Congenital Hydrocephalus

Our Specialists for Congenital Hydrocephalus

Congenital hydrocephalus is a lifelong condition — not a one-and-done operation. The team that matters most is the one that will still be following your child five, ten, and twenty years from now. At UChicago Comer Children's, pediatric neurosurgeons with decades of experience in shunts, endoscopic third ventriculostomy, and revision surgery manage these children from infancy through adulthood.

Arthur J. DiPatri Jr., M.D.

Director, Pediatric Neurosurgery

Dr. DiPatri has been performing pediatric neurosurgery for over 27 years. He's dual board-certified in both neurological surgery and pediatric neurological surgery, and he's Chief of Pediatric Neurosurgery for the Chicagoland Children's Health Alliance, meaning he oversees pediatric brain care across Comer, Advocate, and Endeavor Health. He came to UChicago after 22 years at Lurie Children's Hospital, with additional fellowship training at Boston Children's Hospital. Dr. DiPatri has been managing pediatric hydrocephalus for more than 27 years — placing shunts, performing ETVs, and following those same children through revisions, growth, and adolescence. As Chief of Pediatric Neurosurgery for the Chicagoland Children's Health Alliance, he's the most likely person to operate on a baby with congenital hydrocephalus anywhere in the UChicago-Comer-Advocate-Endeavor network.

Paramita Das, M.D., M.S.

Director, Neurotrauma; Director, Neurosurgical Trauma Fellowship

Dr. Das is Director of Neurotrauma at UChicago's Level 1 Trauma Center and directs the Neurosurgical Trauma Fellowship, one of only a few dedicated neurosurgical trauma fellowships in the country. She manages both head trauma and acute spine pathology and has been named to the Bucksbaum-Siegler Institute for Clinical Excellence. She trained at the University of Minnesota for residency and completed a skull base fellowship at Cleveland Clinic. Dr. Das is a fellowship-trained pediatric neurosurgeon who rounds out the Comer team managing infants and children with hydrocephalus, including complex revisions and endoscopic third ventriculostomy. She works alongside Dr. DiPatri on shunt and ETV cases, and is one of the surgeons families see for long-term follow-up from infancy into school age.

What Is Congenital Hydrocephalus?

Your brain and spinal cord float in a clear fluid called cerebrospinal fluid, or CSF. It's made inside four connected chambers in the middle of the brain called ventricles, and it flows out of the brain and around the spinal cord before being reabsorbed into the bloodstream. In a healthy baby, the body makes about a half cup of fresh CSF every day and reabsorbs the same amount — a perfectly balanced system.

In hydrocephalus, that balance breaks down. Either the fluid can't get out of the ventricles because something is blocking it, or the body can't reabsorb it fast enough. CSF builds up, the ventricles swell, and pressure rises inside the skull. In a baby whose skull bones haven't fused yet, the head gets bigger; in an older child, the pressure has nowhere to go and causes headaches, vomiting, and sleepiness.

Congenital means the condition is present at or near birth. It affects about 1 in 1,000 babies born in the U.S., making it one of the most common reasons a newborn needs brain surgery. Some babies are diagnosed on prenatal ultrasound. Others are picked up in the NICU after a premature birth. And some aren't recognized until the first weeks at home, when parents or the pediatrician notice a head that's growing too fast.

Hydrocephalus is not a single disease — it's a final common pathway for many different underlying problems. The treatment and outlook depend heavily on why it developed in the first place.

What Does It Look Like?

Symptoms depend on your child's age, because a baby's skull can expand to accommodate pressure — an older child's can't.

In newborns and infants (soft spot still open)

• A head that's growing too fast — this is often the first sign. Pediatricians plot head circumference on a growth curve at every visit for exactly this reason.
• A bulging or tense soft spot (fontanelle) on the top of the head
• Veins on the scalp that look unusually prominent
• Eyes that appear to look down ("sunsetting sign")
• Poor feeding, vomiting, or irritability
• Sleepiness or difficulty waking
• Seizures, in some cases

In toddlers and older children (skull bones fused)

• Morning headaches, sometimes with vomiting
• Changes in vision or double vision
• Trouble with balance or walking
• Personality changes, sleepiness, or trouble concentrating
• A drop in school performance
• In babies with existing shunts: the same symptoms may signal that the shunt has stopped working

If your child has a shunt and suddenly develops headaches, vomiting, irritability, or unusual sleepiness, treat it as an emergency and call your neurosurgeon or go to an emergency room. Shunt malfunction can become life-threatening within hours.

How Is It Diagnosed?

Diagnosis starts with imaging of the brain — the specific test depends on your child's age.

