Hydrocephalus presentation

1. Hydrocephalus
Water on Head

2. A 9 year old boy was brought to his
GP by his parents who noted that
he was having difficulty with
balance and was complaining of
head aches.
Sunday, August 31, 2014 2

3. Warm UP

4. CSF is a clear fluid produced by dialysis of blood in
the choroid plexus.
Once produced, CSF is then circulated, due to
hydrostatic pressure, from the choroid plexus of
the lateral ventricles, through the inter-ventricular
foramina into the 3rd ventricle. CSF then flows
through the cerebral into the 4th ventricle. From
the 4th ventricle the CSF circulate in the
subarachnoid space in the brain and spinal cord

5. Large amounts of CSF are drained into venous
sinuses through arachnoid granulations in the
dorsal sagittal sinus. The dorsal sagittal sinus is
located between the folds of dura, known as the
falx cerebri, covering each of the cerebral
hemispheres.
Arachnoid granulations contain many villi that are
able to act as a one way valve helping to regulate
pressure within the CSF, and these arachnoid villi
push through the dura and into the venous
sinuses.

6.  The CSF volume, estimated to be about 150 ml in
adults, is distributed between 125 ml in cranial and
spinal subarachnoid spaces and 25 ml in the
ventricles, but with marked interindividual
variations.
 CSF secretion in adults is around 500 ml per day,
depending on the subject and the method used to
study CSF secretion.

9.  When cerebrospinal fluid pressure increases,
arachnoid villi develop, thereby increasing their
surface of exchange.
 Physiological values of CSF pressure vary
according to individuals and study methods
between 10 and 15mmHg in adults and 3 and
4 mmHg in infants. Higher values correspond to
intracranial hypertension.
CSF pressure varies with the systolic pulse wave,
respiratory cycle, abdominal pressure, jugular
venous pressure, state of arousal, physical
activity and posture.

11. Take off

12. Definition
 Hydrocephalus is a disorder in which an excessive
amount of cerebrospinal fluid (CSF) accumulates
within the cerebral ventricles and/orsubarachnoid
spaces, which are dilated [1,2].
 Aka Hydrodynamic disorder of CSF.
1-Fishman MA. Hydrocephalus. In: Neurological pathophysiology, Eliasson SG, Prensky AL, Hardin WB
(Eds), Oxford, New York 1978.
2-Carey CM, Tullous MW, Walker ML. Hydrocephalus: Etiology, Pathologic Effects, Diagnosis, and Natural
History. In: Pediatric Neurosurgery, 3 ed, Cheek WR (Ed), WB Saunders Company, Philadelphia 1994.

13. Epidemiology
 The prevalence of congenital and infantile
hydrocephalus in the United States and Europe has
been estimated as 0.5 to 0.8 per 1000 live and still
births.
 Mortality/Morbidity
 In untreated hydrocephalus, death may occur by
tonsillar herniation secondary to raised ICP with
compression of the brain stem and subsequent
respiratory arrest.

14.  Sex
Males = females.
EXCEPT in Bickers-Adams syndrome ,an X-linked
hydrocephalus transmitted by females and manifested in
males.
NPH has a slight male preponderance.
 Age
bimodal age curve. One peak occurs in infancy and is related
to the various forms of congenital malformations. Another
peak occurs in adulthood, mostly resulting from NPH. Adult
hydrocephalus represents approximately 40% of total cases
of hydrocephalus.

16. Hydrocephalus results from an imbalance between the
intracranial cerebrospinal fluid (CSF) inflow and
outflow.
It is caused by
1- obstruction of CSF circulation
2- by inadequate absorption of CSF
3- or (rarely) by overproduction of the CSF.
Regardless of the cause, the excessive volume of CSF
causes increased ventricular pressure and leads to
ventricular dilatation

17. Classification
A*
High pressure hydrocephalus
Normal pressure hydrocephalus
B*
Communicating HC = ventricular sys
communicating with SA space
Noncommunicating HC = blockage within the
ventricular system

18.  Normal pressure hydrocephalus (NPH) describes a
condition that rarely occurs in patients younger than 60 years.
Enlarged ventricles and normal CSF pressure at
lumbar puncture (LP) in the absence of papilledema led to the
term NPH.
 intermittent intracranial hypertension has been noted during
monitoring of patients in whom NPH is suspected, usually at
night.
 The classic Hakim triad of symptoms includes gait apraxia,
incontinence, and dementia. Headache is NOT a typical
symptom in NPH

19. NPH

20. Communicating hydrocephalus occurs when full
communication occurs between the ventricles and
subarachnoid space. It is caused by
1. defective absorption of CSF (most often).
2. venous drainage insufficiency (occasionally).
3. overproduction of CSF (rarely).
MCC : infection/ SAH.

