CHAPTER 67  Neurosurgery 1903

include papilledema and sixth cranial nerve palsy with lateral
rectus weakness and side-by-side diplopia, initially worse on far
vision or gaze directed toward the side of the palsy. The papill-
edema is a mostly chronic phenomenon and is not seen acutely.
The sixth nerve palsy of raised ICP can occur regardless of its cause
and does not imply direct involvement by a mass lesion, large or
small, on the sixth nerve. In this situation, the sixth nerve palsy
is a false localizing sign. With raised ICP, there may also be obscu-
rations of vision, in which patients report that their vision tem-
porarily fades or becomes gray, in combination with headache.
Again, these obscurations are caused by the effect of diffusely
increased ICP on the sensitive optic nerves. They do not imply
the presence of a focal mass lesion directly affecting the optic
nerves or pathways. Intuitively, it seems that if there is a slow
increase in a process raising ICP, the pressure would also rise
slowly and evenly, in pace with the evolving process. However, as
first shown by Lundberg in 1960,4 the intermediate stages of
decompensation are characterized by transient pronounced eleva-
tions in ICP (to 60 mm Hg) that characteristically plateau for up
to 45 minutes and then transiently cycle down again to a more
normal range.
years. The risk of hemorrhage rate is up to 4% per year,5 and once
they bleed, they may be even more prone to hemorrhage. They
are typically composed of one or more feeding arteries; a nidus of
varying size, shape, and compactness that is composed of abnor-
mal vessels; and draining veins. They are sometimes associated
with high-flow related aneurysms of the feeding arteries.
Patients typically present with headaches, neurologic deficit,
seizures, or varying combinations of the three. Workup generally
includes computed tomography (CT) and MRI, demonstrating
the lesion (Fig. 67-4). Catheter angiography is then performed to
define the vascular anatomy of the lesion and is used for treatment
planning.
BOX 67-1  Cerebral Vascular Disease
Congenital
Arteriovenous malformation and fistula
Cavernous malformation
Telangiectasis
Venous anomaly (angioma)

Acquired
Traumatic
CEREBROVASCULAR DISORDERS Some arteriovenous fistulas (type I carotid-cavernous fistula)
Traumatic aneurysm
Cerebrovascular disorders encompass a host of disorders: congeni-
Degenerative
tal, acquired, and idiopathic (Box 67-1).
Atherosclerotic, occlusive disease
Arteriovenous Malformations Most cerebral (berry) aneurysms
Some arterial dissections
An AVM is an abnormal collection of blood vessels wherein arte-
Spontaneous intracerebral hemorrhage
rial blood flows directly into draining veins without the normal
Infectious
interposed capillary beds. AVMs are congenital lesions that can
Mycotic aneurysms
enlarge somewhat with age, recruiting new vascular supply, and
often progress from low-flow lesions at birth to higher flow lesions Idiopathic
in adulthood. They present with hemorrhage, ischemia of the Moyamoya
brain parenchyma around the lesion due to a vascular “steal” Some arteriovenous fistulas: dural AVM–like or type II carotid-cavernous
phenomenon, or seizures. They have a prevalence of 15 to fistulas
18/100,000 and typically are manifested before the age of 40

