Hydrocephalus is a medical disorder in which cerebrospinal fluid (CSF) accumulates abnormally in the brain’s ventricles, causing increased pressure inside the skull. The term “hydrocephalus” is derived from the Greek words “hydro,” meaning water, and “cephalic,” meaning head, and refers to the potential damage caused by build up in brain tissue.

Hydrocephalus is a prevalent condition in children due to various developmental, genetic, and environmental factors that influence the flow and absorption of cerebrospinal fluid (CSF).

Factors affecting the growth of hydrocephalus in children

Developmental and congenital Factors :

• Aqueductal :

• The Aqueduct of Sylvius, which restricts CSF flow between the third and fourth ventricles, may be narrowed due to congenital malformations, genetic mutations, or developmental anomalies.

• Neural tube defect :

• Spina bifida is a condition where the spinal cord and its surrounding structures are not formed correctly, leading to defects that can disrupt the normal circulation of CSF.

• Arnold-chair malformation :

• Structural defects at the skull and cerebellum base, which can be congenital, can affect CSF flow and disrupt normal brain and spinal cord development.
• Dandy-walker malformation :

• This congenital malformation, characterized by the expansion of the fourth ventricle, absence of the cerebellar vermis, and cyst formation near the skull base, disrupts normal cerebral spinal fluid (CSF) pathways.

• Genetic conditions : 

• Genetic syndromes and mutations can cause brain structure and CSF circulation abnormalities, leading to hydrocephalus due to a genetic predisposition to structural abnormalities.

• Intraventricular haemorrhage :

• Premature infants frequently experience bleeding in the brain’s ventricular system due to the proneness of fragile blood vessels to rupture, potentially blocking CSF flow.

• Infections :

• Meningitis : The inflammation of the brain and spinal cord membranes can obstruct CSF pathways.
• Encephalitis : The inflammation of brain tissue can lead to swelling and obstruction of CSF flow.

• Brain tumours : 

• Tumors in critical brain areas can physically block CSF pathways, causing swelling and inflammation and potentially obstructing CSF flow.

• Head trauma :

• Trauma can cause bleeding, swelling, or scarring, obstructing CSF flow, and head injuries can disrupt brain structure and function, leading to hydrocephalus.

• Cysts : 

• Arachnoid cysts, fluid-filled sacs, can obstruct CSF pathways, particularly in critical brain areas, thereby impeding CSF circulation.

Brain growth and development in children with Hydrocephalus

• Increased CSF production : 

• As the brain grows, the production of CSF increases and any blockage can lead to increased intracranial pressure.

• Enlarging head size :

• Infants’ flexible skulls with open sutures and fontanelles facilitate rapid head growth, potentially highlighting signs of hydrocephalus, such as an enlarged head.

Diagnosis of Hydrocephalus in children

Hydrocephalus, a common condition in children, is now better diagnosed and managed through advancements in prenatal and neonatal care and imaging techniques.

• Ultrasound scan :

• This method is commonly employed in infants where the fontanelle remains open, enabling visualization of brain structures.

• CT scan : 

• Provides detailed brain images to detect abnormalities such as enlarged ventricles.

• MRI :

• High-resolution images that offer detailed information about brain structures and CSF pathways.

Treatment of Hydrocephalus in children

• Shunt systems : 

• The ventriculoperitoneal shunt is a surgical procedure that redirects cerebrospinal fluid (CSF) from the ventricles to the abdomen.

• Endoscopic third ventriculostomy (ETV):

• A minimally invasive procedure is being implemented to establish a new pathway for the flow of cerebrospinal fluid (CSF) within the brain.

• Treatment for underlying causes:

• The overall treatment strategy may include treating infections, removing tumours, or addressing other underlying causes.

• Medications: 

• Medications may be used in certain cases to manage symptoms or address underlying conditions causing hydrocephalus, as well as to reduce CSF production.

Symptoms of hydrocephalus among infants

• A rapid increase in head size (Macrocephaly): 
  • The infant’s head circumference grows faster than normal due to cerebrospinal fluid accumulation in the brain, causing it to be larger than expected for their age.