22. Noncommunicating hydrocephalus occurs when CSF flow is
obstructed within the ventricular system or in its outlets to the
arachnoid space, resulting in impairment of the CSF from the
ventricular to the subarachnoid space.
The most common form: obstructive and is caused by
intraventricular or extraventricular mass-occupying lesions that
disrupt the ventricular anatomy.
Other causes:
 congenital malf. (Aqueduct stenosis).
 Inflammation.
 Hemorrhage.

23. Noncommunicating obstructive hydrocephalus caused by obstruction of
the foramina of Luschka and Magendie. This MRI sagittal image
demonstrates dilatation of lateral ventricles with stretching of corpus
callosumand dilatation of the fourth ventricle.

24. Noncommunicating obstructive hydrocephalus caused by
obstruction of foramina of Luschka and Magendie. This MRI
axial image demonstrates dilatation of the lateral ventricles.

25. Communicating versus non -communicating hydrocephalus
 Brain imaging can help to distinguish obstructive (non-communicating)
from absorptive (communicating)
hydrocephalus.
 The site of obstructed CSF flow may be suggested by the
pattern of ventricular dilatation.
Stenosis of the aqueduct (a common type of
obstructive hydrocephalus) typically results in dilated
lateral and third ventricles and in a fourth
ventricle of normal size.

26.  In contrast, communicating hydrocephalus (eg, caused
by either extraventricular obstruction or by impaired CSF
absorption) in neonates and infants usually results in
symmetric dilatation of all four ventricles.
 If extraventricular obstruction or impaired CSF
absorption occurs in children and adults, it may cause
benign intracranial hypertension (pseudotumor cerebri)
without ventricular dilatation, because of reduced
compliance of the brain tissue

27. LOOK !!
These forms of hydrocephalus are distinct from two radiographic
findings that include the same word.
The term “hydrocephalus ex-vacuo” refers to dilatation of
the ventricles secondary to brain atrophy or loss of brain tissue
secondary to an insult; hydrocephalus ex-vacuo is not
accompanied by increased ICP.

28. The term “external
hydrocephalus” or “benign
enlargement of the extra-axial spaces”
refers to excessive fluid, usually CSF, in
the subarachnoid spaces
 associated with familial macrocephaly
 Seen in infant and early children.
 The ventricles usually are not enlarged
significantly
 resolution within 1 year is the rule.

29. Hydrocephalus can be congenital or acquired. Both
categories include a diverse group of conditions.

30. Congenital hydrocephalus :
 applies to the ventriculomegaly that develops
in the fetal and infancy periods
 often associated with macrocephaly.
 The most common causes of congenital hydrocephalus are
obstruction of the cerebral aqueduct flow, Arnold-Chiari
malformation or Dandy–Walker malformation.
 these patients may stabilize in later years due to compensatory
mechanisms but may decompensate, especially following minor
head injuries.

31. Infants : S & S
Signs :
 Head enlargement: Head
circumference is at or above the
98th percentile for age.
 Dysjunction of sutures: This can
be seen or palpated.
 Dilated scalp veins: The scalp is
thin and shiny with easily visible veins.
 Tense fontanelle: The anterior
fontanelle in infants who are held erect
and are not crying may be excessively
tense.
 Setting-sun sign: In infants, it is
characteristic of increased intracranial
pressure (ICP). Ocular globes are deviated
downward, the upper lids are retracted,
and the white sclerae may be visible above
the iris.
 Increased limb tone: Spasticity
preferentially affects the lower limbs. The
cause is stretching of the periventricular
pyramidal tract fibers by hydrocephalus.
Symptoms :
 Poor feeding
 Irritability
 Reduced activity
 Vomiting