A B C
FIGURE 67-4  CT angiography with three-dimensional reconstruction (A), MRI (B), and conventional angio-
gram (C) of a large AVM with supply from the middle and posterior cerebral arteries and ill-defined nidus.
Complex deep and superficial venous drainage is present.
1904 SECTION XIII  Specialties in General Surgery
TABLE 67-2  Spetzler-Martin
Grading System
FEATURE POINTS
Nidus size (cm)
  Small (<3) 1 unfortunately, this is not helped by any known intervention.
  Medium (3-6) 2 However inadequate, the venous anomaly represents the only
  Large (>6) 3 venous drainage available to that area of brain, and therefore
Eloquence of adjacent brain
  Noneloquent 0 could lead to a venous infarction with swelling and hemorrhage,
  Eloquent (sensorimotor, language, visual, thalamus, 1 the consequences of which are particularly dangerous in the pos-
hypothalamus, internal capsule, brainstem, cerebellar
peduncles, deep cerebellar nuclei)
Pattern of venous drainage
  Superficially only 0
  Deep 1
Treatment options include craniotomy with surgical resection
of the lesion, embolization, and SRS. Commonly, more than one
modality is used. SRS is usually reserved for compact lesions less
the 2.5 cm in diameter. It can take up to 3 years for irradiated
AVMs to shut down after SRS. The Spetzler-Martin grading
system was developed 30 years ago and continues to be used to
help make treatment decisions (Table 67-2).6
Cavernous Malformations
A cavernous malformation is a well-circumscribed, benign vascu-
lar lesion consisting of irregular thin-walled sinusoidal vascular
channels located within the brain but lacking intervening neural
parenchyma, large feeding arteries, or large draining veins. Char-
acterized by McCormick in an autopsy series,7 these lesions have
a prevalence of approximately 0.5%. There are three familial forms
described. Because these are low-pressure, low-flow lesions, hem-
orrhage is typically not catastrophic unless it is in a highly elo-
quent area of the brain.
Patients usually present with hemorrhage or headaches with or
without a history of new-onset seizures. Although it is often
evident on CT, MRI is the imaging modality of choice and dem-
onstrates a characteristic dark hemosiderin ring. Treatment for
symptomatic lesions includes seizure control and surgical excision.
Radiosurgery has not been shown to significantly alter the natural
history of these lesions.
Capillary Telangiectasia
This lesion is composed of vascular channels with extremely thin
walls similar to those of dilated capillaries. These are usually
grouped in small clusters, generally with prominent intervening
brain tissue. They are often clinically silent and generally do not
appear on imaging studies. They are not evident on conventional
catheter angiography unless they are large, and then only in the
capillary venous phase. They clearly differ from an AVM in that
flow through the lesion is not fast enough to demonstrate arteries
and veins in the same conventional angiographic image. These
lesions are typically not treated surgically.
Developmental Venous Anomaly: Venous Angioma
These lesions are composed of an abnormally configured venous
drainage system converging on a single, enlarged venous outflow
channel. The typical appearance is that of a hydra, with radially
converging veins. A characteristic feature of this lesion appears to
be that the abnormal venous bed is poorly collateralized. The
abnormal venous drainage may or may not be fully adequate to
the needs of the brain tissue supplied. Slowly evolving degenera-
tive changes in the brain tissue supplied can occur as a result, but
removal of the venous anomaly is not recommended. Doing so
terior fossa.
Traumatic Fistula
Both the internal carotid artery and vertebral artery enter the
cranial cavity immediately after passing through a venous network.
The internal carotid artery passes through the cavernous sinus,
which communicates with the superior ophthalmic vein, petrosal
sinus, and sphenoparietal sinus. The vertebral artery passes
through a venous plexus at the occipital-C1 epidural space, which
communicates with the jugular vein, epidural venous plexus, and
paraspinal venous plexus. Trauma leading to a tear in the carotid
or vertebral artery at its tether point passing through the skull
base can lead to fistula with the surrounding venous plexus. The
consequences may vary in severity and suddenness but typically
include periorbital swelling, with proptosis and scleral edema in
the case of the carotid-cavernous fistula (CCF) and prominent
pulsatile bruit in the case of the vertebral-jugular fistula. Intraocu-
lar pressure measurement by tonometry can guide the urgency in
treating CCF. On radiologic examination, dilation of the superior
ophthalmic vein is characteristic (Fig. 67-5). These lesions are
usually treated by endovascular techniques. A catheter is advanced
through the tear in the artery into the venous side of the fistula.
The high flow and large fistulous channel facilitate this process.
Embolic material, a coil, or a detachable balloon is then used to
occlude the venous side of the fistula. When conventional trans-
venous routes fail, a direct approach through transorbital punc-
ture may be required to provide endovascular therapy.8
Aneurysms
Aneurysms are an excessive localized enlargement of a vessel due
to a weakening and subsequent defect in the wall of the artery.
There is an adult prevalence of 2% (this varies according to the
study) with an annual incidence of aneurysmal subarachnoid
hemorrhage of 6 to 8/100,000 with peak age at 50 years. Modifi-
able risk factors for subarachnoid hemorrhage include hyperten-
sion, smoking, and excessive alcohol. Aneurysms may be further
divided into saccular, fusiform, dissecting, infectious, and trau-
matic aneurysms.
Saccular Aneurysms
As the name implies, these aneurysms, also referred to as berry
aneurysms, are usually saccular in form and come off the vessel
wall or at a bifurcation. Many of these are found incidentally,
given the frequency of neuroimaging, but many present with
hemorrhage.9 The classic presentation of subarachnoid hemor-
rhage due to a cerebral aneurysm is that of sudden onset of a
headache, described as the “worst headache of my life.” Workup
typically includes a CT scan, demonstrating a typical distribution
of blood (Fig. 67-6). Between 10% and 15% of subarachnoid
hemorrhages from saccular aneurysms are fatal before the hospital
is even reached. Of those individuals who reach a medical facility,
 CHAPTER 67  Neurosurgery 1905

