More information on Hydrocephalus

Reference: "Hydrocephalus" by Eugenia-Daniela Hord, MD, Instructor, Departments of Anesthesia and Neurology, Massachusetts General Hospital Pain Center, Harvard Medical School

Pathophysiology

Normal CSF production is 0.20-0.35 mL/min; a majority is produced by the choroid plexus, which is located within the ventricular system, mainly the lateral and fourth ventricles. The capacity of the lateral and third ventricles in a healthy person is 20 mL. Total volume of CSF in an adult is 120 mL.

Normal route of CSF from production to clearance is the following: From the choroid plexus, the CSF flows to the lateral ventricle, then to the interventricular foramen of Monro, the third ventricle, the cerebral aqueduct of Sylvius, the fourth ventricle, the 2 lateral foramina of Luschka and 1 medial foramen of Magendie, the subarachnoid space, the arachnoid granulations, the dural sinus, and finally into the venous drainage.

ICP rises if production of CSF exceeds absorption. This occurs if CSF is overproduced, resistance to CSF flow is increased, or venous sinus pressure is increased. CSF production falls as ICP rises. Compensation may occur through transventricular absorption of CSF and also by absorption along nerve root sleeves. Temporal and frontal horns dilate first, often asymmetrically. This may result in elevation of the corpus callosum, stretching or perforation of the septum pellucidum, thinning of the cerebral mantle, or enlargement of the third ventricle downward into the pituitary fossa (which may cause pituitary dysfunction).

The mechanism of NPH has not been elucidated completely. Current theories include increased resistance to flow of CSF within the ventricular system or subarachnoid villi; intermittently elevated CSF pressure, usually at night; and ventricular enlargement caused by an initial rise in CSF pressure; the enlargement is maintained despite normal pressure because of the Laplace law. Although pressure is normal, the enlarged ventricular area reflects increased force on the ventricular wall.

Frequency

United States

Incidence of congenital hydrocephalus is 3 per 1,000 live births, while the incidence of acquired hydrocephalus is not known exactly.

International

Incidence of acquired hydrocephalus is unknown. About 100,000 shunts are implanted each year in the developed countries, but little information is available for other countries.

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.

• Shunt dependence occurs in 75% of all cases of treated hydrocephalus and in 50% of children with communicating hydrocephalus.
• Patients are hospitalized for scheduled shunt revisions or for treatment of shunt complications or shunt failure.
• Poor development of cognitive function in infants and children, or loss of cognitive function in adults, can complicate untreated hydrocephalus. It may persist after treatment.
• Visual loss can complicate untreated hydrocephalus and may persist after treatment.

Sex

Generally, incidence is equal in males and females. The exception is Bickers-Adams syndrome, an X-linked hydrocephalus transmitted by females and manifested in males. NPH has a slight male preponderance.

Age

Incidence of human hydrocephalus presents a 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.

More on Hydrocephalus

Common Causes of Hydrocephalus

Although rare, hydrocephalus can be inherited genetically or may be associated with developmental disorders, including spina bifida (congenital defect of the spine) and encephalocele (hernia of the brain). Other causes can include bleeding within the brain, brain tumors, head injuries, complications of premature birth such as hemorrhage, or diseases such as meningitis or other infections. In some cases, normal flow of CSF within the brain is blocked, resulting in fluid build-up.

Symptoms of hydrocephalus vary greatly from person to person. According to the Hydrocephalus Association, some of the most common symptoms are listed below as a reference.

Symptoms of Hydrocephalus in infants Abnormal enlargement of the head; soft spot (fontanel) is tense and bulging; scalp can appear thin; bones separated in baby's head; prominent scalp veins; vomiting; drowsiness; irritability; downward deviation of baby's eyes; seizures; or poor appetite.

