Cervical Posterior Spinal Artery Syndrome Caused By Spontaneous Vertebral Artery Dissection: Two Case Reports and Literature Review.

Herein, we described 2 patients with posterior spinal artery syndrome (PSAS) caused by vertebral artery dissection. The patients complained of sudden neck pain or walking instability. Neurological examination revealed sensory loss, muscle weakness, and sensory ataxia. Angiography showed double lumen sign or intimal flap in the vertebral artery. T2-weighted imaging and diffusion-weighted imaging of MRI showed a hyperintense lesion in the dorsal side of the cervical spinal cord at different times after onset. Both patients had good outcome after antiplatelet therapy and physiotherapy. A review of previously reported PSAS cases was also conducted in order to improve the understanding and awareness of this rare myelopathy.

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.

Open and endovascular treatment of spinal dural arteriovenous fistulas: a 10-year experience.

OBJECTIVE Vascular malformations of the spine represent rare clinical entities with profound neurological implications. Previously reported studies on management strategies for spinal dural arteriovenous fistulas (sDAVFs) appeared before the advent of modern liquid embolic agents. Authors of the present study review their institutional experience with endovascularly and surgically treated sDAVFs. METHODS The authors performed a retrospective, observational, single-center case series on sDAVFs treated with endovascular embolization, microsurgical occlusion, or both between 2004 and 2013. The mode, efficacy, and clinical effect of treatment were evaluated. RESULTS Forty-seven patients with spinal arteriovenous malformations were evaluated using spinal angiography, which demonstrated 34 Type I sDAVFs (thoracic 20, lumbar 12, and cervical 2). Twenty-nine of the patients (85%) were male, and the median patient age was 63.3 years. Twenty patients underwent primary endovascular embolization (16 Onyx, 4 N-butyl cyanoacrylate [NBCA]), and 14 underwent primary surgical clipping. At a mean follow-up of 36 weeks, according to angiography or MR angiography, 5 patients treated with endovascular embolization demonstrated persistent arteriovenous shunting, whereas none of the surgically treated patients showed lesion persistence (p = 0.0237). Thirty patients (88%) experienced some resolution of their presenting symptoms (embolization 17 [85%], surgery 13 [93%], p = 1.00). CONCLUSIONS Microsurgical occlusion remains the most definitive treatment modality for sDAVFs, though modern endovascular techniques remain a viable option for the initial treatment of anatomically amenable lesions. Treatment of these lesions usually results in some clinical improvement.

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 2005­2009, 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 0­2 on postoperative day 30 was correlated with presymptomatic mRS of 0­2 [P<0.0001, odds ratio (OR): 42.88, 95% confidence interval (CI): 14.83­123.97] and postoperative neurological improvement (P=0.046, OR: 2.57, 95% CI: 1.02­6.48). 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.

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.

Endovascular treatment of adult spinal arteriovenous lesions.

Spinal arteriovenous lesions (SAVLs) are rare disorders, the diagnosis of which can be established using various imaging modalities. To discern the various types of SAVL, spinal angiography of the entire neural axis is required. Surgery is the standard treatment of choice; however, ever advancing endovascular technology can provide a viable alternative. This article discusses the normal anatomy, arterial supply, and venous drainage of the spinal cord. The classification, pathophysiology, epidemiology, clinical presentation, natural history, and pathophysiology of SAVLs in adults are reviewed. Finally, endovascular treatment of these lesions is discussed.

Spinal arteriovenous shunts in children.

Pediatric spinal arteriovenous shunts are rare and, in contrast to those in adults, are often congenital or associated with underlying genetic disorders. These are thought to be a more severe and complete phenotypic spectrum of all spinal arteriovenous shunts seen in the overall spinal shunt population. The pediatric presentation thus accounts for its association with significant morbidity and, in general, a more challenging treatment process compared with the adult presentation.

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.