32. Setting-sun sign

33. Adults : S & S
Symptoms:
 Cognitive deterioration: This can be confused with other types of dementia in the elderly.
 Headaches: more prominent in the morning because cerebrospinal fluid (CSF) is resorbed less
efficiently in the recumbent position. This can be relieved by sitting up. As the condition
progresses, headaches become severe and continuous. Headache is rarely if ever present in
normal pressure hydrocephalus (NPH).
 Neck pain: If present, neck pain may indicate protrusion of cerebellar tonsils into the foramen
magnum.
 Nausea that is not exacerbated by head movements
 Vomiting: Sometimes explosive, vomiting is more significant in the morning.
 Blurred vision (and episodes of "graying out"): These may suggest serious optic nerve compromise, which
should be treated as an emergency.
 Double vision (horizontal diplopia) from sixth nerve palsy
 Difficulty in walking
 Drowsiness
 Incontinence (urinary first, fecal later if condition remains untreated): This indicates significant destruction
of frontal lobes and advanced disease.

34. Signs :
 Papilledema: If raised ICP is not treated, it leads to optic atrophy.
 Failure of upward gaze and of accommodation indicates pressure on
the tectal plate. The full Parinaud syndrome* is rare.
* Parinaud's Syndrome, also known as dorsal midbrain syndrome is a group of
abnormalities of eye movement and pupil dysfunction. It is caused by lesions of the
upper brain stem and is named for Henri Parinaud(1844–1905), considered to be the
father of French ophthalmology
 Unsteady gait is related to truncal and limb ataxia. Spasticity in legs also
causes gait difficulty.
 Large head: The head may have been large since childhood
 Unilateral or bilateral sixth nerve palsy is secondary to increased ICP.

35. NPH : S & S
 Gait disturbance is usually the first symptom and may precede
other symptoms by months or years. Magnetic gait is used to
emphasize the tendency of the feet to remain "stuck to the floor"
despite patients’ best efforts to move them.
 Dementia should be a late finding in pure (shunt-responsive) NPH.
It presents as an impairment of recent memory or as a "slowing of
thinking." Spontaneity and initiative are decreased. The degree can
vary from patient to patient.
 Urinary incontinence may present as urgency, frequency, or a
diminished awareness of the need to urinate.
 Other symptoms that can occur include personality changes and
Parkinsonism. Seizures are extremely rare and should prompt
consideration for an alternative diagnosis.

36. Signs :
 Muscle strength is usually normal.
 NO SENSORY LOSS IS NOTED.
 Reflexes may be increased, and the Babinski response may be found
in one or both feet. These findings should prompt search for vascular
risk factors (causing associated brain microangiopathy or vascular
Parkinsonism), which are common in NPH patients.
 Difficulty in walking varies from mild imbalance to inability to walk
or to stand. The classic gait impairment consists of short steps, wide
base, externally rotated feet, and lack of festinating (hastening of
cadence with progressively shortening stride length, a hallmark of the
gait impairment of Parkinson disease). These abnormalities may
progress to the point of apraxia. Patients may not know how to take
steps despite preservation of other learned motor tasks.
 Frontal release signs such as sucking and grasping reflexes appear in
late stages.

37.  Congenital causes in infants and children:
 Brainstem malformation causing stenosis of the aqueduct of Sylvius: This is
responsible for 10% of all cases of hydrocephalus in newborns.
 Dandy-Walker malformation: This affects 2-4% of newborns with
hydrocephalus.
 Arnold-Chiari malformation type 1 and type 2
 Agenesis of the foramen of Monro
 Congenital toxoplasmosis
 Bickers-Adams syndrome: This is an X-linked hydrocephalus accounting for
7% of cases in males. It is characterized by stenosis of the aqueduct of
Sylvius, severe mental retardation, and in 50% by an adduction-flexion
deformity of the thumb.