Symptoms of Hydrocephalus in toddlers/children
Abnormal enlargement of baby's head; headache; nausea; vomiting; fever; blurred or double vision; unstable balance; irritability; sleepiness; delayed progress in walking or talking; poor coordination; change in personality; inability to concentrate; loss of sensory motor functions; seizures; or poor appetite. Older children may experience difficulty in remaining awake or waking up.

Symptoms of Hydrocephalus in young and middle-aged adults
Headache; difficulty in remaining awake or waking up; loss of coordination or balance; bladder control problems; impaired vision and cognitive skills that may affect job performance and personal skills.

Symptoms of Hydrocephalus in older adults
Loss of coordination or balance; shuffling gait, memory loss; headache; or bladder control problems. Hydrocephalus is often categorized for age groups as either congenital or normal pressure hydrocephalus. Congenital hydrocephalus refers to conditions that are caused by conditions existing at birth. Primary symptoms include headache, nausea, vomiting and drowsiness. Normal pressure hydrocephalus (NPH) is the accumulation of cerebrospinal fluid that causes the ventricles in the brain to become enlarged, with little or no increase in pressure. Adult-onset NPH mainly occurs in adults age 60 and older. Patients with NPH often get misdiagnosed with Alzheimer's disease or dementia, as some of the symptoms mimic these two conditions.

Diagnosing Hydrocephalus

Before your doctor can recommend a course of treatment, he or she will:

• Review your medical history, and perform a physical examination
• Perform a complete neurological examination including diagnostic testing if needed
• Ask specific questions to determine if symptoms are caused by hydrocephalus

The neurological examination will also help to determine the severity of your condition. Further tests such as an ultrasound (if the patient is an infant), computed tomography (CT or CAT scan), or magnetic resonance imaging (MRI) may be ordered. The tests may reveal useful information about the severity of the condition and its likely cause.

When Surgery is Necessary

Hydrocephalus can be treated in a variety of ways. The problem area may be treated directly (by removing the cause of CSF obstruction), or indirectly (by diverting the fluid to somewhere else; typically to another body cavity). Indirect treatment is performed by implanting a device known as a shunt to divert the excess CSF away from the brain. The body cavity in which the CSF is diverted is usually the peritoneal cavity (the area surrounding the abdominal organs).

In some cases, two procedures are performed, one to divert the CSF, and another at a later stage to remove the cause of obstruction (e.g., a brain tumor). Once inserted, the shunt system usually remains in place for the duration of a patient's life (although additional operations to revise the shunt system are sometimes needed). The shunt system continuously performs its function of diverting the CSF away from the brain, thereby keeping the intracranial pressure within normal limits.

An alternative operation called endoscopic third ventriculostomy utilizes a tiny camera to look inside the ventricles, allowing the surgeon to create a new pathway through which CSF can flow.

Recovery

Your neurological function will be evaluated post surgery. If any neurological problems persist, rehabilitation may be required to further your improvement. However, recovery may be limited by the extent of the damage already caused by the hydrocephalus and by your brain's ability to heal.

Because hydrocephalus is an ongoing condition, long-term follow-up by a doctor is required. Follow-up diagnostic tests including CT scans, MRIs and x-rays, are helpful in determining if the shunt is working properly. Do not hesitate to contact your physician if you experience any of the following postoperative symptoms:

• Redness, tenderness, pain or swelling of the skin along the length of the tube or incision
• Irritability or drowsiness
• Nausea, vomiting, headache or double vision
• Fever
• Abdominal pain
• Return of preoperative neurological symptoms

Prognosis

The prognosis for hydrocephalus depends on the cause, the extent of symptoms, and the timeliness of diagnosis and treatment. Some patients show a dramatic improvement with treatment while others do not. In some instances of NPH, dementia can be reversed by shunt placement. Other symptoms such as headaches may disappear almost immediately if the symptoms are related to elevated pressure.

In general, the earlier hydrocephalus is diagnosed, the better the chance for successful treatment. The longer the symptoms have been present, the less likely it is that treatment will be successful. Unfortunately, there is no way to accurately predict how successful surgery will be for each individual. Some patients will improve dramatically while others will reach a plateau or decline after a few months.

