Abstract
Spinal vascular malformations (SVMs) are a heterogeneous group that can cause acute, subacute, or chronic spinal cord dysfunction. The majority of the patients present to neurosurgical attention after a protracted course with severe neurological dysfunction. Spinal vascular lesions comprise approximately 3–4 % of all intradural spinal lesions. They are pathologically similar to their intracranial counterparts, but their clinical impact is often comparatively worse. Early, correct recognition of the pathology is mandatory to halt the progression of the disease and minimize permanent spinal cord injury. The first clinical observation of a SVM was published in 1890, but it was not until 1914 that the first successful surgical treatment of a spinal vascular malformation was reported. Intervention—either by microsurgical or endovascular means—aims to halt or reverse the progressive neurological deterioration by eliminating flow through the abnormal fistulous or nidal connections, and restoring normal spinal cord perfusion and intravascular pressures. In fact, complex spinal arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs) frequently require a multimodality approach that utilizes both microsurgery and endovascular embolization effectively. The goal of this review is to describe the various types of vascular malformations of the spine, their pathophysiology, clinical presentation, treatment strategies, and outcome. For purposes of discussion on the current manuscript, vascular malformations of the spine were divided into arteriovenous fistulas (AVFs) and arteriovenous malformations (AVMs). Spinal cord aneurysms are extremely rare, and the majority of the lesions that come to the neurosurgeon’s attention are concomitant to a spinal AVM.
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The authors would like to thank Suzanne “Jorlam” Truex, medical illustrator, for her invaluable assistance in transforming such an arid topic through her unique artistic drawings.
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Biagia La Pira, Giuseppe Lanzino, Rochester, USA
The authors provide a comprehensive summary on spinal vascular malformations. We like to make a few comments on the type 1 spinal dural arteriovenous fistulas (SDAVFs), which represent the most common spinal vascular malformation. Unfortunately, despite advances and widespread utilization of noninvasive neuroimaging, the diagnosis continues to be delayed [1, 7]. When we compared the time interval from symptom onset to final diagnosis between those patients treated from 1986 to 1999 and those treated between 2000 and 2008, we were surprised to find that the median delay in diagnosis had not changed over the time interval being 12 months in both periods [7]. At times, delays in diagnosis are related to the difficulty of performing a complete spinal angiography in some of these patients who are often elderly with advanced atherosclerosis. We routinely use magnetic resonance angiography (MRA) as a screening tool helpful in narrowing down the segments most likely bearing the fistula [3]. In this manner, catheter angiography can be started on the segments in question. In the exceptional cases where catheter angiography cannot localize the fistula, advanced MRI techniques such as time-resolved MRA and PC-Fiesta imaging can be helpful in identifying the site of the fistula [6].
As noted by the authors, type 1 DAVFs have a striking male predominance (usually in the 7th and 8th decade of life). The presence of clinical symptoms and MRI findings suspicious for a type I DAVFs in a young patient or in a woman, should raise the suspicion of an epidural fistula or a paraspinal AVM with secondary retrograde intradural venous drainage [2]. With increased awareness of this entity and better imagine techniques, epidural AVFs now account for about 30 % of the spinal vascular malformations that we see. Differentiation between an epidural AVF and a type 1 AVF is an important one, as epidural fistulas are amenable to successful and permanent obliteration with endovascular trans-arterial embolization [5]. Moreover, epidural AVFs are often more difficult to obliterate surgically then the classic type I SDAF because the AV shunt is often located in the ventral epidural venous plexus.
After complete obliteration of a type 1 DAVF, the degree of clinical improvement is highly variable. The vast majority of patients experience some degree of improvement of motor function. As noted by the authors of this review, improvement of sensory function and sphincter control is less dramatic. Interestingly, there is no correlation between the degree and pattern of improvement of signal changes on MRI and the degree of clinical improvement [4]. Resolution of the flow voids on MRI and of serpiginous vessels on MRA is an excellent predictor of complete fistula obliteration [4], and in straightforward cases, we rarely perform a postoperative catheter angiography after surgical obliteration.
After successful treatment of a type I DAVF, it is not uncommon in patients who had presented with myelopathy to complain of delayed subjective recurrence of symptoms in association with an intercurrent systemic illness. This is usually related to the loss of the ability to compensate for the lost function in the presence of an intercurrent systemic illness and not to the recurrence of the fistula. Similarly, patients can report a subjective sense of increasing weakness and fatigability 6 to 18 months after successful treatment. This is often related to increased muscular tone which occur as a result of some degree of “spinal cord healing.” These symptoms usually improve with pharmacological treatment of the spasticity. It is important to warn patients about these possible “setbacks” and their significance as to avoid excessive worrying and unnecessary expensive imaging studies.
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Ryan Morton, Louis Kim, Seattle, USA
Flores et al. are to be congratulated for reporting a comprehensive update on the modern classification systems, treatment and management strategies, and outcomes data for spinal vascular malformations. They note that endovascular embolization with Onyx or nBCA is the treatment of choice for dAVF (with the exception of perimedullary or intradural ventral dAVF with single or small feeders) and often can result in cure. Even for spinal AVM, unlike its cranial counterpart, complete embolization can result in a durable cure and even partial embolization can improve neurological function. Before a proper treatment strategy can be developed, however, comprehensive spinal angiography must be performed. The authors dutifully note the obligation of the angiographer to image all of the thoracic intercostal and lumbar radicular arteries not only to look for any fistulous connection, but also to identify the artery of Adamkiewicz. Critical to comprehensive angiography, however, is knowing what to image when the thoracic and lumbar segmental arteries fail to show any fistulous connection. The authors mention multiple strategies including injecting the lateral sacral arteries, arterial supply to the cervical cord and posterior fossa, as well as the bilateral internal iliac arteries. These are very useful pearls to the article readership and are much appreciated.
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Flores, B.C., Klinger, D.R., White, J.A. et al. Spinal vascular malformations: treatment strategies and outcome. Neurosurg Rev 40, 15–28 (2017). https://doi.org/10.1007/s10143-016-0713-z
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DOI: https://doi.org/10.1007/s10143-016-0713-z