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Ventriculomegaly

  • At Boston Children’s Hospital, we’ve already helped many infants who have been diagnosed with fetal ventriculomegaly, a condition in which the fluid-filled spaces in the brain (ventricles) appear larger than normal on a prenatal ultrasound.

    Here are the basics about ventriculomegaly:

    • It occurs in approximately one in 1,000 births.
    • Ventriculomegaly can be detected during the second trimester on a prenatal ultrasound.
    • Ventriculomegaly only needs to be treated if a child also has hydrocephalus, a term that describes the progressive accumulation of cerebrospinal fluid (CSF) within the ventricles leading to worsening ventriculomegaly (progressive enlargement of the ventricles) under pressure. Hydrocephalus has a number of different causes.
    • There is no single identifiable cause for ventriculomegaly, but sometimes enlarged ventricles are associated with decreased surrounding brain tissue from damage or abnormal development.

    How Boston Children's approaches ventriculomegaly

    Clinicians in the Neonatal and Congenital Anomaly Neurosurgery Program here at Boston Children’s specialize in the diagnosis and treatment of ventriculomegaly and other types of disorders relating to the brain and nervous system.

    Typically, ventriculomegaly only requires treatment if it causes hydrocephalus. Since the excessive pressure caused by the buildup of cerebrospinal fluid in hydrocephalus can lead to serious, long-term neurological damage, prompt treatment is a must.

    Boston Children's was the first hospital in the world to treat children with hydrocephalus by shunting. In the years since this important milestone, our physicians have helped design and test the next generation of shunting devices, introduced and refined the use of minimally invasive surgical alternatives to shunt placement, and taught these potentially life-saving techniques to pediatric neurosurgeons around the world.

    Our approaches to treating ventriculomegaly and hydrocephalus are both patient-focused and family-centered. We never lose sight of the fact that your child is, first and foremost, an individual—not merely a patient—and we include your family at every stage of the treatment process.

    Ventriculomegaly: Reviewed by Benjamin C. Warf, MD
    © Boston Children’s Hospital; posted in 2012


    In many cases, ventriculomegaly is an isolated condition, unlinked to other health problems and will resolve on its own. However, your child will be monitored by a pediatric neurologist through a pre-natal and post-natal examination and a physical exam to measure the circumference of the head.
    Boston Children's Hospital 
    300 Longwood Avenue
    Fegan 11 and Hunnewell 2
    Boston MA 02115

     617-355-6388


    Ventriculomegaly that occurs in combination with a condition called hydrocephalus can be treated and managed through our Hydrocephalus Program.
    Boston Children's Hospital 
    300 Longwood Avenue
    Boston MA 02115

     617-355-6008
  • In-Depth

    Infants who require evaluation for ventriculomegaly or possible hydrocephalus are generally seen in the Neonatal and Congenital Anomalies Neurosurgery Programat Boston Children’s Hospital. Here, members of the team work closely with specialists in other departments in the hospital, to develop individual care plans that meet each child’s medical needs.

    Until you meet with our team of doctors, you may find it helpful to read the straightforward information about ventriculomegaly provided in the following pages.

    What does it mean to have ventriculomegaly?

    • Your child’s brain and spinal cord are covered in a clear protective liquid called cerebrospinal fluid (CSF). There are spaces within the brain (ventricles) that are also filled with CSF.
    • In the brain of a healthy fetus, the ventricles are about 10 millimeters wide. However, CSF may become trapped in the spaces, causing them to grow progressively larger.
    • Ventriculomegaly is a term that describes the actual image of the enlarged spaces as it appears on a prenatal ultrasound.

     

    Are there any medical complications associated with ventriculomegaly?

    • Hydrocephalus is the main concern associated with ventriculomegaly. Hydrocephalus occurs when CSF builds up within the ventricles of the brain, causing them to grow progressively larger.
    • Benign macrocrania of infancy is a more common condition in which a baby can have mild ventriculomegaly and a large head, but not hydrocephalus. Although this condition causes the head to grow too fast, the baby is otherwise well, and the condition is self-limiting. It does not require treatment.

    Causes

    What causes ventriculomegaly?

    Ventricles develop early in pregnancy and can be seen on a prenatal ultrasound in the second trimester, at about the 15th week.

    Although there’s not always a single identifiable reason why a child develops ventriculomegaly, there are four main factors that can cause enlargement of the ventricles:

    • A problem that prevents the CSF fluid from circulating and being absorbed normally, which causes the fluid to progressively build up in the ventricles  - the condition known as hydrocephalus.
    • A minor self-limiting imbalance in fluid circulation and absorption that becomes compensated
    • Defects in brain development
    • Damage or loss of brain tissue

    Is ventriculomegaly inherited?

    Ventriculomegaly usually occurs spontaneously, which means that a child does not inherit the condition from the parents. However, benign macrocephaly tends to run in families and there are some rare inherited causes of hydrocephalus. Congenital hydrocephalus can be associated with other anomalies of the brain, or other conditions such as spina bifida or heart defects.

    Symptoms

    What are the symptoms of ventriculomegaly?

    Infants with mild self-limited ventriculomegaly usually don’t have any symptoms. If the ventriculomegaly is progressive the baby may show the signs and symptoms of hydrocephalus once it is born.

    An infant with hydrocephalus may have:

    • Abnormally rapid head growth
    • Abnormally full fontanel
    • Distended scalp veins
    • Eyes that cannot look upward or appear to be staring downward (sunset sign)
    • Developmental delays
    • Irritability or abnormal sleepiness
    • Poor feeding
    • Vomiting

    What is the long-term outlook?

    Most infants with simple, nonprogressive ventriculomegaly in the absence of brain damage or developmental anomalies will have completely normal and healthy development. Likewise, infants with hydrocephalus who do not have other serious brain anomalies or damage have an excellent prognosis for normal development with timely treatment of the condition.

