Spinal subarachnoid hemorrhage and aneurysms.

Spinal subarachnoid hemorrhage (SAH) is a rare disease. Spinal aneurysms are even rarer and mostly undetected unless they rupture and become symptomatic. In this chapter we aim to review the available literature about spinal subarachnoid hematoma with special emphasis on spinal aneurysms. As most reports of spinal aneurysms describe a single case or a small case series, the diagnostic algorithm is often lacking. The outcome is also different based on the etiologies; therefore management strategy must be individualized. We addressed these issues in this chapter. The reported incidence of spinal SAH is less than epidural hematoma and more than subdural hematoma. Spinal aneurysms can present as isolated entity or can be associated with other vascular anomalies. Microsurgical clipping and/or resection is possible, especially when they are located dorsally or dorsolaterally. Endovascular approach is also a feasible option unless negotiation of microcatheter becomes difficult in tortuous small-caliber arteries. Successful obliteration leads to good outcome, especially when present in posterior spinal artery. A detailed knowledge of spinal SAH and spinal aneurysms is important to detect them in time. Clinicians must consider several factors to choose an appropriate treatment strategy to ensure the safety of their patients.

Risk factors of hematomyelia recurrence and clinical outcome in children with intradural spinal cord arteriovenous malformations.

BACKGROUND AND PURPOSE: Few published data are available concerning the risk of re-bleeding of spinal cord AVM after an hematomyelia and concerning the long-term clinical outcome. Our aim was to assess the risk of recurrence and long-term clinical outcome after hematomyelia in children with spinal cord AVMs. MATERIALS AND METHODS: This single-center retrospective study reviewed the clinical and radiologic data of 28 children younger than 18 years of age with arteriovenous malformation who had experienced at least 1 episode of hematomyelia between 1988 and 2012. Long-term clinical outcome was assessed by the American Spinal Injury Association Impairment Scale, and radiologic review included MR imaging and angioarchitecture on angiography (blinded to clinical information) before treatment and at recurrence. RESULTS: Sixteen children (57%) experienced 1 episode of hematomyelia, while 12 children (43%) experienced recurrence. Girls and boys were equally affected (sex ratio, 1:1), and mean clinical follow-up was 5.7 ± 4.4 years. The risk of recurrence was higher for AVMs of the cervical and upper thoracic spine, 12 (100%) versus 11 (69%) (P = .01). A high American Spinal Injury Association scale score at last follow-up was reported for 11 children (39%), and the risk of recurrence tended to be associated with poorer functional prognosis (7 [64%] versus 5 [29%], P = .07). At the time of recurrence, perimedullary venous drainage was the main factor associated with recurrence (P = .002). Occlusion rate ≥50% was associated with a decreased risk of recurrence (P = .047). CONCLUSIONS: In the present series, cervical and upper thoracic spinal cord AVMs and microarchitecture were predictive of the risk of hematomyelia recurrence. Perimedullary venous drainage was one of the main parameters associated with recurrence. Functional prognosis was better in patients with a single episode of hematomyelia.

Endovascular treatment of spinal vascular lesion in Japan: Japanese Registry of Neuroendovascular Therapy (JR-NET) and JR-NET2.

A subgroup analysis of spinal vascular lesions in the Japanese Registry of Neuroendovascular Therapy (JR-NET) and JR-NET2, retrospective registry studies conducted in 2005-2009, was performed to understand the current status of treatment in Japan. Of 201 spinal lesions enrolled, 98 analyzable cases of spinal dural arteriovenous fistula (SDAVF), 43 of spinal perimedullary arteriovenous fistula (SPAVF), and 23 of spinal intramedullary arteriovenous malformation (SIAVM) were assessed. Treatment was radical in the majority (83.6%) of SDAVF, palliative in the majority (70.6%) of SIAVM, and radical and palliative in a similar number of cases of SPAVF. Total occlusion was achieved in 26 (54.2%) SDAVF cases, 9 (29.0%) SPAVF, and 4 (23.5%) SIAVM. Treatment-related complications occurred in 3 (3.1%) SDAVF cases, 7 (16.3%) SPAVF, and 1 (4.3%) SIAVM. Post-treatment neurological improvement was achieved in 49 (50.0%) of SDAVF cases, 15 (34.9%) SPAVF, and 5 (21.7%) SIAVM. The modified Rankin Scale (mRS) of 0, 1, or 2 on postoperative day 30, the primary endpoint, was achieved in 62 (63.3%) SDAVF cases, 26 (60.5%) SPAVF, and 12 (52.2%) SIAVM. The mRS of 0-2 on postoperative day 30 was correlated with presymptomatic mRS of 0-2 [P < 0.0001, odds ratio (OR): 42.88, 95% confidence interval (CI): 14.83-123.97] and postoperative neurological improvement (P = 0.046, OR: 2.57, 95% CI: 1.02-6.48). In Japan, endovascular treatment of spinal vascular lesions was administered safely. Good mRS on postoperative day 30 was highly correlated with good pre-symptomatic mRS, suggesting necessity of early diagnosis and treatment.

Spinal extradural arteriovenous fistulas: clinical article.