A B
FIGURE 67-5  Right internal carotid–cavernous sinus fistula (A, arrow) with dilation of the superior ophthal-
mic drain (B, arrowhead), a typical imaging finding of this pathologic process.

A B C
FIGURE 67-6  A, CT scan of brain showing subarachnoid blood in the basal cisterns. Dilated temporal horns
(arrow) indicate the presence of hydrocephalus. B, Cerebral angiogram shows two aneurysms located at the
junction of the A1 and A2 segments of the anterior cerebral artery (circle). C, CT angiography with three-
dimensional reconstruction showing the relationship of the aneurysms (circle) with the skull base.

one third do not survive (usually because of rebleeding), one third
will survive with varying degrees of neurologic disability, and one
third return to their baseline level.
The major complications of subarachnoid hemorrhage include
rebleeding, hydrocephalus (which is observed in around 15% to
20% of cases), cardiac events in around 50% of patients, vaso-
spasm, hyponatremia, and seizures. Rebleeding is a major cause
of death in patients who reach medical care after their initial
episode of bleeding. Hydrocephalus is caused by disruption in the
function of the arachnoid granulations and is commonly treated
with an external ventricular drain. Vasospasm is a narrowing of
the cerebral arteries thought to be caused by smooth muscle dys-
function related to blood breakdown products in the spinal
fluid. When present, it can contribute to significant ischemia of
the brain and, as such, be a significant cause of morbidity. Treat-
ment of symptomatic vasospasm is typically multimodality and
includes the use of hypervolemia and induced hypertension,
endovascular procedures, and a variety of pharmacologic agents
(Box 67-2).10-12
BOX 67-2  Treatment of Vasospasm
Prevention of Arterial Narrowing
Subarachnoid blood removal
Prevention of dehydration and hypotension
Calcium channel blockers (nimodipine)
Reversal of Arterial Narrowing
Intra-arterial calcium channel blockers
Transluminal balloon angioplasty
Prevention and Reversal of Ischemic Neurologic Deficit
Hypertension, hypervolemia, hemodilution
A classic study of cerebral aneurysms and their treatment docu-
ments the risk for rupture according to size and location as dem-
onstrated in Table 67-3.13 As a means of predicting possible
vasospasm and overall outcomes, the Hunt and Hess clinical
grading scale14 and the World Federation of Neurological
1906 SECTION XIII  Specialties in General Surgery

TABLE 67-3  Risk of Rupture During 5 Years (%) According to International Study of
Unruptured Intracranial Aneurysms
TYPE OF ANEURYSM <7 mm AND NO PRIOR SAH <7 mm AND PRIOR SAH 7-12 mm 13-24 mm >24 mm
Carotid-cavernous 0 0 0 3.0 6.4
Anterior circulation 0 1.5 2.6 14.5 40.0
Posterior circulation 2.5 3.4 14.5 18.4 50.0

SAH, subarachnoid hemorrhage.

