Additional cases of IAs in identical twins 339, 349

as well as familial aggregations

of IAs without a recognized inherited disorder have also been reported but are felt
to be rare ( It has been estimated that < 2 % of IAs are familial. Most reported
casus consist of only 2 family members with IAs, and the are most commonly
siblings. Analysis of case reports reveals that when IAs occur in siblings they tend to
occur at identical or mirror images site, and in comparison to sporadic IAs, familias
IAs tend to rupture at a smaller size and younger age, and that the incidence of
anterior communicating artery aneurysm is lower. It has been postulated that IAs
occuring in sibling may represent a distinct population of IAs.
The Indications and best method for investigation of asymptomatic relatives of a
patient found to harbor an intracranial aneurysm are controversial. Negatives
studies (angeography, DSA, MRA..) do not guarantee that a later date an aneurysm
will not be discovered that either subsequently developed or expanded, or was
simply not detected on the initial study. Cerebral angiography is the most sensitive
study, how ever the risk and expense may not justify its use as screening test in
many cases. Furthermore there is some evidence that aneurysms that rupture tend
to do shortly after their formation which would reduce the value of screening.
Screening recommendations : first degree relatives (especially siblings) are at
higher risk of harboring IAs, and should undergo MRI and MRA screening. Finding
suspicious for IA(s) require follow-up with four vessel
arteriography to confirm
suspected lesions ( MRA has a high false-positive rate of 16 %) and to rule-out
additional IAs.

30.15. Traumatic aneurysms

Traumatic Aneurysms (Tas) comprise < 1 % of intracranial aneurysms. AKA
pseudoaneurysms ( a rupture of all the vessel wall layers with the wall of the
aneurysm being formed by sorrounding cerebral structures. They many occur rarely
in childhood. The mechanism of injury usually falls into one of the following groups.
1. Those arising from penetrating trauma ; usually from gunshot wounds,
although penetration with a sharp object ( which is less common) may be
more prone to cause traumatic aneurysms.
2. Thoe arising from close head injury: more common. Theories of pathogenesis
include traction injury to the reveal wall or entrapment within a fracture. Tend
to occur either :
A. Peripherally
1. Distal anterior cerebral artery aneurysms; secondary to impact against
the falcine edge

2. Distal cortical artery aneurysms : Often associated with an overlying

skull fracture, some times growing skull fracture.
B. At the skull base, usually involving the ICA in one of the following sites :
1. Petrous portion
2. Cavernous carotid artery :
a. Aneurysm enlargement may cause a progressive zinus syndrome;
b. Rupture may lead to a posttraumatic carotid-cavernous fistula (see
page 1113) or to massive epistaxis in the presence of a sphenoid
sinus fracture.
3. Supraclinoid carotid artery
4. Istrogenic : following surgery in or around the skull base, the sinoses or orbita
(icluding following transaphenoidal surgery)
Presentation
1. Delayed
intracranial
hemorrhage
(subdural,
subarachnoid,
intreventricular,
or
intraparenchymal;
The
most
common
presentation. TAs tend to have a high rate of rupture.
2. Recurrent epistaxis
3. Progressive cranial nerve palay
4. Enfarging skull fracture
5. May be incidental finding on CT Scan

Treatment
Although there are case reports of spontaneous resolution, treatment is usually
recommended. ICA aneurysms at the skull base undergo trapping or endovascular
embolization. Peripheral lesions should be treated surgically with clipping of
aneurysm neck, excision of the aneurysm, coiling or wrapping if no other method is
fesible.

30.16. Mycotic aneurysms

The name mycotic originated with Ouler in whose time the term referred to any
infections process rather then the current usage which infers a fungal etiology.
Currently accepted terminology favors infectious aneurysm ( or bacterial

aneurysm). Infectious aneurysms can, however, also occur with fungal infections.
Tend to form indistal (often unnamed) vessels.
EPIDEMOLOGY & PATHOPHYSIOLOGY

Comprise 4 % of intracranial aneurysms

Occurs in 3-15 % of patiens with subacute bacterial endocarditio (SBE)
Most common location distal MCA branches (75 80%)
At least 20% have or develop multiple aneurysms
Increased frequency in immunocompromised patiens (e.g AIDS) and drug
users
Most probably start in the adventitia (outer layer) and upread inward

EVALUATION
Blood cultures and LP may identity the infectious organism. Table 30-15 shows
typical pathogens recovered. Patients with suspected infectious aneurysms should
undergo echocardiography to look for signs of endocarditis.
Organism

