Hydrocephalus in Pregnancy

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    Hydrocephalus in Pregnancy

    Pregnancy is a joyous time for a woman. However, receiving the news that your developing baby has hydrocephalus can be overwhelming, frightening, and sad for expectant women and couples. Know that you’re not alone. We provide programs and support services to help you navigate your child’s journey with hydrocephalus. Through our HydrocephalusCONNECT program, Community Networks, National Conference on Hydrocephalus (HA CONNECT), and WALKs to End Hydrocephalus you can connect with other parents, volunteers, and medical professionals to help build a strong support system.

    Why do Babies Develop Hydrocephalus?

    Hydrocephalus is an abnormal accumulation of fluid in cavities called ventricles inside the brain. Hydrocephalus that is present at birth is referred to as congenital hydrocephalus. Congenital hydrocephalus is caused by a complex interaction of genetic and environmental factors during fetal development. The most common causes of congenital hydrocephalus are:

    • Spina bifida
    • Aqueductal stenosis
    • Brain malformations

    How is Hydrocephalus Diagnosed in Pregnancy?

    Hydrocephalus can be detected using a normal ultrasound examination, which is typically performed throughout a woman’s pregnancy. During ultrasound screening, the technician may see pockets of fluid in the developing brain, indicating enlarged ventricles and the possible presence of hydrocephalus.

    Hydrocephalus can be detected as early as the latter part of the first trimester. Diagnosis has been made as early as 13 weeks. Around 20 to 24 weeks, abnormal dilation of the ventricles are more clearly detectable.

    In certain special cases, fetal magnetic resonance imaging (MRI) may be done, especially when abnormal anatomy is observed with ultrasonography. The MRI provides more detailed images of the brain.

    For more detailed information on Hydrocephalus, visit About Hydrocephalus.

    How is Hydrocephalus Treated in Pregnancy?

    Determining the proper treatment and when to intervene depends on the cause of your baby’s hydrocephalus. During pregnancy, the main treatment method is observation. Throughout the pregnancy, it is important to have a series of ultrasounds to monitor ventricular size. The ultrasound study is a determination of the fetus and the ventricles at that particular time. There is no way to predict whether the ventricular size will increase or decrease in subsequent studies.

    It is generally felt that if ventricular size remains stable, the fetus should be followed to term. If progressive ventriculomegaly is demonstrated (that is, if the ventricles continue to enlarge), action may be taken sooner. Depending on gestational age and assessment of the viability, several options are considered.

    • For fetuses older than 35 weeks, a preterm cesarean section may be performed, and a shunt may be placed postnatally (after birth).
    • For fetuses younger than 32 weeks, the risk of pulmonary (lung) immaturity must be weighed against the potential damage of progressive ventriculomegaly and early delivery.
    • For fetuses with a diagnosis of spina bifida, pre- or post-natal surgery is an option to close the baby’s back before or right after birth. To learn more about pregnancy and spina bifida, visit the Spina Bifida Association’s Expectant Parents Guide.

    Once hydrocephalus in pregnancy is suspected, establishing a relationship with a pediatric neurosurgeon as early as possible is critical. Your baby may need to undergo treatment quickly after birth. Currently, there are three life-saving treatment options that require brain surgery to manage hydrocephalus. A pediatric neurosurgeon will discuss these with you.

    Shunt System
    The most common treatment for hydrocephalus is a medical device called a shunt, a flexible tube, which is placed in the ventricular system of the brain and connected to a valve. A small hole called a burrhole is made in the skull and the tube is placed into the fluid-filled ventricles. The tube is left in the ventricle to allow the cerebrospinal fluid (CSF) to flow to another region of the body, most often the abdominal cavity, or heart, where the CSF can be absorbed into the vascular system. The tube from the brain is connected to a valve that helps to regulate how much fluid leaves the brain.

