Pediatric hydrocephalus is a condition where excess cerebrospinal fluid (CSF) accumulates within the brain’s ventricles. This buildup increases pressure inside the skull, which can adversely affect brain development in children. In infants, this pressure may cause the skull bones to expand, making the head appear larger.
Understanding Pediatric Hydrocephalus
Cerebrospinal fluid (CSF) is a clear liquid continuously produced by the brain. It circulates around the brain and spinal cord, performing several functions, including delivering nutrients, removing waste products, and cushioning these delicate structures. CSF flows through a network of ventricles and channels within the brain before being absorbed into the bloodstream.
A disruption in the normal production, circulation, or absorption of CSF leads to its accumulation. This imbalance causes the ventricles to widen, putting harmful pressure on brain tissues. This pressure can impair brain function and, in severe instances, lead to brain damage.
Hydrocephalus can arise from various factors, categorized as congenital or acquired. Congenital hydrocephalus is present at or shortly after birth, often due to developmental irregularities of the nervous system. Examples include neural tube defects like spina bifida, and aqueductal stenosis, a narrowing of the passages connecting the brain’s ventricles.
Acquired hydrocephalus develops later in life due to factors like bleeding within the brain, such as intraventricular hemorrhage in premature babies. Infections like meningitis, brain tumors, or injuries can also obstruct CSF flow or impair its absorption, leading to the condition.
Identifying Hydrocephalus in Children
The signs and symptoms of hydrocephalus vary with a child’s age, reflecting their skull’s capacity to accommodate increased pressure. In infants, whose skull bones have not yet fused, common indicators include a rapid increase in head circumference or an unusually large head. A bulging or full soft spot (fontanelle) on the top of the head and visible scalp veins are also frequent signs.
Infants may also exhibit symptoms such as irritability, a high-pitched cry, poor feeding, or projectile vomiting. Sleepiness or reduced alertness can occur, and some infants may have seizures or show eyes fixed downward, sometimes called “sun setting” eyes. Developmental delays, such as a slowing or loss of progress in walking or talking, can also be observed.
For older children, whose skull bones have fused, the symptoms are more related to increased intracranial pressure rather than head enlargement. Headaches, blurred or double vision, and nausea or vomiting are common complaints. They might also experience problems with balance, poor coordination, or a decline in school performance.
Diagnosing hydrocephalus involves a thorough physical examination and a neurological assessment. Imaging techniques then confirm the diagnosis and visualize brain structures. Ultrasound is often used for infants, while computed tomography (CT) scans and magnetic resonance imaging (MRI) scans are commonly employed for all age groups to assess ventricle size and identify any obstructions.
Treatment Strategies
Treatment for pediatric hydrocephalus involves surgical interventions to divert or reduce cerebrospinal fluid (CSF). One common procedure is shunt placement, where a shunt is implanted to reroute excess CSF from the brain to another part of the body, usually the abdominal cavity, where it can be absorbed. A shunt consists of a catheter in a brain ventricle, a valve regulating fluid flow and pressure, and another catheter carrying fluid to its destination.
Different types of shunts exist, but the most common is the ventriculoperitoneal (VP) shunt, which drains CSF into the peritoneum (abdominal cavity). Shunts continuously drain CSF, maintaining a more normal pressure within the brain. While effective, shunts can have complications like malfunction or infection, which may necessitate surgical revision.
Another surgical option for specific cases is endoscopic third ventriculostomy (ETV). This procedure creates a small opening in the floor of the third ventricle, allowing CSF to bypass an obstruction and flow more freely within the brain’s natural pathways. ETV is considered for obstructive forms of hydrocephalus with a blockage in the CSF pathway.
ETV offers an alternative to shunt placement for some children, potentially reducing the need for an implanted device. However, ETV’s suitability depends on the specific cause and location of the CSF obstruction. Both shunt placement and ETV aim to alleviate pressure on the brain, with the most appropriate procedure chosen based on individual patient characteristics and the underlying cause of hydrocephalus.
Life with Pediatric Hydrocephalus
Children with pediatric hydrocephalus require ongoing medical management and regular follow-up care. This involves scheduled check-ups with neurosurgeons and neurologists to monitor shunt function, assess neurological development, and address any emerging concerns. Shunt revisions, surgical procedures to repair or replace a malfunctioning shunt, may be necessary throughout a child’s life.
Despite the need for continuous medical oversight, many children with hydrocephalus can lead full and productive lives with appropriate care. The long-term outlook is highly variable, depending on the cause of the hydrocephalus, the extent of any brain damage, and the effectiveness of treatment. Early diagnosis and timely intervention contribute to better outcomes.
A multidisciplinary team supports children with hydrocephalus, addressing various aspects of their development. This team may include neurosurgeons for surgical management, neurologists for ongoing brain health, and physical, occupational, and speech therapists to assist with developmental milestones. Educational specialists and social workers can also provide support for academic and social integration, helping children reach their full potential.