TABLE 67-4  Hunt and Hess Clinical amenable to endovascular techniques.13,17,18 It is generally believed
Grading Scale that treatment of ruptured aneurysms is best accomplished as
soon as possible after the initial hemorrhage.
GOOD
DESCRIPTION GRADE OUTCOME Spontaneous Intracerebral Hemorrhage
Asymptomatic or minimal headache and 1 ≈70 Spontaneous intracerebral hemorrhages into the brain paren-
slight nuchal rigidity chyma are common, accounting for approximately 10% of all
Moderate to severe headache, nuchal 2 ≈70 strokes. They generally occur in older patients, usually because of
rigidity, ± cranial nerve palsy only degenerative changes in the cerebral vessels that are often associ-
Drowsy, confusion, or mild focal deficit 3 ≈15 ated with chronic hypertension (Box 67-3). In younger patients,
Stupor, moderate to severe hemiparesis, 4 ≈15 they are more likely related to drug abuse or vascular malforma-
possibly early decerebrate rigidity tion. They can occur anywhere in the cerebral circulation or
Deep coma, decerebrate rigidity, moribund 5 ≈0 brainstem but are classically described in association with small
appearance degenerative aneurysms (microaneurysms; also known as Charcot-
Bouchard aneurysms) at the junctions of the perforating vessels
and larger vessels at the skull base. They are typically on the
middle cerebral artery junctions with the small perforating len-
TABLE 67-5  World Federation of ticulostriate vessels, leading to hemorrhage into the putamen. The
Neurological Surgeons Clinical Grading Scale clinical presentation is with a stroke pattern of sudden-onset
GRADE GCS SCORE MOTOR DEFICIT neurologic signs and symptoms that depend on the area of brain
affected. Symptoms are more likely to include headache than
I 15 No
ischemic stroke. The diagnosis is with CT, usually done in an
II 13-14 No
emergency department setting. The size and location of the acute
III 13-14 Yes
hematoma are well visualized with CT, as is any associated brain
IV 7-12 Yes or no shift or hydrocephalus (Figs. 67-9A and 67-10). In older patients
V 3-6 Yes or no with a known history of hypertension and classic CT appearance
GCS, Glasgow Coma Scale. of a hematoma in the putamen, thalamus, cerebellum, or pons,
further diagnostic studies are generally not indicated. Rehemor-
rhage is unlikely in that setting. However, further investigation
TABLE 67-6  Fisher Grading for might be warranted with an atypical hematoma location or
Appearance of SAH on Computed appearance, especially if there is any component of subarachnoid
Tomography blood. Also, investigation is usually recommended for younger
patients without known hypertension and those with a potential
GRADE CT FINDINGS underlying cause for hemorrhage (e.g., history of neoplasm, blood
I No hemorrhage evident dyscrasias, bacterial endocarditis).
II Diffuse SAH with vertical layers < 1 mm thick Further investigation is generally done with contrast MRI or
III Localized clots and/or vertical layers of SAH > 1 mm thick magnetic resonance angiography. Any suggestion of aneurysm or
IV Diffuse or no SAH, but with intracerebral or AVM is followed by conventional catheter angiography. In older
intraventricular hemorrhage patients with a history of early dementia and multiple episodes of
more peripherally located intracerebral hematomas, the diagnosis
SAH, subarachnoid hemorrhage.
of amyloid angiopathy needs to be considered.
Most cases of spontaneous intracerebral hemorrhage do not
Surgeons clinical grading scale15 were developed (Tables 67-4 and require an operative procedure. Many hemorrhages are small
67-5). Another method of classifying subarachnoid hemorrhage enough to be well tolerated and do not require surgery. Others
is described by Fisher and colleagues16 and is based on CT scan are so large at the outset that surgery is of little benefit. Relief of
imaging (Table 67-6). any obstructive hydrocephalus by ventricular drainage is usually
Treatment of saccular aneurysms consists of coiling, coiling offered, except in the most impossible cases. Patients who obey
through a stent, or surgical clipping (Figs. 67-7 and 67-8) with commands and can be monitored by changes in their neurologic
subsequent intensive critical care unit management of potential examination can generally be managed conservatively with
vasospasm and comorbidities. Once the standard of care in the hospital observation for at least 5 to 7 days. Peak swelling and
treatment of cerebral aneurysms, open craniotomy and clipping decompensation are probably most likely to occur within that
is now typically reserved for those lesions believed not to be time frame. Surgery for evacuation of the hematoma may be
 CHAPTER 67  Neurosurgery 1907

A B
FIGURE 67-7  A, Subtraction carotid angiogram shows a 4 × 6-mm berry aneurysm (arrow) originating from
the distal internal carotid artery. B, Postoperative carotid angiogram shows clip placement (arrow), with total
obliteration of the aneurysm.

