45 Fusiform Aneurysms of the Posterior Circulation
Abstract
Posterior circulation fusiform aneurysms (PCFAs) are uncommon and challenging to treat. Compared to other locations, PCFAs present more commonly with symptoms of brainstem compression or ischemia and less commonly with subarachnoid hemorrhage. They may occur as small, focal fusiform dilations of the vertebral or cerebellar arteries or giant, dolichoectatic basilar trunk aneurysms. The majority of PCFAs will present with symptoms and therefore warrant treatment. Treatment options are limited given that these aneurysms lack a true neck, thus making clip reconstruction and stand-alone coiling difficult, if not impossible. Other microsurgical treatment options include bypass with aneurysm trapping, bypass with proximal or distal occlusion (if complete trapping is not possible), and aneurysm wrapping in cases of rupture when all other options have failed. Other endovascular treatment options include stent-assisted coiling, flow diversion (FD), and parent vessel occlusion (PVO). PVO is a reasonable option for distal, small caliber cerebellar artery fusiform aneurysms in which occlusion will not cause meaningful ischemia or in cases when all other options have failed. Experience with FD has increased over the last 5 years and the indications have expanded to include PCFAs. The results have been mostly positive, but complications can be catastrophic with severe ischemia or hemorrhage in the brainstem. While FD may be a good option for fusiform vertebrobasilar junctional aneurysms, it may not be safe for giant, dolichoectatic basilar trunk aneurysms in which the brainstem perforators are completely covered by the flow-diverting stent.
Introduction
Fusiform aneurysms are uncommon but remain challenging to neurosurgical treatment. Fusiform aneurysms of the basilar artery (BA) were first described in 1922, and since then several terms have also been used including dolichoectatic aneurysms, transitional aneurysms, and giant serpentine aneurysms. Posterior circulation fusiform aneurysms (PCFAs) are rare, with significant male predominance (~70%). PCFAs most commonly present as posterior circulation ischemic strokes. In addition, they may cause cranial nerve palsies, brainstem compression, and subarachnoid hemorrhage (SAH). Contrary to the more common saccular aneurysms, fusiform aneurysms are associated with high rates of rebleeding and morbidity. In this chapter, PCFAs are reviewed, including natural history, anatomy, pathophysiology, and treatment, with a suggested algorithm for treatment based on review of the literature.
Major controversies in decision making addressed in this chapter include:
Whether treatment is indicated.
Open versus endovascular treatment for ruptured and unruptured PCFA.
The role of flow diverters for PCFA.
Whether to Treat
Unlike the case of saccular aneurysms, our understanding of the natural history of PCFAs is more limited. The natural history of PCFAs depends on the presenting signs and symptoms. However, it appears to be very poor in symptomatic patients, if left without treatment. In such cases, the rebleeding rate is high and ranges between 30 and 85%. In addition, untreated ruptured PCFAs are associated with high rates of mortality, ranging between 23 and 35% in a 5-year follow-up.
In a large prospective study of vertebrobasilar (VB) aneurysms over a 12-year period, the annual rupture rate of fusiform aneurysms was approximately 2%. Aneurysm enlargement is a significant predictor of lesion rupture. In a series of patients with unruptured fusiform aneurysms, a diameter of ≥ 10 mm in VB aneurysms was a significant risk factor for aneurysm growth and it was predictive of future rupture. Therefore, the vast majority of ruptured PCFAs cases should be treated ( 1 , 2, 3 in algorithm ). Additionally, unruptured PCFAs greater than 10 mm also likely warrant treatment.
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Anatomical Considerations
The posterior circulation supplies the occipital lobes, cerebellum, and brainstem. The posterior circulation begins with the vertebral arteries (VAs) that join to form the BA, which then divides into the posterior cerebral arteries (PCA). These main arteries branch off several smaller vessels, including posterior inferior cerebellar arteries (PICAs), anterior inferior cerebellar arteries (AICAs), pontine branches, and superior cerebellar arteries. The VA can be divided into four segments, usually arising from the posterosuperior part of the subclavian artery and ending at lower part of pons to form the BA. The fourth segment (V4) ascends anterior to the roots of the hypoglossal cranial nerve (CN XII). The BA courses from the lower part of pons, next to the exit of the abducens cranial nerve (CN VI), the upper pontine border, and the oculomotor cranial nerve (CN III) where it bifurcates into two PCAs. From its origin at the termination of the BA, the PCA runs toward the occiput that gives several branches and perforators supplying mainly the occipital lobe and posteromedial temporal lobes.