38.  Acquired causes in infants and children:
 Mass lesions account for 20% of all cases of hydrocephalus in
children. These are usually tumors
(eg, medulloblastoma, astrocytoma), but cysts, abscesses, or
hematoma also can be the cause.
 Haemorrhage: Intraventricular hemorrhage can be related
to prematurity, head injury, or rupture of a vascular malformation.
 Infections: Meningitis (especially bacterial) and, in some geographic
areas, cysticercosis can cause hydrocephalus.
 Increased venous sinus pressure: This can be related to
achondroplasia, some craniostenoses, or venous thrombosis.
 Iatrogenic: Hypervitaminosis A, by increasing secretion of CSF or by
increasing permeability of the blood-brain barrier, can lead to
hydrocephalus. As a caveat, hypervitaminosis A is a more common
cause of idiopathic intracranial hypertension, a disorder with
increased CSF pressure but small rather than large ventricles.
 Idiopathic

39.  Causes of hydrocephalus in adults:
 Subarachnoid hemorrhage (SAH) causes one third of these cases by
blocking the arachnoid villi and limiting resorption of CSF. However,
communication between ventricles and subarachnoid space is preserved.
 Idiopathic hydrocephalus represents one third of cases of adult
hydrocephalus.
 Tumors can cause blockage anywhere along the CSF pathways. The most
frequent tumors associated with hydrocephalus are ependymoma,
subependymal giant cell astrocytoma, choroid plexus papilloma,
craniopharyngioma, pituitary adenoma, hypothalamic or optic nerve
glioma, hamartoma, and metastatic tumors.
 Head injury, through the same mechanism as SAH, can result in
hydrocephalus.

40.  Prior posterior fossa surgery may cause hydrocephalus by blocking
normal pathways of CSF flow.
 Meningitis, especially bacterial, may cause hydrocephalus in adults.
 All causes of hydrocephalus described in infants and children are present in
adults who have had congenital or childhood-acquired hydrocephalus.

41. Causes of NPH
 Most cases are idiopathic and are probably
related to a deficiency of arachnoids granulations:
 SAH
 Head trauma
 Meningitis

42. Differential diagnosis
 Brainstem Gliomas
 Childhood Migraine Variants
 Craniopharyngioma
 Epidural Hematoma
 Frontal and Temporal Lobe
Dementia
 Frontal Lobe Epilepsy
 Frontal Lobe Syndromes
 Glioblastoma Multiforme
 Intracranial Epidural Abscess
 Intracranial Hemorrhage
 Meningioma
 Mental Retardation
 Migraine Headache
 Migraine Variants
 Oligodendroglioma
 Pituitary Tumors
 Primary CNS Lymphoma
 Pseudotumor Cerebri
 Pseudotumor Cerebri: Pediatric
Perspective
 Subdural Empyema
 Subdural Hematoma

43. Imaging studies
CT scan:
 Advantages:
fast
reliable
does not interfere with implanted medical devices
 Disadvantage :
radiation exposure

44.  U/S:
 In a newborn, ultrasonography is the preferred technique for
the initial examination because it is portable and avoids
ionizing radiation.
 Ultrasound is good for imaging the lateral ventricles but does
not assess the posterior fossa well
-to assess ventricular size
 ( through the open fontanelle).

45.  MRI:
MRI is generally the imaging modality of choice in
patients with unexplained hydrocephalus

46. Procedures
 Lumbar puncture (LP)
 is a valuable test in evaluating NPH.
 only in communicating
 should be performed only after CT or MRI of the head.
 Normal LP opening pressure (OP) should be less than 180 mm
H2 O (ie, 18 cm H2O). Patients with initial OP greater than 100
mm H2 O have a higher rate of response to CSF shunting than
those with OPs less than 100 mm H2O.
 Continuous CSF drainage through external lumbar drainage
(ELD) is a highly accurate test for predicting the outcome after
ventricular shunting in NPH, although false negative results are
not uncommon.
 EEG if the patient has seizures .

48. Management
MEDICAL :
1-drugs (Diuretics and fibrinolytics) :They have been used for short periods in
slowly progressive hydrocephalus in patients too unstable for surgery
2- Repeat lumbar punctures (LPs):can be performed for cases
of hydrocephalus after intraventricular hemorrhage, since this
condition can resolve spontaneously. If reabsorption does not resume
when the protein content of cerebrospinal fluid (CSF) is less than 100
mg/dL, spontaneous resorption is unlikely to occur.
LP can be performed only in cases of communicating
hydrocephalus.

