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Hydrocephalus

Hydrencephalus

Hydrocephalus is a clinical condition characterized by disturbance of cerebrospinal fluid regulation in the central nervous system. Cerebrospinal fluid regulation has three important steps - formation, flow and absorption.


Presentation

Infants with hydrocephalus presents with complaints such as poor feeding, vomiting, reduced activity and irritability. Children and adults with hydrocephalus present with complaints such as slowing of higher brain functions, headaches in the morning, neck pain, vomiting specially in the morning, visual abnormality, drowsiness, difficulty in walking. In children there may be delayed growth and sexual maturation. Symptoms of normal pressure hydrocephalus (NPH) include gait disturbance, dementia, and inability to control urination [6].

Poor Feeding
  • Infants with hydrocephalus presents with complaints such as poor feeding, vomiting, reduced activity and irritability.[symptoma.com]
  • feeding habits Projectile vomiting Seizures Sleepiness or lack of alertness Very noticeable scalp veins In older children, headaches and visual changes may be seen.[chp.edu]
  • Symptoms may include: Vomiting Poor feeding Listlessness Irritability Constant downward gaze of the eyes Occasional seizures In older children and adults, the bones of the skull have closed.[medtronic.com]
  • Infants present with symptoms such as poor feeding, vomiting, irritability, and reduced activity.[news-medical.net]
  • Infants Large or enlarging head Poor feeding Vomiting Sleepiness Irritability Downward deviation of the eyes (also called "sunsetting") Seizures Children and adults Headache followed by vomiting Nausea Papilledema (swelling of the optic disk which is[neurosurgery.ucla.edu]
Precocious Puberty
  • They may reach puberty earlier than the average child (see precocious puberty ). About one in four develops epilepsy . Cause [ edit ] Congenital [ edit ] One-year-old girl with hydrocephalus showing "sunset eyes", before shunt surgery.[en.wikipedia.org]
Vomiting
  • Infants with hydrocephalus presents with complaints such as poor feeding, vomiting, reduced activity and irritability.[symptoma.com]
  • Three months after the ETV, he presented with repeated vomiting. Neuroimagings showed a 3-cm fourth ventricular mass with progressive hydrocephalus. Surgical resection was performed, which revealed the pathology was medulloblastoma.[ncbi.nlm.nih.gov]
  • Symptoms of increased intracranial pressure may include headaches , vomiting , nausea , papilledema , sleepiness or coma .[en.wikipedia.org]
  • Symptoms may include: Vomiting Poor feeding Listlessness Irritability Constant downward gaze of the eyes Occasional seizures In older children and adults, the bones of the skull have closed.[medtronic.com]
  • In infants the symptoms of shunt malfunction can include the above as well as vomiting, inappropriate head growth and/or sunsetting eyes.[aans.org]
Nausea
  • Clinical presentation A 20-year-old male with a history of tuberous sclerosis, X-linked intellectual disability, and epilepsy presented to the emergency department with two days of nausea, emesis, seizures, and two months of progressive proptosis.[ncbi.nlm.nih.gov]
  • […] and vomiting In adults: Balance changes Cognitive dulling with progression to headache Lethargy Nausea and vomiting The situations in older children and adults described above are considered urgent and may require immediate intervention.[uchospitals.edu]
  • Symptoms include Headache Vomiting and nausea Blurry vision Balance problems Bladder control problems Thinking and memory problems Hydrocephalus can permanently damage the brain, causing problems with physical and mental development.[medlineplus.gov]
  • Primary symptoms include headache, nausea, vomiting and drowsiness. Normal pressure hydrocephalus (NPH) is the accumulation of cerebrospinal fluid that causes the ventricles in the brain to become enlarged, with little or no increase in pressure.[aans.org]
Failure to Thrive
  • This disease is characterized by hypokalemia, hypochloremia, and metabolic alkalosis that is often associated with failure to thrive and recurrent episodes of dehydration. The combination of BS and cholelithiasis in an infant is very rare.[ncbi.nlm.nih.gov]
  • In extreme cases, a child might have " failure to thrive " and miss growth milestones.[kidshealth.org]
