Prognostic factors for the outcome of surgical and conservative treatment of symptomatic spinal cord cavernous malformations: a review of a series of 20 patients

2010 ◽  
Vol 29 (3) ◽  
pp. E13 ◽  
Author(s):  
Hans-Jakob Steiger ◽  
Bernd Turowski ◽  
Daniel Hänggi
Keyword(s):  
Spinal Cord ◽  
Prognostic Factors ◽  
Mr Imaging ◽  
Neurological Deficits ◽  
Mr Images ◽  
Duration Of Symptoms ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Satisfactory Outcome ◽  
History Of

Object In this study, the authors present a review of a series of 20 intramedullary spinal cord cavernous malformations (SCCMs) with particular focus on MR imaging and prognostic factors. Methods Between 1994 and 2009, 20 patients with SCCM were treated under the care of the senior author. The diagnosis was made in all patients after the onset of clinical symptoms. The age of the 9 men and 11 women ranged between 26 and 71 years (median 38.5 years). The duration of symptoms prior to referral ranged from 1 week to 9 years (median 6.5 months). At the time of referral, 4 patients had no significant neurological deficits, 10 patients suffered significant functional restrictions, and 6 patients presented with severe paraparesis and loss of functional strength. None of the patients had complete paraplegia. Seventeen patients underwent microsurgical removal, while 3 patients opted for conservative therapy. For the present analysis, the medical records and MR images and/or reports were reviewed. Classification of length of history, pretreatment status, MR imaging pattern, and treatment modality was done and correlated with outcome. Results The cavernoma was located at the cervical level in 8 patients and between T-1 and L-1 in 12 patients. The cavernoma appeared as mainly T2 hyperintense on MR images in 7 patients, mainly T2 hypointense in 2 patients, and mixed in the remaining 10 patients. The craniocaudal extension of the core varied between 5 and 45 mm. In 2 patients with cervical cavernomas, a distinct T2 signal of the spinal cord cranial and distal to the cavernoma was seen, and in a patient with a large thoracic cavernoma, T2 extinction cranial and caudal to the cavernoma was seen as a sign of hemosiderosis. Neurological deficits improved postoperatively in 12 of the surgically treated patients, remained stable in 2, and deteriorated in 3. The 3 patients who were conservatively treated remained stable over a follow-up of 3–9 years. Postoperative improvement was seen in 5 of 7 surgical patients with a history of symptoms of 2 months or less, 5 of 6 patients with a history of 2–24 months, and in 2 of 4 patients with a history of more than 2 years. Two of the 3 patients with postoperative deterioration had a history of more than 2 years and the third a short history of 1 month. Conclusions Although a satisfactory outcome can be achieved through surgical treatment of SCCMs, some patients worsen after surgery or during the postoperative course. Long-term stability is possible in oligosymptomatic conservatively treated patients. The prevalence and pathophysiological importance of segmental spinal cord edema and hemosiderosis is incompletely understood at the present time.

Coexistence of intracranial and spinal cavernous malformations: a study of prevalence and natural history

2006 ◽  
Vol 104 (3) ◽  
pp. 376-381 ◽  
Author(s):  
Aaron A. Cohen-Gadol ◽  
Jeffrey T. Jacob ◽  
Diane A. Edwards ◽  
William E. Krauss
Keyword(s):  
Family History ◽  
Natural History ◽  
Mr Imaging ◽  
Neurological Deficits ◽  
Cavernous Malformations ◽  
Recurrent Hemorrhage ◽  
Long Term Follow Up ◽  
History Of ◽  
Clinically Significant

