Author's Accepted Manuscript

The ventricular system of the brain: Anatomy and

normal variations
Lindsay Stratchko, Irina Filatova, Amit Agarwal,
Sangam Kanekar MD

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PII: S0887-2171(16)00005-6
DOI: http://dx.doi.org/10.1053/j.sult.2016.01.004
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Semin Ultrasound CT MRI

Cite this article as: Lindsay Stratchko, Irina Filatova, Amit Agarwal, Sangam Kanekar MD,
The ventricular system of the brain: Anatomy and normal variations,
Semin Ultrasound CT MRI , http://dx.doi.org/10.1053/j.sult.2016.01.004

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The Ventricular System of the Brain: Anatomy and Normal

Variations

Lindsay Stratchko1
Irina Filatova1
Amit Agarwal1
Sangam Kanekar, MD1,2
1Department of Radiology,
2Department of Neurology

Penn State University and Hershey Medical Center

Address for correspondence:

Sangam Kanekar, MD
Associate Professor
Department of Radiology & Neurology
The Pennsylvania State University &
Hershey Medical Center,
500 University Drive
Hershey, PA 17033
Tel 717-531-8051
Fax 717-531-0006
skanekar@hmc.psu.edu

INTRODUCTION

The ventricular system is comprised of a set of adjoining cavities that

produce and circulate cerebrospinal fluid (CSF) within the brain. Cerebrospinal

fluid is produced within the ventricular system by specialized ependymal cells

known as choroid plexus. This fluid serves many purposes, including mechanical

protection, regulation of homeostasis within the cerebral interstitial fluid, as well as

facilitation of brain development. Cerebrospinal fluid travels through the

ventricular system in a unidirectional, rostrocaudal fashion and ultimately

communicates with the cranial and spinal subarachnoid space, which is the

predominant location of CSF reabsorption [Fig 1].

Knowledge of anatomical relationships between the ventricular system and

the surrounding brain parenchyma is essential in understanding intraventricular

pathology and the potential risks associated with surgical management. It is

important to be familiar with several anatomic variations in order to avoid false

diagnosis as well as prompt further investigation for associated central nervous

system (CNS) abnormalities. This article aims to review pertinent ventricular

anatomy with emphasis on recent advances in neuroimaging. We provide an

overview of anatomical variations of the ventricular system and their association

with other structural CNS anomalies. Lastly, applied surgical anatomy is discussed,

highlighting operative approach and potential risk to adjacent structures.

ANATOMY

Lateral Ventricles

The paired lateral ventricles are comprised of the body and atria centrally

with the anterior (frontal), inferior (temporal), and posterior (occipital) horns

forming a C-shape, extending peripherally in their respective cerebral lobes. The

lateral ventricles are intimately associated with portions of the diencephalon and

telencephalon, and communicate with the third ventricle through the foramen of

Monroe.

The anterior, or frontal, horns of the lateral ventricle extend from the frontal

lobe, connecting with the body of the lateral ventricle at the level of the foramen of
Monroe [Fig 2]. The corpus callosum wraps around the frontal horn forming the

superior, anterior, and inferior boundaries. The corpus callosum is a broad, flat

group of nerve fibers situated midline in the supratentorial brain. It is the largest

white matter tract and is responsible for interhemispheric communication. The

corpus callosum is divided into the genu, body, and splenium extending from

anterior to posterior, with a small portion termed the rostrum (from the Latin

“beak”) projecting inferior and posterior from the genu. The genu of the corpus

callosum defines the roof and anterior wall of the frontal horn and the rostrum

forms the floor. The frontal horns are separated by the septum pellucidum and they

are bordered laterally by the head of the caudate nucleus [Fig 2].

The bodies of the lateral ventricles are situated superior to the thalamus.

The anterior-most aspect of the body is the foramen of Monroe, and the posterior

boundary is defined by the convergence of the fornix and corpus callosum. The roof

is formed by the body of the corpus callosum, which parallels and defines at least a

portion of the superior border of each segment of the lateral ventricles. The caudate

nucleus forms the lateral border of the lateral ventricle body.

The atrium of the lateral ventricle, also referred to as the trigone, extends

from the posterior aspect of the body and is contiguous with the paired inferior

(temporal) and posterior (occipital) horns. The body, splenium, and tapetum of the

corpus callosum delineate the superior border of the atrium. The tapetum, from the

Latin tapete or “carpet”, is comprised of decussating fibers of the corpus callosum

that radiate laterally, overlying the lateral ventricles, also forming the lateral

boundaries of the trigones as well as the occipital and temporal horns. The caudate
nucleus also contributes to the lateral wall of the atrium, situated anterior to the

tapetum. Medially, the corpus callosum and calcar avis (or hippocampus minor)

form the superior and inferior margin of the atrium, respectively. The floor of the

atrium is defined by the collateral trigone.

The posterior horns, also referred to as the occipital horns of the lateral

ventricles converge medially as they extend into the occipital lobes. In the supine

patient, the occipital horns are the most dependent portion of the lateral ventricles.

