eMedicine Specialties > Neurosurgery > Trauma

Hydrocephalus

Author: Herbert H Engelhard III, MD, PhD, Director, UIC Neuro-Oncology Program, Chief, Division of Neuro-Oncology, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago
Coauthor(s): Kamran Sahrakar, MD, Clinical Professor, Department of Neurosurgery, University of California-Davis; Dachling Pang, MD, FRCS(C), FACS, Clinical Professor of Neurosurgery, Chief of Pediatric Neurosurgery, University of California Davis School of Medicine; Chief, Regional Center for Pediatric Neurosurgery, Kaiser Permanente Hospitals of Northern California
Contributor Information and Disclosures

Updated: Dec 19, 2007

Introduction

History of the Procedure

Hydrocephalus was first described by Hippocrates. Hydrocephalus was not treated effectively until the mid 20th century, when the development of appropriate shunting materials and techniques occurred. Interestingly, at the beginning of the 20th century, doctors (including urologists) attempted to introduce scopes into the ventricular system. Attempts were also made to remove the choroid plexus, which generates much of the cerebrospinal fluid (CSF), in an attempt to treat hydrocephalus. Today, the focus of hydrocephalus research is on pathophysiology, valve design in shunting, and minimally invasive techniques of treatment.

Problem

Hydrocephalus is the abnormal rise in CSF volume and, usually, pressure, that results from an imbalance of CSF production and absorption.

Frequency

The overall incidence of hydrocephalus is unknown. When cases of spina bifida are included, congenital hydrocephalus occurs in 2-5 births per 1000 births. Incidence of acquired types of hydrocephalus is unknown.

Etiology

The etiology of hydrocephalus in congenital cases is unknown. Very few cases (<2%) are inherited (X-linked hydrocephalus). The most common causes of hydrocephalus in acquired cases are tumor obstruction, trauma, intracranial hemorrhage, and infection.

Pathophysiology

Hydrocephalus can be subdivided into the following 3 forms:

  • Disorders of CSF production: This is the rarest form of hydrocephalus. Choroid plexus papillomas and choroid plexus carcinomas can secrete CSF in excess of its absorption.
  • Disorders of CSF circulation: This form of hydrocephalus results from obstruction of the pathways of CSF circulation. This can occur at the ventricles or arachnoid villi. Tumors, hemorrhages, congenital malformations (such as aqueductal stenosis), and infections can cause obstruction at either point in the pathways.
  • Disorders of CSF absorption: Conditions, such as the superior vena cava syndrome and sinus thrombosis, can interfere with CSF absorption. Some forms of hydrocephalus cannot be classified clearly. This group includes normal pressure hydrocephalus and pseudotumor cerebri.

Presentation

The various types of hydrocephalus can present differently in different age groups.

Acute hydrocephalus typically presents with headache, gait disturbance, vomiting, and visual changes. In infants, irritability or poor head control can be early signs of hydrocephalus. When the third ventricle dilates, the patient can present with Parinaud syndrome (upgaze palsy with a normal vertical Doll response) or the setting sun sign (Parinaud syndrome with lid retraction and increased tonic downgaze). Occasionally, a focal deficit, such as sixth nerve palsy, can be the presenting sign. Papilledema is often present, although it may lag behind symptomatology. Infants present with bulging fontanelles, dilated scalp veins, and an increasing head circumference. When advanced, hydrocephalus presents with brainstem signs, coma, and hemodynamic instability.

Normal pressure hydrocephalus has a very distinct symptomatology. The patient is older and presents with progressive gait apraxia, incontinence, and dementia. This triad of symptoms defines normal pressure hydrocephalus.

Indications

Most cases of symptomatic hydrocephalus need to be treated before permanent neurologic deficits result or neurologic deficits progress.

When an etiologic factor is known, hydrocephalus can be treated with temporary measures while the underlying condition is treated. Examples of temporary treatment measures are ventriculostomy until a posterior fossa tumor is resected or lumbar punctures in a neonate with intraventricular hemorrhage until the blood is absorbed and normal cerebrospinal fluid (CSF) absorption resumes.

Relevant Anatomy

See Intraoperative details for a discussion of relevant anatomy.

Contraindications

Few cases of hydrocephalus should not be treated. Cases in which treatment should not be implemented include the following:

  • The patient in whom a successful surgery would not affect the outcome (eg, a child with hydranencephaly)
  • In ventriculomegaly of senescence, the patient who does not have the symptom triad
  • Ex vacuo hydrocephalus is merely the replacement of lost cerebral tissue with cerebrospinal fluid. Because no imbalance in fluid production and absorption exists, this technically is not hydrocephalus.
  • Arrested hydrocephalus is defined as a rare condition in which the neurologic status of the patient is stable in the presence of stable ventriculomegaly. The diagnosis must be made extremely carefully because children can present with very subtle neurological deterioration (eg, slipping school performance) that is difficult to document.
  • Benign hydrocephalus of infancy is found in neonates and young infants. The children are asymptomatic, and head growth is normal. CT scan shows mildly enlarged ventricles and subarachnoid spaces.