Shunt malfunction or failure may occur. The valve can become clogged or the pressure in the shunt may not match the needs of the patient, requiring additional surgery. In the event of an infection, antibiotic therapy may be needed. A shunt malfunction may be indicated by headaches, vision problems, irritability, fatigue, personality change, loss of coordination, difficulty in waking up or staying awake, a return of walking difficulties, mild dementia or incontinence. Fortunately, most complications can be dealt with successfully.

Source: NeurosurgeryToday.Org

The effects of hydrocephalus on intelligence quotient in children with localized infratentorial ependymoma before and after focal radiation therapy

By: THOMAS E. MERCHANT, D.O., PH.D., HEATHER LEE, M.D., JUNHONG ZHU, M.S.,
XIAOPING XIONG, PH.D., GREGORY WHEELER, M.B.B.S., SEAN PHIPPS, PH.D.,
FREDERICK A. BOOP, M.D., AND ROBERT A. SANFORD, M.D.

Departments of Radiation Oncology and Biostatistics, St. Jude Children’s Research Hospital,
Memphis, Tennessee


Object. The goal of this study was to determine the influence of hydrocephalus on intelligence quotient (IQ) in children with infratentorial ependymoma before and after the administration of focal radiation.

Methods. Measurements of ventricular size, including Evans index (EI), cella media index (CMI), frontal horn diameter (FHD), and ventricular angle, were performed using magnetic resonance imaging at the time of diagnosis and again at 3, 6, 9, and 12 months after the initiation of radiation therapy. Of the 59 patients (median age at time of radiation treatment, 4.1 years), the clinical diagnosis established in 50 (85%) was hydrocephalus and 23 (39%) required placement of a cerebrospinal fluid (CSF) shunt. Extent of resection was gross or near total in 50 (85%). Before and after radiation treatment, IQ was measured using age-appropriate testing. The correlation between multiple ventricular measurements and IQ was investigated using standard regression techniques and a generalized linear model.
Patients with a higher EI (p = 0.04), CMI (p = 0.001), and FHD (p = 0.0002) at the time of diagnosis were more likely to have lower IQ scores before radiation treatment. Patients with higher CMI (p = 0.04) and FHD (p = 0.01) at the time of diagnosis were more likely to exhibit an increase in IQ score after radiotherapy. The rate of change in IQ after radiation treatment was positively correlated with the CMI intercept (p = 0.015) and negatively correlated with the rate
of FHD change (p = 0.006).

Conclusions. Changes in IQ score before and after radiation treatment are significantly influenced by the extent and treatment of hydrocephalus at the time of diagnosis. Hydrocephalus is an important factor to include when analyzing the effects of treatment. Patients who undergo a second surgery for ependymoma are more likely to require the placement of a CSF shunt (p = 0.0001).


KEY WORDS • ependymoma • hydrocephalus • cognition • radiation treatment • pediatric neurosurgery

Complete article

Congenital Hydrocephalus: Treatment

Early treatment-within a baby's first 3 to 4 months-is important to help limit or prevent brain damage. The long-term effects of congenital hydrocephalus depend largely on the cause of the condition, its severity, and the response to treatment.

Treatment generally consists of surgically inserting a flexible tube (shunt) in the brain to drain the cerebrospinal fluid. The shunt will remain in the brain permanently but may need to be repaired or replaced if a problem develops.

Endoscopic third ventriculostomy (ETV) is sometimes performed instead of surgical shunt placement. ETV is a surgical procedure in which a small hole is made in a ventricle in the brain, allowing CSF to flow freely. It is often done when hydrocephalus seems to be a result of a blockage between ventricles. If used as treatment for congenital hydrocephalus, it is more likely to be tried after a shunt malfunction or infection. ETV was once thought to be a permanent solution for redirecting CSF flow, but it has been shown to fail over time. Babies younger than 6 months of age usually are not good candidates for this procedure.

