Hydrocephalus

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Last Updated: March 14, 2002

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Synonyms and related keywords: abnormal rise in cerebrospinal fluid volume, abnormal rise in
cerebrospinal fluid pressure, CSF, imbalance of cerebrospinal fluid production and absorption,
spinal bifida, congenital hydrocephalus, acquired hydrocephalus, intracranial tumor obstruction,
intracranial trauma, intracranial hemorrhage, intracranial infection, disorders of cerebrospinal fluid
production, disorders of cerebrospinal fluid circulation, disorders of cerebrospinal fluid absorption,
cerebrospinal fluid diversion, third ventriculostomy

AUTHOR INFORMATION

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Author: Kamran Sahrakar, MD, Clinical Professor, Department of Neurosurgery,

University of California-Davis
Coauthor(s): Dachling Pang, MD, Chief of Pediatric Neurosurgery, Kaiser
Permanente Hospitals; Clinical Professor, Department of Neurosurgery, University
of California at Davis
Kamran Sahrakar, MD, is a member of the following medical societies: Alpha
Omega Alpha, American Association of Neurological Surgeons, American Medical
Association, California Medical Association, Florida Medical Association, and
Nevada State Medical Association
Editor(s): Duc Hoang Duong, MD, Director of Neuroscience Physician Assistant
Program, Associate Professor, Departments of Neurological Surgery and
Neuroscience, Epilepsy Center, Charles R Drew University; Francisco Talavera,
PharmD, PhD, Senior Pharmacy Editor, Pharmacy, eMedicine; Ryszard M Pluta,
MD, PhD, Associate Professor, Neurosurgical Department Medical Research
Center, Polish Academy of Sciences at Warsaw, Poland; Senior Researcher,
Surgical Neurology Branch, National Institute of Neurological Disorders and
Stroke, NIH; Herbert H Engelhard, MD, PhD, Chief, Division of Neuro-Oncology,
Associate Professor, Departments of Neurosurgery and Molecular Genetics,
University of Illinois at Chicago; and Michael Dogali, MD, CM, Professor,
Department of Neurological Surgery, University of Southern California School of
Medicine
INTRODUCTION

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History of the Procedure: Hydrocephalus was first described by Hippocrates.

Hydrocephalus was not treated effectively until the 20th century, when the
development of appropriate shunting materials and techniques occurred. Today,
the focus of hydrocephalus research is on pathophysiology, valve design in
shunting, and minimally invasive techniques of treatment.

Problem: Hydrocephalus is the abnormal rise in cerebrospinal fluid volume and,

usually, pressure, that results from an imbalance of cerebrospinal fluid production
and absorption.
Frequency: The overall incidence of hydrocephalus is unknown. When cases of
spina bifida are included, congenital hydrocephalus occurs in 2-5 births per 1000
births. Incidence of acquired types of hydrocephalus is unknown.
Etiology: The etiology of hydrocephalus in congenital cases is unknown. Very
few cases (<2%) are inherited (X-linked hydrocephalus). The most common
causes of hydrocephalus in acquired cases are tumor obstruction, trauma,
intracranial hemorrhage, and infection.
Pathophysiology: Hydrocephalus can be subdivided into 3 forms.

Disorders of cerebrospinal fluid production: This is the rarest form of

hydrocephalus. Choroid plexus papillomas and choroid plexus carcinomas
can secrete cerebrospinal fluid in excess of its absorption.
Disorders of cerebrospinal fluid circulation: This form of hydrocephalus
results from obstruction of the pathways of cerebrospinal fluid circulation.
This can occur at the ventricles or arachnoid villi. Tumors, hemorrhages,
congenital malformations, and infections can cause obstruction at either
point in the pathways.
Disorders of cerebrospinal fluid absorption: Conditions, such as the
superior vena cava syndrome and sinus thrombosis, can interfere with
cerebrospinal fluid absorption. Some forms of hydrocephalus cannot be
classified clearly. This group includes normal pressure hydrocephalus and
pseudotumor cerebri.