Classification
Only few classification systems attempted to guide clinical decision in fusiform aneurysms by stratifying patients into risk groups. Mizutani et al′s classification system is composed of four types based on a combination of clinical features, imaging findings, lesional patterns of the internal elastic lamina (IEL), and the state of the intima. Type I is classic dissecting aneurysm characterized by disruption of the IEL without intimal thickening, presentation with SAH, and high rates of rebleeding. Type II is segmental ectasia, with a benign clinical course compared to type I. This type is pathologically characterized by extended and/or fragmented IEL with intimal thickening. In addition, the luminal surface is smooth without thrombus formation. Type III is dolichoectatic dissecting aneurysm. This type is distinguished pathologically from type II by dissections in the thickened intima, and organized luminal thrombus. Type III is frequently associated with hemorrhage and 50% mortality rate. Lastly, type IV is saccular aneurysm. Similar to the case of types I and III, type IV is associated with high rates of rebleeding and mortality.
Flemming′s classification for nonsaccular VB aneurysms is based on radiographic appearance. All lesions were defined as having an arterial dilation 1.5 times normal diameter: fusiform (14%)—aneurysmal dilation of the involved segment, without identifiable neck; dolichoectasia (45%)—uniformed dilation of the involved segment; transitional (19%)—uniform aneurysmal dilation with superimposed dilation of a portion of the involved arterial segment; and, indeterminate type (20%). Fusiform and transitional types were most likely to be symptomatic and dolichoectatic type. Similar to the case of dolichoectatic aneurysms (type II) in the classification of Mizutani et al, dolichoectatic aneurysms in Flemming′s classification are benign lesions with no significant neurological sequelae. ▶ Fig. 45.1 shows Flemming′s classification for nonsaccular VB aneurysms.
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Workup
Clinical Evaluation
Posterior circulation fusiform aneurysms tend to present with ischemic strokes rather than just hemorrhage. PCFAs are usually diagnosed in male patients older than 50 years. The most common presentations are posterior circulation ischemic strokes with cranial nerve palsy, brainstem compression, and/or SAH. The most common cranial nerve palsies involved in PCFAs are CN V–VIII, presenting as trigeminal neuralgia and hemifacial spasm. Deficits involving lower cranial nerves are rare in PCFAs. Headache and obstructive hydrocephalus are also common at the presentation.
Imaging
Diagnostic cerebral angiography (DSA) with three-dimensional reconstruction is the gold standard for diagnosis. Magnetic resonance angiography (MRA) and computed tomography angiography (CTA) can also be used for large aneurysms.
Treatment
Choice of Treatment and the Influence of Intracerebral Hematoma
Classically, all PCFAs have been treated with open surgery. The surgical treatment modalities include proximal/parent artery occlusion (Hunterian ligation), trapping procedures with mural hematoma decompression for cases with acute mass effect, surgical bypass, and clip reconstruction techniques. Recently, endovascular treatments have been successfully used in treating PCFAs with good outcomes. The endovascular options include parent vessel coil occlusion, stenting alone, stenting with coiling, flow-diverting stents.
Cerebrovascular Management—Operative Nuances
Microsurgical treatment of PCFAs is becoming less common given the recent advancements of endovascular therapy, especially flow diversion. In fact, microsurgical treatment is generally reserved for cases that cannot be treated with endovascular therapy. Because these aneurysms, by definition, do not have a neck and usually involve a circumferentially diseased vessel, clip reconstruction is generally not the treatment of choice (▶ Figs. 45.2 and ▶ 45.3 ). Complicating matters further, these aneurysms can be partially calcified/thrombosed and the surgical corridor is obstructed by a labyrinth of neurovascular structures. Thus, trapping with or without bypass is the main microsurgical modality. Operative nuances are discussed within each chapter for specific anatomical aneurysm location (see Chapters 37–44).
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