When hydrocephalus accompanies an encephalocele, a separate procedure may be needed to manage the fluid and pressure. This article explains shunt surgery and its alternative.
Why a separate procedure may be needed
Closing an encephalocele addresses the defect, but it doesn't always resolve hydrocephalus — and sometimes hydrocephalus appears or worsens after the repair, as CSF dynamics change. Managing this pressure protects the brain and the encephalocele repair itself.
The ventriculoperitoneal (VP) shunt
The most common treatment is a VP shunt — a thin, soft tube placed under the skin that drains excess CSF from the brain's ventricles to the abdomen, where the fluid is reabsorbed. A small valve controls the flow. The shunt is usually not visible from the outside once healed, though it can sometimes be felt under the skin.
The ETV alternative
For some children, an endoscopic third ventriculostomy (ETV) is an option. Instead of a tube, the surgeon creates a small opening inside the brain to allow CSF to drain along a new internal pathway, avoiding a permanent device. Whether ETV is suitable depends on the child's anatomy and the cause of the hydrocephalus; your neurosurgeon will advise.
Timing relative to encephalocele repair
Shunt or ETV timing varies:
- Before or at the time of encephalocele repair, if significant hydrocephalus is already present.
- After repair, if hydrocephalus develops once the defect is closed.
Close monitoring around surgery — head circumference, the soft spot, and signs of pressure — guides the timing.
Living with a shunt: what to watch for
Shunts are highly effective but can malfunction or get infected over time, sometimes needing revision. Parents are taught to watch for shunt-problem signs, which can include vomiting, unusual sleepiness or irritability, headache (in older children), a bulging soft spot, rapid head growth, fever, or redness along the shunt tract. Report these promptly — shunt problems need timely evaluation.
Sources
- StatPearls (NCBI Bookshelf) — Encephalocele (shunts for CSF management)
- Cleveland Clinic — Hydrocephalus / Encephalocele
- CHOP — Hydrocephalus / Encephalocele
- NINDS / NIH — Hydrocephalus