Children with congenital hydrocephalus are at increased risk for developmental disabilities and may require treatment such as speech therapy or physical therapy.

Congenital Hydrocephalus: Diagnosis

Most cases of congenital hydrocephalus are diagnosed during a physical exam soon after birth based on the larger-than-normal size of the baby's head. In rare cases, a diagnosis is made later in childhood. There may be reasons other than congenital hydrocephalus for why a baby has a larger-than-normal head.
With sophisticated imaging technologies, congenital hydrocephalus can be detected in a fetus as early as the third or fourth month of pregnancy. By the fifth or sixth month, abnormal dilation of brain cavities is more clearly detectable. Tests to identify the condition include:

• Ultrasound -- Performed by a radiologist or perinatologist, this test will establish if there is an abnormal collection of fluid but may not show the obstruction. Imaging tests are usually done to see whether congenital hydrocephalus is a possibility.

• Amniocentesis -- This, or a needle aspiration of intrauterine fluid, may be performed to detect the presence of other birth defects associated with hydrocephalus.

Congenital hydrocephalus, however, is frequently diagnosed at birth or shortly after. Sometimes it's not diagnosed until after birth.

A computed tomography (CT) scan, magnetic resonance imaging (MRI), or ultrasound may be done to help confirm the diagnosis or to provide a more detailed picture of the brain and its structures.

Congenital Hydrocephalus: Signs and Symptoms

Signs and Symptoms

The most common cause of congenital hydrocephalus is obstruction of the cerebral aqueduct -- the long, narrow passageway between the third and fourth ventricle or cavity of the brain. This condition may result from a blockage, infection, hemorrhage, tumor or arachnoid cyst. Other medical problems associated with this form of hydrocephalus are:

* Chiari malformations, an abnormality at the base of brain where the spinal column joins the skull
* Craniosynostosis, when the bones in the skull fuse together before the brain has stopped growing
* Dandy-Walker syndrome, when the fourth ventricle is enlarged because of partial or complete closure of its outlets
* Hydranencephaly, a rare condition in which the brain's cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid
* Neural tube defects or spina bifida, when the spinal cord is exposed at birth and is often lacking cerebrospinal fluid
* Schizencephaly, an extremely rare disorder characterized by abnormal slits, or clefts, in the brain's cerebral hemispheres
* Vein of Galen malformations, abnormal connections between arteries and the deep draining veins of the brain that develop before birth

Symptoms in infants may include:

* A very large head or a head that's growing very quickly in relation to the rest of the body
* Vomiting
* Sleepiness
* Irritability
* Downward deviation of the eyes, called "sunsetting"
* Seizures

Source: UCSF

Types of hydrocephalus and their etiologies

Hydrocephalus can be caused by impaired cerebrospinal fluid (CSF) flow, reabsorption, or excessive CSF production.

• The most common cause of hydrocephalus is CSF flow obstruction, hindering the free passage of cerebrospinal fluid through the ventricular system and subarachnoid space (e.g., stenosis of the cerebral aqueduct or obstruction of the interventricular foramina - foramina of Monro secondary to tumors, hemorrhages, infections or congenital malformations).
• Hydrocephalus can also be caused by overproduction of cerebrospinal fluid (relative obstruction) (e.g., papilloma of choroid plexus).

Based on its underlying mechanisms, hydrocephalus can be classified into communicating, and non-communicating (obstructive). Both forms can be either congenital, or acquired.

Communicating hydrocephalus

Communicating hydrocephalus, also known as non-obstructive hydrocephalus, is caused by impaired cerebrospinal fluid resorption in the absence of any CSF-flow obstruction. It has been theorized that this is due to functional impairment of the arachnoid granulations, which are located along the superior sagittal sinus and is the site of cerebrospinal fluid resorption back into the venous system. Various neurologic conditions may result in communicating hydrocephalus, including subarachnoid/intraventricular hemorrhage, meningitis, Chiari malformation, and congenital absence of arachnoidal granulations (Pacchioni's granulations).