OBJECT: Our understanding of spinal extradural arteriovenous fistulas (eAVFs) is relatively limited. In this study the authors aimed to provide the demographics, natural history, and treatment results of these rare lesions. METHODS: The authors performed a pooled analysis of data in the PubMed database through December 2012. Individualized patient data were extracted to elucidate demographic, clinical, and angioarchitectural features of spinal eAVFs as well as outcomes following different treatment strategies. RESULTS: Information on 101 patients was extracted from 63 eligible studies. The mean patient age was 45.9 years, and there was no significant overall sex predilection. Only 3% of the lesions were incidental, whereas 10% occurred in patients who had presented with hemorrhage. None of the 64 patients with at least 1 month of untreated follow-up sustained a hemorrhage over a total of 83.8 patient-years. Patients with lumbosacral eAVFs were significantly older (mean age 58.7 years, p < 0.0001), were significantly more often male (70% male, p = 0.02), had significantly worse presenting Aminoff-Logue motor and bladder scores (p = 0.0008 and < 0.0001, respectively), and had the greatest prevalence of lesions with intradural venous drainage (62% of cases, p < 0.0001). Neurofibromatosis Type 1 (30% of cases, p < 0.0001) and subarachnoid hemorrhage (9% of cases, p = 0.06) were associated with and exclusively found in patients with cervical eAVFs. The overall complete obliteration rate was 91%. After a mean follow-up of 1.7 years, the clinical condition was improved in 89% of patients, the same in 9%, and worse in 2%. Obliteration rates and outcome at follow-up did not significantly differ between surgical and endovascular treatment modalities. CONCLUSIONS: Spinal eAVFs are rare lesions with a low risk of hemorrhage; they cause neurological morbidity as a result of mass effect and/or venous hypertension. Their treatment is associated with a high rate of complete obliteration and improvement in preoperative symptoms.

Spinal arteriovenous metameric syndrome: clinical manifestations and endovascular management.

BACKGROUND AND PURPOSE: SAMS is a rare form of SCAVM. We discuss the clinical presentation, endovascular management, and outcome of this disease in our series. MATERIALS AND METHODS: Retrospective review was performed in patients with SCAVM and SAMS who underwent angiography with intent to treat at our institution from 1980 to 2010. RESULTS: One hundred forty-eight SCAVMs were identified, and 28 (19%) of these were SAMS. Of these 28 patients, 24 had nidus-type AVMs and 4 had fistulas. SAMS were more prevalent in females (71% versus 48%), and also presented earlier than non-SAMS SCAVMs. Intradural hemorrhage (SAH or hematomyelia) was the most common presentation and more common than in non-SAMS lesions. Twenty-six patients underwent embolization of the intradural lesion in 50 sessions. Thirteen patients underwent treatment of intradural aneurysms in 16 sessions. Six patients underwent embolization of extradural lesions in 16 sessions. Twenty-three patients had an average of 94 months of clinical follow-up (3-309 months) after the first treatment, during which 5 patients had hemorrhages. Angiographic follow-up was performed in 20 patients at an average of 85 months (range, 3-309 months), which showed new development or enlargement of an aneurysm in 5 patients. This type of angiographic progression was more common in patients with SAMS. CONCLUSIONS: SAMS most commonly presents with hemorrhage from the SCAVM. Endovascular embolization can be performed safely with good functional outcome, though most patients clinically deteriorate in the long term. Periodic angiographic follow-up with intent to perform preventive target embolization is important to control the disease.

National trends in spinal arteriovenous malformations.

OBJECT: Spinal arteriovenous malformations (AVMs) are rare and understudied vascular lesions that cause neurological insult by mass effect, venous obstruction, and vascular steal. These lesions are challenging entities to treat because of their complicated anatomy and physiology. Current management options include open microsurgery, endovascular embolization, and stereotactic radiosurgery. METHODS: Our study used the National Inpatient Sample database to analyze outcome data for spinal AVMs treated nationwide over an 11-year period from 1995 through 2006. Trends in procedural management, hospital course, and epidemiology of spinal AVMs are investigated. RESULTS: Annually, an average of 300 patients presented with spinal AVMs requiring hospital treatment. The average length of hospital stay for this treatment has declined from more than 9 days in 1995 to 6 days in 2006. However, the average cost of a hospital stay has increased from < $30,000 to nearly $70,000. Whereas one-half of spinal AVMs were treated operatively in 1995, one-third were managed operatively in 2006. CONCLUSIONS: Spinal AVMs are being increasingly treated by endovascular, radiosurgical, or combined means. A discussion of modern strategies to treat these disorders is presented.

Neurovascular complications of marfan syndrome: a retrospective, hospital-based study.

BACKGROUND AND PURPOSE: Small case series have associated Marfan syndrome with cerebral and spinal ischemia or hemorrhage. However, there has been no investigation of the frequency and etiology of neurovascular disorders in a large series of Marfan patients. METHODS: We conducted a retrospective, hospital-based study of all Marfan syndrome patients seen in an 8-year period. Records were reviewed in detail, and clinical characteristics of those with and without a neurovascular diagnosis compared. RESULTS: Of 513 patients, 18 (3.5%) had a neurovascular diagnosis, as follows: transient ischemic attack (11), cerebral infarction (2), spinal cord infarction (2), subdural hematoma (2), and spinal subarachnoid hemorrhage (1). A cardioembolic source was identified in 12 of 13 patients with cerebral ischemia, as follows: prosthetic heart valves (9), mitral valve prolapse (2), and atrial fibrillation (1). Chronic anticoagulant therapy was a likely cause in 2 of 3 patients with hemorrhagic events. Compared with other Marfan syndrome patients, those with neurovascular events were older (39.6 versus 31.7 years, P=0.04) and more likely to be in atrial fibrillation (22.2% versus 3.2%, P=<0.01), to have prosthetic heart valves (61.1% versus 7.7%, P=0.001), and to be taking anticoagulant therapy (72.2% versus 16.1%, P<0.001). Aortic disease, a putative factor in the etiology of neurovascular complications, was present in equal measure in Marfan patients with and without neurovascular complications (78% versus 65%, P=NS). CONCLUSIONS: Neurovascular complications of Marfan syndrome are rare during 8 years of follow-up, and generally are ischemic in nature. A high-risk cardiac source was identified in the majority. A significant association with vascular dissection was not established.