Object The purpose of this study was to examine the prevalence of intracranial cavernous malformations (CMs) in a large series of predominantly Caucasian patients with spinal cord CMs. The authors also studied the natural history of spinal CMs in patients who were treated nonoperatively. Methods The medical records of 67 consecutive patients (32 female and 35 male patients) in whom a spinal CM was diagnosed between 1994 and 2002 were reviewed. The patients’ mean age at presentation was 50 years (range 13–82 years). Twenty-five patients underwent resection of the lesion. Forty-two patients in whom the spinal CM was diagnosed using magnetic resonance (MR) imaging were followed expectantly. Thirty-three (49%) of 67 patients underwent both spinal and intracranial MR imaging. All available imaging studies were reviewed to determine the coexistence of an intracranial CM. Fourteen (42%) of the 33 patients with spinal CMs who underwent intracranial MR imaging harbored at least one cerebral CM in addition to the spinal lesion. Six (43%) of these 14 patients did not have a known family history of CM. Data obtained during the long-term follow-up period (mean 9.7 years, total of 319 patient-years) were available for 33 of the 42 patients with a spinal CM who did not undergo surgery. Five symptomatic lesional hemorrhages (neurological events), four of which were documented on neuroimaging studies, occurred during the follow-up period, for an overall event rate of 1.6% per patient per year. No patient experienced clinically significant neurological deficits due to recurrent hemorrhage. Conclusions As many as 40% of patients with a spinal CM may harbor a similar intracranial lesion, and approximately 40% of patients with coexisting spinal and intracranial CMs may have the nonfamilial (sporadic) form of the disease. Patients with symptomatic spinal CMs who are treated nonoperatively may have a small risk of clinically significant recurrent hemorrhage. The findings will aid in evaluation of surveillance images and in counseling of patients with spinal CMs, irrespective of family history.

Intramedullary ependymoma of the spinal cord

1990 ◽  
Vol 72 (4) ◽  
pp. 523-532 ◽  
Author(s):  
Paul C. McCormick ◽  
Roland Torres ◽  
Kalmon D. Post ◽  
Bennett M. Stein
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Tumor Recurrence ◽  
Neurological Deficits ◽  
Functional Improvement ◽  
Consecutive Series ◽  
Intramedullary Spinal Cord ◽  
Content Type ◽  
Fine Print

✓ A consecutive series of 23 patients underwent operative removal of an intramedullary spinal cord ependymoma between January, 1976, and September, 1988. Thirteen women and 10 men between the age of 19 and 70 years experienced symptoms for a mean of 34 months preceding initial diagnosis. Eight patients had undergone treatment prior to tumor recurrence and referral. Mild neurological deficits were present in 22 patients on initial examination. The location of the tumors was predominantly cervical or cervicothoracic. Radiological evaluation revealed a wide spinal cord in all cases. Magnetic resonance (MR) imaging was the single most important radiological procedure. At operation, a complete removal was achieved in all patients. No patient received postoperative radiation therapy. Histological examination revealed a benign ependymoma in all cases. The follow-up period ranged from 6 to 159 months (mean 62 months) with seven patients followed for a minimum of 10 years after surgery. Fourteen patients underwent postoperative MR imaging at intervals ranging from 8 months to 10 years postoperatively. No patient has been lost to follow-up review and there were no deaths. No patient showed definite clinical or radiological evidence of tumor recurrence during the follow-up period. Recent neurological evaluation revealed functional improvement from initial preoperative clinical status in eight patients, no significant change in 12 patients, and deterioration in three patients. The data support the belief that long-term disease-free control of intramedullary spinal ependymomas with acceptable morbidity may be achieved utilizing microsurgical removal alone.

The Natural History of Conservatively Managed Symptomatic Intramedullary Spinal Cord Cavernomas

Neurosurgery ◽  
2007 ◽  
Vol 60 (5) ◽  
pp. 865-872 ◽  
Author(s):  
Siddharth Kharkar ◽  
John Shuck ◽  
James Conway ◽  
Daniele Rigamonti
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Duration Of Symptoms ◽  
Intramedullary Spinal Cord ◽  
Additional Information ◽  
History Of ◽  
The Mean ◽  
Spinal Cavernomas ◽  
Time Of Presentation

Abstract OBJECTIVE The presentation and natural history of untreated, symptomatic intramedullary spinal cavernomas at our institution were analyzed. The objective is to provide additional information regarding the natural history of conservatively managed, symptomatic, intramedullary spinal cord cavernous malformations. METHODS The medical records of patients treated in our institution between 1989 and 2002 were reviewed to identify those with intramedullary cavernomas. The medical, radiological, surgical, and pathological records from these patients were retrospectively reviewed and analyzed. RESULTS Fourteen patients were included in the study. The mean age at presentation was 42 years. Four lesions (29%) were located in the cervical region and 10 lesions (71%) were present in the thoracolumbar spinal cord. All patients were symptomatic at the time of presentation. In this cohort of 14 patients, 10 patients (71%) were conservatively managed. For these patients, the mean duration of symptoms before presentation was 10 months. The mean duration of follow-up from the time of presentation was 80 months. The median McCormick grade for conservatively treated patients at presentation was II. During this period, none of the conservatively managed patients had an acute intramedullary bleed. In nine patients, the McCormick grade at the last follow-up evaluation was the same as or better than their score at presentation. Four patients (29%) were treated surgically. The mean duration of symptoms before presentation was 33 months. The mean duration of follow-up from the time of presentation was 42 months. In two surgical patients, the McCormick grade at the last follow-up evaluation remained unchanged compared with their score at presentation, whereas the McCormick grade improved in one patient and deteriorated in another patient. CONCLUSION This cohort of conservatively managed patients with symptomatic, intramedullary spinal cord cavernomas was clinically stable throughout the follow-up period. In this series, patients harboring symptomatic spinal cord cavernous malformation did not have significant, permanent neurological decline during the follow-up period when treated with the conservative approach of observation. This data provides additional information for determining the appropriate treatment strategy for patients with intramedullary spinal cavernomas.

Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data

2014 ◽  
Vol 21 (4) ◽  
pp. 662-676 ◽  
Author(s):  
Jetan H. Badhiwala ◽  
Forough Farrokhyar ◽  
Waleed Alhazzani ◽  
Blake Yarascavitch ◽  
Mohammed Aref ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Individual Patient Data ◽  
Clinical Course ◽  
Meta Analysis ◽  
Single Center ◽  
Total Resection ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Neurological Outcomes ◽  
History Of

Object Information pertaining to the natural history of intramedullary spinal cord cavernous malformations (ISCCMs) and patient outcomes after surgery is scarce. To evaluate factors associated with favorable outcomes for patients with surgically and conservatively managed ISCCMs, the authors performed a systematic review and metaanalysis of the literature. In addition, they included their single-center series of ISCCMs. Methods The authors searched MEDLINE, EMBASE, CINAHL, Google Scholar, and The Cochrane Library for studies published through June 2013 that reported cases of ISCCMs. Data from all eligible studies were used to examine the epidemiology, clinical features, and neurological outcomes of patients with surgically managed and conservatively treated ISCCMs. To evaluate several variables as predictors of favorable neurological outcomes, the authors conducted a meta-analysis of individual patient data and performed univariate and multivariate logistic regression analyses. Variables included patient age, patient sex, lesion spinal level, lesion size, cerebral cavernomas, family history of cavernous malformations, clinical course, presenting symptoms, treatment strategy (operative or conservative), symptom duration, surgical approach, spinal location, and extent of resection. In addition, they performed a meta-analysis to determine a pooled estimate of the annual hemorrhage rate of ISCCMs. Results Eligibility criteria were met by 40 studies, totaling 632 patients, including the authors' institutional series of 24 patients. Mean patient age was 39.1 years (range 2–80 years), and the male-to-female ratio was 1.1:1. Spinal levels of cavernomas were cervical (38%), cervicothoracic (2.4%), thoracic (55.2%), thoracolumbar (0.6%), lumbar (2.1%), and conus medullaris (1.7%). Average cavernoma size was 9.2 mm. Associated cerebral cavernomas occurred in 16.5% of patients, and a family history of cavernous malformation was found for 11.9% of evaluated patients. Clinical course was acute with stepwise progression for 45.4% of patients and slowly progressive for 54.6%. Symptoms were motor (60.5%), sensory (57.8%), pain (33.8%), bladder and/or bowel (23.6%), respiratory distress (0.5%), or absent (asymptomatic; 0.9%). The calculated pooled annual rate of hemorrhage was 2.1% (95% CI 1.3%–3.3%). Most (89.9%) patients underwent resection, and 10.1% underwent conservative management (observation). Outcomes were better for those who underwent resection than for those who underwent conservative management (OR 2.79, 95% CI 1.46–5.33, p = 0.002). A positive correlation with improved neurological outcomes was found for resection within 3 months of symptom onset (OR 2.11, 95% CI 1.31–3.41, p = 0.002), hemilaminectomy approach (OR 3.20, 95% CI 1.16–8.86, p = 0.03), and gross-total resection (OR 3.61, 95% CI 1.24–10.52, p = 0.02). Better outcomes were predicted by an acute clinical course (OR 1.72, 95% CI 1.10–2.68, p = 0.02) and motor symptoms (OR 1.76, 95% CI 1.08–2.86, p = 0.02); poor neurological recovery was predicted by sensory symptoms (OR 0.58, 95% CI 0.35–0.98, p = 0.04). Rates of neurological improvement after resection were no higher for patients with superficial ISCCMs than for those with deep-seated ISCCMs (OR 1.36, 95% CI 0.71–2.60, p = 0.36). Conclusions Intramedullary spinal cord cavernous malformations tend to be clinically progressive. The authors' findings support an operative management plan for patients with a symptomatic ISCCM. Surgical goals include gross-total resection through a more minimally invasive hemilaminectomy approach within 3 months of presentation.