• Bulging or tense fontanelle: 
  • The soft spot (fontanelle) on the top of a baby’s head may appear bulging or feel tense to the touch.
• Vomiting:
  • Frequent and unexplained vomiting, not related to feeding or illness, is a common issue.
• Poor appetite:
  • The individual may experience difficulties in feeding, a decrease in appetite, or a decrease in their usual feeding habits.
• Irritability:
  • The individual experienced increased fussiness or crying, particularly when being handled.
• Sleepiness or lethargy:
  • The term “excessive sleepiness” refers to a condition where one experiences difficulty waking up or lethargy.
• Downward deviation of the eyes (sunsetting eyes):
  • The infant’s eyes may appear to be looking downward, with the upper part of the iris covered by the lower eyelid.
• Seizures:
  • The occurrence of seizures or unusual movements.
• Delayed developmental milestones: 
  • Delays in achieving developmental milestones like sitting, crawling, or walking.

Symptoms of hydrocephalus among older children

• Headache: 
  • Headaches, often persistent or severe, are more severe in the morning or after lying down.
• Nausea and vomiting:
  • The individual experiences nausea and vomiting, particularly in the morning.
• Blurred or double vision:
  • Increased intracranial pressure can cause visual disturbances like blurred or double vision.
• Problems with balance and coordination:
  • The individual is experiencing difficulty walking, poor balance, or clumsiness.
• Changes in personality or behaviour:
  • Unconfirmed alterations in personality, behaviour, or mood.
• Decline in school performance:
  • The issue may involve a decline in academic performance, difficulty concentrating, or memory issues.
• Increased head size:
  • Head size can increase in older children, although it is less common.
• Urinary incontinence:
  • The individual may experience a loss of bladder control, which may manifest as bedwetting or frequent urination.

Hydrocephalus, a common condition in children, disrupts cerebrospinal fluid flow, causing developmental delays, learning disabilities, and physical impairments. Early diagnosis and treatment are crucial for improving outcomes and quality of life. Parents and caregivers should seek medical attention if symptoms persist or worsen, as early diagnosis and treatment can prevent long-term complications.

About Dr Vishakha

Dr. Vishakha Karpe, a highly skilled Senior Paediatric Neurosurgeon at Rainbow Children’s Hospital, Banjara Hills, and Hyder Nagar in Hyderabad, is one of India’s leading paediatric neurosurgeons with extensive experience in paediatric neurosurgery. With over nine years of dedicated practice, she is among the few in India working extensively in this field.

With extensive experience in paediatric neurosurgical conditions, she focuses on comprehensive care, including precise surgery and educating parents about the complete case management protocol. She is an efficient and passionate medical professional, pursuing ethical practice and ensuring patient care after surgery.

Proficiency of Dr Vishaka:

Hydrocephalus (increased fluid in the brain): The procedure involves an endoscopic third ventriculostomy and CSF diversion (VP shunt) to treat complex hydrocephalus.

1. Craniosynostosis (abnormal head shape due to untimely cranial sutures fusion) surgeries: Helmet therapy is a technique that is used in both endoscopic and open surgery.
2. Spinal dysraphisms(Spina Bifida)- (spinal abnormalities present by birth) – surgical repair
3. Encepahaocles repair surgery.
4. Vascular conditions and stroke surgeries: revascularization surgeries for moya moya disease.
5. Pediatric brain and spine tumour surgeries.
6. Pediatric brain and spine infection surgeries: Endoscopic and open surgeries for brain and spine infections.
7. Pediatric traumatic brain and spinal injury.
8. Antenatal counselling for congenital fatal neurosurgical conditions.

Dr Vishaka specializes in craniosynostosis surgery, which is only done in a few centres in India. Dr Vishaka Patil, M.B.B.S, DNB (AIIMS) New Delhi, M.Ch (IPGMER SSKM) became a Member of  “The Royal College of Surgeons, Edinburgh” (U.K.) a highly successful and best pediatric neurosurgeon in Hyderabad, Telangana with 13 years of experience, is among the topmost pediatric neurosurgeons in the Rainbow group of hospitals at Hyder Nagar and Banjara Hills.