Medical Management Considerations
Hydrocephalus (also known as “hydrocephaly” and commonly referred to as “water on the brain”) is a condition caused by an abnormal accumulation of cerebrospinal fluid (CSF) that leads to dilation of the ventricles and increased intracranial pressure. Hydrocephalus can occur by one of two mechanisms: (1) the pathways for the flow of CSF is obstructed (noncommunicating hydrocephalus), or (2) CSF is not being reabsorbed (communicating hydrocephalus). If the normal drainage pathways are blocked or if absorption is hindered, CSF accumulates in the ventricles inside the brain, causing them to swell. This results in increased intracranial pressure and compression of the surrounding brain tissue. In young children, when the bones of the head are not yet fused, the head will grow abnormally large.
Hydrocephalus is not a disease in itself, but the result of buildup of CSF. Congenital hydrocephalus is caused by prenatal brain disorders. Acquired hydrocephalus occurs after birth, when a variety of postnatal disorders block the flow or absorption of cerebrospinal fluid.
Noncommunicating hydrocephalus results from a blockage of circulation of CSF in the ventricular cavities, usually due to acqueductal stenosis.
Communicating hydrocephalus occurs when the circulation of CSF around the brain and its absorption is disturbed; resulting from a blockage in the subarachnoid space.
Normal pressure hydrocephalus occurs when an accumulation CSF enlarges the ventricles of the brain, but occurs with little or no increase in pressure.
The diagnosis of hydrocephalus has traditionally been based on the enlargement of the lateral ventricles in the brain. Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as magnetic resonance imaging (MRI), cranial ultrasonography (US), and computerized tomography (CT). A physician selects the appropriate diagnostic tool based on the person’s age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord. Unlike CT, MRI is particularly useful for diagnosing the presence of congenital malformations such as Chiari, Dandy-Walker, or heterotopias; CT will not always show developmental malformations that accompany hydrocephalus. Congenital hydrocephalus can also be diagnosed pre-natally by ultrasound.
In babies and infants, the head will enlarge at an abnormally fast rate. In older children and adults, the head size cannot increase since the bones that form the skull are completely joined together, and symptoms of increased intracranial pressure will be present.
Some forms of hydrocephalus require no specific treatment. Other forms are temporary and resolve spontaneously. An infant’s pediatrician should measure the young child’s head circumference at each office visit. Nonetheless, most forms do require treatment, and this is usually surgical, most often insertion of a shunt. Seldom does removing the cause of the condition, such as a tumor or other blockage, treat the condition.
Certain drugs like the carbonic anhydrase inhibitors, such as acetazolamide, may temporarily control communicating hydrocephalus. Acetazolamide has been shown to reduce CSF production by the choroid plexus. Drugs have been used for many years but they may have unpleasant side effects and are not often successful.
The usual treatment is to insert a shunting device. This reduces the amount of CSF and, hence, the pressure on the brain. Shunts, which consist of tubing and valves, are surgically inserted into a ventricleto divert the accumulated CSF from the obstructed pathways to another part of the body where it is absorbed. The CSF flow is regulated by a unidirectional shunt valve so that a constant intracranial pressure (ICP) is maintained within the brain. Treating physicians must be aware of the seriousness of shunt failure or infection and how to evaluate and treat it, emphasizing to people with shunts and their families/caregivers the need to take all symptoms and signs of potential shunt failure or infection seriously and report it to the doctor immediately.
Figures Pertaining to Shunts
An alternative treatment for noncommunicating hydrocephalus may be endoscopic third ventriculostomy (ETV). In this procedure, a hole is made in the floor of the third ventricle. The procedure effectively allows the CSF to bypass the block between ventricles three and four and flow outside the brain. The initial complication rate of ETV is higher than that for shunt placement, but, if successful, the procedure eliminates the need for a shunt as well as the associated risks of shunt malfunction. Nonetheless, not all types of hydrocephalus can be treated by this method and only about 25% of people with all forms of hydrocephalus will be suitable candidates. People with aqueductal stenosis benefit most from third ventriculostomy, and the older the person, the greater the chance of success of the endoscopic procedure. The overall success rate of endoscopic ventriculostomy for all people with hydrocephalus is 66%.
