Hydrocephalus (Fluid in the Brain): Prevention, Diagnosis and Treatment
Overview of Hydrocephalus
Traumatic injuries to a baby’s head and oxygen deprivation can lead to bleeding inside the infant’s brain ventricles, and a condition called hydrocephalus can result. When hydrocephalus occurs, the enlarged, fluid-filled ventricles push against the brain.
The fluid in the ventricles consists of cerebral spinal fluid (CSF) and other blood and cellular products. The swollen ventricles cause the brain to be pushed against the skull. This often results in the baby having an enlarged head.
Hydrocephalus can cause brain damage through a number of mechanisms, which include 1.) direct damage of the brain’s white matter (WM) by CSF getting into the WM tissues, and by decreased blood flow in the WM, 2.) destruction of the membrane that covers the brain, which causes malfunction of the brain’s vascular system (vessels), 3.) damage to the vessels and cells of the nervous system, which causes further WM damage.
Although other conditions can cause hydrocephalus, it many times begins with a brain hemorrhage, such as a germinal matrix hemorrhage (GMH) or intraventricular hemorrhage (IVH). A GMH is a type of IVH. The germinal matrix is a very important structure in the fetus because it helps create the fully-formed brain; cells develop and migrate out of this structure during brain development.
This is how hydrocephalus progresses: 1.) IVH (or other brain insult) 2.) disturbance in CSF dynamics 3.) swelling of the ventricles 4.) hydrocephalus 5.) brain damage.
The period around the time of birth is the most significant in the prevention of GMH and IVH. Listed below are prenatal and postnatal strategies for prevention. In order to prevent GMH, IVH and resultant hydrocephalus, research shows that the following should be prevented or minimized:
- Cerebral blood flow fluctuations (constant changes in blood flow and pressure in the brain)
- Hypertension (high blood pressure)
- Hypotension (low blood pressure)
- Hypoxia (decreased oxygen getting to tissues)
- Hyperoxia (baby receiving too much oxygen)
- Hypercapnia (abnormally high carbon dioxide (CO2) level in blood)
- Hypocarbia (abnormally low CO2 level in blood)
- Acidosis (blood is acidic due to poor gas exchange / high CO2)
- Increased venous pressure (high blood pressure)
Research shows that prenatal strategies to prevent GMH and IVH must include good obstetric management and smooth delivery, smart use of C-section delivery, and administration of steroids and vitamin K.
- Optimal obstetric management and smooth delivery. This includes close monitoring of the mother and baby during pregnancy to ensure the health of the baby and to learn about any situations that might put the baby at risk around the time of birth. Preventing the baby from getting an infection, and prevention of situations that could cause trauma or oxygen deprivation in the baby are crucial. This means that physicians should prevent the following conditions when possible and be aware of their existence at the earliest possible time in order to affect prompt and proper treatment:
- Cephalopelvic disproportion (CPD) (baby’s head or body cannot fit through birth canal)
- Macrosomia (large baby)
- Malpresentation of the baby, such as a breech position
- Problems with the umbilical cord, such nuchal cord and cord prolapse
- Oligohydramnios (low fluid in womb)
- Problems that affect the placenta, such as placental abruption, placenta previa and preeclampsia
- Ruptured uterus
- Infections in the mother, such as syphilis, cytomegalovirus (CMV) and Group B Strep (GBS)
To prevent IVH and GMH, the physician should do the following before and during birth: 1.) give steroids and magnesium sulfate to the mother if there is suspicion that the baby will be premature, 2.) transfer the mother to a high risk perinatal facility before the baby is born, 3.) avoid prolonged labor, which means moving on to a C-section when indicated (e.g., if the baby is showing any distress), 4.) wait for 30 seconds or more to clamp / cut the umbilical cord. After the baby is born, indomethacin should be given to premature infants to close a major blood vessel that typically is closed in term infants, and is supposed to be closed for proper blood circulation and oxygenation. Vitamin K should be given to all newborns to prevent bleeding in the brain and other problems.