A B
FIGURE 67-8  A, Subtraction vertebral angiogram shows a basilar tip aneurysm. B, Subtracted vertebral
angiogram after the placement of coils demonstrates excellent obliteration of the aneurysm and preservation
of adjacent vessels.

BOX 67-3  Causes of Spontaneous small subarachnoid spaces have a lower compliance, with lower
Intracerebral Hemorrhage tolerance, than older patients with cerebral atrophy and generous
ventricles and subarachnoid spaces.
Hypertension Cerebral amyloid angiopathy The Surgical Trial in Intracerebral Hemorrhage has noted a
Vascular anomaly Coagulopathy lack of clinical outcome difference in comparing early surgery
Cerebral aneurysm Tumors with conservative management.19 If indicated, surgical evacua-
Arteriovenous malformation Drug abuse tion is usually done by craniotomy over the most accessible part
Cavernous malformation Other of the hematoma (Fig. 67-9B). Intraoperative ultrasound is often
Cerebral infarction (stroke) helpful in finding hematomas that do not quite come to the
transformation cortical surface and in monitoring the progress of the evacuation.
The goal of surgery is decompression more than complete
removal, but it is generally done as far as safely practical. The
appropriate in a small group of patients with intermediate-sized wall of the hematoma cavity is inspected for any underlying
hemorrhages in accessible locations who appear to tolerate the cause, and a biopsy specimen is taken, if indicated. Putamen
hematoma initially but then deteriorate in a delayed fashion with hemorrhage can sometimes be evacuated with minimal surgical
edema, despite medical therapy. Steroids have not demonstrated damage to the overlying brain by a trans–sylvian fissure–
benefit. Attempts to predict which patients will deteriorate solely transinsular approach. Stereotactic aspiration and methods with
on the basis of hematoma volume have been frustrated by the fibrinolytic agents are being developed and may be a consider-
broad spectrum of intracranial compliance exhibited by different ation for patients with hematomas in deep locations that are
patients. In general, younger patients with smaller ventricles and otherwise difficult to access.
1908 SECTION XIII  Specialties in General Surgery
A B
FIGURE 67-9  Nonenhanced CT scan of the head. A, Spontaneous hypertensive intracerebral hematoma in
the right basal ganglia, with extension to the frontal and temporal lobes. B, Immediate postoperative CT scan
shows near-total removal of the intracerebral hematoma.
FIGURE 67-10  Nonenhanced CT scan of the brain shows a large,
hypertensive, intracerebellar hematoma with obstruction of the fourth
ventricle and enlargement of the temporal horns, indicating obstructive
hydrocephalus.
A special situation to consider is the patient with cerebellar
hemorrhage (Fig. 67-10). Surgery is offered more readily in these
cases because the danger of sudden deterioration from brainstem
compression is more of a concern and because even extensive
damage to the cerebellum itself is generally survivable, with good
functional outcome. Patients with fourth ventricular obstruction
and hydrocephalus from cerebellar hemorrhage can sometimes be
treated with ventricular drainage alone but are usually offered
surgical evacuation of the hematoma by suboccipital craniotomy
because of the risk for brainstem compression.
Mycotic Aneurysms
These aneurysms are associated with a systemic infection capable
of showering small particles of bacteria-infected material into the
cerebral vascular bed. Subacute bacterial endocarditis and some
pulmonary infections can do this. A distinguishing feature of
these aneurysms is that they are generally found more distal in
the cerebral vascular bed, as opposed to berry aneurysms, which
are usually found on larger vessels near the circle of Willis. There
can also be many of them. When the bacterial emboli lodge in
distal cerebral arterial branches, they can erode through the wall
of these smaller vessels, often creating a hemorrhage contained by
the perivascular tissue. Maximal antibiotic treatment is essential
at the outset. The presence of an intracerebral hematoma may
force immediate craniotomy for evacuation. Operation on the
aneurysm at this early stage often reveals a component of sub-
arachnoid hemorrhage and an early inflammatory reaction in the
subarachnoid space, with only a blood collection covering the
erosion defect in the wall of the small artery. Attempts to dissect
and to define a neck are frustrated by a lack of developed fibrous
tissues, and intraoperative hemorrhage is then common. Typically,
the diseased arterial segment must be occluded and resected when
it is operated on in this early stage. The need for arterial bypass
to maintain blood flow to critical cerebral areas should be antici-
pated, but this is not always possible.
If the mycotic aneurysms are discovered or treated at some later
stage, a fibrous wall to the aneurysm may have had time to
develop, and clipping can then be a possibility. However, the
surgeon needs to be forewarned that it may be difficult to find
the aneurysm in a distal location, often buried deep in a cerebral
sulcus thickened with reactive fibrous scar tissue.
Moyamoya Disease
Moyamoya disease is a cerebrovascular disorder that is character-
ized by an idiopathic nonatherosclerotic narrowing or occlusion
of major intracranial blood vessels with the development of a

