What is hydrocephalus?
It is an important medical condition that results from abnormalities in the flow and resorption of cerebrospinal fluid (CSF); that is why it is also called as hydrodynamic CSF disorder. It is characterized by dilation of brain ventricles.
It is actually a combination of heterogeneous complex and multifactorial disorders. Genetic factors are also involved in the causation of it. The estimated incidence of it is 1 in 1500 births1. This disorder can be classified into:
Congenital hydrocephalus- it is present at the time of birth and may be associated with developmental defects. Recent studies show that almost 40% of such cases have possible genetic etiology1.
Hydrocephalus in adults:
(acquired type) – it occurs after the development of brain and its ventricular system1.
Normal pressure hydrocephalus or idiopathic hydrocephalus in adults- it is characterized by enlargement of cerebral ventricles and gait dysfunction in the absence of another cause. It is relatively rare but a potential cause of it in elders. It is a reversible condition if diagnosed timely. Sometimes, it is also called idiopathic adult hydrocephalus syndrome as intracranial pressure is not always normal as in normal pressure hydrocephalus2,5.
The symptoms and signs of it are affected by following factors3:
- Age of the patient
- Cause of the disability
- Location of obstruction
- Duration of obstruction
- Mode of onset of obstruction
Symptoms in infants include:
- Poor feeding
- Reduced activity3
Symptoms for it in children and adults include3:
- Morning headache
- Vomiting especially in the morning
- Neck pain, indicating tonsillar herniation
- Blurred vision due to papilledema and optic atrophy
- Double vision due to unilateral or bilateral 6th nerve palsy
- Spascity leading to difficulty in walking especially affecting the lower limbs as the periventricular pyramidal tract is stretched by increased CSF.
In addition to the above children with this condition may have following features:
- Stunted growth
- Sexual maturation problems
Short Documentary: Living with Hydrocephalus: Emma’s story
In addition to above described symptoms of it, adults with this disorder may have the following features:
- Nausea aggravated by head movements
- Urinary incontinence
- Fecal incontinence
The following are the clinical features of normal pressure hydrocephalus4,5:
- Gait dysfunction is usually the first symptom of normal pressure hydrocephalus and it may occur months or years before the appearance of other symptoms. The term magnetic gait means the tendency of feet to remain “stuck to floor” despite the patient’s best efforts to lift them up. Dementia may be the late symptom in this type and it presents as an impairment of recent memory.
- Urinary incontinence, frequency, urgency and diminished awareness of the need to urinate are some other symptoms of normal pressure hydrocephalus.
- Headache and fits are rare symptoms4.
Examination of infants may result in the following findings indicating the diagnosis of it3,4:
- Enlarged head
- Tense fontanelle
- Dilated scalp veins
- Dysjunction of sutures
- Setting-sun sign
- Spascity of lower limbs
In adults, the following findings may be seen:
- Failure of upward gaze
- Enlarged head
- Unsteady gait
- Unilateral or bilateral 6th nerve palsy
Patients having normal pressure hydrocephalus may have the following findings4,5:
- Up going planter
- Difficulty in walking
- Frontal release sign
- Normal muscle tone
The following tests may be required in case of hydrocephalus3:
- Genetic testing
- CSF examination in case of post meningitis hydrocephalus
- EEG if seizures are present
- CT scanning to access the size of ventricles
- Skull radiography to detect erosion of sella-turcica
- MRI cine to measure the CSF stroke volume in 3rd ventricle
- Diffusion tensor imaging
- Radionuclide cisternography
Surgical treatment is the treatment of choice for hydrocephalus. The following types of shunts can be placed after surgery3:
- Ventriculoperitoneal (VP)
- Ventriculoatrial (VA)
- Lumboperitoneal shunt
- Torkildsen shunt
- Ventriculopleural shunt
Acute onset hydrocephalus is an emergency and the following surgical procedures can be performed depending upon the specific case:
- Ventricular tap
- Open ventricular drainage
- Lumbar puncture (LP)
- VP or VA shunt
Several alternative procedures can also be applied such as:
- Choroid plexectomy
- Opening of a stenosed aqueduct
- Endoscopic fenestration of the floor of the third ventricle
Conservative treatment is carried out in hydrocephalus as a temporary measure to delay the surgical intervention. Drugs used as conservative therapy include furosemide and acetazolamide3.
Benjamin Warf , MD, Neurosurgeon at Boston Children’s Hospital, explains the condition hydrocephalus and our innovative approach to treating the condition.
1. Zhang J, William MA, Rigamonti D. Genetics of human hydrocephalus. J Neurol 2006; 253(10):1255-66.
2. Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep 2008;8(5):371-6.
3. Nelson SNJr. Hydrocephalus [Internet]. [Last updated 2013 Aug 5; cited 2013 Aug 19]. Available from:
4. Kiefer M, Unterberg A. The differential diagnosis and treatment of normal-pressure hydrocephalus. Dtsch Arztebl Int 2012;109(1-2):15-26.
5. Williams MA, Wilson RK. Evidence that congenital hydrocephalus is a precursor to idiopathic normal pressure hydrocephalus in only a subset of patients. J Neurol Neurosurg Psychiatry 2007;78(5):508-11.