Clinical features and treatment outcomes of the spinal arteriovenous fistulas and malformation: clinical article.

OBJECT: Spinal vascular diseases, such as spinal dural arteriovenous fistulas (DAVFs), perimedullary arteriovenous fistulas (AVFs), and spinal arteriovenous malformations (AVMs), are very rare. The authors analyzed the features and treatment outcomes of these conditions. METHODS: Data from 64 patients were retrospectively reviewed. There were 33 spinal DAVFs (1 patient had 2 lesions), 20 perimedullary AVFs, and 12 spinal AVMs. Clinical features, radiological findings, treatment results, and clinical outcomes were evaluated according to the diseases, subtypes, and treatment modalities. The median duration of follow-up was 20, 42, and 56 months for spinal DAVFs, perimedullary AVFs, and spinal AVMs, respectively. RESULTS: Spinal DAVFs showed faster progression of symptoms (median 5, 12, and 36 months for spinal DAVFs, perimedullary AVFs, and spinal AVMs, respectively) and worse neurological status at diagnosis (poor neurological status in 56%, 65%, and 33%, respectively). On MRI, signal voids were demonstrated in all except 1 spinal DAVF. At the last follow-up, 94% of spinal DAVFs, 68% of perimedullary AVFs, and 50% of spinal AVMs were completely obliterated. Favorable clinical outcomes were achieved in 91%, 95%, and 58%, respectively. In detail, the majority (78%) of spinal DAVFs were embolized, resulting in complete obliteration in 92% and favorable clinical outcomes in 92%. Most Type IVa and IVb perimedullary AVFs were surgically treated (71% and 88%), with complete obliterations of 86% and 71%, and favorable clinical outcomes in 100% and 86%, respectively. All Type IVc lesions were embolized with a low cure rate of 40%; however, clinical outcomes were satisfactory. Spinal AVMs were generally embolized (67%), and only glomus-type lesions attained a satisfactory cure rate (80%) and clinical outcome (100%). CONCLUSIONS: Embolization produced satisfactory outcomes in spinal DAVFs and glomus-type spinal AVMs. Surgery is advantageous in Type IVa and IVb perimedullary AVFs. Palliative embolization can be effective in Type IVc perimedullary AVFs and juvenile spinal AVMs.

Management considerations in ruptured isolated radiculopial artery aneurysms. A report of two cases and literature review.

Little is known on the natural history of ruptured isolated aneurysms of the posterior spinal artery (PSA). To date, only a few of such cases have been described in the literature. This paper aims to assess the most appropriate management strategy, based on the available literature and two new cases. In one of these, treatment was postponed until day 33, when angiography showed slight growth of the aneurysm. In the other, conservative treatment, requested by the patient, was successful. From these data, we conclude that treatment strategies for ruptured PSA aneurysms may vary. Aside from the recommendation by others to perform prompt surgical treatment, we suggest an alternative clinical paradigm allowing for the evaluation of the early clinical course. This may preclude the unnecessary treatment of spontaneously regressing lesions and still allows for appropriate treatment for persistent lesions.

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.

Hemorragia intramedular (Hematomielia)no traumática: revisión / Non traumatic intramedullary hemorrhage (hematomyelia): a review

La hemorragia intramedular (hematomielia) no traumática es el sangrado producido en el interior del tejido medular en ausencia de trauma, su primera descripción clínica aparece reportada por Tellegen en 1850, se le considera la más infrecuente de las hemorragias intraespinales y rara si la comparamos con la frecuencia en que ocurren las hemorragias intracerebrales. La incidencia es alta en los varones (relación hombre-mujer 1,5:1) y muy relacionada con la entidad patológica condicionante. Esta obedece a múltiples causas: malformaciones arteriovenosas, cavernomas, tumores, cuagulopatias, inflamatoria, etc. La disfunción aguda del cordón espinal junto a las imágenes por resonancia magnética son elementos decisivos para el diagnóstico. El tratamiento aun sujeto de controversias, médico o quirúrgico (precoz o tardío), dependerá de la etiología del sangrado y del estatus neurológico en el momento del diagnóstico, hallándose los resultados más favorables en aquellos pacientes a los cuales se les realizó un pronto diagnóstico seguidos de una cirugía temprana.