conspicuous compensatory collateral rete vessel network, which
allows continued cerebral perfusion around the occluded or
severely narrowed segment. The disorder is usually bilateral,
although not necessarily exactly symmetrical. Although generally
rare, the disease is more common in persons of Asian ancestry and
was first recognized from cases studied with angiography in Japan
before the advent of CT and MRI. The term moyamoya comes
from the Japanese word for “puff of smoke” or mist. The actual
disease is sometimes confused with the less conspicuous collateral
vascular networks seen around severe narrowing of common ath-
erosclerotic origin in persons of Western origin. In the juvenile
form, moyamoya typically is manifested as cognitive decline, with
deteriorating school performance and evidence of multiple
infarcts. Angiography reveals the internal carotid artery, proximal
middle cerebral artery, or proximal anterior cerebral artery with
severe narrowing or occlusion and, generally, multiple clusters of
fine collateral vessels. In the adult form of moyamoya disease, the
rete vessels cause subarachnoid or basal ganglia hemorrhage, the
most common presentations. The hemorrhage can usually be
treated conservatively. Some form of extracranial to intracranial
bypass is generally attempted to take the load off the collateral
vascular network. In younger patients, the results are good, with
an onlay interposition of the superficial temporal artery sewed
into the dura after a strip craniotomy. A feature of the disorder is
the vigor with which collaterals form from the onlay transposed
vessel. In the adult form, a microvascular anastomosis with the
superficial temporal artery or grafted vessel may be preferred.
Dural Arteriovenous Malformations
Dural AVMs (type II CCF is a subtype) are not often seen in
younger patients. The lesions seem to occur only in adults and are
probably acquired lesions that follow a dural sinus thrombosis,
usually of the cavernous sinus or sigmoid-transverse sinus junction
area. With subsequent healing, the thrombosed segment triggers
a neovascular response that evolves to an AVM configuration with
fistulous channels that can gradually enlarge. Usually, there is
associated stenosis of the affected dural segment, suggesting the
earlier thrombosis. The lesions are generally not dangerous unless
they cause retrograde venous drainage into the cerebral circulation.
The risk for intracranial hemorrhage is then fairly high; it is
important at least to separate the dural AVM drainage from the
cerebral circulation when that occurs. In the case of transverse-
sigmoid sinus dural AVM, the patient usually complains of a bruit,
and embolization or resection is optional, depending on symptom
tolerance. In the case of type II CCF, the problem is usually
intraocular and intraorbital venous hypertension, with proptosis,
chemosis, and sometimes threatened vision. Ocular tonometry
can help determine the extent of the threat to vision. Treatment
of type II CCF involves endovascular embolization of prominent
feeders, followed by occlusion of the affected venous dural sinus.
As long as the dural AVM drainage is separated from the cerebral
circulation, occlusion of the affected venous drainage is safe and
curative. The affected stenotic transverse-sigmoid sinus segment
can be reached by endovascular techniques through the jugular
vein. The cavernous sinus can be reached by the petrosal sinus or,
with neurosurgical assistance, through the superior orbital vein.
CENTRAL NERVOUS SYSTEM TUMORS
Intracranial Tumors
Intracranial tumors can be classified as primary versus secondary,
as pediatric versus adult, by cell of origin, or by location in the