Before birth

Prenatal ultrasound can often pick up enlarged ventricles (called ventriculomegaly) as early as the second trimester. If hydrocephalus is suspected, a fetal MRI is usually done next — it gives a much more detailed look at the brain and can identify specific causes like aqueductal stenosis, Dandy-Walker malformation, or spina bifida.

In newborns and young infants

Because the soft spot is still open, we can use it as a window: a cranial ultrasound through the fontanelle is quick, safe, and requires no sedation. This is the first test for most premature babies in the NICU with bleeding in the brain. If more detail is needed, a fast brain MRI — designed specifically for infants and usually done without sedation — gives a much clearer picture.

In older children

An MRI of the brain is the standard. It shows the ventricles, any blockages, and the anatomy of the flow pathways. Special MRI sequences can actually show CSF moving — which helps us figure out whether an ETV is likely to work. A CT scan is sometimes used in emergencies or to check shunt placement, but MRI is preferred for planning treatment because it avoids radiation.

Beyond imaging, your child will be examined by a pediatric neurosurgeon, and often a developmental pediatrician and genetic specialist — because the cause of hydrocephalus points to what else needs to be watched.

Types of Congenital Hydrocephalus

Hydrocephalus gets sorted two different ways — by how the fluid is backing up (communicating vs. non-communicating) and by why it developed (the underlying cause). Both matter for treatment.

Communicating vs. non-communicating

• Non-communicating (obstructive) hydrocephalus — there is a physical blockage somewhere inside the ventricular system, so CSF can't flow out. The classic example is aqueductal stenosis, where the narrow channel between the third and fourth ventricles is too tight. This type is often a good candidate for ETV, because we can bypass the blockage without putting in hardware.
• Communicating hydrocephalus — the fluid flows out of the ventricles just fine, but the body can't reabsorb it downstream (usually across the membranes over the brain's surface). Bleeding from prematurity and infection are the most common causes. This type usually needs a shunt, though ETV with choroid plexus cauterization can succeed in select infants.

By underlying cause

• Post-hemorrhagic hydrocephalus of prematurity (PHH) — the most common cause in the U.S. Babies born very premature can bleed into their ventricles (intraventricular hemorrhage, or IVH); the blood products scar the fluid-absorbing membranes, and fluid backs up. Higher IVH grade (grade III or IV) carries the highest risk. These are often the most medically complex children we treat.
• Aqueductal stenosis — a narrowing of the cerebral aqueduct, often present before birth. May be isolated or run in families (X-linked hydrocephalus from L1CAM gene mutations). ETV has a strong track record for these children.
• Dandy-Walker malformation — a developmental malformation of the cerebellum and fourth ventricle. Often diagnosed prenatally. Usually requires a shunt, sometimes with additional surgery to the cyst.
• Myelomeningocele-associated hydrocephalus — roughly 80% of babies born with open spina bifida also develop hydrocephalus, usually linked to a Chiari II malformation crowding the back of the brain. Fetal surgery for spina bifida reduces the need for a shunt, and ETV with choroid plexus cauterization has become a good alternative to shunting in many centers.
• Post-infectious hydrocephalus — follows meningitis or in-utero infection (such as CMV or toxoplasmosis). More common in low-income countries but still seen here.
• Genetic and syndromic causes — increasing numbers of congenital hydrocephalus cases are linked to specific gene mutations, which is why genetic testing is becoming part of the workup.

How Is It Treated?

Hydrocephalus is a plumbing problem, and there's no medication that fixes it. Treatment is surgical and comes in two main flavors: shunt the fluid out of the brain to somewhere else in the body, or create a new pathway inside the brain so the fluid can escape on its own.

Ventriculoperitoneal (VP) shunt — the workhorse

A VP shunt is the most common treatment for hydrocephalus in children and has been the standard for more than 60 years. It's a soft silicone tube with three parts: a ventricular catheter that sits inside one of the ventricles, a valve that controls how fast fluid drains, and a distal catheter that runs under the skin, down the neck and chest, and into the abdomen — where the body reabsorbs the extra fluid. The whole system is invisible under the skin once it's in.

Shunts work, and they work immediately. They're also the right answer for most babies with post-hemorrhagic or communicating hydrocephalus. But they have two big downsides that every family needs to understand up front:

• Shunts fail. Roughly half of all shunts placed in children need a revision within 2 years, and most children will need several revisions across their lifetime. The tubing can clog, break, or get disconnected; the valve can malfunction.
• Shunts can get infected — historically in 8-10% of surgeries, now closer to 5% at centers using standardized infection prevention protocols. An infected shunt has to be removed, the infection treated with antibiotics, and a new shunt placed.