• Normal pressure hydrocephalus (NPH) is a particular form of communicating hydrocephalus, characterized by enlarged cerebral ventricles, with only intermittently elevated cerebrospinal fluid pressure. The diagnosis of NPH can be established only with the help of continuous intraventricular pressure recordings (over 24 hours or even longer), since more often than not, instant measurements yield normal pressure values. Dynamic compliance studies may be also helpful. Altered compliance (elasticity) of the ventricular walls, as well as increased viscosity of the cerebrospinal fluid, may play a role in the pathogenesis of normal pressure hydrocephalus.

Main article: normal pressure hydrocephalus

• Hydrocephalus ex vacuo also refers to an enlargement of cerebral ventricles and subarachnoid spaces, and is usually due to brain atrophy (as it occurs in dementias), post-traumatic brain injuries and even in some psychiatric disorders, such as schizophrenia. As opposed to hydrocephalus, this is a compensatory enlargement of the CSF-spaces in response to brain parenchyma loss - it is not the result of increased CSF pressure.

Non-communicating hydrocephalus

Non-communicating hydrocephalus, or obstructive hydrocephalus, is caused by a CSF-flow obstruction (either due to external compression or intraventricular mass lesions).

• Foramen of Monro obstruction may lead to dilation of one or, if large enough (e.g., in colloid cyst), both lateral ventricles.
• The aqueduct of Sylvius, normally narrow to begin with, may be obstructed by a number of genetically or acquired lesions (e.g., atresia, ependymitis, hemorrhage, tumor) and lead to dilatation of both lateral ventricles as well as the third ventricle.
• Fourth ventricle obstruction will lead to dilatation of the aqueduct as well as the lateral and third ventricles.
• The foramina of Luschka and foramen of Magendie may be obstructed due to congenital failure of opening (e.g., Dandy-Walker malformation).
• The subarachnoid space surrounding the brainstem may also be obstructed due to inflammatory or hemorrhagic fibrosing meningitis, leading to widespread dilatation, including the fourth ventricle.

Congenital hydrocephalus

The cranial bones fuse by the end of the third year of life. For head enlargement to occur, hydrocephalus must occur before then. The causes are usually genetic but can also be acquired and usually occur within the first few months of life, which include 1) intraventricular matrix hemorrhages in premature infants, 2) infections, 3) type II Arnold-Chiari malformation, 4) aqueduct atresia and stenosis, and 5) Dandy-Walker malformation.

Main articles: Arnold-Chiari malformation and Dandy-Walker malformation

In newborns and toddlers with hydrocephalus, the head circumference is enlarged rapidly and soon surpasses the 97th%. Since the skull bones have not yet firmly joined together, bulging, firm anterior and posterior fontanelles may be present even when the patient is in an upright position.

The infant exhibits fretfulness, poor feeding, and frequent vomiting. As the hydrocephalus progresses, torpor sets in, and the infant shows lack of interest in his surroundings. Later on, the upper eyelids become retracted and the eyes are turned downwards (due to hydrocephalic pressure on the mesencephalic tegmentum and paralysis of upward gaze). Movements become weak and the arms may become tremulous. Papilledema is absent but there may be reduction of vision. The head becomes so enlarged that the child may eventually be bedridden.

About 80-90% of fetuses or newborn infants with spina bifida - often associated with meningocele or myelomeningocele - develop hydrocephalus.^[6]

Acquired hydrocephalus

This condition is acquired as a consequence of CNS-infections, meningitis, brain tumors, head trauma, intracranial hemorrhage (subarachnoid or intraparenchymal) and is usually extremely painful for the patient.

Source: Wikipedia

normal pressure hydrocephalus

What is normal pressure hydrocephalus?

Normal pressure hydrocephalus, or NPH, is an acquired hydrocephalus that most often occurs in people over age 60. NPH is different from typical hydrocephalus in that it may not cause an obvious increase of pressure in the head, but may have fluctuations in CSF pressure from high to normal to low.