Knowing this anatomic relationship is imperative in the evaluation of subtle acute

pathology, such as layering intraventricular hemorrhage. The confines of the

occipital horns are largely the same as the atrium with the roof and lateral walls

bordered by the tapetum, the floor comprised of the collateral trigone, and the

medial wall defined by the bulb of the corpus callosum superiorly and the calcar avis

inferiorly.

Lastly, the inferior (temporal) horns extend inferiorly from the atrium,

wrapping around the pulvinar nuclei of the thalamus before terminating in the

anterior temporal lobes. The temporal horns are the largest horns of the lateral

ventricles. As stated previously, the tapetum covers the lateral aspect of the

temporal horn roof, outlining the lateral walls. The medial roof is formed by the

thalamus and caudate tail. The floor is comprised of several structures, including the

hippocampus and the fimbria hippocami, which is a prominent white matter tract

extending along the medial margin of the hippocampus. The lateral aspect of the

floor is formed by the collateral eminence, which is continuous with the collateral
trigone. The choroidal fissure is located along the medial wall of the inferior horns,

separating the temporal lobes from the optic tract.

Foramen of Monro

The interventricular foramen of Monro connects the lateral ventricles and

the midline third ventricle. The anterior columns and body of the fornix bound the

anterior aspect of the foramina, and the thalami form the posterior border. The

foramen of Monro contains choroid plexus and associated vessels. The medial

posterior choroidal artery, arising from the posterior cerebral artery, travels

through the foramen of Monro, supplying the choroid plexus. After passing through

the interventricular foramen, it forms an anastamosis with branches of the lateral

posterior choroidal artery, a more distal branch of the posterior cerebral artery.

The superior choroidal vein also traverses the Foramen of Monro, joining with the

superior thalamostriate vein and anterior vein of the caudate nucleus at the

confluence of the internal cerebral vein.3

Third Ventricle

The midline third ventricle is a narrow cavity situated between the thalami

[Fig 3]. The third ventricle communicates with the lateral ventricles via the foramen

of Monro and the inferior fourth ventricle through the cerebral aqueduct of Sylvius.

The convex superior margin of the third ventricle extends from the foramen of

Monroe to the suprapineal recess. The posterior wall of the third ventricle, from

superior to inferior, includes the habenular commissure, the pineal gland and its
associated recess, and the posterior commissure. The posterior commissure is an

important fiber tract situated at the junction of the midbrain and diencephalon that

connects the pretectal nuclei and is involved in pupillary reflex.2

The columns of the fornix form the anterior wall of the third ventricle. The

rostral portion of the third ventricle contains two distinct projections above and

below the optic chiasm termed the supraoptic and infundibular recesses,

respectively [Fig 4]. Medial fibers of the optic nerves receive input from the nasal

aspect of the retina and decussate at the level of the optic chiasm. Lateral fibers

from the temporal portion of the retina converge at the optic chiasm, however, do

not cross to the contralateral side. The anterior commissure and lamina terminalis

extend along the anterior third ventricle from the foramen of Monro to the

supraoptic recess.

The floor can be subdivided into three different zones: the anterior

premammillary portion, the intermediate or interpeduncular portion, and the

posterior peduncular portion. The premammillary portion consists of the

supraoptic and infindibular recesses as well as the tuber cinerium of the

hypothalamus. The interpeduncular portion spans from the mammillary bodies to

the posterior aspect of the interpeduncular space. Finally, the peduncular space is

made up of the midbrain tegmentum, situated just above the cerebral peduncles,

which extends to the cerebral aqueduct.4

The lateral walls of the third ventricle are bordered superiorly by the

anterior thalami and inferiorly by the hypothalamus and subthalamus. The thalami

are conjoined by a band of gray matter termed the massa intermedia, or

interthalamic adhesion. The interthalamic adhesion is often, but not always present,

and is typically larger in females than males.5

Aqueduct of Sylvius

The cerebral aqueduct of Sylvius serves as a communication for

cerebrospinal fluid flow from the third ventricle to the fourth ventricle. Being the

narrowest portion of the ventricular system, the aqueduct of Sylvius is susceptible

to obstruction and resultant hydrocephalus. The cerebral aqueduct traverses the

dorsal aspect of the midbrain, surrounded by the periaqueductal gray matter, which

is situated anterior to the tectum and posterior to the tegmentum.

Fourth Ventricle

The fourth ventricle is a diamond-shaped midline cavity between the

brainstem and the cerebellum in the infratentorial region. Cerebrospinal fluid

enters the fourth ventricle from the rostral system via the cerebral aqueduct and

exits into the subarachnoid space by the paired lateral foramina of Luschka and the

single midline foramen of Magendie [Fig 5]. The fourth ventricle is comprised of a

floor, roof and two lateral recesses. The superior and inferior most portions of the

fourth ventricle are relatively narrow, expanding centrally to the lateral margins of

the brainstem at the level of the lateral recesses.

The dorsal roof is divided into the superior and inferior segments by the

fastigium. The superior medullary velum and the medial aspects of the superior

cerebellar peduncles shape the superior roof of the fourth ventricle. The foramen of
Magendie connects the ventricular system with the cisterna magna at the caudal

aspect of the inferior medullary velum, which forms the inferior roof.