  • The latter manifests itself as failure to thrive and developmental delays. Other signs include: Rapid increase in head circumference or head circumference is in the 98th percentile for the age or greater.[patient.info]
Blurred Vision
  • Older patients, who have less ability to compensate for increased intracranial pressure, are known to experience dizziness, blurred vision, nausea, loss of motor function and changes in cognition and personality.[fusfoundation.org]
  • vision Diplopia (double vision) Sunsetting of the eyes, problems with balance Poor coordination Drowsiness Elderly (Normal pressure hydrocephalus) Progressive mental impairment and dementia Problems with walking Impaired bladder control leading to urinary[neurosurgery.ucla.edu]
  • Symptoms in older children may include: Awkward gait Balance problems Blurred vision Double vision Downward deviation of the eyes Drowsiness or lethargy Headache Incontinence Irritability Memory loss Nausea with or without vomiting Show More During your[ucsfbenioffchildrens.org]
  • Children and adults presents with symptoms such as slowing of higher brain functions, headaches in the morning, neck pain, vomiting specially in the morning, blurred vision, double vision, difficulty in walking or drowsiness.[symptoma.com]
  • Symptoms of hydrocephalus The damage to the brain can cause a wide range of symptoms, including: headache being sick blurred vision difficulty walking Different types of hydrocephalus can cause specific symptoms.[nhs.uk]
Diplopia
  • […] feeding Vomiting Sleepiness Irritability Downward deviation of the eyes (also called "sunsetting") Seizures Children and adults Headache followed by vomiting Nausea Papilledema (swelling of the optic disk which is part of the optic nerve) Blurred vision Diplopia[neurosurgery.ucla.edu]
  • Symptoms may include: Headache Vomiting and/or nausea Papilledema (swelling of the optic disk which is part of the optic nerve) Visual disturbances: blurred vision, diplopia (double vision), and sun setting of the eyes Difficulty with balance, poor coordination[aesculapusa.com]
Neck Pain
  • A 58-year-old lady presented with neck pain and difficulty in walking and numbness of all 4 limbs of 1-year duration. Diagnostics with magnetic resonance imaging of the head and the cervical spine were done in the referring hospital.[ncbi.nlm.nih.gov]
  • Children and adults with hydrocephalus present with complaints such as slowing of higher brain functions, headaches in the morning, neck pain, vomiting specially in the morning, visual abnormality, drowsiness, difficulty in walking.[symptoma.com]
  • Toddlers, older children, and adults show decreased mental capacity and may experience headaches, neck pain, vision disturbances, drowsiness, and unstable balance.[news-medical.net]
  • Some of the main symptoms of hydrocephalus include: Headache, neck pain Nausea and vomiting Blurred or double vision Confusion Some individuals may also experience: Poor coordination Difficulty with balance Difficulty walking Urinary and/or bowel incontinence[news-medical.net]
  • Acquired and idiopathic forms of hydrocephalus can occur at any time in life and typical symptoms include: Headaches Neck pain Nausea Vomiting Drowsiness Confusion Poor co-ordination Blurred or double vision Difficulty walking Bladder and/or bowel incontinence[headway.org.uk]
Muscle Spasticity
  • spasticity ( spasm ); Slow growth (child 0–5 years); Slow or restricted movement; Vomiting. [6] Because hydrocephalus can injure the brain, thought and behavior may be adversely affected.[en.wikipedia.org]
  • Symptoms of continued hydrocephalus include irritability and muscle spasticity .[encyclopedia.com]
Skeletal Dysplasia
  • Hydrocephalus is also a frequent component of skeletal dysplasias and craniosynostosis syndromes in which there is a disparity between brain size and skull size.[neuropathology-web.org]
Incontinence
  • A child with congenital hydrocephalus may experience difficulty walking or with eye-hand coordination whereas an elderly person with hydrocephalus may experience incontinence.[hydrocephalus.ca]
  • The most commonly occurring symptoms are difficulty walking, incontinence and problems with processing information.[news-medical.net]
  • NPH presents with the classic triad of impaired gait, urinary incontinence, and mental status changes. It is an important disorder to recognize as it is potentially treatable by shunting.[casemed.case.edu]