Non traumatic intramedular hemorrhage (hematomyelia) is the blood found in the medullar tissues in absence of trauma, its first clinical description was reported by Tellegen in 1850.Is considered to be the most infrequent of intraespinales hemorrhage. The incidence is high in men and is related with conditionate pathological entity. Its do to multiples causes: Arteriovenos malformation, cavernomas, tumors, coagulopathies, inflammations, etc. The acute dysfunction of the spinal cord and magnetic resonance imaging are decisive in the diagnosis. The treatment though subject to controversies, medical or surgery (early or late), will depend on the bleeding etiology and the neurological status in the moment of the diagnosis, finding the most favorable result in those patients that were diagnosed earlier following an early surgery.

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.

Acute paraplegia due to spinal arteriovenous fistula in two patients with hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVM). Of these, spinal AVM is a rare manifestation that concerns mainly children. In this report, we describe two cases of spinal AVM revealed by acute paraparesis due to subarachnoid hemorrhage in children with HHT and reviewed the literature on spinal arteriovenous malformations in HHT. In most of the cases reported, the clinical presentation was acute in the pediatric population and insidious during adulthood. The prognosis of spinal AVM mainly depends on the presence or not of medullar signs and symptoms and on the delay before treatment. In conclusion, any child with a family history of HHT should be considered at risk for spinal AVM in order to improve management of such complications and to decrease the risk of neurological sequellae.

Successful embolization of a giant perimedullary arteriovenous fistula of the cervical spine in a 6-year-old child.

CASE: We report a case of a 6-year-old boy with a giant perimedullary arteriovenous fistula of the cervical spine who presented with progressive tetraparesis. METHOD: Transarterial glue embolization was performed and complete occlusion of the fistula was achieved. RESULT: The patient's symptoms resolved completely during the next year.

Spinal cord intradural arteriovenous fistulae: anatomic, clinical, and therapeutic considerations in a series of 32 consecutive patients seen between 1981 and 2000 with emphasis on endovascular therapy.

OBJECTIVE: To review our series of intradural spinal cord arteriovenous fistulas (SCAVFs), analyzing symptoms and related angioarchitecture, and to study the morphological and clinical results of embolization. METHODS: Thirty-two SCAVFs (in 22 adults and 10 children) were treated between 1981 and 2000. These lesions were classified as microarteriovenous fistulas (mAVFs) or macroarteriovenous fistulas (MAVFs) according to shunt morphology. Location, architecture, presenting symptoms, and age group were detailed. The selection of patients for endovascular versus surgical treatments was analyzed, as were the anatomic and clinical results obtained by embolization with n-butylcyanoacrylate. Clinical status was evaluated according to the Karnofsky Performance Scale score. RESULTS: Ten SCAVFs were found in the pediatric population (four mAVFs and six MAVFs). All four mAVFs presented with acute symptoms. Three mAVFs (two cervical and one thoracic) presented hematomyelia; in one patient with a thoracic AVF, subarachnoid hemorrhage was suspected. All six MAVFs were located in the thoracolumbar cord (five associated with hereditary hemorrhagic telangiectasias). Four of the six MAVFs presented with hemorrhage. In the adult population, there were 21 mAVFS (95%) and one MAVF (5%). Only two mAVFs were found in the cervical cord, all other shunts affecting the thoracolumbar region. Hemorrhage was present in 6 of the 22 cases seen in adults (27%). The symptoms of SCAVFs did not differ from those found in spinal cord arteriovenous shunts of nidus type. Pial venous reflux and congestion were the most frequently encountered features in both the adult and pediatric groups. Arterial aneurysms (different from false aneurysms) were not found in association with hemorrhagic presentation of SCAVFs. Mean follow-up in our series was 3.3 years. Of the MAVFs, 86% were embolized, with 67% cured. The others had more than 75% occlusion. All patients followed up improved significantly. Of the mAVFs, 48% were treated endovascularly. Successful embolization was performed in 75% of patients. One patient was not embolized because of vasospasm, whereas 67% percent of mAVFs were completely occluded, 22% were more than 90% occluded, and 11% were 75% occluded. Complementary surgery was deemed unnecessary. All patients with mAVFs improved significantly at follow-up. Transient complications occurred in 22% of all patients, with no permanent morbidity or mortality. No patient bled or rebled after embolization. Thirty-six percent of mAVFs were operated on because of anticipated technical difficulties for endovascular approach or distal localization of the shunt. CONCLUSION: Endovascular treatment of SCAVFs stabilizes, normalizes, or improves neurological symptoms in all patients at long-term follow-up, with no bleeds or rebleeds. Embolization of SCAVFs with glue is a safe treatment that compares favorably with other approaches and significantly improves the poor natural history of the disease.

