degenerated ligamentum flavum. Postoperatively, the lower limb pain improved immediately. At the 6-month follow-up, the pain and numbness of the lower limb disappeared, and the muscle strength of both legs recovered completely with normal gait.

Spontaneous SSDH with ligamentum flavum hematoma was caused by a sudden increase of intravenous pressure, resulting from a marked surge in the intra-abdominal or intrathoracic pressure. Consecutive MRI scans provided valuable information, leading to a diagnosis of spontaneous SSDH 16).


Oh et al. presented a case of acute nontraumatic SSDH presenting with transient left hemiplegia for 4 hours. A magnetic resonance imaging study of cervical spine confirmed SSDH with C3-6 cervical cord compression at the left side. The patient had conservative management without recurrence. Although hemiplegia is an unusual clinical manifestation of SSDH, it should be differentiated from that of cerebrovascular origin promptly. Conservative management may be an alternative therapeutic option for selective cases with transient neurological deficits 17).


1) , 7)

Raymaekers V, Beck T, Goebel S, Janssens F, Van den Branden L, Menovsky T, Plazier M. An Acute Spinal Intradural Hematoma after an Extraforaminal Wiltse Approach: A Case Report and Review of the Literature. J Neurol Surg A Cent Eur Neurosurg. 2020 Oct 21. doi: 10.1055/s-0040-1714432. Epub ahead of print. PMID: 33086421.
2) , 4)

Ardebol J, Cahueque M, Lopez W, Azmitia E. Spontaneous thoracic spinal subdural hematoma associated with apixaban therapy. J Surg Case Rep. 2019 Apr 27;2019(4):rjz115. doi: 10.1093/jscr/rjz115. eCollection 2019 Apr. PubMed PMID: 31044059; PubMed Central PMCID: PMC6486654.
3)

Aissa I, Elkoundi A, Andalousi R, Benakrout A, Chlouchi A, Moutaoukil M, Laaguili J, Bensghir M, Balkhi H, Lalaoui SJ. Unusual localization of bleeding under acenocoumarol: Spinal subdural hematoma. Int J Surg Case Rep. 2019;59:15-18. doi: 10.1016/j.ijscr.2019.04.053. Epub 2019 May 10. PubMed PMID: 31100481; PubMed Central PMCID: PMC6522769.
5)

Rothrock RJ, Li AY, Rumsey J, Fifi JT, Kellner CP, Roonprapunt C. Aneurysmal Subarachnoid Hemorrhage with Spinal Subdural Hematoma: A Case Report and Systematic Review of the Literature. World Neurosurg. 2019 May 16. pii: S1878-8750(19)31343-9. doi: 10.1016/j.wneu.2019.05.069. [Epub ahead of print] Review. PubMed PMID: 31103768.
6) , 8)

Gan CW, Chen SY, Chang CS, Liu JD. Spontaneous Spinal Subdural Hematoma: Case Report of 2 Years’ Clinical and Radiologic Findings. World Neurosurg. 2019 Jul;127:275-278. doi: 10.1016/j.wneu.2019.04.063. Epub 2019 Apr 13. PubMed PMID: 30986583.
9)

Sanchez K, Glener S, Esplin NE, Okorie ON, Parikh A. A Case of Reverse Takotsubo Cardiomyopathy Incited by a Spinal Subdural Hematoma. Case Rep Neurol Med. 2019 Jul 22;2019:9285460. doi: 10.1155/2019/9285460. eCollection 2019. PubMed PMID: 31428488; PubMed Central PMCID: PMC6679891.
10)

Farzan A, Pourbakhtyaran E, Moosavian T, Moosavian H. Spinal Subdural Hematomas in a Normal Child without Trauma History: A Case Report. Iran J Child Neurol. 2019 Summer;13(3):121-124. PubMed PMID: 31327977; PubMed Central PMCID: PMC6586447.
11)

Hong CS, Camara-Quintana J, Kundishora AJ, Diluna ML, Kahle KT. Teaching NeuroImages: Spinal subdural hematoma in pediatric nonaccidental trauma. Neurology. 2019 Jul 30;93(5):e522-e523. doi: 10.1212/WNL.0000000000007869. PubMed PMID: 31358679.
12)

