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orrhage, but one that is often disastrous Saccular aneurysms are also called berry aneurysms; actually they take the form of small, thin-walled blisters protruding from arteries of the circle of Willis or its major branches Their rupture causes a ooding of the subarachnoid space with blood under high pressure As a rule, the aneurysms are located at vessels bifurcations and branchings (Fig 34-22) and are generally presumed to result from developmental defects in the media and elastica An alternate theory holds that the aneurysmal process is initiated by focal destruction of the internal elastic membrane, which is produced by hemodynamic forces at the apices of bifurcations (Ferguson) As a result of the local weakness, the intima bulges outward, covered only by adventitia; the sac gradually enlarges and may nally rupture Saccular aneurysms vary in size from 2 mm to 2 or 3 cm in diameter, averaging 75 mm (Wiebers et al) Those that rupture usually have a diameter of 10 mm or more (by angiography), but rupture also occurs, albeit less often, in those of smaller size Aneurysms vary greatly in form Some are round and connected to the parent artery by a narrow stalk, others are broad-based without a stalk, and still others take the form of narrow cylinders The site of rupture is usually at the dome of the aneurysm, which may have one or more secondary sacculations A review of the subject by Schievink is recommended for further details In routine autopsies, the incidence of unruptured aneurysms is almost 2 percent excluding minor outpouchings of 3 mm or less Aneurysms are multiple in 20 percent of patients It has been estimated that 400,000 Americans harbor unruptured aneurysms and that there are an estimated 26,000 subarachnoid hemorrhages from them per year (Sahs et al) In childhood, rupture of saccular aneurysms is rare, and they are seldom found at routine postmortem examination; beyond childhood, they gradually increase in frequency to reach their peak incidence between 35 and 65 years (average 49 years) Therefore they cannot be regarded as fully formed congenital anomalies; rather, they appear to develop over the years on the basis of either a developmental or acquired arterial defect.

Post inf cerebellar A Vertebral A Figure 34-22 Diagram of the circle of Willis showing the principal sites of saccular aneurysms Approximately 90 percent of aneurysms are on the anterior half of the circle The sizes of the aneurysms depicted correspond roughly to the frequency of occurrence at those sites

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There is an increased incidence of congenital polycystic kidneys, bromuscular dysplasia of the extracranial arteries, moyamoya, and coarctation of the aorta among persons with saccular aneurysms A saccular aneurysm occurs in approximately 5 percent of cases of arteriovenous malformation, usually on the main feeding artery of the malformation Numerous reports have documented a familial occurrence of saccular aneurysms, lending support to the idea that genetic factors play a role in their development The number of rst-degree relatives found to harbor an unsuspected aneurysm has been about 4 percent in most series This low rate, the nding that half of the discovered aneurysms are small, and the complications of surgery make routine screening of siblings, children, and parents of patients with ruptured aneurysms impractical, according to the Magnetic Resonance Angiography in Relatives of Patients with Subarachnoid Hemorrhage Study Group However, since aneurysms of the familial variety tend to be larger at the time of rupture and more numerous than in patients who have sporadic ones, there are exceptions to this statement (Ruigrok et al) While hypertension is more frequently present than in the general population, nevertheless aneurysms most often occur in persons with normal blood pressure Pregnancy does not appear to be associated with an increased incidence of aneurysmal rupture, although there is always concern about the possibility of rupture during the straining of natural delivery Atherosclerosis, though present in the walls of some saccular aneurysms, probably plays no part in their formation or enlargement Approximately 90 to 95 percent of saccular aneurysms lie on the anterior part of the circle of Willis (Fig 34-22) The four most common sites are (1) the proximal portions of the anterior communicating artery, (2) at the origin of the posterior communicating artery from the stem of the internal carotid, (3) at the rst major bifurcation of the middle cerebral artery, and (4) at the bifurcation of the internal carotid into middle and anterior cerebral arteries Other sites include the internal carotid artery in the cavernous sinus, at the origin of the ophthalmic artery, the junction of the posterior communicating and posterior cerebral arteries, the bifurcation of the basilar artery, and the origins of the three cerebellar arteries Aneurysms that rupture in the cavernous sinus may give rise to an arteriovenous stula (page 749) There are several types of aneurysm other than saccular, eg, mycotic, fusiform, diffuse, and globular The mycotic aneurysm is caused by a septic embolus that weakens the wall of the vessel in which it lodges (page 727) The others are named for their predominant morphologic characteristics and consist of enlargement or dilatation of the entire circumference of the involved vessels, usually the internal carotid, vertebral, or basilar arteries The latter are also referred to as arteriosclerotic aneurysms, since they frequently show atheromatous deposition in their walls, but it is likely that they are at least partly developmental in nature Some are gigantic and press on neighboring structures or become occluded by thrombus; they rupture only infrequently (see further on) Clinical Syndrome With rupture of the aneurysm, blood under high pressure is forced into the subarachnoid space (usually in relation to the circle of Willis), and the resulting clinical events assume one of three patterns: (1) the patient is stricken with an excruciating generalized headache and vomiting and falls unconscious almost immediately; (2) headache develops in the same manner but the patient remains relatively lucid the most common syndrome; (3) rarely, consciousness is lost quickly with-.

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