The volar floor of the fourth ventricle is formed superiorly by the pons and

inferiorly by the medulla. The floor can be further divided into three segments. The

superior (pontine) portion extends from the cerebral aqueduct to the level of the

cerebellar peduncles. The intermediate (junctional) section is a thin band between

the inferior aspect of the superior cerebellar peduncles and the taenia of the fourth

ventricle. The inferior (medullary) segment extends from the taenia to the obex,

which is in close proximity to the foramen of Magendie. The medial eminence and

the sulcus limitans are paired structures oriented vertically along the floor of the

fourth ventricle. The medial eminence contains the facial colliculus, which is formed

by the motor fibers of the facial nerve as they pass over the abducens nuclei. The

medial eminence also houses the hypoglossal and vagal trigones, which overlie their

respective cranial nerve nuclei. The superior and inferior fovea arise from the sulcus

limitans as well as the locus coeruleus, which plays an important role in the stress

response.

The junction of the roof and the floor of the fourth ventricles form the lateral

recesses. The recesses communicate with the subarachnoid space at the level of the

cerebellopontine angles via the foramina of Luschka.

Choroid Plexus

The choroid plexus serves a vital role in the production of cerebrospinal

fluid. Typical cerebrospinal fluid production in adults ranges between 400 and 600
mL per day with a cerebrospinal fluid volume totaling 150 mL at any given time.

Additionally, the choroid plexus serves a filtration function and secretes several

growth factors and nutrients such as vitamins B1, B12, C and folate to support and

maintain homeostasis.18 The choroid is located along the walls of the lateral

ventricles, sparing the frontal and occipital horns; it extends through the foramen of

Monro into the roof of the third ventricle, and is also located along of the roof of the

fourth ventricle.

Branches of the anterior and posterior cerebral circulation provide blood

supply to the choroid plexus. The anterior choroidal arteries from the middle

cerebral arteries and the posterior choroidal arteries from the posterior cerebral

arteries supply the choroid within the lateral ventricles. The posterior choroidal

arteries also supply the third ventricle. Choroid within the fourth ventricle receives

blood via the anterior and posterior inferior cerebellar arteries arising from the

vertebrobasilar system.19

Histology

The ventricles are lined by specific glial cells termed ependymocytes. This

ependyma is comprised of ciliated columnar epithelium, which aids in circulation of

cerebrospinal fluid throughout the ventricular system. A specialized type of

ependymal cell, known as choroid plexus, produces cerebrospinal fluid. Choroid

plexus is comprised of vascularized branching pia mater surrounded by a network

of loose connective tissue. A thin basement membrane separates the connective

tissue core from the layer of simple cuboidal epithelium. Numerous permeable
capillaries are situated within the connective tissue matrix, which provide the blood

supply from which the cerebrospinal fluid is made. Surface area is increased in the

relatively small ventricular system secondary to villi and a brush border of

microvilli around the capillaries.16 Plasma is passively filtered from the capillaries

to the connective tissue interstitium. From this point, the choroidal epithelium

actively transports several ions (including K+, Na+, Cl-, and HCO3-) into the

ventricular lumen, allowing water to follow via an osmotic gradient, ultimately

forming CSF.18

Anatomy on Transcranial Ultrasonography

Neonatal cranial ultrasonography has been utilized as a fast, non-invasive,

and cost effective way to evaluate the infant brain. Lack of ionizing radiation makes

this study a preferred method of assessing neurologic status of an infant, whose

exam and clinical symptoms may be nonspecific and challenging to interpret.

Cranial sonography is often performed at bedside, using either a sector or linear

array transducer. The acoustic window is generally the anterior fontanelle due to

its larger size and relative closer proximity to the supratentorial brain. The smaller

posterior fontanelle can also be used, particularly if there is interest in evaluating

the posterior structures such as the occipital lobes or cerebellum. A general

principle in cranial sonography is that normal white matter appears hyperechoic

and gray matter is hypoechoic.

Standard coronal and sagittal planes are imaged in cranial sonography to

allow for accurate and reproducible images for evaluation and comparison. An
important coronal view in the evaluation of the ventricular system is at the level of

the frontal horns of the lateral ventricles [Fig 6]. At this level, the cavum septum

pellucidum is typically visualized as a thin, anechoic structure situated between the

anterior horns. The corpus callosum is a hypoechoic band that crosses midline,

connecting the cerebral hemispheres above the level of the anterior horns. The next

standard coronal image is at the level of the foramen of Monro and third ventricle,

which is a slit-like anechoic structure just inferior to the septum pellucidum. This

image better delineates the thalami and basal ganglia structures, which are situated

along the floor of the lateral ventricles and lateral aspect of the third ventricle. Due

to the orientation of the transducer, portions of the brainstem are included in the

field of view at this level. The echogenic choroid plexus becomes more visible at the

roof of the third and fourth ventricles as the transducer is angled more posteriorly.

The quadrigeminal plate cistern is an important landmark used in neonatal cranial

sonography, at this level the temporal horns of the lateral ventricles are well seen.