Vertebral body ischemia in the posterior spinal artery syndrome: case report and review of the literature.

STUDY DESIGN: A case of posterior spinal cord syndrome in which magnetic resonance images showed predominant T2 hyperintense signal in the adjacent vertebral body is reported. OBJECTIVES: To present the case for abnormal bone marrow magnetic resonance signal in the radiologic diagnosis of posterior spinal cord syndrome and to review its significance. SUMMARY OF BACKGROUND DATA: Infarction in the region of posterior spinal arteries has been rarely described. This is attributable not only to the infrequent occurrence of infarction of posterior spinal arteries, but also to a lack of well-established diagnostic procedures. It is of clinical value to define diagnostic images of posterior spinal cord syndrome, especially early in the course of the disease. METHODS: The subject was a 52-year-old man who was presented with acute nontraumatic myelopathy. Magnetic resonance imaging, performed serially after onset of the disorder from 5 hours to 11 months, was evaluated in comparison with neurologic findings. The literature was reviewed to discuss the magnetic resonance images of spinal cord infarction. RESULTS: The neurologic findings were consistent with posterior spinal cord syndrome. A magnetic resonance image taken at 5 hours after onset of the syndrome showed T2 hyperintense signal in the T12 vertebral body. At 3 days after onset, T2 hyperintense signal became obvious in the posterior portion of the spinal cord at T9-T12 vertebral levels. Follow-up magnetic resonance imaging at 41 days, 8 months, and 11 months showed a decrease in the size and intensity of the T2 signal change in the spinal cord and T12 vertebral body. In the literature, T2 hyperintense bone marrow signal was defined in one case of posterior spinal cord syndrome and seven cases of anterior spinal cord syndrome. CONCLUSIONS: Associated bone marrow abnormalities likely reflect the underlying pathology of the blood supply to the vertebral body, and may be an additional key sign for radiologic diagnosis of posterior spinal cord syndrome.

Venous manifestations of spinal arteriovenous fistulas.

Spinal dural AVFs, the most common type of spinal arteriovenous malformations, are symptomatic because of venous hypertension and congestion. This has been referred to as venous congestive myelopathy. The typical MRI findings that reflect venous congestive myelopathy include peripheral T2 hypointensity that outlines a T2 hyperintensity within a swollen spinal cord. Enlarged perimedullary vessels are typically present. Contrast-enhanced MRA has become instrumental in localizing the site of these fistulas. Spinal epidural AVFs and the perimedullary spinal cord AVFs may also present with a congestive myelopathy and have similar findings on MRI. Angiography remains the gold standard for characterization of the angioarchitecture of spinal vascular malformations. [figure: see text] Multidisciplinary treatment planning is mandatory and requires knowledge of the natural history of these vascular lesions.