That's why where — and with whom — your child has shunt surgery matters. Centers that participate in the Hydrocephalus Clinical Research Network (HCRN) follow a standardized shunt insertion protocol shown to significantly cut infection rates.

Endoscopic third ventriculostomy (ETV)

ETV is a different approach entirely. Instead of implanting hardware, the neurosurgeon uses a small endoscope — a tiny camera on a wand — to make a small opening in the floor of the third ventricle. CSF then drains through this new hole into the natural fluid spaces around the brain, where it can be reabsorbed normally. No tubing, no valve, no permanent implant.

ETV works beautifully for the right patient — typically an older child with non-communicating hydrocephalus from aqueductal stenosis or a tumor. But it doesn't work for everyone. Success depends on your child's age, the cause of their hydrocephalus, and whether they've had a shunt before. This is captured by the ETV Success Score (ETVSS), a tool developed by Dr. Kulkarni and colleagues that predicts the chance of ETV success before you even do the surgery.

ETV with choroid plexus cauterization (ETV/CPC)

For infants — historically considered poor ETV candidates — Dr. Benjamin Warf developed a variation in Uganda called ETV with choroid plexus cauterization (ETV/CPC). During the same endoscopic surgery, the surgeon uses a small electrocautery wand to shrink the choroid plexus, the tissue that produces CSF inside the ventricles. The combination reduces production and creates a new drainage path at the same time. This technique has transformed care for babies with hydrocephalus in sub-Saharan Africa — where shunts are dangerous because families live far from specialized centers — and it's now used selectively in the U.S. for infants with aqueductal stenosis, myelomeningocele, and Dandy-Walker malformation.

Treating the underlying problem

Some causes of hydrocephalus have their own specific treatments. Fetal surgery to close a spina bifida defect before birth (MOMS trial) reduces the risk of needing a shunt by about half. A tumor blocking CSF flow can sometimes be removed to fix the hydrocephalus. A symptomatic Dandy-Walker cyst may need its own drainage procedure.

What to expect after surgery

Most babies who have a shunt or ETV go home within 2-4 days. They need lifelong follow-up with pediatric neurosurgery — typically an office visit and imaging every 6-12 months in the first few years, then less often as they stabilize. Developmental evaluations and school-age assessments are part of the long game: many children with congenital hydrocephalus need extra help with learning, attention, or motor skills, and the earlier that's identified, the better.

What Are the Outcomes?

The outcomes from congenital hydrocephalus vary enormously based on the underlying cause, the child's age at treatment, and complications along the way. Two things matter most: how the shunt or ETV holds up over time, and how the child's brain develops.

How long do shunts last?

Shunt survival is sobering. Large prospective data from the Hydrocephalus Clinical Research Network (HCRN) show that roughly 40-50% of first-time shunts fail within 2 years of placement. Infants under 6 months, those with prior infections, and those with complex medical problems are at the highest risk. Most children will have multiple revisions across a lifetime — the reality of living with a shunt is that it's not a cure, it's an ongoing partnership with a neurosurgical team.

ETV success rates by ETV Success Score

The ETV Success Score, built from data on hundreds of children at 12 international centers, predicts 6-month ETV success based on three factors: age, cause of hydrocephalus, and whether a shunt was placed previously. Here's roughly how it shakes out:

Adding choroid plexus cauterization (CPC) pushes ETV success rates higher in infants, particularly those with aqueductal stenosis and myelomeningocele. In Dr. Warf's landmark prospective series, ETV/CPC succeeded in roughly 65-75% of carefully selected infants, comparable to shunt in many causes. A randomized trial in Ugandan infants with post-infectious hydrocephalus found no significant difference in 12-month cognitive outcomes between ETV/CPC and shunt.

Developmental outcomes

Most children with treated hydrocephalus attend regular schools, though many need support services. Developmental outcomes are shaped far more by the underlying cause than by which operation was done:

• Children with isolated aqueductal stenosis treated early often have near-normal development
• Children with post-hemorrhagic hydrocephalus of prematurity face higher rates of cerebral palsy, learning differences, and visual issues — driven by the underlying brain injury, not the shunt itself
• Children with myelomeningocele generally have normal intelligence but often need help with attention, executive function, and math
• Shunt infection is an independent predictor of worse cognitive outcomes, which is why infection prevention is non-negotiable

The most important thing you can do for your child's long-term outcome is choose a pediatric neurosurgery program that will stay with you — one that sees enough hydrocephalus to keep revision numbers down, infection rates low, and follow-up consistent from infancy through the teenage years.

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