  • […] other changes in personality or cognition including memory loss Normal Pressure Hydrocephalus Symptoms of NPH include progressive mental impairment and dementia, problems with walking, and impaired bladder control leading to urinary frequency and/or incontinence[aesculapusa.com]
  • Turning around, or either starting or stopping suddenly leads to imbalance. sphincter disturbance (incontinence ): on occasions, the person is incontinent of urine and sometimes faeces.[sophysa.com]
Headache
  • Despite a ventriculoperitoneal shunt placement, he continued to have headaches and was soon found to have several skull base subarachnoid lesions, which were later diagnosed as the sequelae of an active neurocysticercosis infection.[ncbi.nlm.nih.gov]
  • He reported a 10-day history of fever, photophobia, headache and fatigue. His medical history included hypothyroidism and migraine. He was a non-smoker, did not consume alcohol and denied a history of drug use.[ncbi.nlm.nih.gov]
  • Headache; difficulty in remaining awake or waking up; loss of coordination or balance; bladder control problems; impaired vision and cognitive skills that may affect job performance and personal skills.[aans.org]
  • […] unrelieved by pain medication visual disturbances, fainting gait disturbances: clumsiness, difficulty walking on uneven surfaces and stairs cognitive problems: dependent on lists, decline in academic or work performance * The headaches experienced by[hydrocephalus.ca]
Irritability
  • She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia.[ncbi.nlm.nih.gov]
  • She remained seizure-free until age five months, when seizures reappeared in the context of increasing head size and irritability. A cranial ultrasound showed hydrocephalus, for which she underwent ventriculoperitoneal shunting.[ncbi.nlm.nih.gov]
  • Infants with hydrocephalus presents with complaints such as poor feeding, vomiting, reduced activity and irritability.[symptoma.com]
  • , exciting that system into violent action by morbid sympathy; or from the effects of something of an irritating nature having been received into, and circulating with the blood, and thereby directly applied to the internal... ‎[books.google.es]
  • Manifestations can include enlarged head, bulging fontanelle, irritability, lethargy, vomiting, and seizures. Diagnosis is by ultrasonography in neonates and young infants with an open fontanelle and by CT or MRI in older infants and children.[merckmanuals.com]
Lethargy
  • Symptoms can include vomiting, lethargy, headache, enlarged head or even seizures. There is no cure, but treatment may provide long-term relief. Definition The term "hydrocephalus" is derived from two Greek words: hydro (water) and kephale (head).[medtronic.com]
  • Manifestations can include enlarged head, bulging fontanelle, irritability, lethargy, vomiting, and seizures. Diagnosis is by ultrasonography in neonates and young infants with an open fontanelle and by CT or MRI in older infants and children.[merckmanuals.com]
  • […] outweigh the risks, potential complications can arise, including: Infection Shunt malfunction, resulting in underdrainage or overdrainage of the CSF Bleeding Fever Vomiting Irritability Redness Swelling along the area of the tubing Decreased alertness or lethargy[chp.edu]
  • Toddlers and older children Among toddlers and older children, signs and symptoms may include: Physical signs and symptoms Headache Blurred or double vision Eyes fixed downward (sunsetting of eyes) Abnormal enlargement of a toddler's head Sleepiness or lethargy[mayoclinic.org]
Abnormal Gait
  • Other symptoms include abnormal gait (difficulty walking), inability to hold urine ( urinary incontinence ), and, occasionally, inability to control the bowels. The dementia symptoms of NPH can be similar to those of Alzheimer's disease .[webmd.com]
  • Other symptoms include abnormal gait (difficulty walking ), inability to hold urine ( urinary incontinence ), and, occasionally, inability to control the bowels. The dementia symptoms of NPH can be similar to those of Alzheimer disease.[emedicinehealth.com]
Difficulty Concentrating
  • concentrating Normal pressure hydrocephalus (NPH) This form of the condition usually begins slowly and is more common in adults over the age of 60.[healthline.com]