CHAPTER 67  Neurosurgery 1909
nervous system. Primary tumors arise from tissues in the nervous
system, whereas secondary tumors originate from tissues outside
the nervous system and metastasize secondarily to the brain. They
may represent local extension of regional tumors, such as chor-
doma or scalp cancer, but usually reach the nervous system
through the hematogenous route.
In general, the incidence of primary brain tumors is higher in
whites than in blacks, and mortality is higher in males than in
females. According to the Central Brain Tumor Registry of the
United States (CBTRUS), the overall incidence of primary brain
tumors was 14.8/100,000 person-years between 1998 and 2002
(CBTRUS statistical report, 2005-2006). On the other hand,
secondary tumors outnumber primary brain tumors by 10:1 and
occur in 20% to 40% of cancer patients.20 Because no national
cancer registry documents brain metastases, the exact incidence is
unknown, but it has been estimated that 98,000 to 170,000 new
cases are diagnosed in the United States each year.21
Clinical Presentation
The clinical manifestations of various brain tumors can be divided
into those caused by focal compression and irritation by the tumor
itself and those attributed to secondary consequences, namely,
increased ICP, peritumoral edema, and hydrocephalus. Usually,
symptoms are caused by a combination of these factors.
The clinical presentation does not differ much by tumor histol-
ogy; rather, rate of growth and location of the tumor contribute
to the clinical features. A meningioma peripherally located in a
relatively silent area of the brain, with a slow rate of growth, may
enlarge to a significant size in a neurologically intact patient
because the brain can accommodate to a slowly growing lesion.
On the other hand, a small metastatic lesion at the foramen of
Monro or in the sensorimotor strip can cause acute hydrocephalus
or seizures, respectively.
Headache occurs in 50% to 60% of primary brain tumors and
in 35% to 50% of metastatic tumors. It is classically described as
being worse in the morning, probably because of hypoventilation
during sleep, with consequent elevation of the PCO2 and cerebro-
vascular dilation. The headache is associated with nausea and
vomiting in 40% of patients and may be temporarily relieved by
vomiting as a result of hyperventilation. Seizures may be the first
symptom of a brain tumor. Patients older than 20 years presenting
with a new-onset seizure are aggressively investigated for a brain
tumor.
Infratentorial lesions may be manifested with headache, nausea
and vomiting, gait disturbance and ataxia, vertigo, cranial nerve
deficits leading to diplopia (abducens nerve), facial numbness and
pain (trigeminal nerve), unilateral hearing deficit and tinnitus
(vestibulocochlear nerve), facial weakness (facial nerve), dysphagia
(glossopharyngeal and vagus nerves), and CSF obstruction causing
hydrocephalus and papilledema. Supratentorial lesions may be
manifested with different symptoms, depending on the location.
Frontal lobe lesions are manifested as personality changes, demen-
tia, hemiparesis, or dysphasia. Temporal lobe lesions may be mani-
fested with memory changes, auditory or olfactory hallucinations,
or contralateral quadrantanopia. Patients with parietal lobe lesions
may develop contralateral motor or sensory impairment, apraxia,
and homonymous hemianopia, whereas those with occipital lobe
lesions may show contralateral visual field deficits and alexia.
Imaging Studies
The initial workup generally involves a relatively inexpensive diag-
nostic tool, a CT scan of the brain. CT provides a rapid means
1910 SECTION XIII  Specialties in General Surgery

