Overview
What is hydrocephalus?
The term hydrocephalus is derived from two Greek words hydro means water and cephalus means head.1 It is a rare condition in which excess cerebrospinal fluid (CSF) builds up within or around the brain.4 CSF is a fluid present between the meninges (layers of the brain) and it has a vital role in protecting the brain and spinal cord from external shock. Hydrocephalus is caused by the increased production and reabsorption of cerebrospinal fluid within the brain.4 It can occur at any age but mainly happens in infants and older people.2
Hydrocephalus in infants often occurs as a result of brain injury during vaginal delivery. Increased pressure from CSF damages brain tissue as a result the baby's brain can not work properly or even death may occur if it is not treated on time.3 In the US two out of 1000 infants are affected by hydrocephalus and the chances of survival is only 50%.1
The main purpose of this article is to create awareness among common people to understand this condition and start treatment before it's too late. Furthermore, this article gives a brief review of the diagnosis, treatment options and management of hydrocephalus. Surgeries maintain a balanced production of cerebrospinal fluid in the brain and therapies aid in managing symptoms of hydrocephalus.2
Types of hydrocephalus in infants
In infants mainly hydrocephalus are of two types:
- Congenital hydrocephalus
- Acquired hydrocephalus
- Congenital hydrocephalus: it means hydrocephalus present by birth. Different genetic and environmental factors contributed to congenital hydrocephalus when the baby was present in the mother's womb1
- Acquired hydrocephalus: it occurs after birth and it is caused by head injury or trauma1
It is also classified as communicating and non-communicating hydrocephalus.
- Communicating hydrocephalus: In communicating hydrocephalus when CSF leaves the ventricle, it obstructs the site of the brain. It is called communicating because CSF flows through the ventricles1
- Non- communicating hydrocephalus: It is also known as obstructive hydrocephalus. The cerebrospinal fluid causes obstruction when it flows through the ventricle1
Diagnosis of hydrocephalus in infants
Diagnosis is based on two things:
- Symptoms
- Diagnostic test (brain-imaging or other diagnostic test)3
Clinical presentation of hydrocephalus in infants
Symptoms of hydrocephalus in infants are as follows:3
- Enlargement of baby’s head
- Soft spot present at the top of the head
- Vomiting and nausea
- Irritability and fussiness
- Sleepiness or extreme tiredness
- Not able to feed properly
- Always looking at downward position (also known as sunsetting of eyes)
- Delayed development
- Seizures3
Diagnostic test to detect hydrocephalus
Doctors may perform different diagnostic techniques to detect this condition. It can be diagnosed before the birth of a baby during pregnancy or a test may be performed when an infant shows the symptoms.5
Brain scanning test
- Ultrasound: ultrasound shows an image of the presence of fluid in the brain’s ventricle with the help of sound waves. It has no effects on a child's brain and it is a painless technique, waves easily penetrate the brain and show clear structure5
- MRI-scan: In MRI scans magnetic fields and radio-frequency waves are used to create an image of the brain’s structure, its tissues, brain malfunction, fluid-filled ventricles, and detect the intracranial pressure. Some procedures may require mild sedation5
- CT-scan: It is the most reliable diagnostic test in which X-rays are passed through the patient. It gives a clear picture of brain structure, ventricle, CSF accumulation. This procedure can be performed without sedation5
Other diagnostic tests
- Lumbar puncture test: a sample of cerebrospinal fluid is taken from the spinal cord to check the pressure6
- Lumbar drainage test: a tube is inserted between the bones of the spinal cord to drain a large quantity of fluid. It is performed under local anaesthesia6
- Lumbar infusion test: fluid is infused into the lower part of the spinal cord and checks its pressure and absorption6
Complications
Less severe hydrocephalus is usually treated by surgery but if it progresses to an advanced stage it can lead to permanent brain damage, and physical and mental retardation in infants. Complications arise due to hydrocephalus, its treatment and therapies.7
- Visual impairment
- Hernia of temporal lobe
- Cognitive and behavioural issues
- Delaying in normal development
- Difficulty in movement
- Shunt complications (blockage, over and under shunting)
- Septicemia
- Seizures7
Treatment options for hydrocephalus in infants
Surgical interventions
The treatment of hydrocephalus depends upon the condition of the infant. Often it is treated with surgery. An infant is completely monitored by doctors after birth and based on disease condition treatment options are selected.8
Two types of treatment are used to treat hydrocephalus in infants, the result of both treatments is to decrease intracranial pressure.8
- Shunt: the placement of a shunt is the most common type of surgical intervention. It is a plastic tube-like device inserted into the brain’s ventricles to release pressure and maintain the flow of cerebrospinal fluid. It decreases the pressure by reabsorbing the extra fluid into the abdominal cavity. This device aids in managing normal flow of fluid, decreasing pressure inside the brain, and healthy growth of the child's brain. An infant is closely monitored by a doctor through their childhood8
- Endoscopic third ventriculostomy: this treatment option is opted for those infants who are having blockage in the ventricles of the brain. In this procedure the doctors make a small opening in the ventricle. This opening helps in releasing the pressure and normalizing flow of CSF8
Long- term management of hydrocephalus in infants
Successful treatment of hydrocephalus requires long term management and proper followup. Routine checkup and brain imaging test i.e CT-Scan or MRI should be done after every 3 months. An infant with shunt should be regularly monitored even after surgery because there is a chance of relapse, occurrence of infection, complication related to shunt. If symptoms present, the patient should immediately contact the doctor without any negligence because hydrocephalus children may face development issues in future too.8,9
Advancement and future of hydrocephalus treatment in infants
In the past, when hydrocephalus had no treatment, infants and children died and those who survived left with permanent brain abnormality, gait problem, and mental illness throughout their lives.10 As time passes, a tube-like device called shunt is introduced and successful surgeries are performed by using a shunting device.