Widespread central nervous system cavernous malformations associated with café-au-lait skin lesions

2003 ◽  
Vol 99 (2) ◽  
pp. 412-415 ◽  
Author(s):  
Kiran Musunuru ◽  
Virany Huynh Hillard ◽  
Raj Murali
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Novel Mutation ◽  
Skin Lesions ◽  
Neurological Deficits ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Content Type ◽  
Brain And Spinal Cord

✓ The simultaneous presence of cavernous malformations in the brain and spinal cord is a very rare finding and is typically associated with familial cavernous malformations. Although they are uncommon, various skin lesions can manifest in patients with familial cavernous malformations. The authors report on a 60-year-old man in whom more than 100 lesions consistent in appearance with cavernous malformations, including several intramedullary spinal cord lesions, were found throughout the neuraxis. This patient also displayed prominent café-au-lait skin lesions, but had no additional signs of neurofibromatosis or other neurocutaneous disorders. Analysis of his DNA revealed a novel mutation in the KRIT1/CCM1 gene, thereby confirming the diagnosis of familial cavernous malformation. The presence of these lesions in every major compartment of this patient's central nervous system underscores their indiscriminate nature and the need to screen throughout the neuraxis in patients in whom familial cavernous malformations are suspected. The findings in this case add to the growing list of skin lesions associated with genetically confirmed familial cavernous malformations. In patients presenting with seizures, focal neurological deficits, or hemorrhagic stroke, the presence of unusual skin lesions should prompt consideration of familial cavernous malformations, and appropriate screening should be performed.

Prognostic relevance of the postoperative evolution of intramedullary spinal cord changes in signal intensity on magnetic resonance imaging after anterior decompression for cervical spondylotic myelopathy

2007 ◽  
Vol 7 (6) ◽  
pp. 615-622 ◽  
Author(s):  
Luciano Mastronardi ◽  
Ahmed Elsawaf ◽  
Raffaelino Roperto ◽  
Alessandro Bozzao ◽  
Manuela Caroli ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Magnetic Resonance ◽  
Mr Imaging ◽  
Signal Intensity ◽  
Cervical Spondylotic Myelopathy ◽  
Mr Images ◽  
Intramedullary Spinal Cord ◽  
Signal Changes ◽  
T1 And T2 ◽  
T2 Weighted Images

Object Areas of intramedullary signal intensity changes (hypointensity on T1-weighted magnetic resonance [MR] images and hyperintensity on T2-weighted MR images) in patients with cervical spondylotic myelopathy (CSM) have been described by several investigators. The role of postoperative evolution of these alterations is still not well known. Methods A total of 47 patients underwent MR imaging before and at the end of the surgical procedure (intraoperative MR imaging [iMRI]) for cervical spine decompression and fusion using an anterior approach. Imaging was performed with a 1.5-tesla scanner integrated with the operative room (BrainSuite). Patients were followed clinically and evaluated using the Japanese Orthopaedic Association (JOA) and Nurick scales and also underwent MR imaging 3 and 6 months after surgery. Results Preoperative MR imaging showed an alteration (from the normal) of the intramedullary signal in 37 (78.7%) of 47 cases. In 23 cases, signal changes were altered on both T1- and T2-weighted images, and in 14 cases only on T2-weighted images. In 12 (52.2%) of the 23 cases, regression of hyperintensity on T2-weighted imaging was observed postoperatively. In 4 (17.4%) of these 23 cases, regression of hyperintensity was observed during the iMRI at the end of surgery. Residual compression on postoperative iMRI was not detected in any patients. A nonsignificant correlation was observed between postoperative expansion of the transverse diameter of the spinal cord at the level of maximal compression and the postoperative JOA score and Nurick grade. A statistically significant correlation was observed between the surgical result and the length of a patient's clinical history. A significant correlation was also observed according to the preoperative presence of intramedullary signal alteration. The best results were found in patients without spinal cord changes of signal, acceptable results were observed in the presence of changes on T2-weighted imaging only, and the worst results were observed in patients with spinal cord signal changes on both T1- and T2-weighted imaging. Finally, a statistically significant correlation was observed between patients with postoperative spinal cord signal change regression and better outcomes. Conclusions Intramedullary spinal cord changes in signal intensity in patients with CSM can be reversible (hyperintensity on T2-weighted imaging) or nonreversible (hypointensity on T1-weighted imaging). The regression of areas of hyperintensity on T2-weighted imaging is associated with a better prognosis, whereas the T1-weighted hypointensity is an expression of irreversible damage and, therefore, the worst prognosis. The preliminary experience with this patient series appears to exclude a relationship between the time of signal intensity recovery and outcome of CSM.