What are the symptoms of NPH?

There are three classic symptoms of NPH. They are referred to as the classic triad of symptoms:

• Difficulty walking — This problem can be mild or severe. In many cases, people with NPH have trouble picking up their feet. Some describe it as feeling like their feet are stuck to the floor. This can lead to a shuffling walk, and problems going up stairs and curbs. It also increases the risk of falling.
• Dementia — This often involves confusion, short-term memory loss, and a lack of interest in daily activities.
• Problems with bladder control — Problems include urinary incontinence (the inability to hold urine), frequent urination, and a strong feeling of needing to urinate.

Most patients with NPH do not have headaches, which are common in patients with obstructive hydrocephalus.

What causes NPH?

Many cases of NPH have no known cause. Some cases of NPH are linked to bleeding in the brain or a blockage in CSF flow through and around the brain and spinal cord. It is believed that blockages are linked to a history of infection, stroke, or head injury.

How common is NPH?

Because the symptoms of NPH are similar to those of other diseases, people with NPH are often diagnosed with disorders such as Alzheimer's or Parkinson's disease, or the symptoms may be attributed to the aging process. For that reason, it is difficult to know how many people actually have NPH. However, it is estimated that as many as 10 percent of people with dementia attributed to other disorders may actually have NPH.

How is NPH diagnosed?

A careful review of symptoms, a medical history, and various tests are used to diagnose NPH. Tests used may include:

• Computed tomography (CT) — A CT scan is a diagnostic tool that uses X-rays and a computer to create pictures of structures inside the body. A CT scan can provide images that show the size of the ventricles.
• Lumbar puncture for NPH — Also called a spinal tap, this procedure is used to remove a sample of the CSF. For NPH, this test is used to determine if a person’s symptoms improve after removing a large amount of fluid. About 1 to 1½ oz. of fluid is removed. However, this test is not definitive.
• NPH protocol — The protocol entails a series of screening procedures, including a gait analysis, blood work, and neuropsychiatric testing. It also involves the removal of CSF through a special catheter (tube) over a 36-hour period. It is expected that following removal of CSF, there will be a dramatic, temporary relief of symptoms. The protocol also provides the surgeon with information about the potential benefit of implanting a shunt, which is a device that drains excess CSF away from the brain and spinal cord, diverting it to another part of the body, such as the abdomen or heart, where the body can absorb it.
• Magnetic resonance imaging (MRI) — An MRI scan uses a magnet and radio waves, instead of X-rays, to produce images.
• Gait analysis (walking) — This is a timed walk test. The patient is watched as he or she walks 10 meters (about 30 feet).
• Neuropsychological testing — This involves a series of questions used to see if there is a loss of brain function caused by NPH.

How is NPH treated?

NPH may be treated using an implantable shunt to drain excess CSF away from the brain and spinal cord.

What complications are linked to NPH treatment?

Complications of NPH treatment are those associated with any surgical procedure. They include bleeding, infection, and reaction to the anesthesia used during surgery. Patients might also experience mild abdominal pain. Seizures also may occur as surgery on the brain can affect very sensitive areas of the brain. Fortunately, these complications are not common, and in most cases can be successfully treated.

What is the outlook for people with NPH?

With treatment, the symptoms of NPH can be partially or even fully reversible. On the other hand, the outlook is poor when the disorder is not treated appropriately. Without treatment, the symptoms can continue to worsen and lead, eventually, to death.

Is there any way to prevent NPH?

Right now, there is no known way to prevent NPH. However, getting treatment as soon as symptoms appear can improve those symptoms and increase the chance for a full or partial recovery.