In the present era, a hydrocephalus's survivor has symptoms free to live throughout adulthood. Although the shunting device has its own complications like infections, drainage, and obstruction therefore, researchers found novel technologies with less side effects.10 Moreover, ultrasound during the prenatal stage helps in diagnosing hydrocephalus at an early stage.
Doctors performed intrauterine surgery which prevents hydrocephalus to further progress and reduces its morbidity and mortality rate. Intrauterine surgery is a better option as compared to placement of shunt. Recent advancement in brain imaging techniques gives better understanding of disease. Furthermore, researchers have studied gene therapy, molecular biology and neurodegeneration to provide more advancement in treatment.10
FAQs
When to see a doctor?
If the child's head is bigger than normal or you see rapid enlargement in the child's head, eyes are continuously in a downward position, or seizure; immediately consult with a neurosurgeon, neurologist, or paediatrician.
Can hydrocephalus be prevented?
In some cases it cannot be prevented, your child has to live with it, but one thing parents can do is to protect their child from head injury or trauma.
How long can a patient survive with hydrocephalus?
The survival rate of hydrocephalus is low. 50% of the patients died before 3 years and 80% died before adulthood.
Summary
In simple words, hydrocephalus is a swelling of the brain due to the accumulation of cerebrospinal fluid in the ventricles. It is caused by the imbalance of production and reabsorption of CSF and it can affect both children and adults.
Hydrocephalus in infants has a high morbidity and mortality rate. Enlargement of head size, the downward position of the eye, a soft spot on the top of the head, resistant feeding, vomiting, and seizures are signs and symptoms of hydrocephalus. Moreover, Ultrasound, MRI and CT scans are diagnostic tests of hydrocephalus which show the imaging of the brain, its tissue, and the build-up of fluid. Once the diagnosis is confirmed, the next step is to start treatment.
Often it is treated by surgery and the most common surgery for hydrocephalus is the placement of a shunt. Follow-up and long-term management is necessary even after the successful treatment because complications associated with disease and treatment can take place anytime. It should be treated on time otherwise it could lead to permanent brain damage and even death may occur.
Reference
- Cerebral Palsy Guide [Internet]. 2023 [cited 2024 Jun 12]. Hydrocephalus in infants | symptoms, treatment & legal help. Available from: https://www.cerebralpalsyguide.com/birth-injury/hydrocephalus/
- Hydrocephalus in children [Internet]. 2021 [cited 2024 Jun 11]. Available from: https://www.hopkinsmedicine.org/health/conditions-and-diseases/hydrocephalus/hydrocephalus-in-children
- Mayo Clinic [Internet]. [cited 2024 Jun 12]. Hydrocephalus - Symptoms and causes. Available from: https://www.mayoclinic.org/diseases-conditions/hydrocephalus/symptoms-causes/syc-20373604
- James HE. Hydrocephalus in infancy and childhood. Am Fam Physician. 1992 Feb;45(2):733–42.
- Hydrocephalus in infants and children: diagnosis & treatment [Internet]. 2023 [cited 2024 Jun 12]. Available from: https://www.hydroassoc.org/hydrocephalus-in-infants-and-children/
- nhs.uk [Internet]. 2018 [cited 2024 Jun 12]. Hydrocephalus - diagnosis. Available from: https://www.nhs.uk/conditions/hydrocephalus/diagnosis/
- Koleva M, De Jesus O. Hydrocephalus. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 [cited 2024 Jun 15]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK560875/
- Hydrocephalus fetal to newborn care center - dayton [Internet]. Dayton Children’s Hospital. [cited 2024 Jun 13]. Available from: http://fetaltonewborn.org/hydrocephalus/
- Washington JG PharmD Candidate Washington State University College of Pharmacy Spokane, Washington Joshua J Neumiller, PharmD, CDE, CGP, FASCP Assistant Professor of Pharmacotherapy Washington State University College of Pharmacy Spokane. Review of the treatment & management of hydrocephalus [Internet]. [cited 2024 Jun 15]. Available from: https://www.uspharmacist.com/article/review-of-the-treatment-management-of-hydrocephalus
- Upadhyaya P. Hydrocephalus: past, present and future. Indian J Pediatr. 1997;64(6 Suppl):4–14.