Arain AR, Moral M, Shams S, Desai K, Kalsa K. Atypical Presentation of Atraumatic Spinal Subdural Hematoma Associated with Warfarin: A Case Report and Review of the Literature. Case Rep Orthop. 2019 May 20;2019:4037916. doi: 10.1155/2019/4037916. eCollection 2019. PubMed PMID: 31236299; PubMed Central PMCID: PMC6545747.
13)

Benyaich Z, Laghmari M, Lmejjati M, Aniba K, Ghannane H, Benali SA. Acute lumbar spinal subdural hematoma inducing paraplegia after lumbar spinal manipulation: A case report and literature review. World Neurosurg. 2019 May 9. pii: S1878-8750(19)31275-6. doi: 10.1016/j.wneu.2019.05.002. [Epub ahead of print] PubMed PMID: 31078801.
14)

Lee Y, Lim J, Han S, Choi SW, Youm JY, Koh HS. Spontaneous Spinal Subdural and Subarachnoid Hemorrhage with Concomitant Intracerebral Hemorrhage: A Case Report. Korean J Neurotrauma. 2019 Apr 19;15(1):34-37. doi: 10.13004/kjnt.2019.15.e7. eCollection 2019 Apr. PubMed PMID: 31098347; PubMed Central PMCID: PMC6495584.
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Go T, Tsutsui T, Iida Y, Fukutake K, Fukano R, Ishigaki K, Tsuchiya K, Takahashi H. A Case of Spontaneous Spinal Subdural Hematoma Complicated by Cranial Subarachnoid Hemorrhage and Spinal Adhesive Arachnoiditis. Case Rep Orthop. 2019 Mar 13;2019:7384701. doi: 10.1155/2019/7384701. eCollection 2019. PubMed PMID: 31001442; PubMed Central PMCID: PMC6436331.
16)

Li X, Yang G, Wen Z, Lou X, Lin X. Surgical treatment of progressive cauda equina compression caused by spontaneous spinal subdural hematoma: A case report. Medicine (Baltimore). 2019 Mar;98(12):e14598. doi: 10.1097/MD.0000000000014598. PubMed PMID: 30896615.
17)

Oh SH, Han IB, Koo YH, Kim OJ. Acute spinal subdural hematoma presenting with spontaneously resolving hemiplegia. J Korean Neurosurg Soc. 2009 Jun;45(6):390-3. doi: 10.3340/jkns.2009.45.6.390. Epub 2009 Jun 30. PubMed PMID: 19609426; PubMed Central PMCID: PMC2711240.

High-grade arteriovenous malformation

High-grade arteriovenous malformation

High-grade arteriovenous malformations (AVMs), such as Spetzler-Martin AVM grading system 4 and 5, 1) are generally considered difficult to cure using any modalities such as surgery, embolization, and/or radiosurgery 2). However, endovascular treatment potentially offers an advantage over the other two methods because of the ability to immediately target certain areas of an AVM. Partially targeted embolization could be effective in controlling the bleeding point when treating high-grade AVMs; however, it is not curative 3) 4).

Grade 4 and 5 AVMs with supply from lenticulostriate, choroidal, thalamic deep perforating arteries or deep meningeal recruitment may be best treated conservatively or possibly by multimodality treatment utilising radiotherapy and embolisation combined with surgery 5).

Untreated high grade AVMs presenting with hemorrhage have a significant risk of subsequent rupture, and their rupture carries a higher risk of case fatality and permanent morbidity than AVMs in general. The risks associated with their treatment should be appraised in light of perilous natural history 6).

A retrospective analysis of a prospectively maintained database was performed in children with treated and nontreated pediatric AVMs at the University of California, San Francisco, from 1998 to 2017. Inclusion criteria were age ≤ 18 years at time of diagnosis and an AVM confirmed by a catheter angiogram.