Additionally, the posterior fossa structures are visualized, including the cisterna

magna in the far field, at the level of the cerebellar hemispheres. One of the final

coronal images displays the trigones of the lateral ventricles containing their

hyperechoic choroid plexus, which should be more echogenic than the surrounding

periventricular white matter.

The transducer can then be rotated ninety degrees to further image the

infant brain in a sagittal/parasagittal plane [Fig 7]. The true midline sagittal image

highlights the corpus callosum, previously described as a longitudinal hypoechoic

band, which is surrounded superiorly by the echogenic cingulate gyrus and

inferiorly by the anechoic cavum septum pellucidum. In the far-field of the initial

midline image, the anechoic third and fourth ventricles are well-visualized. Central

echogenic brainstem (most prominently the pons) and cerebellar fossa are seen

along the anterior and posterior margins of the fourth ventricle, respectively.

Paired parasagittal views can then be obtained, highlighting the lateral ventricles

and their internal echogenic choroid plexus. The junction of the caudate nucleus

and the thalamus, referred to as the caudothalamic groove, can be identified along

the floor of the fourth ventricle in this plane. The caudothalamic groove is an

important landmark in neonatal cranial sonography; it is located at the level of the

foramen of Monro and it demarcates the anterior-most border of the choroid plexus.

Echogenic material anterior to the caudothalamic groove on this parasagittal view

suggests germinal matrix hemorrhage. Far lateral images can be obtained, further

delineating the lateral thalami, temporal lobes and their accompanying lateral

ventricular horns, and intraventricular choroid plexus.14

White Matter Tract Relationships with the Ventricular System

Diffusion tensor imaging (DTI) is very useful in outlining the various white

matter tracts in the supra- and infratentorial brain parenchyma. Many of these fiber

tracts are closely related to the ventricular system. Knowledge of these tracts,

normal anatomic variations and displacement by intrinsic brain pathology is vital

for neurosurgeons and neurologist. In respect to the ventricular system, these

fibers may be categorized into projection fibers (cortex–spinal cord, cortex-

brainstem, and cortex-thalamus connections), association fibers (cortex-cortex

connections), limbic system tracts (which are predominantly located in the

temporal horns), and commissural fibers (right-left hemispheric connections).

Commissural fibers comprise the largest bundles of white matter, which are

most intimately associated with the lateral ventricles. Prominent commissural

fibers include the corpus callosum and anterior and posterior commissures [Fig 8,

9]. The main function of the corpus callosum is interhemispheric sensorimotor and

auditory connectivity. The projections from the genu of the corpus callosum form

the forceps minor; those from the splenium form the forceps major. Projections

from the splenium, which pass inferiorly along the lateral margin of the posterior

horn of the lateral ventricle to the temporal lobes, are called the tapetum. The

anterior commissure is a compact bundle of fibers between the anterior and

posterior columns of the fornix.

Projection fibers are afferent and efferent tracts that interconnect areas of

the cortex with the brainstem, deep nuclei, cerebellum, and spinal cord. Major

projection fibers, which are easily identifiable on DTI include the corticospinal,

corticobulbar, corticopontine, and geniculocalcarine tracts (optic radiations). Of

these fibers, portions of the corticospinal and optic radiations are in close

anatomical relationship with the ventricular system. The optic radiations connect

the lateral geniculate body to the primary visual cortex. The anterior-most fiber of

the optic radiation wraps around the anterior temporal lobe, projecting posteriorly

to its final termination in the calcarine sulcus; this important tract is commonly

referred to as Meyer’s loop [Fig 10]. Precise identification of Meyer’s loop is

particularly important during planning for epilepsy surgery as inadvertent damage

can result in homonymous upper quadrantanopia. The central bundle of the optic

radiation passes laterally, crossing over the roof of the temporal horns before

coursing along the lateral walls and roof of the trigones and occipital horns of the

lateral ventricles. The posterior bundle courses over the roof of the trigones and

occipital horns, terminating at the upper lip of the calcarine fissure. The lateral

walls of the temporal and occipital horns are in close proximity to the optic

radiation bundles, separated from the ependyma by a thin layer of the corpus

callosum, known as the tapetum.

The cingulum, fornix, and stria terminalis form the three major white matter

tracts of the limbic system. The cingulum begins below the rostrum of the corpus

callosum and terminates at the parahippocampal gyrus and uncus. Its course arches

over the corpus callosum, situated inferior to the cingulate gyrus. The fornix carries

afferent and efferent information between the hippocampus, the septal area, the

hypothalamus, and mammillary body. The fornix branches into two columns near

the anterior commissure and projects into the dorsal regions of the hippocampi.

The brainstem and cerebellum represent a crossroad between the fiber bundles of

the spinal cord, midbrain and cerebral hemispheres. There are five major white matter

tract connections seen which include the superior (SCP), middle (MCP), and inferior

cerebellar peduncles (ICP), the corticospinal tract, and the medial lemniscus (ML). At

different levels some of these fibers are in close proximity to the fourth ventricle [Fig

11].
NORMAL ANATOMICAL VARIANTS

Asymmetric Size

In the majority of individuals, ventricular size is symmetric, however, in

approximately 20-40% of infants one lateral ventricle may be slightly larger.14

Typically, when encountered, the left lateral ventricle is marginally larger than the

right and it is often associated with a more prominent ipsilateral choroid plexus.12

The difference in size is usually most evident in the occipital horn of the lateral

ventricles. Colpocephaly, the term given to disproportionate dilation of the occipital

horns, can be differentiated from normal variant asymmetric ventricular size by

examining the frontal horns of the lateral ventricles and overall ventricular

morphology.