Preventing GMH and IVH is of utmost importance. But when it occurs, quick recognition and treatment is essential. Experts indicate that all premature infants in a neonatal intensive care unit (NICU) are at risk of hemorrhage and should therefore be screened for hydrocephalus. In addition, hydrocephalus should be suspected in any infant whose head is enlarged at birth or in whom serial measurements of the head indicate excessive head growth compared to other babies. Indeed, infants at greatest risk of hemorrhage and hydrocephalus should be screened frequently; an initial ultrasound should be done before day 5 of life, a second one during the second week, and a third one on day 28. Generally, at least one ultrasound per week should detect all cases of GMH and IVH and show the ventricular size status.
The time course from the initial hemorrhage to clinically evident ventricle enlargement and subsequent progressive hydrocephalus varies from days to weeks, depending on the cause of the hydrocephalus. Experts state that it is mandatory to closely evaluate and follow up with these babies by using diagnostic tools such as ultrasonography and MRI. Clinical data alone is insufficient for evaluation, monitoring and follow up.
Diagnostic tools include the following:
Ultrasonography (ultrasound scan). Ultrasound is the procedure of choice for the diagnosis of hemorrhages and hydrocephalus because it is portable and avoids radiation. Ultrasonography uses sound waves to take pictures of soft tissue in the brain.
An ultrasound can help identify the following issues pertaining to hemorrhages:
- Identification of hemorrhage
- Grading the severity of hemorrhage (The severity of IVH is based on the presence and amount of blood in the germinal matrix.)
- Timing of hemorrhage
A brain ultrasound provides direct and accurate information about ventricular size, exact location and extension of hematoma (bleeding in between the tissues that surround the brain), cortical mantle thickness (thickness of the tissue that surrounds the largest part of the brain), and periventricular white matter condition. Combined with clinical evidence, an ultrasound is used to detect initial hemorrhage, which more often occurs during the first week after birth. Ultrasonography also detects further ventricular dilation (swelling), which usually results in progressive hydrocephalus, usually after the third week. This time interval is important because ventricular dilation can occur without clinical evidence, and it contributes to an otherwise preventable brain damage. In other words, ultrasonagraphy is essential because it often is the only way of discovering ventricular dilation. Once ventricular dilation is discovered, treatment can begin and some or much of the brain damage can be prevented. Furthermore, stopping or decreasing ventricular dilation can help prevent progression to hydrocephalus.
There are many different methods for measuring ventricular size (to determine dilation) with an ultrasound. The most widely used is the ventricular index (VI), which is the measurement of a specific distance near the ventricles. Physicians compare this VI to reference ranges in order to distinguish normal size from abnormal. Progressive ventricular enlargement as indicated by a VI that exceeds a certain number is an indication that physicians need to drain CSF from the ventricles.
Lumbar puncture. Lumbar puncture (a spinal tap) is a procedure whereby physicians use a needle to remove CSF and detect CSF abnormalities, which can be critical in early recognition of intracranial hemorrhage. Also, the higher the CSF protein level, the more severe the hemorrhage.
Magnetic Resonance Imaging Scan (MRI). MRI uses computer-generated radio waves and a magnetic field to produce detailed images of tissue, organs, bones, and nerves. MRI has been employed in recent years in infants with GMH and IVH. Although it may be difficult for a baby to endure an MRI, it provides very useful information. The exact condition of periventricular white matter can be clarified, and this can help physicians predict the developmental and neurodevelopmental outcome of the baby.
MRI can help distinguish ventricular enlargement due to atrophy (destruction) from ventricular dilation due to CSF build up. Round “ballooned” ventricular horns and other features of hydrocephalus can be seen on an MRI.
