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Hydrocephalus

What is Hydrocephalus?

Hydrocephalus is a medical condition characterized by the accumulation of excess cerebrospinal fluid (CSF) within the cavities (ventricular system) of the brain. CSF is a clear fluid that surrounds and cushions the brain and spinal cord, providing protection and nourishment. In hydrocephalus, the buildup of CSF can lead to increased pressure within the skull, potentially causing damage to brain tissue and impairing normal brain function.

Hydrocephalus in a child
What Might Cause Hydrocephalus in Children?

Hydrocephalus is typically caused by an imbalance between the production and absorption of cerebrospinal fluid (CSF) in the brain. When there is an abnormal accumulation of CSF within the brain's ventricles, it can lead to increased intracranial pressure and the development of hydrocephalus. The underlying causes of this imbalance can vary, and hydrocephalus can be congenital (present at birth) or acquired later in life. Here are some common causes of hydrocephalus:

  1. Congenital Causes:

    • Developmental Abnormalities: Structural abnormalities in the brain that affect the normal flow of CSF, such as aqueductal stenosis (narrowing of the passage connecting the ventricles), can lead to hydrocephalus.

    • Neural Tube Defects: Certain conditions that result in incomplete closure of the neural tube during fetal development, such as myelomeningocele (spina bifida), can be associated with hydrocephalus.

    • Dandy-Walker Syndrome: A rare congenital condition characterized by a cystic enlargement of the fourth ventricle, leading to hydrocephalus.

  2. Acquired Causes:

    • Infections: Infections of the brain or its surrounding tissues, such as meningitis or encephalitis, can cause inflammation and scarring that disrupt the normal flow and absorption of CSF.

    • Tumors: Brain tumors or other growths can block the flow of CSF or cause overproduction of CSF, leading to hydrocephalus.

    • Head Injuries: Traumatic brain injuries that result in bleeding or swelling can obstruct CSF flow or disrupt the normal balance between production and absorption.

    • Bleeding: Intraventricular or subarachnoid bleeding, often due to conditions like vascular pathologies, trauma, infection, inflammation , other causes can lead to hydrocephalus.

    • Vascular Malformations: Abnormal blood vessels in the brain can interfere with CSF circulation and contribute to hydrocephalus, as seen in vein of Galen malformation.

    • Idiopathic: In some cases, the specific cause of hydrocephalus may not be identified, leading to a diagnosis of idiopathic hydrocephalus.

Spina Bifida caused by Hydrocephalus
Tumor caused by Hydrocephalus
 What Symptoms does Hydrocephalus Cause in Children?

Hydrocephalus can present with a range of symptoms in children, which can vary depending on the age of the child, the rate of fluid accumulation, and the underlying cause of the condition. Early detection and treatment are crucial to prevent complications and promote healthy development. Common symptoms of hydrocephalus in children include:

Hydrocephalus Causing Enlarged Head Size, Bulging Fontenelles, and Sunset Eyes
Hydrocephalus Causing Vomiting in a Child
Hydrocephalus Causing Coordination Issues in a Child
  1. In Infants:

    • Enlarged Head Size: One of the most prominent signs of hydrocephalus in infants is an abnormally rapid increase in head circumference (macrocephaly). The head may appear disproportionately larger than the rest of the body.

    • Bulging Fontanelles: The fontanelles, or soft spots on the baby's head, may be tense, raised, or visibly bulging due to the accumulation of cerebrospinal fluid.

    • Irritability and Fussiness: Infants with hydrocephalus may display increased irritability, fussiness, or difficulty consoling.

    • Poor Feeding: Feeding difficulties can arise due to increased intracranial pressure.

    • Vomiting: Frequent vomiting, especially following feeding, can be a sign of elevated intracranial pressure.

    • Sunset Eyes: The baby's eyes may appear to be facing downward, which is often referred to as "sunsetting eyes." This can be indicative of increased pressure on the brainstem and cranial nerves.