Source: Cleveland Clinic

Minimally Invasive Endoscopic Surgery Can Safely Remove Deep Brain Tumors in Patients Without Hydrocephalus

NEW YORK, NY -- October 6, 2005 -- A research study by a New York-Presbyterian Hospital/Weill Cornell Medical Center neurosurgeon demonstrates that surgeons who use an endoscope to biopsy or resect intraventricular brain tumors in patients without hydrocephalus achieve equal if not better results than similar cases where hydrocephalus is present -- demonstrating that the field of minimally invasive brain surgery is advancing.

Minimally invasive surgery, where possible, is generally preferred to open brain surgery because it is associated with less morbidity, less scarring, and shorter recovery time.

The study appears in the October issue of Operative Neurosurgery.

Using an endoscope to remove intraventricular brain tumors (tumors within the brain's ventricles or cavities) is seen as one of the most challenging aspects of neurosurgery because the ventricles are located near the center of the brain. And, in patients without hydrocephalus (swelling of the brain) the challenge is actually much greater because patients lack the enlarged ventricles that allow surgeons wider pathways to the tumor site and more room to operate once there.

Only a few medical centers in the U.S. have surgeons with enough training to remove intraventricular brain tumors in patients with or without hydrocephalus, and most patients end up undergoing a traditional craniotomy -- where a portion of the skull is removed in order to gain access to the brain -- and enduring the greater risk, longer recovery times, and greater scarring the procedure involves.

"Our study demonstrates that minimally invasive brain surgery is gaining ground," says Dr. Mark Souweidane, the study's author and vice chairman of the department of neurological surgery and director of pediatric neurological surgery at NewYork-Presbyterian/Weill Cornell, and associate professor of clinical neurological surgery at Weill Cornell Medical College. "We know that endoscopic surgery is safer than opening a patient's brain -- a craniotomy -- and now, from our study, we know that even one of the most difficult types of endoscopic surgery -- intraventricular tumors in patients without hydrocephalus -- is becoming safer."


Dr. Souweidane hopes his study will encourage hospitals and neurosurgeons to invest in endoscopic training, and will encourage patients with brain tumors to ask questions and explore their surgical options.

With endoscopic surgery, after a small 14 mm hole is made in the skull for access, surgeons guide the endoscope, a slender catheter-like camera through the brain's normal fluid-filled compartments, which visually often looks like swimming through a soft coral reef. Surgeons watch the image on a nearby monitor, and when the tumor is located, the tools that will biopsy or resect are deployed through a 2 mm tunnel in the scope.

Because endoscopic surgery allows surgeons to reach the center of the brain without significant cutting of tissue or draining of intracranial fluid, which can cause the brain to "sag" away from the skull and bleed, the procedure is safer than traditional methods for operating upon intraventricular brain tumors.

In Dr. Souweidane's study, 80 patients at NewYork-Presbyterian/Weill Cornell who underwent endoscopic management for an intraventricular brain tumor were identified from a prospective database. Of these patients, 15 had an intraventricular tumor without concomitant hydrocephalus and underwent primary endoscopic surgery for biopsy or resection. The surgical technique, the success rate, and patient outcome were assessed and then compared with the 65 hydrocephalic patients who underwent similar procedures.

All patients in the study were treated between December 1995 and December 2004. Patients in the comparison group (with hydrocephalus), ranged in age from 3 months to 80 years, with the mean being 33.1 years. Thirty-six were male and 29 were female. In the study group (without hydrocephalus), patients ranged in age from 13 to 80 years, with the mean being 40.3 years. Eight were male and seven were female.

Of the 15 patients without hydrocephalus, 11 had tumors located in the third ventricle (direct center of brain) and four had tumors located in the lateral ventricle (slightly above the center and forward). In all 15 patients, the ventricular compartment was successfully cannulated (entered) and the intended goal was accomplished: 12 patients had successful diagnostic sampling (a biopsy), and three had complete resection of a colloid cyst. There were no operative complications related to the endoscopic procedure, nor were there any recognized neurological complications, seizures, or surgically related infections or deaths. No patient required subsequent intervention for hydrocephalus.