Benign variations in lateral ventricular size generally maintain normal

configuration throughout the remainder of the ventricular system [Fig 12]. Follow-

up examinations will not show interval increase in size or asymmetry.12

Colpocephaly, on the other hand, shows significant enlargement of the occipital

horns when compared to the frontal horns and there is typically loss of normal

ventricular configuration.13 The distinction between normal variation in ventricular

size and colpocephaly is important because the latter is associated with other

central nervous system abnormalities and should prompt further investigation [Fig

13]. Colpocephaly has reported associations with Chiari II malformation and

agenesis of the corpus callosum.14

Absent Septum Pellucidum

 Absence of the septum pellucidum is a relatively uncommon variant,

occurring in approximately 2 to 3 per 100,000 people [Fig 14].7 Frequently, absence

of the septum pellucidum is associated with additional structural abnormalities of

the central nervous system, however, there have been reports of isolated absence.6

Documented associations include, but are not limited to, septo-optic dysplasia,

holoprosencephaly, agenesis of the corpus callosum, and Chiari II malformation.6

Cavum Septum Pellucidum and Vergae

The cavum septum pellucidum is a normal fetal CSF filled cavity between the

septal laminae [Fig 15]. This structure normally begins to close in utero and its

resolution may continue into the first several months of postnatal life. Cavum

vergae is a similar entity to cavum septum pellucidum with the differentiating

feature being the location. Cavum septum pellucidum is an anterior structure

occurring between the genu of the corpus callosum to the anterior columns of the

fornix. Alternatively, cavum vergae is located posterior to the anterior columns of

the fornix and may extend to the level of the splenium of the corpus callosum [Fig

16]. Several studies have attempted to discern whether a persistent cavum septum

pellucidum was associated with neuropsychiatric diseases, particularly

schizophrenia. Results are varied with some researchers suggesting that a large (≥6

mm) cavum septum pellucidum is more prevalent in schizophrenic patients.11

Fifth Ventricle

The fifth ventricle is a term used to describe an ependyma-lined expansion of

the caudal portion of the central canal of the spinal cord. The fifth ventricle is also

referred to as the terminal ventricle and is located at or in close proximity to the

conus medullaris. In a retrospective analysis of over four hundred normal pediatric

spine MRI examinations, isolated terminal ventricle was reported in 2.6%, all in

children less than five years of age.9 Persistence of the fifth ventricle into adulthood

is a rare entity, particularly in the absence of other structural malformations of the

central nervous system. On imaging, a terminal ventricle is an intramedullary ovoid

cystic lesion at the level of the conus medullaris [Fig 17]. The lesion follows CSF

signal intensity and does not enhance after contrast administration. Clinical

manifestations vary and conservative management is typically favored over surgery

unless significant associated symptoms are present.10

Choroid Plexus Cysts and Xanthogranulomas

Cystic structures may be encountered in the intraventricular and

periventricular regions, typically along the lateral ventricles of neonates. The

differentiation between intraventricular choroid plexus cysts and periventricular

cysts are important. Both entities can be seen in benign conditions, often

representing normal anatomical variants, and they are often self-limiting. Abnormal

periventricular cysts are typically encountered at the caudothalamic groove and can

be seen in the setting of evolving germinal matrix hemorrhage (subependymal

cysts) and metabolic disorders (germinolytic cysts). These cysts typically cannot be
differentiated by imaging characteristics. Periventricular leukomalacia is an entity

seen in preterm infants as a sequela of hypoxic ischemic insult, which leads to white

matter cysts adjacent to the lateral ventricles.14

Choroid plexus cysts are considered inconsequential in fetal or neonatal

imaging when single, not associated with other central nervous system

abnormalities, and measure less than 1 cm in size. These cysts typically regress by,

or shortly after birth. Chromosomal abnormalities (typically trisomy 18, trisomy 21,

and Klinefelter syndrome) should be considered when choroid plexus cyst number

or appearance is atypical.14

Adult choroid plexus cysts and xanthogranulomas are similar entities and

imaging features frequently overlap. Choroid plexus cysts are epithelium-lined

cysts that typically result from degenerative lipid accumulation over time. Most are

less than 1 cm in size and their frequency is relatively common, occurring in up to

50% of autopsy specimens.15 Choroid plexus cysts are often bilateral and located in

the atria of the lateral ventricles. Imaging characteristics vary and cysts usually

demonstrate increased signal intensity on T1 and T2 imaging compared to

cerebrospinal fluid (occasionally T1 signal may be isointense to CSF) [Fig 18]. The

cysts will not completely suppress on fluid-attenuated inversion-recovery

sequences and most will restrict diffusion on diffusion-weighted imaging.15

Computed tomography may be helpful in detection as some choroid plexus cysts

show peripheral calcification.