Clinical presentation and surgical outcome of intramedullary spinal cord cavernous malformations

2012 ◽  
Vol 16 (3) ◽  
pp. 308-314 ◽  
Author(s):  
Xianzeng Tong ◽  
Xiaofeng Deng ◽  
Huan Li ◽  
Zhuang Fu ◽  
Yulun Xu
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Surgical Outcome ◽  
Clinical Presentation ◽  
Acute Onset ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Recent Onset ◽  
Characteristic Imaging ◽  
Microsurgical Resection

Object Intramedullary spinal cord cavernous malformations (CMs), once thought to be extremely rare, have been diagnosed more frequently since the advent of MR imaging. In the literature, however, only a few studies include more than 10 cases. The aim of this study was to discuss the clinical presentation of intramedullary spinal cord CMs and the outcome of microsurgery for these histologically benign but clinically progressive lesions. Methods The authors retrospectively reviewed the records of 20 patients who underwent microsurgery for intramedullary spinal cord CMs. All patients had undergone pre- and postoperative MR imaging, and they were all treated using microsurgical resection. The diagnosis of spinal cord CMs was based on pathological criteria. The pre- and postoperative neurological states of the patients were classified according to the McCormick scale and Frankel scale. The microsurgical outcomes are presented and discussed. Results In most cases, CMs can be diagnosed on the basis of MR imaging findings, since these lesions have certain characteristic imaging patterns. Patients with intramedullary spinal cord CMs may present with either a rapid, acute onset of symptoms or slow, progressive neurological decline. The CMs in 19 of 20 patients in this series were totally resected, and most patients neurologically improved postoperatively. As previously reported, the authors confirm that the treatment of choice for symptomatic intramedullary CMs is total removal of the lesion to avoid recurrence and the possibility of further hemorrhage. Conclusions This study has defined the clinical features of symptomatic intramedullary spinal cord CMs. Surgery is the mainstay treatment. Surgical outcome is associated with low mortality with a high probability of functional recovery, especially when symptoms are not severe and are of relatively recent onset.

Clinical presentation and surgical management of intramedullary spinal cord cavernous malformations

2010 ◽  
Vol 29 (3) ◽  
pp. E12 ◽  
Author(s):  
Daniel C. Lu ◽  
Michael T. Lawton
Keyword(s):  
Surgical Approach ◽  
Clinical Symptoms ◽  
Preoperative Imaging ◽  
Surgical Approaches ◽  
Duration Of Symptoms ◽  
Intramedullary Spinal Cord ◽  
Cavernous Malformations ◽  
Satisfactory Outcome ◽  
Estimated Blood Loss

Object Intramedullary cavernous malformation (CM) is a rare entity, accounting for 5% of all intraspinal lesions. The objective in this study was to define the clinical characteristics of this disease, detail the surgical approach and technique, and present the clinical outcome. Methods Retrospective chart review was performed in 22 patients with histologically confirmed CMs. The authors used a laminectomy approach for midline dorsal lesions, with unilateral radical facetectomy and dentate ligament resection for laterally or ventrally located lesions. Patient profiles, operative indications, surgical approaches, operative findings, complications, and long-term follow-up were reviewed. Results The average age of patients in the cohort was 43 ± 14 years, the average duration of symptoms was 7 ± 7 months, and the average follow-up was 6 ± 4 years. The average size of the lesion was 1 ± 0.4 cm, the average surgical time was 4 ± 0.96 hours, and the average estimated blood loss was 350 ± 131 ml. The rate of complication was 5% (1 patient; due to a wound infection). According to the McCormick classification, the score for the cohort was 1.8 ± 1.2 preoperatively, 2.1 ± 1.2 postoperatively, and 1.3 ± 0.65 at late follow-up. (All preceding values are given as the mean ± SD.) There was a significant neurological improvement at follow-up compared with the preoperative state (p < 0.05). The majority of patients (50%) had a stable outcome compared with their preoperative state, with a large proportion (41%) having an improved outcome. A minority of patients (9%) had a worsened outcome due to dysesthetic pain. Patients with dysesthesia had a longer duration of clinical symptoms prior to surgery compared with patients without dysesthesia (p < 0.05). Conclusions The authors demonstrated the safety, efficacy, and durability of their surgical approach for resection of spinal intramedullary CM. Proper examination, preoperative imaging, and prompt surgical intervention were necessary for a satisfactory outcome.