More evidence of hydrocephalus can be elicited by cerebral blood flow measurement (how well the blood is flowing) and apparent diffusion coefficient (ADC) values. (ADC values reflect blood flow, and they take into account the fact that the diffusion of blood between the vessels in the tissues is affected by many mechanisms.) Distinction between progressive and compensated hydrocephalus is crucial in premature infants and it has been found that normal CBF and low ADC values (measured by MRI) are associated with a compensated-arrested state (the body has compensated for some of the negative effects of hydrocephalus and the hydrocephalus is not getting any worse). Knowing that the hydrocephalus is not getting worse can support a conservative (less aggressive) treatment approach.
Management of Hemorrhages and Hydrocephalus
Therapeutic approaches for an infant suffering from GMH and IVH should focus on two objectives: 1.) to protect the brain tissue from damage caused by increased brain pressure / intracranial pressure (ICP), and 2.) to avoid the placement of a permanent shunt with the long-term complications and undesirable shunt dependency that this leads to.
If ventricular dilation is allowed to progress, permanent brain damage may occur. The physician has to find the golden median between the two objectives during the first weeks of the infant’s life. When ventricular dilation is noticed, VI measurements typically are used to determine the ventricular size and whether intervention is necessary. Other clinical data are also taken into consideration, such as head size increase and neurological status (how well the brain is functioning).
When progressive ventricular dilation is verified, initial interventions aim at removing CSF, which lowers the pressure within the ventricles. Blood degradation
products as well as other products released when the body is injured also are removed. This can be done with the following procedures:
- Lumbar puncture (LP).
- Ventricular taps. This is when a needle is used to remove fluid directly from the brain ventricles, either free handed or with the help of an ultrasound. This is a short term option for infants with rapidly enlarging ventricles who cannot have a lumbar puncture because it is contraindicated.
- External ventricular drainage (EVD). This is a type of shunt that is used to quickly decrease pressure in the ventricles and eliminate blood-filled CSF. Specifically, an EVD is placed by a neurosurgeon and it relieves elevated pressure in the brain (ICP) and hydrocephalus when the normal flow of CSF around the brain is obstructed. A plastic tube is placed in a ventricle to drain fluid from the ventricles to keep them decompressed (not swollen). The tube also monitors the ICP.
- Subcutaneous reservoirs (or ventricular access devices- VAD). These reservoirs are connected to a ventricular catheter (tube that goes into the ventricles) that allows for drainage of CSF, even in very low birthweight infants. The reservoir sits on the surface of the skull under the tissue that covers the top part of the brain. The reservoir can be punctured to withdraw CSF to keep the ventricular system decompressed.
- Ventricular subgaleal shunt. This is an alternative to VAD and gives a temporary benefit of about a month. With this shunt, physicians perform surgery to create a pocket in the brain between the skull and the scalp that allows free drainage of CSF. This pocket is connected to the ventricles This shunt is most frequently used as a temporizing measure in the premature infant with hydrocephalus caused by a hemorrhage within the ventricle. The shunt allows CSF and blood to clear from the ventricles before a permanent shunt is inserted, since shunts tend to malfunction in the presence of blood. It also allows the premature infant to mature. A catheter is placed in the ventricle and connected to a low pressure valve. The valve is placed under the scalp and the incision is closed. The shunt is allowed to drain under the scalp. Fluid under the scalp indicates the shunt is functioning.
Initial interventions aim to stabilize the progressive ventricular dilation and ongoing brain damage. Interventions offer time for the infant to gain weight and for the physician to deal with the concurrent health problems of the baby, and to help the baby’s immature immune system gain strength.
If the interventions listed above fail to control progressive ventricular dilation, then the next and well-established treatment option is VP shunting. VP shunt systems have been developed to simulate the normal CSF absorption rate. The baby must weigh over 2,000 grams, have certain levels of CSF proteins, have no evidence of infection, and have no abdominal impairment. As a general rule, a low profile valve, low pressure setting, and minimal interconnections (two-piece shunt system) is sufficient. The VP shunt system must be surgically implanted. It uses tubes to redirect the flow of fluid from an area of buildup in the brain to the abdominal cavity, where it can be absorbed as part of the circulatory process. Physicians can use the valve in the system to adjust flow and normalize pressure.