    • Drowsiness: Decrease in mentation or activity level of the child in conjunction other symptoms can be a sing of hydrocephalus.

  2. In Older Children and Adolescents:

    • Headache: Children may complain of persistent or severe headaches, which can be a result of increased intracranial pressure.

    • Nausea and Vomiting: Nausea and vomiting, particularly upon awakening or with changes in head position, are common symptoms.

    • Visual Changes: Visual disturbances, such as blurred vision, double vision, or difficulty focusing, may occur due to pressure on the optic nerves.

    • Balance and Coordination Issues: Children may experience difficulties with balance, coordination, and motor skills. They may appear unsteady or have trouble walking.

    • Cognitive Changes: Cognitive impairments, memory problems, changes in concentration, and alterations in school performance may be observed.

    • Personality and Behavior Changes: Hydrocephalus can sometimes lead to changes in behavior, personality, or mood. Children may become irritable, withdrawn, or exhibit emotional changes.

    • Urinary Incontinence: In some cases, children with hydrocephalus may experience urinary incontinence or changes in urinary habits.

    • Regression of Milestones: Any regression in milestones prompt a medical workup. Hydrocephalus can present as a change in psychomotor or neurocognitive performace of a child.

How is Hydrocephalus Treated in Children?

Once your child’s has been diagnosed with hydrocephalus, I (as a pediatric neurosurgeon in New York) will work with you, your child, and other medical professionals (e.g., neurologists and pediatricians) at Children's Hospital at Montefiore (CHAM) to formulate a customized treatment strategy tailored to your child’s needs.

This customized treatment typically involves surgical intervention to address the underlying cause of the condition and to restore normal cerebrospinal fluid (CSF) flow and absorption. The primary goal of treatment is to relieve intracranial pressure, prevent further brain damage, and promote healthy brain development. The management and treatment of hydrocephalus depend on the underlying cause, the individual's age, and the severity of symptoms. Treatment often involves surgical intervention to address the cause of the fluid imbalance and restore normal CSF circulation. This may include the placement of a shunt to redirect CSF to another part of the body where it can be absorbed, or other surgical procedures to remove obstructions or correct underlying issues. Early diagnosis and appropriate treatment are crucial to prevent complications and promote healthy brain development. Here are the main treatment options:

  1. Surgical Shunt Placement: The most common and effective treatment for hydrocephalus in children is the surgical placement of a shunt. A shunt is a medical device that diverts excess CSF from the brain's ventricles to another part of the body where it can be absorbed and eliminated. The shunt typically consists of a one-way valve and a tube that is tunneled under the skin to a different body cavity, such as the abdomen (ventriculoperitoneal shunt), heart (ventriculoatrial shunt), pleura (ventriculopleural shunt), galea (subgaleal shunt), or gall bladder.

  2. Endoscopic Third Ventriculostomy (ETV): In some cases, particularly when the obstruction is at the level of the ventricular outlet or the aqueduct of Sylvius, an endoscopic procedure may be performed. During an ETV, a small hole is made in the floor of the third ventricle to create an alternate pathway for CSF to flow, bypassing the obstruction. ETV is a less invasive option compared to shunt placement and may be considered in certain cases.

  3. Surgical Correction of Underlying Cause: If hydrocephalus is caused by a specific structural abnormality, such as a tumor or cyst blocking CSF flow, surgical removal or correction of the underlying cause may be necessary.

  4. Monitoring and Adjustments: Children with shunts require ongoing monitoring to ensure proper function. The pressure settings of the shunt may need to be adjusted periodically, especially as the child grows. Regular medical follow-up appointments are important to assess shunt function and address any complications.

Hydrocephalus Treated by VP Shunt
Dr. Behbahani operating on a child having hydrocephalus

Early diagnosis and timely intervention are crucial for optimizing outcomes and promoting a child's overall well-being.

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