Spinal cord grey matter lesions in multiple sclerosis detected by post-mortem high field MR imaging

2008 ◽  
Vol 15 (2) ◽  
pp. 180-188 ◽  
Author(s):  
CP Gilmore ◽  
JJG Geurts ◽  
N Evangelou ◽  
JCJ Bot ◽  
RA van Schijndel ◽  
...  
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Mr Imaging ◽  
High Field ◽  
Grey Matter ◽  
Great Majority ◽  
Proton Density ◽  
Post Mortem ◽  
Mr Images ◽  
The Brain

Background Post-mortem studies demonstrate extensive grey matter demyelination in MS, both in the brain and in the spinal cord. However the clinical significance of these plaques is unclear, largely because they are grossly underestimated by MR imaging at conventional field strengths. Indeed post-mortem MR studies suggest the great majority of lesions in the cerebral cortex go undetected, even when performed at high field. Similar studies have not been performed using post-mortem spinal cord material. Aim To assess the sensitivity of high field post-mortem MRI for detecting grey matter lesions in the spinal cord in MS. Methods Autopsy material was obtained from 11 MS cases and 2 controls. Proton Density-weighted images of this formalin-fixed material were acquired at 4.7Tesla before the tissue was sectioned and stained for Myelin Basic Protein. Both the tissue sections and the MR images were scored for grey matter and white matter plaques, with the readers of the MR images being blinded to the histopathology results. Results Our results indicate that post-mortem imaging at 4.7Tesla is highly sensitive for cord lesions, detecting 87% of white matter lesions and 73% of grey matter lesions. The MR changes were highly specific for demyelination, with all lesions scored on MRI corresponding to areas of demyelination. Conclusion Our work suggests that spinal cord grey matter lesions may be detected on MRI more readily than GM lesions in the brain, making the cord a promising site to study the functional consequences of grey matter demyelination in MS.

Clinical presentation and prognostic factors of spinal dural arteriovenous fistulas: an overview

2012 ◽  
Vol 32 (5) ◽  
pp. E17 ◽  
Author(s):  
Jennifer E. Fugate ◽  
Giuseppe Lanzino ◽  
Alejandro A. Rabinstein
Keyword(s):  
Spinal Cord ◽  
Clinical Symptoms ◽  
Neurological Deficits ◽  
Intramedullary Spinal Cord ◽  
Lower Extremity Weakness ◽  
Arteriovenous Fistulas ◽  
Signal Abnormality ◽  
Dural Arteriovenous Fistulas ◽  
Sensory Disturbances ◽  
Spinal Dural Arteriovenous Fistulas

Spinal dural arteriovenous fistulas (AVFs), the most common type of spinal cord vascular malformation, can be a challenge to diagnose and treat promptly. The disorder is rare, and the presenting clinical symptoms and signs are nonspecific and insidious at onset. Spinal dural AVFs preferentially affect middle-aged men, and patients most commonly present with gait abnormality or lower-extremity weakness and sensory disturbances. Symptoms gradually progress or decline in a stepwise manner and are commonly associated with pain and sphincter disturbances. Surgical or endovascular disconnection of the fistula has a high success rate with a low rate of morbidity. Motor symptoms are most likely to improve after treatment, followed by sensory disturbances, and lastly sphincter disturbances. Patients with severe neurological deficits at presentation tend to have worse posttreatment functional outcomes than those with mild or moderate pretreatment disability. However, improvement or stabilization of symptoms is seen in the vast majority of treated patients, and thus treatment is justified even in patients with substantial neurological deficits. The extent of intramedullary spinal cord T2 signal abnormality does not correlate with outcomes and should not be used as a prognostic factor.

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