Typical Approach for Hemorrhage and Hydrocephalus
For babies with GMH / IVH that involve hemorrhages extending into the ventricles and dilation of the ventricles, serial lumbar punctures (LP’s) should be performed. The LP’s usually are performed 2 – 3 times a week, and the CSF amount removed is about 10ml/kg of the baby’s weight. LP’s are effective for a week or two and must be accompanied by close clinical and ultrasound investigations (ultrasound every 2 – 3 days). Next, a low profile subcutaneous reservoir (VAD) is inserted so that physicians can tap into the reservoir and withdraw small amounts (10ml/kg) of CSF, usually once a day. Clinical and ultrasound monitoring are critical in order to determine if the ventricles remain enlarged and if further treatment is necessary. This period can last up to 3 – 4 weeks. When a baby is at term, an MRI scan can be performed so that a more detailed imaging of the brain tissue, white matter, and area in the skull near the brainstem can be assessed.
As discussed earlier, persistent hydrocephalus or hydrocephalus that keeps getting worse is treated with VP shunting, as long as there are no other current morbidities (severe health / organ problems), and CSF cell count is normal. If CSF protein levels are abnormal, an obstruction in the shunt can occur, and this can be dangerous or fatal for the baby. Shunts are associated with many complications, including infection and obstruction.
Long-Term Outcomes for Children with Hydrocephalus
Many children with hydrocephalus grow up without any problems. Some children suffer from seizures, and some have brain damage that causes a low IQ score. Brain disabilities are mainly influenced by the extent of the brain tissue injury. Spasticity (which includes spastic cerebral palsy) and intellectual deficits occur when certain areas of brain tissue die because blood vessels become damaged, blocked or they leak. Periventricular leukomalacia (PVL) is mainly responsible for high and constant tightness of muscles in the limbs (spasticity). Intellectual disabilities, seizures and cerebral palsy are the most frequent disabilities. The timing of the GMH / IVH seems to affect neurodevelopmental outcome as infants who develop a hemorrhage at less than 6 hours of life have a higher chance of suffering cerebral palsy and lower IQ scores. Follow up should extend to childhood or even adolescence in order to unveil minor cognitive and neuropsychological deficits.
Brain Hemorrhages, Hydrocephalus and Medical Malpractice
Physicians must do everything they can to prevent injury to a baby’s brain, especially if the baby is premature. Failure to follow standards of care to prevent dangerous complications, and failure to recognize and properly manage serious conditions, such as infection, cord prolapse and placental problems, is negligence. Failure to properly monitor and screen a baby, and failure to recognize a brain hemorrhage constitute negligence. It also is negligent when hydrocephalus occurs and is not timely diagnosed and treated. When physicians act negligently in their diagnosis, monitoring and treatment of hemorrhages and hydrocephalus, and this results in permanent brain damage in the baby, it is medical malpractice.
The longer hydrocephalus is allowed to occur without proper treatment, the more severe the brain damage. When risk factors for hydrocephalus or signs and symptoms of the condition occur, close monitoring of the baby is essential. Due to the severity of the condition, diagnostic tests must be performed when there is any suspicion of brain hemorrhage or hydrocephalus.
If you are seeking the help of a hydrocephalus lawyer, it is very important to choose a lawyer and firm that focus solely on birth injury cases. Reiter & Walsh ABC Law Centers is a national birth injury law firm that has been helping children for decades.
If your child was diagnosed with a birth injury, such as cerebral palsy, a seizure disorder or hypoxic ischemic encephalopathy (HIE), the award-winning hydrocephalus lawyers at ABC Law Centers can help. We have helped children throughout the country obtain compensation for lifelong treatment, therapy and a secure future, and we give personal attention to each child and family we represent. Our nationally recognized birth injury firm has numerous multi-million dollar verdicts and settlements that attest to our success and no fees are ever paid to our firm until we win your case. Email or call Reiter & Walsh ABC Law Centers at 888-419-2229 for a free case evaluation. Our firm’s hydrocephalus lawyers are available 24 / 7 to speak with you.