Proper management should include a complete assessment of the individual’s neuropsychologic function, since the likelihood of underlying cognitive impairments contributing to observed problems is high. The neurologist and developmental pediatrician, along with psychologists and educators, can best benefit people with hydrocephalus and their families by helping them to understand the proper evaluation, interpretation, and treatment/management of the cognitive/academic problems associated with hydrocephalus. Spasticity can be managed with occupational therapy and physical therapy. An orthopedist and/or neurologist can also provide medical management for spasticity in the use of medications such as baclofen and Botulinum Toxin (Bo-Tox).
People with hydrocephalus may also show social and emotional problems as well as patterns of learning deficiencies—including problems with attention, organizational skill, and problem solving—that may severely affect their ability to reach their educational and vocational potential. It is imperative that the clinician diligently screen for, identify, and address these issues and help families and schools recognize the impairments as being neurologically based rather than the result of laziness or lack of motivation in a person.
(1997). About normal pressure hydrocephalus. San Francisco: Hydrocephalus Association.
Alvisi, C., Cerisoli, M., & Giulioni, M. (1986). The Surgical Treatment of Congenital Hydrocephalus. Journal of Neurosurgical Sciences, 30(3), 107-113.
Mataro, M., Junque, C., Poca, M. A., Sahuquillo, J. (2001). Neuropsychological findings in congenital and acquired childhood hydrocephalus [Abstract]. Neuropsychology Review, 11(4), 169-178.
Partington, M. D. (2001). Congenital hydrocephalus. Neurosurgery Clinics of North America, 36(4), 737-742.
Shiminski-Maher, T., & Disabato, J. (1994). Current trends in the diagnosis and management of hydrocephalus in children. Journal of Pediatric Nursing, 9(2), 74-82.
Sussman, M. (2004). Slit ventricle syndrome update. Hydrocephalus Association Newsletter, 7.
Vintzileos, A. M., Ingardia, C. J., & Nochimson, D. J. (1983). Congenital hydrocephalus: A review and protocol for perinatal management. Obstetrics and Gynecology, 62(5), 539-549.
Chauvel, P. J., & Kinsman, S. L. (1996). Spina bifida and hydrocephalus. In A. J. Capute & P. J. Accardo (Eds.), Developmental disabilities in infancy and childhood: Vol. 2. The spectrum of developmental disabilities (2nd ed., pp. 179-187). Baltimore: Paul H. Brookes.
Fenichel, G. (2001). Clinical pediatric neurology (4th ed., pp. 357-361). Philadelphia: W.B. Saunders Company.
Gascon, G. G., & Leech, R. W. (1991). Medical Evaluation. In R. W. Leech & R. A. Brumback (Eds.), Hydrocephalus: Current clinical concepts (pp. 105-128). St. Louis, MO: Mosby Year Book.
Gilmore, H. E. (1990). Medical Treatment of Hydrocephalus. In R. M. Scott (Ed.), Hydrocephalus: Vol. 3. Concepts in neurosurgery (pp. 37-46). Baltimore: Williams & Wilkins.
Hudgins, R.J. and Boydston, W.R. (2001/2002). Hydrocephalus: Clinical Presentation of Hydrocephalus. Retrieved December 20, 2005, from http://www.choa.org/default.aspx?id=906
Hyman-Newman Institute for Neurology and Neurosurgery (INN). (n.d.). Hydrocephalus: CSF production. Retrieved December 17, 2002, from http://nyneurosurgery.org/hydrocsf.htm
Kinsman, S.L. (1996). Childhood-Acquired Hydrocephalus. In A. J. Capute & P. J. Accardo (Eds.), Developmental disabilities in infancy and childhood: Vol. 2. The spectrum of developmental disabilities (2nd ed., pp. 189-197). Baltimore: Paul H. Brookes.
Lynch, D.R., & Batshaw, M.L. (1997). The Brain and Nervous System: Our Computer. In M. L. Batshaw (Ed.), Children with Disabilities (4th ed., pp. 293-314). Baltimore: Paul H. Brookes.
Nickel, R. E. (2000). Meningomyelocele and Related Neural Tube Defects. In R. E. Nickel & L. W. Desch (Eds.), The Physician’s Guide to Caring for Children with Disabilities and Chronic Conditions (pp. 425-476). Baltimore: Paul H. Brookes.
