Overview

Hydrocephalus is a medical condition characterised by an abnormal accumulation of cerebrospinal fluid (CSF) in the brain.¹ This accumulation can lead to pressure which causes various health and neurological complications.¹ While hydrocephalus can occur at any age, it often begins in infancy or early childhood, but can also be developed by adults.² For individuals assigned female at birth (AFAB)with hydrocephalus who are pregnant or planning to get pregnant, there are some special things to consider to ensure a healthy pregnancy and baby. This article aims to provide a comprehensive overview of these considerations, offering guidance on managing hydrocephalus during pregnancy to ensure the best possible outcomes for both parent and child.

Understanding hydrocephalus 

• Congenital hydrocephalus is a type of hydrocephalus that is present from birth. It is a complicated interplay between genetic and environmental variables that occurs throughout foetal development and results in congenital hydrocephalus² 
• When the cerebrospinal fluid (CSF), which envelops the brain and spinal cord, accumulates inside the brain it causes hydrocephalus 
• This accumulation leads to an increase in intracranial pressure, resulting in symptoms such as headaches, nausea, blurred vision, and difficulty thinking or moving³

How is hydrocephalus diagnosed?

• Hydrocephalus is typically diagnosed through imaging techniques such as ultrasound, MRI, or CT scans⁴ 
• The ultrasound will show pockets of fluid in the developing brain of the baby, which may indicate the presence of hydrocephalus⁴ 
• This condition can be detected as early as the last part of the first trimester (13 weeks). Around 20 to 24 weeks abnormalities can be more clearly detectable⁵ 

How is hydrocephalus treated?

• The goal of treatment is to reduce the pressure inside the brain by draining the excess cerebrospinal fluid which often involves surgical intervention⁶ 
• The most common form of treatment is a shunt, which is a tiny tube that is used to transfer excess fluid from the brain to another area of the body where it can be absorbed⁷
• Another therapeutic approach involves performing an endoscopic third ventriculostomy (ETV) operation, which creates a new channel for the CSF fluid to exit the brain and flow freely⁸ 

Pregnancy considerations for AFAB individuals with hydrocephalus

• AFABswho have hydrocephalus must carefully plan and manage their pregnancy 
• The main issues include the consequences of elevated intracranial pressure, the operation of shunt systems, the influence of drugs, and the possibility of hydrocephalus being passed down via families
• Preconception counselling is crucial for AFABs with hydrocephalus. This involves a thorough evaluation by a multidisciplinary team, including neurologists, neurosurgeons, obstetricians, and genetic counsellors⁹ 

Key aspects of preconception counselling include:

Assessment of shunt functionality

• Ensuring that the shunt is functioning properly before conception is critical. This may involve imaging studies and clinical evaluations to check for any signs of shunt malfunction or infection¹⁰

Genetic counseling

• Since some forms of hydrocephalus can be hereditary, genetic counselling can help assess the risk of transmission to the offspring. This can also provide information about potential genetic testing and options for prenatal diagnosis¹¹

Medication review

• Reviewing and adjusting medications that manage symptoms of hydrocephalus is essential. Some medications may need to be altered or discontinued to avoid potential teratogenic effects on the developing foetus

Managing pregnancy with hydrocephalus 

Once pregnant, continuous monitoring and specialised care are required to manage hydrocephalus effectively. Some things to consider during pregnancy include:

Regular monitoring of shunt function

• It's important to have frequent check-ups to make sure your shunt is still working properly and to monitor your brain pressure. If you experience headaches, nausea, or changes in vision, contact your doctor right away

Management of intracranial pressure

• Pregnancy can sometimes exacerbate symptoms of hydrocephalus due to fluid retention and increased blood volume. Monitoring intracranial pressure and managing symptoms through medical or surgical interventions may be necessary

Collaborative care

• A collaborative approach involving obstetricians, neurologists, and neurosurgeons is essential for managing both pregnancy and hydrocephalus. This team can address any complications promptly and ensure coordinated care throughout pregnancy

Delivery and postpartum considerations 

Deciding how to deliver your baby and taking care of yourself after birth are important considerations:

Mode of delivery

• The decision between vaginal delivery and cesarean section (C-section) should be made on a case-by-case basis 
• While many AFAB individuals with hydrocephalus can safely have a vaginal delivery, a C-section may be recommended in cases where there are concerns about increased intracranial pressure or shunt functionality¹²

Postpartum monitoring

• After delivery, careful monitoring of the parent is crucial. The physical strain of childbirth can potentially impact the function of the shunt, and postpartum hormonal changes can also affect intracranial pressure

Breastfeeding and medication

• Decisions about breastfeeding should consider the safety of medications used to manage hydrocephalus. Some medications may be excreted in breast milk and could affect the newborn

Case study and research findings 

Several case studies and research findings shed light on how pregnant AFAB individuals with hydrocephalus should be managed. These studies highlight the value of tailored care and the positive results that can be obtained with effective management.

Case study: Successful management of pregnancy-associated hydrocephalus with VP shunt ¹²

• A 28-year-old AFAB individual, pregnant with their second child, came to the clinic at 8 weeks pregnant with headaches, vomiting, and worsening vision. An eye exam showed swelling and damage to their optic nerve. They had no history of brain infections or injuries. A brain scan revealed they had hydrocephalus. Initially, they were treated with bed rest and medications, but these did not help
• At 13 weeks pregnant, they had surgery to insert a VP shunt, a device that helps drain the excess fluid from the brain. They received antibiotics during the surgery, which went smoothly, and their symptoms gradually improved afterwards. Their eye condition also got better
• At 38 weeks pregnant, they had a cesarean section due to their previous C-section and the baby's umbilical cord being wrapped around its neck. The C-section was done under epidural anaesthesia after confirming they had no signs of increased pressure in their brain. The delivery went well, and they had a healthy baby. They received more antibiotics for two days after delivery
• Six months later, a follow-up brain scan showed that their brain ventricles had decreased in size, indicating the shunt was working properly

Recommendations for pregnant AFAB individuals hydrocephalus

For AFABswith hydrocephalus, the following recommendations can help manage pregnancy effectively:

Seek preconception counseling

• Engage with a multidisciplinary team to assess shunt functionality, review medications, and discuss genetic risks before attempting to conceive

Regular monitoring during pregnancy

• Schedule frequent check-ups to monitor shunt function and intracranial pressure. Be vigilant for any signs of shunt malfunction or increased intracranial pressure

Summary

AFAB individuals with hydrocephalus who are pregnant or planning to become pregnant face unique challenges that require careful planning and management. Preconception counselling, regular monitoring of shunt function, management of intracranial pressure, and a collaborative approach to care are essential components of ensuring a safe pregnancy and delivery. By addressing these considerations, AFABs with hydrocephalus can achieve successful pregnancy outcomes and minimize risks to both themselves and their babies.