Workup

Examination in infants may reveal head circumference ≥98th percentile for age, disjunction of sutures, dilated scalp veins, tense fontanelle, setting-sun sign, and lower limb spasticity.

Papilledema, failure of upward gaze, unsteady gait, large head, sixth nerve palsy and Macewen sign may be observed in examination of children and adults. Patients with NPH may exhibit increased reflexes and Babinski response in one or both feet. Variable difficulty in walking, sucking and grasping reflexes in adults may occur [7].

Size of ventricles and other structures could be assessed by computed tomography scanning (CT). Malformations or tumors are detected by magnetic resonance imaging (MRI). Ultrasonography through anterior fontanelle in infants may diagnose subependymal and intraventricular hemorrhage. Skull radiography detects erosion of sella turcica or beaten copper cranium seen in craniosynostosis to confirm position. MRI cine measures CSF stroke volume in the cerebral aqueduct. Diffusion tensor imaging (DTI) detects microstructural changes in periventricular white matter.

CSF examination is recommended in suspected case of hydrocephalus after hemorrhage and meningitis. Electroencephalography is recommended in patients with seizures. If X-linked hydrocephalus is suspected, genetic testing and counseling is recommended [8].

Slowing
  • We present a case of a 6-month-old infant with an occipital fracture and slow enlargement of a posterior fossa subdural hygroma that culminated in obstructive hydrocephalus.[ncbi.nlm.nih.gov]
  • Children and adults with hydrocephalus present with complaints such as slowing of higher brain functions, headaches in the morning, neck pain, vomiting specially in the morning, visual abnormality, drowsiness, difficulty in walking.[symptoma.com]
  • He eventually began to talk and now at age 7 hasn’t slowed down! (click here to continue) JayLynn was diagnosed with hydrocephalus in utero at 21 weeks.[hydrosupport.org]
  • Symptoms in children and adults include the following: Slowing of mental capacity, cognitive deterioration Headaches (initially in the morning) Neck pain, suggesting tonsillar herniation Vomiting, more significant in the morning Blurred vision: A consequence[emedicine.medscape.com]
Generalized Slowing
  • They may have a general slowing of movements or may complain that their feet feel "stuck."[aesculapusa.com]
  • An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel “stuck.”[chasa.org]
  • An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel "stuck."[ninds.nih.gov]

Treatment

A surgical shunt placement is the preferred therapeutic option. Ventriculoperitoneal shunt is the most common procedure.

Ventriculoatrial shunt, lumboperitoneal shunt (used for communicating hydrocephalus, CSF fistula, or pseudotumor cerebri), Torkildsen shunt (in acquired obstructive hydrocephalus) and ventriculopleural shunt are other possible procedures [9].

Hydrocephalus of rapid-onset needs immediate attention which is managed by ventricular tap in infants, open ventricular drainage in children and adults, lumbar puncture (LP) in posthemorrhagic and postmeningitic hydrocephalus or ventriculoperitoneal or ventriculoatrial shunt.

Alternatives to shunting include choroid plexectomy, opening of a stenosed aqueduct and endoscopic fenestration of the floor of the third ventricle (contraindication is communicating hydrocephalus). Medical treatment with acetazolamide or furosemide may be advised in premature infants with non–acute posthemorrhagic hydrocephalus for delaying surgical intervention [10].

Prognosis

Hydrocephalus requires regular and long term follow-up. There could be complications after treatment such as, infection and choking or collapse of shunt causing rise in CSF pressure in which additional surgeries are considered. Children with hydrocephalus have developmental defects of motor function, mental retardation, defective vision, and risk for epilepsy. Patients with epilepsy have poor prognosis and are more likely to have an IQ lower than 90. About 60% of children with hydrocephalus attend the school and approximately 40% of children will lead normal lives [5].

Etiology

Congenital hydrocephalus occurring in infants may be idiopathic, as a result of premature or obstructed delivery, a brain defect restricting the flow of cerebrospinal fluid, spina bifida, X-linked mutation, Dandy Walker malformation and arachnoid cysts the brain and spinal cord [2].

Acquired hydrocephalus occurs usually due to a blockage between the ventricles of the brain as a result of an injury or illnesses such as hemorrhage, meningitis, brain tumors, head injury, stroke in adults or children. In older people after brain injury, bleeding or infection, without any clear reason normal pressure hydrocephalus (NPH) may develop.

Epidemiology

Approximately hydrocephalus accounts for 0.6 percent of all pediatric hospital admissions and three out of every thousand are born with hydrocephalus. The incidence of acquired hydrocephalus is not known. There is an equal incidence in both sexes with exceptions of X–linked disorders and normal pressure hydrocephalus. The disease shows two peaks one at infancy and other at adulthood on age curve. Adult cases account for 40% of total cases [3].

Sex distribution
Age distribution

Pathophysiology

CSF is produced by the choroid plexus within brain ventricles at the rate of 0.20-0.35 ml/min and total volume in an adult is 120 ml. CSF flows from the lateral ventricle into the venous drainage through various foramina and ventricles in brain. The intracranial pressure increases if CSF is produced excessively or there is increased resistance to CSF flow, or there is increased venous sinus pressure.

First, temporal and frontal horns dilate asymmetrically resulting in elevation of the corpus callosum, followed by stretching of the septum pellucidum, thinning of the cerebral mantle, or enlargement of the third ventricle into the pituitary fossa which may result in pituitary dysfunction [4].

The mechanism of NPH is unclear and may be due to resistance to flow of resulting in intermittently elevation of CSF pressure, at night and ventricular enlargement is maintained despite normal pressure. This reflects increased force on the ventricular wall.

Prevention

Hydrocephalus is not preventable. The risk could be minimized by regular antenatal check up and preventing premature labor. Posthemorrhagic and postmeningitic hydrocephalus could be prevented by avoiding head injuries and prompt treatment with antibiotics respectively.

Summary

Hydrocephalus is also termed as a hydrodynamic cerebrospinal fluid (CSF) disorder and is characterized by increase in volume of this fluid. If it progresses naturally without interventions, it leads to tonsillar herniation and compression of the brain stem which may cause respiratory arrest and death. This condition is diagnosed based on clinical findings, ophthalmic examination, CSF examination and CT scan of the head.