Dr. Souweidane is among several NewYork-Presbyterian/Weill Cornell surgeons in the department of neurological surgery who routinely perform endoscopic surgery on patients with intraventricular brain tumors.


SOURCE: NewYork-Presbyterian Hospital/Weill Cornell Medical Center

What is shunt?

A shunt is a surgically implanted device that diverts cerebrospinal fluid (CSF) in a controlled manner from fluid compartments in the brain or near the spine to another part of the body, such as the abdomen or the heart. For nearly half a century, hydrocephalus has been treated by surgical placement of a (CSF) diverting shunt.
Shunts typically consist of three components:
1. An inflow (proximal) catheter, which drains CSF from the ventricles or the lumbar subarachnoid space, to a valve;
2. A valve mechanism, which regulates differential pressure or controls flow through the shunt tubing;
3. An outflow (distal) catheter, which directs CSF from the valve to the peritoneum, heart or other suitable drainage site.

Other shunt components may include reservoirs and/or antechambers for sampling or injecting medications; or dyes, on/off devices, anti-siphon or other flow-compensating devices, auxiliary catheters, etc., to modify performance or adapt the basic system to the patient’s specialized needs. In selected cases (such as when extraventricular fluid collections are drained), a shunt may not contain a valve.

Hydrocephalus

From Hydrocephalus Association:

Hydrocephalus comes from Greek words: hydro means water, cephalus means head. Hydrocephalus is an abnormal accumulation of fluid—cerebrospinal fluid, or CSF—within cavities called ventricles inside the brain. This condition may occur at any age. CSF is produced in the ventricles, circulates through the ventricular system in the brain and is absorbed into the bloodstream. CSF is in constant circulation and has many functions. It surrounds the brain and spinal cord and acts as a protective cushion against injury. CSF contains nutrients and proteins that are needed for the nourishment and normal function of the brain. It carries waste products away from surrounding tissues. Hydrocephalus occurs when there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed. As the CSF builds up, it causes the ventricles to enlarge and the pressure inside the head to increase.

While there's no known cure for hydrocephalus, the future holds promise. Recent research gives great hope that we may one day find a cure. In the meantime, gradual advances in technology as well as diagnostic and treatment protocols are helping more and more people with hydrocephalus to lead full and active lives.

Although hydrocephalus (hi-dro-sef-a-lus) is not yet a household word, it is NOT uncommon and you are not alone. Over a million people of all ages currently live with hydrocephalus in the United States, though it occurs all over the world. Hydrocephalus knows no socioeconomic, gender or ethnic boundaries. Of every 1,000 babies born in this country, one or two will have hydrocephalus. It is the most common reason for brain surgery in children, but it can also be diagnosed in any decade of life. Experts estimate that hundreds of thousands of older adults have normal pressure hydrocephalus (NPH), which often goes undetected or is misdiagnosed as untreatable dementia, Alzheimer’s or Parkinson’s.

How is Hydrocephalus treated?
There is no known way to prevent or cure hydrocephalus. To date, the most common treatment is surgical insertion of a shunt. A shunt is a flexible tube placed into the ventricular system of the brain which diverts the flow of CSF into another region of the body, most often the abdominal cavity, where it can be absorbed. A valve within the shunt regulates the flow of CSF.

The shunt procedure is performed by a neurosurgeon. Shunts can become clogged or malfunction and surgical revisions are often required. Endoscopic Third Ventriculostomy (ETV) is a procedure in which a small perforation is made in the thinned floor of the third ventricle, allowing movement of CSF out of the blocked ventricular system. It is a promising technique that can treat hydrocephalus without a shunt. ETV is not a cure for hydrocephalus and it is not an appropriate choice of treatment for many types of hydrocephalus. Each case must be evaluated individually by experienced medical professionals.


The association provides a family book about Hydrocephalus. You can get it here.


Useful Links

Hydrocephalus Association

Pediatric Neurosurgery - Hydrocephalus

Hydrocephalus Fact Sheets