APPLIED SURGICAL ANATOMY

Management of Hydrocephalus

Hydrocephalus occurs when there is an imbalance between cerebrospinal

fluid production and absorption, resulting in fluid accumulation and increased

intracranial pressure. Congenital and acquired causes of hydrocephalus include, but

are not limited to, stenosis of the cerebral aqueduct, trauma, infection, and tumor.

Classically, treatment of hydrocephalus has included cerebrospinal fluid diversion

from the ventricular system to the heart, pleura or peritoneal cavity via a shunt

catheter. Recent advances in surgical management allow intraventricular

cerebrospinal fluid to drain to the subarachnoid space through instrumented

perforations in the floor of the third ventricle, creating a connection with the

suprasellar cistern. This procedure is termed endoscopic third ventriculostomy and

is considered the procedure of choice in the appropriate patient population for the

management of obstructive hydrocephalus given its lack of indwelling catheters and

their associated infection and complication risk.

Several approaches can be utilized to gain access to the ventricular system.

Kocher’s point is often used to access the frontal horn of the lateral ventricle. The

insertion point is just anterior to the coronal suture, approximately 2-3 cm from

midline at the midpupillary line. The trajectory is perpendicular to the skull, aiming

toward the intersection of an anteroposterior line at the ipsilateral medial canthus

and a horizontal line from the tragus. This approach allows for avoidance of the
motor cortex and ultimate catheter placement in the region of the foramen of

Monro, decreasing catheter obstruction by choroid plexus.

Frazier’s point is used to reach the occipital horn of the lateral ventricle.

Entry point is located approximately 6-7 cm above the inion and 3-4 cm lateral to

midline. The trajectory is parallel to the skull base, pointing towards the middle of

the forehead. A greater length of shunt tubing can be advanced through the length

of the lateral ventricle body when Frazier’s point is used. Frazier’s point is superior

and lateral to a previously described posterior approach described by Walter Dandy

in the early twentieth century, which is more frequently utilized in the pediatric

population. Dandy’s point is at the level of the lambdoid suture at the midpupillary

line, however, it is associated with a higher rate of visual impairment given the more

medial approach.

A less frequently used approach known as Keen’s point allows for entrance to

the trigone of the lateral ventricle. When performed, there is a more direct route of

shunt tubing passage from the scalp into the peritoneum. Keen’s entry point is

located approximately 3 cm posterior and superior to the pinna of the ear with

trajectory perpendicular to the skull.17

External ventricular drain placement (also referred to as ventriculostomy) is

a common procedure used to manage acute hydrocephalus as well as monitor

intracranial pressure. It can be performed utilizing the access points detailed above.

As previously described, endoscopic third ventriculostomy is a procedure

that has been gaining popularity as a management option, particularly for non-

communicating (obstructive) hydrocephalus. The lateral ventricle is accessed from

an anterior approach just medial to the midpupillary line. An endoscope is then

advanced through the foramen of Monro into the third ventricle. A small

perforation made in the third ventricle floor between the infundibular recess and

mammillary bodies, which is enlarged with aid of a balloon catheter. Preoperative

planning is crucial to evaluate the thickness of the third ventricular floor as well as

the location of the basilar artery in relation to the planned ventriculostomy site.17

Intraventricular Endoscopy for Miscellaneous Conditions

Endoscopic procedures can be performed to aid in the diagnosis and

management of several intraventricular pathologies. Endoscopy can be utilized in

biopsy, excision and/or management of obstructive hydrocephalus associated with

intraventricular tumors. Individual operative approaches can be planned

depending on the lesion’s location. General principles are used when planning

surgical trajectory: avoidance of eloquent areas, allow access to blood supply and

the lesion’s ventricular attachment, and allow management of hydrocephalus, if

present. Typically, approach is planned to allow a small amount of normal ventricle

between the entry point and the lesion in order to provide better visualization of the

mass and surrounding anatomy.17

Additional non-neoplastic processes can be endoscopically managed in the

appropriate patient population. Colloid cysts as well as arachnoid cysts can be

managed via complete excision, aspiration or fenestration. Neurocysticercotic cysts

from the parasite Taenia solium can be removed endoscopically, however, care must
be taken to avoid cyst rupture as it may result in sterile meningitis.17 Surgical

approach is similar to tumor management and largely dependent on cyst location.

