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Vascular Lesions of the Spinal Cord, pp. 375-396 $100.00
Authors:  Robert H. Andres, Raphael Guzman, Alain Barth, Marwan El-Koussy, Arjun V. Pendharkar and Gerhard Schroth
Arteriovenous malformations (AVMs), arteriovenous fistulas (AVFs), and
cavernomas are rare vascular lesions of the spinal cord that are difficult to diagnose and
can lead to considerable morbidity if left untreated. According to the most widely used
classification, spinal arteriovenous lesions are divided in 4 major categories based on
their anatomical and pathophysiological features: Type I, dural AVFs; Type II, glomus or
intramedullary AVMs; Type III, juvenile or combined intra- and extradural AVMs; and
Type IV, intradural perimedullary AVMs. Spinal cavernomas constitute an additional
entity of almost exclusively intramedullary lesions.
Type I AVFs constitute the vast majority of spinal vascular malformations and
usually become symptomatic by slowly progressive congestive myelopathy. Hemorrhage
due to these lesions is considerably rare. In contrast, Type II-IV AVMs and cavernomas
can cause venous congestion, space-occupying effects, and subarachnoid or
intramedullary hemorrhage. Due to the variable and nonspecific presentation of these
lesions, the threshold for performing diagnostic workup should be low.
Neuroradiological evaluation should include magnetic resonance imaging of the whole
spine and selective spinal catheter angiography to confirm diagnosis.
Available treatment options depend on the structural and hemodynamic properties of
each lesion, as well as the relationship to the spinal cord and vasculature. For Type I
AVFs, treatment usually consists of surgical or endovascular occlusion of the draining
vein at the fistulous point. Type II AVMs may be treated by surgery or embolization, and
in most cases, Type III and IV AVMs are treated by endovascular therapy. If complete
occlusion of an AVM or AVF is achieved, the majority of cases will result in good
clinical outcome. Symptomatic cavernomas should be totally resected if possible.
Complete resection improves neurological recovery in most patients and eliminates the
risk of recurrent hemorrhage.
The heterogeneous presentation and clinical nature of spinal vascular malformations
presents special diagnostic and therapeutic challenges. As a result, the optimal treatment
strategy for these lesions should be determined for each patient on an individual basis by
an experienced interdisciplinary neurovascular team. 

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Vascular Lesions of the Spinal Cord, pp. 375-396