The Resource Foundation for Children with Challenges. (n.d.). Hydrocephalus. In Disorder Zone Archives. Retrieved December 30, 2002, from the Special Child website: http://www.specialchild.com/archives/dz-018.html
Romero, R., et al (1988). Hydrocephalus. In Prenatal diagnosis of congenital anomalies (1st ed.). Norwalk, CT: Appleton & Lange.
University of California, San Francisco (UCSF) Children’s Hospital. (2002). Congenital hydrocephalus: Signs and symptoms. Retrieved December 16, 2002, from http://www.ucsfhealth.org/childrens/medical_services/neuro/hydrocephalus/conditions/congenital/signs.html
University of Minnesota, Department of Pediatrics, Division of Neonatology. (2000). Congenital hydrocephalus.
Wardinsky, T. D. (2002, October). A primer on hydrocephaly. Presentation conducted at the Alta California Regional Center Staff Conference, Sacramento, CA.
American Academy of Neurology, 651-695-2717, 800-879-1960, Email: [email protected],
American Association of Neurological Surgeons and Congress of Neurological Surgeons, “Neurosurgery://On-Call”,
Association for Spina Bifida and Hydrocephalus,
Birth Defect Research for Children, Inc., 407-895-0802,
Email: abу[email protected],
Guardians of Hydrocephalus Research Foundation, 718-743-4473, 800-458-8655
HYCEPH-L Listserv,
http://www.geocities.com/hycephl/
Hydrocephalus: A Guide for Patients, Families, and Friends by Chuck Toporek & Kellie Robinson, resource list,
http://www.patientcenters.com/hydrocephalus/news/hydro_organizations.html
Hydrocephalus Association, 415-732-7040, 888-598-3789,
Hydrocephalus Foundation, Inc. (HyFI), 781-942-1161,
Email: [email protected],
http://www.hydrocephalus.org
Hydrocephalus Support Group, Inc., 636-532-8228
Hyman-Newman Institute for Neurology and Neurosurgery (INN), 212-870-9600, http://nyneurosurgery.org
International Federation for Hydrocephalus and Spina Bifida (IFsbh),
March of Dimes Birth Defects Foundation,
914-428-7100, 888-663-4637,
Email: [email protected],
Massachusetts General Hospital, Pediatric Neurosurgery links,
http://neurosurgery.mgh.harvard.edu/pedi/lnkpedi.htm#Hydrocephalus
National Hydrocephalus Foundation, 562-402-3523, 888-857-3434,
National Information Center for Children and Youth with Disabilities (NICHCY), 800-695-0285 (Voice/TTY),
Email: [email protected],
http://www.nichcy.org
NIH/National Institute of Neurological Disorders and Stroke (NINDS), 301-496-5751, 800-352-9424,
National Organization for Rare Disorders (NORD), 203-744-0100, 800-999-NORD (voicemail), 203-797-9590 (TTY),
Email: [email protected],
O’Reilly and Associates, Inc. Hydrocephalus Center,
http://www.patientcenters.com/hydrocephalus/
Special Child: For Parents and Caregivers of Children with Special Needs,
http://www.specialchild.com/index.html
The Tarjan Center for Developmental Disabilities at UCLA,
http://www.tarjancenter.ucla.edu
Robin L. Hansen, M.D.
Theodore A. Kastner, M.D., M.S.
Felice Weber Parisi, M.D., M.P.H.
Patrick J. Maher, M.D.
Jaime Mejlszenkier, M.D., F.A.A.N.
James R. Popplewell, M.D.
Terrance D. Wardinsky, M.D.
Funded by a grant from the California Department of Developmental Services
For more information, contact:
Center for Health Improvement
1330 21st Street, Suite 100
Sacramento, CA 95814
(916) 901-9645
This document does not provide advice regarding medical diagnosis or treatment for any individual case, and any opinions or statements contained in this document are not intended to serve as a standard of medical care. Physicians are encouraged to view the considerations presented in this document in light of evolving scientific information. This document is not intended for use by the layperson. Reproduction of this document may be done with proper credit given to the California Department of Developmental Services and the Center for Health Improvement.