Resources:
1. Hikosaka, O., S. R. Sesack, L. Lecourtier, and P. D. Shepard. "Habenula:
Crossroad between the Basal Ganglia and the Limbic System." Journal of
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Interhemispheric Connections and Laterality." The Brain and Behavior: An
Introduction to Behavioral Neuroanatomy. Cambridge, UK: Cambridge UP,
2005. 226-33. Print.
3. Tubbs, R. Shane, Peter Oakes, Ilavarasy S. Maran, Christian Salib, and
Marios Loukas. "The Foramen of Monro: A Review of Its Anatomy, History,
Pathology, and Surgery." Childs Nerv Syst Child's Nervous System 30.10
(2014): 1645-649. Web.
4. Corrales, M., and G. Torrealba. "The Third Ventricle. Normal Anatomy and
Changes in Some Pathological Conditions." Neuroradiology 11.5 (1976):
271-77. Web.
5. 5. Allen, Laura S., and Roger A. Gorski. "Sexual Dimorphism of the Anterior
Commissure and Massa Intermedia of the Human Brain." The Journal of
Comparative Neurology J. Comp. Neurol. 312.1 (1991): 97-104. Web.
6. Supprian, T., J. Sian, A. Heils, E. Hofmann, M. Warmuth-Metz, and L.
Solymosi. "Isolated Absence of the Septum Pellucidum." Diagnostic
Neuroradiology (1999): 563-66. Springer-Verlag. Web.
7. Barkovich, Aj, and D. Norman. "Absence of the Septum Pellucidum: A
Useful Sign in the Diagnosis of Congenital Brain Malformations." American
Journal of Roentgenology 152.2 (1989): 353-60. Web.
8. Mortazavi, M. M., N. Adeeb, C. J. Griessenauer, H. Sheikh, S. Shahidi, R. I.
Tubbs, and R. S. Tubbs. "The Ventricular System of the Brain: A
Comprehensive Review of Its History, Anatomy, Histology, Embryology,
and Surgical Considerations." Childs Nerv Syst Child's Nervous System30.1
(2013): 19-35.
9. Coleman, Lee, Robert Zimmerman, and Lucy Rorke. "Ventriculus
Terminalis of the Conus Medullaris: MR Findings in Children." American
Journal of Neuroradiology (1995): 1421-426. Print.
10. Liccardo, G., F. Ruggeri, L. De Cerchio, R. Floris, and P. Lunardi. "Fifth
Ventricle: An Unusual Cystic Lesion of the Conus Medullaris." Spinal
Cord 43.6 (2005): 381-84. Web.
 11. Trzesniak, Clarissa, Irismar R. Oliveira, Matthew J. Kempton, Amanda
Galvão-De Almeida, Marcos H.n. Chagas, Maria Cecília F. Ferrari, Alaor S.
Filho, Antonio W. Zuardi, Daniel A. Prado, Geraldo F. Busatto, Phillip K.
Mcguire, Jaime E.c. Hallak, and José Alexandre S. Crippa. "Are Cavum
Septum Pellucidum Abnormalities More Common in Schizophrenia
Spectrum Disorders? A Systematic Review and Meta-
analysis." Schizophrenia Research 125.1 (2011): 1-12. Web.
12. Enríquez, Goya, Flavia Correa, Javier Lucaya, Joaquim Piqueras, Celestino
Aso, and Aranzazu Ortega. "Potential Pitfalls in Cranial Sonography." Ped
Radiol Pediatric Radiology 33.2 (2003): 110-17. Web.
13. Levine, Deborah, Isabelle Trop, Tejas S. Mehta, and Patrick D. Barnes. "MR
Imaging Appearance of Fetal Cerebral Ventricular
Morphology1."Radiology 223.3 (2002): 652-60. Web.
14. Kristin Fickenscher, Zachary Bailey, Megan Saettele, Amy Dahl and Lisa
Lowe (2012). Pediatric Cranial Ultrasound: Techniques, Variants and
Pitfalls, Neuroimaging - Methods, Prof. Peter Bright (Ed.), ISBN: 978- 953-
51-0097-3, InTech
15. Osborn, Anne G., and Michael T. Preece. "Intracranial Cysts: Radiologic-
Pathologic Correlation and Imaging Approach 1." Radiology 239.3 (2006):
650-64. Web.
16. Ovalle, William K., Patrick C. Nahirney, and Frank H. Netter. "Chapter 5
Nervous Tissue." Netter's Essential Histology. Philadelphia, PA:
Elsevier/Saunders, 2013. N. pag. Print.
17. Ellenbogen, Richard G., Saleem I. Abdulrauf, and Laligam N. Sekhar.
Principles of Neurological Surgery. Philadelphia, PA: Saunders/Elsevier,
2012. Print.
18. Sakka, L., G. Coll, and J. Chazal. "Anatomy and Physiology of Cerebrospinal
Fluid." European Annals of Otorhinolaryngology, Head and Neck
Diseases 128.6 (2011): 309-16. Web.
19. Damkier, H. H., P. D. Brown, and J. Praetorius. "Cerebrospinal Fluid
Secretion by the Choroid Plexus." Physiological Reviews 93.4 (2013): 1847-
892. Web.

LEGENDS

Fig1. CSF flow study. Sagittal images of the CSF flow study (A,B) shows normal flow
in the prepontine cistern through the foramen magnum into the cervical canal.

Fig2. Normal anatomy and relationships of lateral ventricle. Midline sagittal T1 WI

image (A) shows corpus callosum (arrow) which forms the superior border of the
lateral ventricles, fornix (arrowhead) which forms the superior margin of the third
ventricle. T is thalamus. Axial T2 WI (B) shows frontal horns (arrowheads) and
trigone (spiral arrows) of the lateral ventricle lined laterally by caudate head (ch).
Lateral drain in the third ventricle (thick arrow) via foramen Monroe (thin arrow).
p=putamen, and t= thalamus. Axial T1WI slight superior to image B shows body of
the lateral ventricles (arrows) surrounded by body of the caudate nucleus
(arrowheads).

