Severity & associated findings

Encephalocele and Hydrocephalus

Fluid, Pressure, and Shunts

· 2 min read · 305 words

In short: Encephalocele and Hydrocephalus

  • Hydrocephalus (excess CSF and pressure) is a common companion to encephalocele, especially occipital types.
  • It can be present at birth or develop later, sometimes after repair, so monitoring is ongoing.
  • It's associated with a more guarded outlook, but it's treatable.
  • Treatment is usually a shunt (often VP) or sometimes ETV.

One of the most common conditions associated with encephalocele is hydrocephalus — sometimes called "water on the brain." Understanding it helps you understand a big part of your child's care.

What hydrocephalus is

The brain makes cerebrospinal fluid (CSF) that normally flows around and through the brain and is reabsorbed. In hydrocephalus, fluid builds up, enlarging the fluid spaces (ventricles) and raising pressure inside the skull. In babies, this can cause a rapidly enlarging head, a bulging soft spot, vomiting, sleepiness, and downward-deviated eyes, among other signs.

Why it occurs with encephalocele

Encephaloceles — especially occipital ones — can disturb the normal flow or reabsorption of CSF, so hydrocephalus may be present at birth or develop afterward, sometimes after the encephalocele is repaired. That's why teams monitor closely before and after surgery.

Why it matters for outlook

The presence of hydrocephalus is associated with a more guarded outlook for encephalocele. Importantly, though, treating hydrocephalus is a well-established part of care, and managing it well is a key way the team protects the brain.

How it's treated

The most common treatment is a shunt — a thin tube that drains excess CSF from the brain to another part of the body (often the abdomen), where it's reabsorbed. This is called a ventriculoperitoneal (VP) shunt. An alternative in some children is an endoscopic third ventriculostomy (ETV), which creates a new internal drainage pathway. Your neurosurgeon will recommend the best option.

Timing with encephalocele surgery

Sometimes hydrocephalus is treated before or at the same time as the encephalocele repair; sometimes it appears afterward and is treated then. Untreated hydrocephalus can also stress the encephalocele repair, which is another reason close monitoring matters.

Sources

  • StatPearls (NCBI Bookshelf) — Encephalocele (hydrocephalus)
  • Cleveland Clinic — Encephalocele / Hydrocephalus
  • CHOP — Encephalocele
  • Child's Nervous System / peer-reviewed literature — hydrocephalus and outcome

Questions people ask

Will my baby definitely need a shunt??

Not necessarily — only if hydrocephalus is present or develops. Your team monitors for it. Is a shunt permanent? Many shunts are long-term; they can need revision over time. ETV is an alternative in some children. What signs should I watch for? Rapid head growth, bulging soft spot, vomiting, unusual sleepiness, or downward-looking eyes — report these promptly.

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