More on Hydrocephalus

Overview: Hydrocephalus
Workup: Hydrocephalus
Treatment: Hydrocephalus
Follow-up: Hydrocephalus
References
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References

  1. Hahn YS, Engelhard H, McLone DG. Abdominal CSF pseudocyst. Clinical features and surgical management. Pediatr Neurosci. 1985-1986;12(2):75-9. [Medline].

  2. Aronyk KE. The history and classification of hydrocephalus. Neurosurg Clin N Am. Oct 1993;4(4):599-609. [Medline].

  3. Black PMcL, Ojemann RG. Hydrocephalus in adults. In: Youman JR, ed. Neurological Surgery. 3rd ed. Philadelphia, Pa:. WB Saunders Co;1990:927-944.

  4. Gleason PL, Black PM, Matsumae M. The neurobiology of normal pressure hydrocephalus. Neurosurg Clin N Am. Oct 1993;4(4):667-75. [Medline].

  5. McLone DG, Partington MD. Arrest and compensation of hydrocephalus. Neurosurg Clin N Am. Oct 1993;4(4):621-4. [Medline].

  6. Milhorat T. Hydrocephalus: Pathophysiology and Clinical Features. Neurosurgery. 1996;3:3625-3632.

  7. Pang D, Altschuler E. Low-pressure hydrocephalic state and viscoelastic alterations in the brain. Neurosurgery. Oct 1994;35(4):643-55; discussion 655-6. [Medline].

  8. Sainte-Rose C. Hydrocephalus in childhood.In: Youmans JR, ed. Neurological Surgery. Philadelphia, Pa:. WB Saunders Co;1996:890-926.

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Further Reading

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Keywords

hydrocephalus, abnormal rise in cerebrospinal fluid volume, abnormal rise in cerebrospinal fluid pressure, CSF, imbalance of cerebrospinal fluid production and absorption, spinal bifida, congenital hydrocephalus, acquired hydrocephalus, aqueductal stenosis, intracranial tumor obstruction, intracranial trauma, intracranial hemorrhage, intracranial infection, disorders of cerebrospinal fluid production, disorders of cerebrospinal fluid circulation, disorders of cerebrospinal fluid absorption, cerebrospinal fluid diversion, third ventriculostomy, normal pressure hydrocephalus

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Contributor Information and Disclosures

Author

Herbert H Engelhard III, MD, PhD, Director, UIC Neuro-Oncology Program, Chief, Division of Neuro-Oncology, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago
Herbert H Engelhard III, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, American Society for Cell Biology, American Society of Clinical Oncology, Chicago Medical Society, Congress of Neurological Surgeons, Illinois State Medical Society, Society for Neuro-Oncology, and Society for Neuroscience
Disclosure: Nothing to disclose.

Coauthor(s)

Kamran Sahrakar, MD, Clinical Professor, Department of Neurosurgery, University of California-Davis
Kamran Sahrakar, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Medical Association, California Medical Association, Florida Medical Association, and Nevada State Medical Association
Disclosure: Nothing to disclose.

Dachling Pang, MD, FRCS(C), FACS, Clinical Professor of Neurosurgery, Chief of Pediatric Neurosurgery, University of California Davis School of Medicine; Chief, Regional Center for Pediatric Neurosurgery, Kaiser Permanente Hospitals of Northern California
Dachling Pang, MD, FRCS(C), FACS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American College of Surgeons, Congress of Neurological Surgeons, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Duc Hoang Duong, MD, Associate Professor, Director of Neuroscience Physician Assistant Program, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles R Drew University
Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association and North American Skull Base Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ryszard M Pluta, MD, PhD, Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences at Warsaw, Poland; Senior Researcher, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH
Disclosure: Nothing to disclose.

CME Editor

Herbert H Engelhard III, MD, PhD, Director, UIC Neuro-Oncology Program, Chief, Division of Neuro-Oncology, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago
Herbert H Engelhard III, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, American Society for Cell Biology, American Society of Clinical Oncology, Chicago Medical Society, Congress of Neurological Surgeons, Illinois State Medical Society, Society for Neuro-Oncology, and Society for Neuroscience
Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD, Medical Director, Northstar Neuroscience, Inc
Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons
Disclosure: Nothing to disclose.

 
 
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