Fig3. III ventricle and its relationship. Axial T2 WI (A) images shows III ventricle in
the midline lined bilaterally by thalami (T). Anteriorly it is lined by crux of the fornix
and anterior commissure (thin white arrow) and posteriorly by posterior
commissure (black arrow). CH=caudate head, P=putamen, and GP= globus pallidus.
Sagittal T1 WI shows anterior commissure (arrowhead), posterior commissure
(spiral arrow) and pineal gland (thin arrow). Aqueduct lies between tegmentum (tg)
and tectal plate (arrow).

Fig4. Anatomy of the floor of the III ventricle. Magnified view of the sagittal T 2 WI
though midline shows mammillary body (black arrowhead), tuber cinerium (thin
white arrow), superior optic recess (spiral white arrow), infundibular recess (spiral
black arrow), optic chiasm (fat white arrow) and suprasellar cistern (curved white
arrow).

Fig5. Normal anatomy and relationships of IV ventricle. Sagittal T1 WI shows

CC=corpus callosum, T= thalamus, and aqueduct of Silvius (arrow) which drain into
the fourth ventricle (star). CSF space superior to the cerebellum is supracerebellar
cistern, anterior to the pons is prepontine cistern (fat arrow) and anterior to
medulla is medullary cistern (spiral arrow).

Fig6. Cranial ultrasound. Coronal images of the cranial ultrasonography through the
frontal horn of lateral ventricle (a), third ventricle (b) and the trigone (c). Image (A)
shows caudate head (small arrows) lateral to the frontal horn of the lateral ventricle
and corpus callosum superiorly (arrow). Note echogenic interhemispheric fissure
(arrowhead). Image (B) shows interhemispheric fissure (arrowheads) and third
ventricle (arrow) between thalami (T). Image C through the atria shows echogenic
choroid plexus (arrows). PL=parietal lobes.

Fig7. Cranial ultrasound. Sagittal images of the cranial ultrasonography through the
midline (A) shows lateral ventricles (LV), surrounded superiorly by corpus callosum
(arrow) and cingulate gyrus (arrowhead). P= pons, C= cerebellum, OL= occipital
lobe. Parasagittal sagittal image (B) shows lateral ventricle (LV), choroid plexus
(arrow) and caudo-thalamic groove (arrowhead). T= thalamus.

Fig8. Transverse DT imaging color maps at the level of lateral ventricles shows genu
of the corpus callosum (arrow), minor forceps of corpus callosum (curved arrows),
posterior limb of internal capsule (fat arrows), splenium of corpus callosum (double
arrows), major forceps of corpus callosum (spiral arrows) and optic radiation
(arrowheads).

Fig9. Coronal DT imaging color maps in patient with right periventricular neoplasm
shows corpus callosum (a), corticospinal tracts (b), cingulate gyrus (c), posterior
limb of the internal capsule (d), t=thalami. Infiltrating glioblastoma (star) is seen
infiltrating and causing destruction of corticospinal tracts and ependymal lining of
the right lateral ventricle (curved arrow).

Fig10. DTI maps superimposed on the functional image at the level of temporal
lobes shows Meyer loop (white arrow), optic radiation (spiral arrow) and visual
cortex (vc).

Fig11. Transverse DT imaging color maps of the posterior fossa. Section shows the
close relation of the white matter tracts with the fourth ventricle (a) with the middle
cerebellar peduncle (b), corticospinal tracts (c) and medial lemniscus (d), red fibers
between c and d is middle cerebellar peduncle.

Fig12. Asymmetric size of the lateral ventricle. Axial T1 (A) and T2 (B) WIs show
asymmetric dilatation of the right lateral ventricle as compared to the left. Rest of
the configuration of the ventricle is normal.

Fig13. Colpocephaly with agenesis of corpus callosum. Axial T2 WI (A) shows

bilateral dilation of the atria and occipital horns of the lateral ventricle. Sagittal T1
WI (B) shows complete agenesis of the corpus callosum.

Fig14. Absent septum pellucidum. Axial (A) and coronal (B) T2 WIs show complete
absence of the septum pellucidum (black arrow)

Fig15. Cavum Septum Pellucidum. Axial FLAIR (A) show well defined cystic filled
space between the medial wall of the lateral ventricles within the unfused septal
laminae.

Fig16. Cavum vergae. Sagittal T1 (A) and axial FLAIR (B) images show cystic lesion
posterior to the fornix bounded posteriorly by splenium of the corpus callosum
(arrow). Note is also made of cavum septum pellucidum on axial image
(arrowhead).

Fig17. Fifth ventricle in six month old child. Sagittal (A) and axial (B) T2 WIs show
intramedullary ovoid CSF signal intensity lesion at the level of the conus medullaris.

Fig18. Xanthogranulomas. Axial T2 (A), and FLAIR (B) MR images show well defined
hyperintense masses (arrows) in the choroid plexus bilaterally. These lesions how
mild restricted diffusion on the DWI (C). There is no evidence of hydrocephalus.