of evaluating changes in brain density, such as calcifications,

hyperacute hemorrhages (<24 hours old), and skull lesions. MRI
of the brain, however, is the “gold standard” modality for diagno-
sis, presurgical planning, and post-therapeutic monitoring of
brain tumors. Gadolinium contrast enhancement with MRI is
more sensitive in demonstrating defects in the blood-brain barrier
and localizing small metastases (up to 5 mm). It can be used in
patients allergic to iodine and those with renal failure. Advances
in MRI techniques have evolved from strictly morphology-based
imaging to a modality that encompasses function, physiology, and
anatomy. Diffusion-weighted imaging can help distinguish
between gliomas and abscesses, and perfusion-weighted imaging
can predict response to radiotherapy in low-grade gliomas. Func-
tional MRI can be used in planning of surgery for tumors in A
eloquent areas of the brain to enable radical resection with less
morbidity. Diffusion tensor imaging can demonstrate the effect
of a tumor on white matter tracts. Magnetic resonance angiogra-
phy is used more routinely as a noninvasive modality to evaluate
the vascularity of a tumor or anatomic relationship of a tumor to
normal cerebral vasculature.22

Surgery
Dexamethasone is recommended for the management of brain
tumors because of its propensity to reduce peritumoral edema by
stabilizing the cell membrane. An antiepileptic drug is also recom-
mended for tumors close to the sensorimotor strip. Mannitol
is often administered before dural opening and operative
resection.
Technical advances have made tumor surgery safer and more
effective. The intraoperative microscope provides superior illumi-
nation and magnification, thereby allowing the surgeon to resect
tumors from critical areas through small cranial openings. The
cavitational ultrasonic surgical aspirator simultaneously breaks up
and sucks away firm tumors while protecting vital neural and
vascular structures. Intraoperative ultrasonography provides real-
time imaging of tumors and cysts in subcortical and deep areas
of the brain. Intraoperative CT or MRI is standard practice in
some centers, enabling on-table imaging of the extent of resection
(Fig. 67-11A). CT and MRI also allow real-time visualization of
a biopsy needle within a target. Image-guided (CT or MRI) fra-
meless surgical navigation allows instant and accurate localization
of the tip of a probe during a craniotomy by displaying that point
on a preoperative CT or MRI scan (Fig. 67-11B).
The primary goals of surgery include histologic diagnosis and
reduction of mass effect by removal of as much tumor as is safely
possible to preserve neurologic function. The decision between a
needle biopsy and more radical surgical resection depends on the
location and size of the tumor, its sensitivity to radiation or che-
motherapy, the preoperative Karnofsky performance score of the
patient, and the systemic status of the primary cancer in case of
metastatic brain lesions.
PRIMARY BRAIN TUMORS
Primary tumors of the brain are divided into intra-axial (those
arising from within the brain parenchyma) and extra-axial (those
arising from outside the brain parenchyma).
Intra-Axial Brain Tumors
Intra-axial brain tumors develop from the glia, or supportive
structures, of the neurons and are collectively called gliomas. Total
B
FIGURE 67-11  Technologic advances in the operating room. A, Intra-
operative CT scanner. B, Computer-guided surgical navigation showing
real-time location of a surgical probe tip on the preoperative MRI study
during resection of clival chordoma.
surgical resection of gliomas is extremely rare because of their
ability to infiltrate widely along the white matter tracts and to
cross the corpus callosum into the contralateral hemisphere. Radi-
ation therapy and chemotherapy options vary according to the
histology of the brain tumor. Therapy involving surgically
implanted carmustine-impregnated polymer combined with post-
operative radiation therapy has a role in the treatment of de novo
and recurrent high-grade gliomas. Biologic therapies are currently
under evaluation for patients with brain tumors and include den-
dritic cell vaccination, tyrosine kinase receptor inhibitors, farnes-
yltransferase inhibitors, virus-based gene therapy, and oncolytic
viruses. An ideal therapy will target rapidly growing malignant
glioma cells along with infiltrating tumor cells, with minimal
toxicity to normal cells. This requires that the therapeutic vehicle
of choice have access to all cells in the brain and be able to dis-
tinguish invasive or quiescent tumor cells from normal cells.23
The current histopathologic classification of brain tumors was
recently updated by the World Health Organization (WHO).24
WHO classifies intra-axial brain tumors by cell type and grades
them on a scale of I to IV based on light microscopy characteristics
that include the degree of cellularity, pleomorphism, mitotic
figures, endothelial proliferation, and necrosis. The higher the
grade, the more aggressive and malignant is the tumor.
An exhaustive review of neuro-oncology is beyond the scope
of this chapter. What follows is an overview of the most com-
monly encountered and unique tumors of the central and periph-
eral nervous systems.