The authors evaluated 189 pediatric patients with AVMs over the study period, including 119 ruptured (63%) and 70 unruptured (37%) AVMs. The mean age at diagnosis was 11.6 ± 4.3 years. With respect to Spetzler-Martin (SM) grade, there were 38 (20.1%) grade I, 40 (21.2%) grade II, 62 (32.8%) grade III, 40 (21.2%) grade IV, and 9 (4.8%) grade V lesions. Six patients were managed conservatively, and 183 patients underwent treatment, including 120 resections, 82 stereotactic radiosurgery (SRS), and 37 endovascular embolizations. Forty-four of 49 (89.8%) high-grade AVMs (SM grade IV or V) were treated. Multiple treatment modalities were used in 29.5% of low-grade and 27.3% of high-grade AVMs. Complete angiographic obliteration was obtained in 73.4% of low-grade lesions (SM grade I-III) and in 45.2% of high-grade lesions. A periprocedural stroke occurred in a single patient (0.5%), and there was 1 treatment-related death. The mean clinical follow-up for the cohort was 4.1 ± 4.6 years, and 96.6% and 84.3% of patients neurologically improved or remained unchanged in the ruptured and unruptured AVM groups following treatment, respectively. There were 16 bleeding events following initiation of AVM treatment (annual rate: 0.02 events per person-year).

Coordinated multidisciplinary evaluation and individualized planning can result in safe and effective treatment of children with AVMs. In particular, it is possible to treat the majority of high-grade arteriovenous malformations with an acceptable safety profile. Judicious use of multimodality therapy should be limited to appropriately selected patients after thorough team-based discussions to avoid additive morbidity. Future multicenter studies are required to better design predictive models to aid with patient selection for multimodal pediatric care, especially with high-grade AVMs 7).


Long-term Outcomes With Planned Multistage Reduced Dose Repeat Stereotactic Radiosurgery for Treatment of Inoperable High-Grade Arteriovenous Malformations: An Observational Retrospective Cohort Study 8).


Treatment of Spetzler-Martin Grade IV and V brain arteriovenous malformations (ie, high-grade AVMs) carries a high risk of morbidity and even mortality. However, little is known about the behavior of these lesions if left untreated.

Objective: To investigate the natural history of patients with high-grade AVMs.

Methods: Patients with untreated high-grade AVMs admitted to our center between 1952 and 2005 were followed from admission until death, AVM rupture, or initiation of treatment. Rates of rupture and various risk factors were analyzed using Kaplan-Meier life table analyses and Cox proportional hazards models. Functional outcome was assessed 1 year after possible AVM rupture using the Glasgow Outcome Scale.

Results: Sixty-three patients with a mean follow-up time of 11.0 years (range, 1 month to 39.6 years) were identified. Twenty-three patients (37%) experienced a subsequent rupture. The average annual rate of rupture was 3.3%. In patients with hemorrhagic presentation, the annual rate was 6.0%, compared to 1.1% in patients with unruptured AVMs (P = .001, log-rank test; hazard ratio, 5.09 [1.40-18.5, 95% CI]; P = .013, multivariate Cox regression model). One year after the first subsequent rupture, 6 patients (26%) had died, and 9 (39%) had moderate or severe disability.

Untreated high grade AVMs presenting with hemorrhage have a significant risk of subsequent rupture, and their rupture carries a higher risk of case fatality and permanent morbidity than AVMs in general. The risks associated with their treatment should be appraised in light of perilous natural history 9).


Jayaraman et al. examined the prospective annual risk of hemorrhage in patients harboring Spetzler-Martin grades IV and V arteriovenous malformations (AVMs) before and after initiation of treatment.

Medical records of 61 consecutive patients presenting with Spetzler-Martin grades IV and V AVMs were retrospectively reviewed for demographics, angiographic features, presenting symptom(s), and time of all hemorrhage events, before or after treatment initiation. Pretreatment hemorrhage rates (excluding hemorrhages at presentation) and posttreatment rates were subsequently calculated. Modified Rankin Scale (mRS) scores before and after treatment were recorded.

The annual pretreatment hemorrhage rate for all patients was 10.4% per year (95% CI, 2.2 to 15.4%), 13.9% (95% CI, 3.5 to 22.1%) in patients with hemorrhagic presentation and 7.3% (2.6 to 14.3%) in patients with nonhemorrhagic presentation. Posttreatment hemorrhage rates were 6.1% per year (95% CI, 2.5 to 13.2%) for all patients, 5.6% (95% CI, 2.1 to 11.8%) for patients presenting with hemorrhage and 6.4% (95% CI, 1.6 to 10.1%) in patients with nonhemorrhagic presentation. A noninferiority test showed that the posttreatment hemorrhage rate was less than or equal to the pretreatment hemorrhage rate (P<0.0001), with some indication that the reduction was greatest in patients with hemorrhagic presentation. Of the 62 patients, 51 (82%) had an mRS score of 0 to 2 before treatment, and 47 (76%) had an mRS score of 0 to 2 at the last follow-up after treatment.