Such patients are treated with a shunt. Patients are reassessed for shunt revisions, complications and failure. In infants and children, hydrocephalus may cause poor development of cognitive function. It may cause loss of cognitive function in adults [1]. Persistent visual loss if occurs may persist even after treatment.

Patient Information

Definition

Hydrocephalus is a clinical condition characterized by disturbance of cerebrospinal fluid regulation (CSF) in the central nervous system ie. production, flow and absorption.

Cause

Hydrocephalus may be inborn without any cause or secondary to complications of delivery such as delay or obstruction, genetic disorders, spinal and brain malformations. 
In adults or children there is usually acquired hydrocephalus due to a blockage between the ventricles of the brain as a result of an injury or illnesses such as subarachnoid hemorrhage, venous thrombosis, meningitis, brain tumors, head injury or stroke.

Symptoms

Infants with hydrocephalus presents with complaints such as poor feeding, reduced activity, irritability, and vomiting. Children and adults presents with symptoms such as slowing of higher brain functions, headaches in the morning, neck pain, vomiting specially in the morning, blurred vision, double vision, difficulty in walking or drowsiness

Diagnosis

Hydrocephalus may be diagnosed on history and clinical examination along with investigations such as X-ray, CT, MRI, and USG. Other investigations such as CSF examination, electroencephalography, genetic screening may be advised if required based on the suspected cause.

Treatment

A surgical shunt placement is the preferred therapeutic option. Hydrocephalus with rapid-onset is an emergency which is managed by ventricular tap or drainage in infants and children or adults respectively. Lumbar puncture (LP) is treatment procedure for posthemorrhagic and postmeningitic hydrocephalus. Choroid plexectomy and opening of a stenosed aqueduct are alternatives to shunting, endoscopic fenestration of the floor of the third ventricle. Medical treatment with acetazolamide or furosemide may be advised in premature infants with non–acute posthemorrhagic hydrocephalus for delaying surgical intervention. 

References

Article

  1. Kahlon B, Annertz M, Stahlberg F, Rehncrona S. Is aqueductal stroke volume, measured with cine phase-contrast magnetic resonance imaging scans useful in predicting outcome of shunt surgery in suspected normal pressure hydrocephalus?. Neurosurgery. Jan 2007;60(1):124-9; discussion 129-30.
  2. Hamilton MG. Treatment of hydrocephalus in adults. Semin Pediatr Neurol. Mar 2009;16(1):34-41.
  3. Lacy M, Oliveira M, Austria E, Frim MD. Neurocognitive outcome after endoscopic third ventriculocisterostomy in patients with obstructive hydrocephalus. J Int Neuropsychol Soc. May 2009;15(3):394-8. 
  4. Partington MD. Congenital hydrocephalus. Neurosurg Clin N Am. Oct 2001;12(4):737-42, ix
  5. Larsson A, Moonen M, Bergh AC, Lindberg S, Wikkelso C. Predictive value of quantitative cisternography in normal pressure hydrocephalus. Acta Neurol Scand. Apr 1990;81(4):327-32. 
  6. Lee JH, Back DB, Park DH, Cha YH, Kang SH, Suh JK. Increased Vascular Endothelial Growth Factor in the Ventricular Cerebrospinal Fluid as a Predictive Marker for Subsequent Ventriculoperitoneal Shunt Infection : A Comparison Study among Hydrocephalic Patients. J Korean Neurosurg Soc. Jun 2012;51(6):328-33. 
  7. Damasceno BP, Carelli EF, Honorato DC, Facure JJ. The predictive value of cerebrospinal fluid tap-test in normal pressure hydrocephalus. Arq Neuropsiquiatr. Jun 1997;55(2):179-85.
  8. Chang CC, Kuwana N, Noji M, Tanabe Y, Koike Y, Ikegami T. Cerebral blood flow in patients with normal pressure hydrocephalus. Nucl Med Commun. Feb 1999;20(2):167-9.
  9. Garvey MA, Laureno R. Hydrocephalus: obliterated perimesencephalic cisterns and the danger of sudden death. Can J Neurol Sci. May 1998;25(2):154-8
  10. Tanaka A, Kimura M, Nakayama Y, Yoshinaga S, Tomonaga M. Cerebral blood flow and autoregulation in normal pressure hydrocephalus. Neurosurgery. Jun 1997;40(6):1161-5; discussion 1165-7. 

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Last updated: 2018-06-22 12:20