49. Surgical
Shunting :
allows CSF to flow from the ventricles into the systemic
circulation or to the peritoneum where it is absorbed,
bypassing the site of mechanical or functional obstruction
to absorption.

50. Types of shunting
 Ventriculo-peritoneal
shunting (VP shunting):
 Most commonly used.
 It is preferred for growing children
and most adults.
 Lateral ventricle is the usual
proximal location.
 Advantages:
 The ability of the peritoneal cavity to
accept a large loop of tubing and to
accommodate the axial growth.
 Specific complications:
 Inguinal hernia in > l5%
 Hydrocele
 Peritonitis.
 Volvulus
 Peritoneal cyst
 Intestinal obstruction.

51. 2. Ventriculo-atrial shunting (VA shunting)
3. Lumbo-peritoneal shunting (LP shunting)
VP shunt VA shunt

52. Contraindications:
 Active ventriculitis.
 Fresh ventricular hemorrhage
 Active systemic infection.
 Open meningeocele (source of infection if not
closed)

53. Complication of shunt
o Infections:
 2-3 months post-op
 Staph. Epidermis and Staph.
Aureus.
o Obstruction of the catheter
o Intracerebral /subdural
Hemorrhage
o Over shunting (VP shunts)
o Misplacement
o Seizures
 Approximately 40percent
of standard shunts
malfunction within the first
year after placement

54. Third ventriculostomy — Endoscopic third ventriculostomy
(ETV) is a procedure in which a perforation is made to
connect the third ventricle to the subarachnoid space.
 This has been used in the initial treatment of selected cases
of obstructive hydrocephalus and as an alternative to shunt
revision. Some experts consider it the treatment of choice for
aqueductal stenosis, although about 20 percent of patients
still require shunting.
 ETV is not useful for patients with communicating
hydrocephalus. The success of the procedure depends upon
the cause of hydrocephalus and upon previous complications
.
 When successful, ETV provides a treatment for
hydrocephalus that is relatively low-cost and durable.

56. Complications of third ventriculostomy
 are mainly perioperative
 and include inability to complete the procedure,
hemorrhage, hypothalamic dysfunction (diabetes
insipidus, syndrome of inappropriate antidiuretic hormone
secretion, or precocious puberty), meningitis, and cerebral
infarction.
 In a systematic review, permanent morbidity after the
procedure was 2.1 percent, and mortality was 0.22 percent

57. Complications of hydrocephalus
 Related to progression of hydrocephalusVisual changes
 Occlusion of posterior cerebral arteries secondary to downward
transtentorial herniation
 Chronic papilledema injuring the optic disc
 Dilatation of the third ventricle with compression of optic chiasm
 Cognitive dysfunction
 Incontinence
 Gait changes
 Related to medical treatmentElectrolyte imbalance
 Metabolic acidosis

58. Prognosis
 Long-term outcome is related directly to the cause of hydrocephalus.
 Survival in untreated hydrocephalus is poor. Approximately 50 percent of
affected patients die before three years of age, and 77 to 80 percent die
before reaching adulthood [27].
 Treatment markedly improves the outcome for hydrocephalus not
associated with tumor
 Functional outcome depends upon factors including :
degree of prematurity
central nervous system (CNS) malformations
other congenital abnormalities
and epilepsy, as well as sensory and motor impairments

59. A 9 year old boy was brought to his GP by his parents who noted
that he was having difficulty with his balance and was
complaining of head aches.
 He was referred to the neurosurgical unit and a CT
scan was perform of his head region.
 This showed the presence of dilated lateral and III
ventricles with a normal IV ventricle. There was
effacement of the overlying cortical sulci in the brain
and a diagnosis of Hydrocephalus was made.
 MRI showed the cerebral aqueduct was stenosed.
 The boy subsequently had a III ventriculostomy
performed, and his symptoms resolved rapidly.
Sunday, August 31, 2014 59

60. Reference:
 http://www.uptodate.com/contents/search?sp=0&source=USER
_PREF&search=Hydrocephalus&searchType=PLAIN_TEXT
 http://emedicine.medscape.com/article/1135286-overview
 http://www.sciencedirect.com/science/article/pii/S18797296110
01013

61. جزاكم الله خيرا