Streptococcus
Staphylococcus
Miscellaneous
Multiple
No growth
No info
total

44 %
18 %
6%
5%
12 %
14 %
99 %

Comment

TREATMENT
These aneurysms usually have fusiform morphology and are usually very friable,
therefore surgical treatment in difficult and/or risky. Most cases are treated acutely
with antibiotics which are continued 4-6 weeks. Serial angiography ( at 7-10 days
and 1.5,3,6 and 12 months, even if aneurysms seem to be getting smaller, they
may subsequently increases and new ones may form) helps document effectiveness
of medical therapy (Serial MRA may be a viable alternative in some cases ).
Aneurysms may continue to shrink following completion of antibiotic therapy.
Delayed clipping may be more feasible,indication include :

Patients with SAH

Increasing size of aneurysms while on antibiotics ( controversial, some say
not mandatory).
Failure of aneurysms to reduce in size after 4-6 weeks of antibiotics.
Patients with SBE requiring valve replacement should have bioprothetic (i.e
tissue valves instead of mechanical valves to eliminate the need for risky
anticoagulation.

30.17. Giant aneurysms

Risk of rebleeding
Overall rebleed rate is 0,5%/yr, which is lower then with aneurysmal SAH or
rebleeding from AVMs. There also a small risk of delayed cerebral ischemia
(vasospasm). Neurological outcome is likewise better.
MANAGEMENT
General measures
These patients are still at risk for the same complications of SAH as with
aneurysmal SAH: Vasospasm, hydrocephalus, hyponatremia, rebleeding, etc.(see
page 1040) and should be managed as any SAH (see page 1040). Some subgroups
may be at lower risk for complications and may be managed accordingly (e.g. see
Pretruncal nonaneurysm SAH (PNSAH) below.
Repeat angiography
Yield of positive second angiogram after technically adequate negative study: 1.89.8 %)370 in early (pre-CT) studies, 2-24% quoted more recently 369,371,372 . CT
scan findings are helpful in the decision to repeat angiography373. 70% of cases
with diffuse SAH and thick layering of blood in the anterior interhemispheric fissure
were associated with an AcoA aneurysm that show up on repeat angiography 367.
The absence of blood on CT (performed within 4 days of SAH), or thick blood in the
perimesencephalic cisterns alone (see below) were unlikely to be associated with a
missed aneurysm.
Recomendations regarding repeat angio :
1. repeat angio after = 10 -14 days ( allow pasospasm & some clot to resolve) A
A. Technically adequate 4 vessel angiogram is negative, and evidence for
SAH is strong
B. Original angio was incomplete or if there are suspicious findings

2. If CT localizes blood clot to particular area, place special attention to this area
on repeat angio
3. Do not repeat angio for classic pretruncal SAH (see below) or if no blood on
CT
4. Patients are usually kept in the hospital 10-14 days while waiting for repeat
angio (to watch for and manage complication of SAH or bleeding)
Other studies
1. imaging studies of the brain: MRI (with MRA if avaliable) or CT (with angioCT if avaliable). This may visualize an aneurysm that fails to show up on
angiography, and may identify other sources of SAH such as
angiographically occult vascular information ( see page 1105), tumor......
2. tests to rule-out spinal AVM: a rare cause of intracebral SAH (see page
507)
A. spinal MRI: cervical, thoracic and lumbar
B. spinal angiography : too difficult and risky to be justified in most cases
of angio negative SAH. Consider in cases with high suspicion of spinal
source.
Surgical exploration
Advocated by some for cases of SAH with CT findings compatible with an
aneurysmal source in which a suspicious area is demonstrated angiographically
with carefull explanation to the patient and family of the possibility of negative
operative findings.

369

30.19. Nonaneurysmal SAH

For etiologies of SAH other aneurysm, see page 1034
PRETRUNCAL NONANEURYSMAL SAH (PNSAH)
Nee perimesencephalic nonaneurysmal SAH

374

. The suggestion to change the name

to pretruncal non aneurysmal SAH was proposed because improved neuroimaging
techniques have shown the true anatomic localization of the blood to be in front of
the brain stem (truncus cerebri) centered in front of the pons rather than
perimesencephalic 375.
A distinct entity considered to be a benign condition with good outcome and less risk of
bleeding and vasospasm than other patients with SAH of unknown etiology 376 (no
rebleeding occured in 37 patients with PNSAH and 45 months mean follow-up 377 , nor in 169
patients with 8-51 months follow-up372, vasospasm has been reported in only 3 patients and
may have been related to cerebral angiography rather than the PNSAH, and although it is
slow, the incidence of angiographic vasospasm may be higher than oroginally though 378).
The actual etiology has yet to be determined, but iy may be secondary to rupture of a small
perimesencephalic vein or capillary378.

Presentation
Patients may present with severe paroxysmal HA, meningismus, photophobia, and nausea.
Loss of consciousness is rare. These patients are usually not critically ill ( all were grade 1 or
2), however, complications such as hyponatremia or cardiac abnormalities may occur.
Preretinal Hemorrhages and sentinel H/A have not occured.