Clinical: The various types of hydrocephalus can present differently in different

age groups.
Acute hydrocephalus typically presents with headache, gait disturbance,
vomiting, and visual changes. In infants, irritability or poor head control can be
early signs of hydrocephalus. When the third ventricle dilates, the patient can
present with Parinaud syndrome (upgaze palsy with a normal vertical Doll
response) or the setting sun sign (Parinaud syndrome with lid retraction and
increased tonic downgaze). Occasionally, a focal deficit, such as sixth nerve
palsy, can be the presenting sign. Papilledema often is present, although it may
lag behind symptomatology. Infants present with bulging fontanelles, dilated
scalp veins, and an increasing head circumference. When advanced,
hydrocephalus presents with brainstem signs, coma, and hemodynamic
instability.

Normal pressure hydrocephalus has very distinct symptomatology. The patient is

older and presents with progressive gait apraxia, incontinence, and dementia.
This triad of symptoms defines normal pressure hydrocephalus.
INDICATIONS

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Most cases of symptomatic hydrocephalus need to be treated before permanent

neurologic deficits result or neurologic deficits progress.
When an etiologic factor is known, hydrocephalus can be treated with temporary
measures while the underlying condition is treated. Examples of temporary
treatment measures are ventriculostomy until a posterior fossa tumor is resected
or lumbar punctures in a neonate with intraventricular hemorrhage until the blood
is absorbed and normal cerebrospinal fluid absorption resumes.
RELEVANT ANATOMY AND
CONTRAINDICATIONS

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Relevant Anatomy: See Intraoperative details for a discussion of relevant

anatomy.
Contraindications: Few cases of hydrocephalus should not be treated. Cases in
which treatment should not be implemented include the following:

The patient in whom a successful surgery would not affect the outcome
(eg, a child with hydranencephaly)
In ventriculomegaly of senescence, the patient who does not have the
symptom triad
Ex vacuo hydrocephalus is merely the replacement of lost cerebral tissue
with cerebrospinal fluid. Because no imbalance in fluid production and
absorption exists, this technically is not hydrocephalus.
Arrested hydrocephalus is defined as a rare condition in which the
neurologic status of the patient is stable in the presence of stable
ventriculomegaly. The diagnosis must be made extremely carefully
because children can present with very subtle neurological deterioration
(eg, slipping school performance) that is difficult to document.
Benign hydrocephalus of infancy is found in neonates and young infants.
The children are asymptomatic, and head growth is normal. CT scan
shows mildly enlarged ventricles and subarachnoid spaces.

Imaging Studies:

CT scan of the head delineates the degree of ventriculomegaly and, in

many cases, the etiology. When performed with contrast, it can show
infection and tumors causing obstruction. It also helps with operative
planning. Ventricles usually are dilated proximal to the point of obstruction.
In pseudotumor cerebri, the CT scan findings usually are normal.

Perform MRI scan of head in most, if not all, congenital cases of

hydrocephalus. This delineates the extent of associated brain anomalies
such as corpus callosum agenesis, Chiari malformations, disorders of
neuronal migration, and vascular malformations. Some tumors, for
example the midbrain tectal gliomas, only can be detected with this study.
T2-weighted images can show transependymal flow of cerebrospinal fluid.

Fetal and neonatal cranial ultrasound is a good study for monitoring

ventricular size and intraventricular hemorrhage in the neonatal ICU
setting. Certainly, prior to treatment, perform other imaging studies.

Diagnostic Procedures:

Lumbar puncture can be used to measure intracranial pressure, but it

should only be performed after imaging studies rule out an obstruction. A
diagnostic high-volume lumbar puncture in normal pressure
hydrocephalus can assist in making decisions regarding shunting. Spinal
fluid can show the type and severity of infection (see Meningitis).

TREATMENT

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Medical therapy: Medical therapy usually is a temporizing measure. In transient

conditions, such as sinus occlusion, meningitis, or neonatal intraventricular
hemorrhage, medical therapy can be effective.