  • Tests

    The Neonatal and Congenital Anomaly Neurosurgery Program at Boston Children’s Hospital was created to diagnose ventriculomegaly and other neurologic congenital anomalies as early as possible. We know that an early and accurate diagnosis  gives newborns the chance for optimal treatment and recovery. As always, we will be in close communication with you and your family every step of the way.

    How is ventriculomegaly diagnosed?

    Ventriculomegaly can be detected during pregnancy, on prenatal tests, or after the baby is born.

    Prenatal tests

    The different types of prenatal tests used to diagnose ventriculomegaly include:

    • Fetal ultrasounds are used to diagnose hydrocephalus as well as other conditions when a baby is still in the womb. This diagnostic imaging technique uses high-frequency sound waves and a computer to create images of blood vessels, tissues and organs. The evaluation provides general information about the size and position of fetal organs and how they are functioning. We perform fetal imaging services for pregnant women who are referred to the Advanced Fetal Care Center (AFCC)at Boston Children’s.
    • Amniocentesis, a prenatal diagnostic method in which a needle is inserted through the mother’s abdomen and into the amniotic sac that surrounds the baby. Amniocentesis is most often used to detect chromosomal abnormalities, which fetuses with ventriculomegaly might have. This test is usually done in the second trimester.

    Testing after birth

    The following tests can be used to diagnose hydrocephalus in infants and older children who have ventriculomegaly:

    • Magnetic resonance imaging (MRI): This imaging procedure uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of the brain without exposing the child to x-rays.

    What should I do after my unborn child is diagnosed with ventriculomegaly?

    Once the imaging studies are completed, radiologists in the Advanced Fetal Care Center (AFCC) at Children’s will review them with a team of nursing staff and pediatric specialists.

    Following this review, you will meet with the members of the multidisciplinary team to go over the results, discuss what the findings mean for the fetus for the remainder of the pregnancy and review expected outcomes after birth related to surgery, hospitalization and long-term health. During this time, you will also have the opportunity to review the images and ask questions.

    To schedule an appointment or consultation at the Advanced Fetal Care Center, please use our online request form or contact us at:

    Phone:  617-355-3896

    Email: afcc@childrens.harvard.edu

  • Clinicians in the Neonatal and Congenital Anomaly Neurosurgery Program at Boston Children's Hospital are experienced in treating hydrocephalus. When surgery is necessary, we use minimally invasive techniques to avoid placement of a shunt whenever we can. Our experts are highly experienced in all of these procedures, and will work with you and your family to determine which approach best suits your child.

    How is ventriculomegaly treated?

    Ventriculomegaly only needs to be treated if a child also has hydrocephalus, a term that describes any number of problems that cause cerebrospinal fluid (CSF) to either build up abnormally in the brain.

    Hydrocephalus may be treated by:

    • shunt placement :a process in which a small tube is implanted while the child is under anesthesia . This provides continual internal drainage of cerebrospinal fluid from the ventricles of the brain into the  abdominal cavity.
    • endoscopic third ventriculostomy (ETV): This minimally invasive procedure creates an opening in the floor of the third ventricle in the brain, allowing the entrapped fluid to escape from the ventricles into its normal circulation pathway.
    • combined endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC), a treatment developed by Benjamin C. Warf, MD,director of the Neonatal and Congenital Anomaly Neurosurgery Programat Children's. This technique is effective for infants, in whom ETV alone is not as successful as in older children. CPC reduces the rate of fluid production, while ETV restores the normal fluid circulation.

    Coping & support

    We know that this information about ventriculomegaly may be anxiety-provoking and even overwhelming. Remember that it's normal and acceptable to go through a range of emotions when your child has a medical condition. Fortunately, there is a lot of help available to you.

    This list outlines some of the resources in the wider community that may be helpful to you:

    Outside resources:

    Please note that neither Boston Children's Hospital, the Hydrocephalus Program nor the Neonatal and Congenital Anomaly Neurosurgery Program at Children's unreservedly endorses all of the information found at the sites listed below.

    • HyFI: Hydrocephalus Foundation, Inc. is a non-profit organization dedicated to providing support and educational resources for children and families affected by hydrocephalus.
    • Hydrocephalus Association is a non-profit organization dedicated to providing support and educational resources for children and families affected by hydrocephalus, and also promotes new areas of research.
  • Research & Innovation

    At Boston Children’s Hospital, important advances are being made in the treatment and research of ventriculomegaly, hydrocephalus and other disorders of the brain, spinal cord and nervous system

    Changing the delivery of care for children with hydrocephalus

    A new approach to brain surgery for hydrocephalus offers an alternative to the risky standard treatment of installing a permanent shunt. Learn how Benjamin C. Warf, MD,director of the Neonatal and Congenital Anomaly Neurosurgery Program at Children’s, is working to change the delivery of care for children with hydrocephalus.

    Children’s neurosurgeon Benjamin Warf, MD, brings life-saving care to Uganda

    Dr. Warf is also training pediatric neurosurgeons in Africa in hiscombined endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) technique. Learn more about how his innovative surgical procedure is improving hydrocephalus treatment in developing countries like Uganda.

    Creating noninvasive ways to measure intracranial pressure

    Thanks to a grant from the Boston-based nonprofit Center for Integration of Medicine and Innovative Technology (CIMIT), Children's neurosurgeon Joseph Madsen, MD, is creating a system to noninvasively measure pressure inside the skulls of patients with  hydrocephalus, head injuries, subarachnoid hemorrhage and other related conditions. His goal is to create a portable device that can be used by emergency technicians, as well as by medics in battlefield situations.

    Learn more about scientific research at Children’s.

    Find out about clinical trials happening at Children's.

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