Congenital Hydrocephalus: Causes, Symptoms, and Treatment Options

Hydrocephalus, Congenital

Introduction

Hydrocephalus is a medical condition characterized by an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain. This excess fluid can put pressure on the brain and spinal cord, leading to a variety of symptoms, including:

• Increased head size
• Bulging fontanelle (soft spot) on the head
• Downward deviation of the eyes (sunset eyes)
• Vomiting
• Irritability
• Seizures
• Developmental delays

Congenital hydrocephalus is a type of hydrocephalus that is present at birth. It is the most common type of hydrocephalus, accounting for about 80% of cases.

Causes

The exact cause of congenital hydrocephalus is often unknown. However, it is thought to be caused by a combination of factors, including:

• Genetic factors: Some people are born with a genetic predisposition to hydrocephalus.
• Birth defects: Hydrocephalus can be caused by birth defects that block the flow of CSF.
• Infections: Infections during pregnancy or infancy can increase the risk of developing hydrocephalus.
• Head injuries: Head injuries can also lead to hydrocephalus.

Symptoms

The symptoms of congenital hydrocephalus can vary depending on the severity of the condition. Mild cases may not cause any noticeable symptoms, while severe cases can lead to life-threatening complications.

Common symptoms of congenital hydrocephalus include:

• Increased head size
• Bulging fontanelle (soft spot) on the head
• Downward deviation of the eyes (sunset eyes)
• Vomiting
• Irritability
• Seizures
• Developmental delays

Diagnosis

Congenital hydrocephalus is typically diagnosed based on the results of a physical examination and imaging tests.

• Physical examination: The doctor will look for physical signs of hydrocephalus, such as an enlarged head, bulging fontanelle, or downward deviation of the eyes.
• Imaging tests: Imaging tests, such as an ultrasound or MRI, can help to confirm the diagnosis of hydrocephalus. These tests can also help to determine the cause of the hydrocephalus and to assess the severity of the condition.

Treatment

The treatment for congenital hydrocephalus depends on the severity of the condition. Mild cases may not require any treatment, while severe cases may require surgery.

Treatment options for congenital hydrocephalus include:

• Observation: Mild cases of hydrocephalus may be monitored closely without treatment. The doctor will check the child’s head circumference and developmental progress regularly to make sure that the hydrocephalus is not getting worse.
• Medication: Medications can be used to reduce the production of CSF or to increase the absorption of CSF.
• Surgery: Surgery is the most common treatment for congenital hydrocephalus. The goal of surgery is to create a pathway for the CSF to flow out of the brain. There are several different types of surgery that can be used to treat hydrocephalus, including:
  • Ventricular shunting: This is the most common type of surgery for hydrocephalus. A shunt is a small tube that is placed in the brain to drain the excess CSF. The shunt is connected to a reservoir that is placed under the skin. The CSF flows from the brain into the reservoir and then out through the shunt.
  • Endoscopic third ventriculostomy (ETV): This is a less invasive type of surgery that is used to create a new pathway for the CSF to flow out of the brain. ETV is performed using a small camera and instruments that are inserted into the brain through a small incision in the skull.
  • Choroid plexus cauterization: This is a type of surgery that is used to destroy the choroid plexus, which is the part of the brain that produces CSF. Choroid plexus cauterization is performed using a laser or a heated probe.

Prognosis

The prognosis for congenital hydrocephalus depends on the severity of the condition and the type of treatment that is used. With early diagnosis and treatment, most children with congenital hydrocephalus can live full and healthy lives.

However, some children with hydrocephalus may have long-term problems, such as:

• Learning disabilities
• Developmental delays
• Speech problems
• Motor problems
• Seizures

Prevention

There is no sure way to prevent congenital hydrocephalus. However, some things that can help to reduce the risk of the condition include:

• Getting vaccinated: Vaccinations can help to prevent infections that can lead to hydrocephalus.
• Avoiding head injuries: Head injuries can increase the risk of developing hydrocephalus.
• Getting prenatal care: Prenatal care can help to identify and treat problems that could lead to hydrocephalus.