Acetazolamide (25 mg/kg/d in 3 doses): Careful monitoring of respiratory

status and electrolytes is crucial. Treatment beyond 6 months is not
recommended.
Furosemide (1 mg/kg/d in 3 doses): Again, electrolyte balance and fluid
balance need to be monitored carefully.
Lumbar punctures: In neonates recovering from intraventricular
hemorrhage, serial lumbar punctures can resolve hydrocephalus in some
cases. If possible, this is the preferred method of treatment.
Removal of the underlying cause resolves hydrocephalus in most cases.

Surgical therapy: As performance of cerebrospinal fluid diversion has increased

in frequency, so has awareness of pitfalls of the procedure. Recently, a
resurgence of interest in third ventriculostomies has occurred.
Preoperative details: Make every effort to identify the cause of hydrocephalus
prior to considering a diversion procedure. Make every effort to treat
hydrocephalus medically.
Do not consider diversion in patients with infection or high cerebrospinal fluid
protein (>150 mg/dL).
Obtain some idea of brain compliance in order to select the optimum valve
pressure and decide if the pressure-programmable valve should be used.
Use one dose of preoperative prophylactic antibiotics.
Intraoperative details:

Third ventriculostomy: Reserve this procedure for obstructive cases in

patients who have normal or near normal spinal fluid absorptive capacity.
Use a blunt instrument to penetrate the floor of the third ventricle. Sharp
instruments or lasers can cause vascular injury. Leaving a clamped drain
in place postoperatively might be prudent. The burr hole placed on the
coronal suture allows a straight trajectory to the foramen of Monro.
Stereotactic guidance is not needed if endoscopic techniques are used.
Ventriculoperitoneal shunting: This procedure is by far the most common
procedure for cerebrospinal fluid diversion. The abdomen should be able
to absorb fluid. The ventricular catheter can be placed more reliably from
the coronal approach. Some surgeons still prefer parietooccipital
catheters. The proximal catheter tip should lie anterior to the choroid
plexus in the frontal horn of the lateral ventricle.
Ventriculoatrial shunting: This procedure usually is the first choice for
patients who are unable to have abdominal distal catheters (eg, multiple
operations, recent abdominal sepsis, known malabsorptive peritoneal
cavity). The procedure carries more risk. Long-term complications are
more serious (eg, renal failure, great vein thrombosis). Fluoroscopic
guidance is necessary to prevent catheter thrombosis (short distal
catheter) or cardiac arrhythmias (long distal catheter).
Ventriculopleural shunting: Reserve this procedure for patients with failed
peritoneal and atrial shunts.
Torkildsen shunts or internal shunts are straight tubes that communicate to
cerebrospinal fluid spaces without a valve. Their effectiveness and longterm efficacy are not proven.
Lumboperitoneal shunts are used in communicating hydrocephalus,
especially if ventricles are small. Pseudotumor cerebri is the classical
indication of this method of shunting.

Postoperative details: ICU observation after third ventriculostomy is advised.

In patients with high brain compliance, gradual assumption of the upright position
and slow mobilization may reduce the incidence of early subdural hematoma
formation.
Plain radiographs of the entire hardware system confirm good position and serve
as excellent baseline studies for the future.
Wounds should remain dry for at least 3 days postoperatively, until
epithelialization has occurred.
In patients with pleural shunts, perform an early postoperative chest radiograph
to ensure adequate absorption of fluid. Large effusions can occur in short
periods, and respiratory problems can ensue.
Follow-up care:

Remove stitches by 2 weeks postsurgery.

Perform CT scan for baseline at 2-4 weeks postsurgery.
Monitor all children with shunts every 6-12 months. Carefully monitor head
growth in infants. Check distal tubing length with plain radiographs when
the child grows. Appropriate specialists should carefully assess child
development.
What happens to ventricular size in patients who have a third
ventriculostomy or Torkildsen shunt is not known. Other methods of
assessment of patency need to be used, such as MRI flow studies and
clinical evaluations (eg, detailed funduscopic examinations).
In patients with pseudotumor cerebri, visual acuity and fields should be
monitored by the appropriate specialist.