The annual rate of hemorrhage in grades IV and V AVMs is higher in this series than reported for all AVMs, which may reflect some referral bias in this single-center study. Nevertheless, initiation of treatment does not appear to increase the rate of subsequent hemorrhage. Treatment for these lesions may be warranted, given their poor natural history 10).


Between July 1997 and May 2000, 73 consecutive patients with Grades IV and V AVMs were evaluated prospectively by the cerebrovascular team at Barrow Neurological Institute. Treatment recommendations given to the patients or referring physicians were classified as complete treatment, partial treatment, and no treatment. Retrospectively, the hemorrhage rates associated with these treatment groups were also calculated. In the prospective portion of the study (the intention-to-treat analysis), no treatment of the AVM, was recommended for 55 patients (75%) and partial treatment was recommended for seven patients (10%). Aneurysms associated with an AVM were obliterated by surgical or endovascular treatment in seven patients (10%), and complete surgical removal was recommended for four patients (5%). The overall hemorrhage rate for Grades IV and V AVMs was 1.5% per year. The annual risk of hemorrhage was 10.4% among patients who previously had received incomplete treatment, compared with patients without previous treatment.

The hemorrhage risk of 1.5% per year, which was associated with Grades IV and V AVMs appears to be lower than that reported for Grades I through III AVMs. The authors recommend that no treatment be given for most Grades IV and V AVMs. No evidence indicates that partial treatment of an AVM reduces a patient’s risk of hemorrhage. In fact, partial treatment may worsen the natural history of an AVM. The authors do not support palliative treatment of AVMs, except in the specific circumstances of arterial or intranidal aneurysms or progressive neurological deficits related to vascular steal. Complete treatment is warranted for patients with progressive neurological deficits caused by hemorrhage of the AVM. This selection process plays a significant role in the relatively low combined morbidity and mortality rates for Grade IV and Grade V AVMs (17 and 22%, respectively) reported by the cerebrovascular group in both retrospective and prospective studies. 11).


The aim of a study was to compare operatively and non-operatively managed high-grade arteriovenous malformations (AVMs) and to identify risk factors for surgical morbidity. Three hundred and ninety-one consecutively enrolled patients with AVMs were graded using the Spetzler Martin grading scheme. Forty-six of these patients had grade 4 or 5 AVMs. Twenty-nine patients underwent surgery and 17 were conservatively managed. During an average of 33 months follow-up the non-operative group experienced a decline in function in 27% of cases followed. These deteriorations were due to haemorrhage, progressive neurological deficits and seizures. In the surgical group completing treatment there was a mortality and morbidity impacting on self-care of 15%. In those without deep perforating arterial supply the morbidity was 10% and with deep perforating arterial supply or deep meningeal recruitment there was a combined morbidity and mortality of 44%. This difference in outcome was statistically significant (P<0.01). We conclude that high-grade AVMs have a high operative morbidity. However, these lesions often have a poor natural history and with careful selection (based on the presence or absence of deep perforating arterial supply) a group can be selected that benefits from surgery. Grade 4 and 5 AVMs with supply from lenticulostriate, choroidal, thalamic deep perforating arteries or deep meningeal recruitment may be best treated conservatively or possibly by multimodality treatment utilising radiotherapy and embolisation combined with surgery 12).


1)

Spetzler RF, Martin NA.: A proposed grading system for arteriovenous malformations. J Neurosurg 65: 476– 483, 1986.
2)

Ogilvy CS, Stieg PE, Awad I, Brown RD, Jr, Kondziolka D, Rosenwasser R, Young WL, Hademenos G, Special Writing Group of the Stroke Council American Stroke Association : AHA Scientific Statement: Recommendations for the management of intracranial arteriovenous malformations: a statement for healthcare professionals from a special writing group of the Stroke Council, American Stroke Association. Stroke 32: 1458– 1471, 2001.
3)

Krings T, Hans FJ, Geibprasert S, Terbrugge K.: Partial “targeted” embolisation of brain arteriovenous malformations. Eur Radiol 20: 2723– 2731, 2010.
4)

Le Feuvre D, Taylor A.: Target embolization of AVMs: identification of sites and results of treatment. Interv Neuroradiol 13: 389– 394, 2007.
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