    Endoscopic Third Ventriculostomy (ETV)
    A second surgical treatment option is called an Endoscopic Third Ventriculostomy (ETV). This is typically used for children over the age of 2 with non-communicating (obstructive) hydrocephalus; hydrocephalus caused by a blockage in the brain like aqueductal stenosis. Similar to shunt surgery, a small hole called a burrhole is made in the skull and an endoscope is gently guided through the brain into one of the lateral ventricles. With the use of a camera, the endoscope then passes down into the third ventricle and punctures the membrane on the floor of the third ventricle. This creates an alternative pathway for CSF to flow out of the ventricles and around the brain. A shunt is not needed.

    Not everyone is a candidate for ETV treatment. Learn more, watch our video.

    ETV with Choroid Plexus Cauterization (CPC)
    The third treatment option involves an ETV with the addition of a procedure called choroid plexus cauterization (CPC). This treatment is primarily used in children under 2. Once inside the brain, the neurosurgeon uses a device to burn or cauterize choroid plexus tissue to reduce the amount of fluid being introduced into the ventricles. The choroid plexus is vascular tissue within the ventricles of the brain and is the source of CSF production. The fluid then passes normally through the opening made during the ETV and into the subarachnoid space surrounding the surface of the brain. Not everyone is a candidate for ETV/CPC treatment. ETV/CPC is a very technical surgery and should be performed by a neurosurgeon trained and experienced in the procedure.

    With early detection and effective treatment, the outlook for children with hydrocephalus is promising. Many people with hydrocephalus lead normal lives with few or any limitations. Research and experience show that children with hydrocephalus have excellent opportunities to attain their full potential through programs that stimulate their development.

    To find a pediatric neurosurgeon, visit our Physician Directory.

    What is the Prognosis for Babies with Hydrocephalus?

    Many babies born with hydrocephalus go on to lead full lives, though the road you envisioned for your child might not be the road you journey down together. Hydrocephalus is a complex and unpredictable condition and parents caring and nurturing a baby and then a child with hydrocephalus need to prepare for the road ahead with careful education and planning. We also recommend a network of other hydrocephalus parents you can turn to for both information and friendship.

    So what can you expect? Each child is different and hydrocephalus can present varying degrees of cognitive and physical challenges based on the cause of the hydrocephalus and its effective management. It is not uncommon for babies who have shunts to need repeated shunt revisions in the first weeks, months, or years as their bodies adjust to the condition and treatment. Repeated brain surgeries can impact a baby’s normal development for many reasons including repeated intervention in the brain and time away from being able to grow and learn. Also, in addition to hydrocephalus, some infants may be diagnosed with other medical conditions including but not limited to seizures, cortical visual impairment, cerebral palsy, spina bifida, dandy-walker syndrome, and Chiari malformation, among others.

    Some general challenges we see in babies as they grow include, but are not limited to, delays in meeting milestones, sensory issues, problems with chewing and/or swallowing, eye-hand coordination, visual-spatial deficits, delayed speech, and delayed gross motor development like rolling over, crawling, and walking.

    Do not despair! Babies with hydrocephalus grow into resilient and determined children. With advances in medical treatments, therapies, educational support services, and an entire industry around toys and games to stimulate the brain and encourage learning, most children grow up to fulfill their dreams of making friends, playing sports, winning academic awards, graduating from high school, and going to college.

    For resources on raising a child with hydrocephalus visit, Caring for your Child.

    Research and Hydrocephalus

    As the leading private funder of hydrocephalus research in the country, we are investing in research to improve outcomes as well as prevent the development of hydrocephalus and, ultimately, find a cure. The Hydrocephalus Clinical Research Network (HCRN) is a network of children’s hospitals that conduct clinical research to improve treatment and care for babies and children with hydrocephalus.

    You can be a part of the research. Enroll in HAPPIER, the only hydrocephalus patient-powered registry in the country, and help our doctors and scientists better serve you!

    The Hydrocephalus Association regularly supports our scientists and doctors by assisting in gathering data through surveys as well as through helping enroll patients in current research studies.

    Information you can trust! This article was produced by the Hydrocephalus Association, copyright 2021. We would like to thank the following individuals for their valuable contributions and expert input: Marion L. (Jack) Walker, MD, and Cathy Cartwright, DNP, RN-BC, PCNS, FAAN.