COMPLICATIONS

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The most common complications differ depending on the type of shunt and the
underlying pathophysiology.
Infection is the most feared complication in the young age group. The
overwhelming majority of infections occur within 6 months of the original
procedure. Common infections are staphylococcal and propionibacterial. Early
infections occur more frequently in neonates and are associated with more
virulent bacteria such as Escherichia coli. Infected shunts need to be removed,
the cerebrospinal fluid needs to be sterilized, and a new shunt needs to be
placed. Treatment of infected shunts with antibiotics alone is not recommended

because bacteria can be suppressed for extended periods and bacteria can
resurface once antibiotics are stopped.
Subdural hematomas occur almost exclusively in adults and children with
completed head growth. Incidence of subdural hematomas can be reduced by
slow postoperative mobilization. This allows for brain compliance reduction. The
treatment is drainage and may require temporary occlusion of the shunt.
Shunt failure is mostly due to suboptimal proximal catheter placement.
Occasionally, distal catheters fail. Suspect infection if the distal catheter is
obstructed with debris. Abdominal pseudocysts are synonymous with low-grade
shunt infection.
Overdrainage is more common in lumboperitoneal shunts and manifests with
headaches in the upright position. In most cases, overdrainage is a self-limiting
process. However, revision to a higher-pressure valve or a different shunt system
occasionally may be necessary.
Slit ventricle syndrome is an extremely rare condition in which brain compliance
is unusually low. It mostly occurs in the setting of prior ventriculitis or shunt
infection. The patient may develop high pressures without ventricular dilatation.
The slit ventricle syndrome does not imply overdrainage, and the symptoms
usually are those of high pressure rather than low pressure. Most experts also
agree that slit ventricles predispose the patient to a higher incidence of
ventricular catheter failure. Repeated ventricular blockage by the coapted
ventricular wall may be helped by performing a subtemporal decompression that
creates an artificial pressure reservoir and induces slight reenlargement of the slit
ventricle.
OUTCOME AND PROGNOSIS

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In general, outcome is good. A typical patient should return to baseline after

shunting. The neurologic function of children is optimized with shunting.
The best long-term results in the most carefully selected patients are no better
than 60% in normal pressure hydrocephalus. Few complete recoveries occur.
Often, gait and incontinence respond to shunting, but dementia responds less
frequently.
Often, various other neurologic abnormalities associated with hydrocephalus are
the limiting factor in patient recovery. Examples are migrational abnormalities and
postinfectious hydrocephalus.

FUTURE AND CONTROVERSIES

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Hydrocephalus research and treatment have advanced tremendously in the last

20 years. Examples are the development of new shunt materials and, more
recently, programmable valve technology. Current research categories include
the following:

Transplantation of tissue, such as vascularized omentum, to reestablish

normal cerebrospinal fluid could be the best method to treat
communicating hydrocephalus.
Third ventriculostomies and aqueductoplasty eliminate the need for
shunting in noncommunicating cases of hydrocephalus. New optics and
smaller scopes have expanded this field over the last 5 years.

BIBLIOGRAPHY

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Author Information Introduction Indications Relevant Anatomy And Contraindications Workup Treatment Complications
Outcome And Prognosis Future And Controversies Bibliography

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Neurological Surgery. 3rd ed. Philadelphia, Pa: WB Saunders Co; 1990:
927-944.
Gleason PL, Black PM, Matsumae M: The neurobiology of normal
pressure hydrocephalus. Neurosurg Clin N Am 1993 Oct; 4(4): 66775[Medline].
McLone DG, Partington MD: Arrest and compensation of hydrocephalus.
Neurosurg Clin N Am 1993 Oct; 4(4): 621-4[Medline].
Milhorat T: Hydrocephalus: Pathophysiology and Clinical Features.
Neurosurgery 1996; 3: 3625-3632.
Pang D, Altschuler E: Low-pressure hydrocephalic state and viscoelastic
alterations in the brain. Neurosurgery 1994 Oct; 35(4): 643-55; discussion
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Sainte-Rose C: Hydrocephalus in childhood. In: Youmans JR, ed.
Neurological Surgery. Philadelphia, Pa: WB Saunders Co; 1996: 890-926.