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Neurosurgery for Hydrocephalus

  • Author: Herbert H Engelhard, III, MD, PhD, FACS; Chief Editor: Brian H Kopell, MD  more...
 
Updated: Oct 28, 2015
 

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.

An image depicting hydrocephalus can be seen below.

Noncommunicating obstructive hydrocephalus caused Noncommunicating obstructive hydrocephalus caused by obstruction of the foramina of Luschka and Magendie. This MRI sagittal image demonstrates dilatation of lateral ventricles with stretching of corpus callosum and dilatation of the fourth ventricle.
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Problem

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

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Epidemiology

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.

Tanaka et al concluded that the incidence of idiopathic normal pressure hydrocephalus was 1.4% in their study of an elderly Japanese population.[1]

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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.

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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.
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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.

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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.

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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.
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Contributor Information and Disclosures
Author

Herbert H Engelhard, III, MD, PhD, FACS Director, UIC Neuro-Oncology Program, Chief, Division of Neuro-Oncology, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago College of Medicine

Herbert H Engelhard, III, MD, PhD, FACS is a member of the following medical societies: American Association for Cancer Research, Society for Neuroscience, Society for Neuro-Oncology, American Society for Cell Biology, Congress of Neurological Surgeons, Chicago Medical Society, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Illinois State Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kamran Sahrakar, MD, FACS Clinical Professor, Department of Neurosurgery, University of California, San Francisco, School of Medicine

Kamran Sahrakar, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, Congress of Neurological Surgeons, American Association of Neurological Surgeons

Disclosure: Nothing to disclose.

Dachling Pang, MD, FRCSC, FACS, FRCSE Professor 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, FRCSC, FACS, FRCSE is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Ontario Medical Association, Congress of Neurological Surgeons, American Association of Neurological Surgeons, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ryszard M Pluta, MD, PhD Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Polish Society of Neurosurgeons, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from St Jude Neuromodulation for consulting; Received consulting fee from MRI Interventions for consulting.

Additional Contributors

Duc Hoang Duong, MD Professor, Chief Physician, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles Drew University of Medicine and Science

Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association, Congress of Neurological Surgeons, North American Skull Base Society

Disclosure: Nothing to disclose.

Acknowledgements

The author would like to thank Dr. Yoon Hahn and Dr. David McLone for their guidance in treating patients with hydrocephalus.

References
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  2. Klimo P Jr, Van Poppel M, Thompson CJ, Baird LC, Duhaime AC, Flannery AM, et al. Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 6: Preoperative antibiotics for shunt surgery in children with hydrocephalus: a systematic review and meta-analysis. J Neurosurg Pediatr. 2014 Nov. 14 Suppl 1:44-52. [Medline].

  3. Whitehead WE, Riva-Cambrin J, Wellons JC 3rd, Kulkarni AV, Holubkov R, Illner A, et al. No significant improvement in the rate of accurate ventricular catheter location using ultrasound-guided CSF shunt insertion: a prospective, controlled study by the Hydrocephalus Clinical Research Network. J Neurosurg Pediatr. 2013 Oct 11. [Medline].

  4. Janson CG, Romanova LG, Rudser KD, Haines SJ. Improvement in clinical outcomes following optimal targeting of brain ventricular catheters with intraoperative imaging. J Neurosurg. 2013 Oct 11. [Medline].

  5. Mihajlovic M, Bogosavljevic V, Nikolic I, Mrdak M, Repac N, Scepanovic V, et al. Surgical Treatment Problems of Hydrocephalus Caused by Spontaneus Intraventricular Hemorrhage in Prematurely Born Children. Turk Neurosurg. 2013. 23(5):593-599. [Medline].

  6. Nocun A, Mosiewicz A, Kaczmarczyk R, Kazalska T, Czekajska-Chehab E, Chrapko B, et al. Early brain perfusion improvement after ventriculoperitoneal shunt surgery in patients with idiopathic normal pressure hydrocephalus evaluated by 99mTc-HMPAO SPECT - preliminary report. Nucl Med Rev Cent East Eur. 2015. 18 (2):84-8. [Medline].

  7. Reddy GK, Bollam P, Caldito G. Long-term outcomes of ventriculoperitoneal shunt surgery in patients with hydrocephalus. World Neurosurg. 2014 Feb. 81 (2):404-10. [Medline].

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  9. Kazui H, Miyajima M, Mori E, Ishikawa M, SINPHONI-2 Investigators. Lumboperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (SINPHONI-2): an open-label randomised trial. Lancet Neurol. 2015 Jun. 14 (6):585-94. [Medline].

  10. Rasul FT, Marcus HJ, Toma AK, Thorne L, Watkins LD. Is endoscopic third ventriculostomy superior to shunts in patients with non-communicating hydrocephalus? A systematic review and meta-analysis of the evidence. Acta Neurochir (Wien). 2013 May. 155(5):883-9. [Medline].

  11. Williams TA, Leslie GD, Dobb GJ, Roberts B, van Heerden PV. Decrease in proven ventriculitis by reducing the frequency of cerebrospinal fluid sampling from extraventricular drains. J Neurosurg. 2011 Nov. 115(5):1040-6. [Medline].

  12. Eide PK, Sorteberg W. Outcome of surgery for idiopathic normal pressure hydrocephalus: Role of pre-operative static and pulsatile intracranial pressure. World Neurosurg. 2015 Sep 28. [Medline].

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

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

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

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

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

 
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Noncommunicating obstructive hydrocephalus caused by obstruction of the foramina of Luschka and Magendie. This MRI sagittal image demonstrates dilatation of lateral ventricles with stretching of corpus callosum and dilatation of the fourth ventricle.
Communicating hydrocephalus with surrounding "atrophy" and increased periventricular and deep white matter signal on fluid-attenuated inversion recovery (FLAIR) sequences. Note that apical cuts (lower row) do not show enlargement of the sulci, as is expected in generalized atrophy. Pathological evaluation of this brain demonstrated hydrocephalus with no microvascular pathology corresponding with the signal abnormality (which likely reflects transependymal exudate) and normal brain weight (indicating that the sulci enlargement was due to increased subarachnoid cerebrospinal fluid [CSF] conveying a pseudoatrophic brain pattern).
 
 
 
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