Neurosurgery for Hydrocephalus

Neurosurgery for Hydrocephalus

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Hydrocephalus, a condition first described by Hippocrates, is the abnormal rise in cerebrospinal fluid (CSF) volume and, usually, pressure, that results from an imbalance of CSF production and absorption.

Hydrocephalus was not treated effectively until the mid-20th century, when the development of appropriate shunting materials and techniques occurred. 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 CSF, in an attempt to treat hydrocephalus. 

Hydrocephalus research and treatment have advanced tremendously in the last 20 years. Today, the focus of hydrocephalus research is on pathophysiology, shunting (eg, new shunt materials and programmable valve design), and minimally invasive techniques of treatment. Areas of research include the following:

Hydrocephalus can be subdivided into the following three forms:

Some forms of hydrocephalus cannot be classified clearly. This group includes normal-pressure hydrocephalus and pseudotumor cerebri.

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, [1] trauma, intracranial hemorrhage, and infection.

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

In general, outcome is good. A typical patient should return to baseline after shunting, unless prolonged elevated intracranial pressure (ICP) or brain herniation has occurred. The neurologic function of children is optimized with shunting. Infection, especially if repeated, may affect cognitive status.

The best long-term results in the most carefully selected patients are no better than 60% in normal-pressure hydrocephalus. Few complete recoveries occur. Often, gait and incontinence respond to shunting, but dementia responds less frequently.

Often, various other neurologic abnormalities associated with hydrocephalus are the limiting factor in patient recovery. Examples are migrational abnormalities and postinfectious hydrocephalus.

In a study of responders and nonresponders to shunt surgery for idiopathic normal-pressure hydrocephalus, responders were found to have much higher preoperative pulsatile ICP than nonresponders did. [3]

Abecassis IJ, Hanak B, Barber J, Mortazavi M, Ellenbogen RG. A Single-Institution Experience with Pineal Region Tumors: 50 Tumors Over 1 Decade. Oper Neurosurg (Hagerstown). 2017 Oct 1. 13 (5):566-575. [Medline].

Tanaka N, Yamaguchi S, Ishikawa H, Ishii H, Meguro K. Prevalence of possible idiopathic normal-pressure hydrocephalus in Japan: the Osaki-Tajiri project. Neuroepidemiology. 2009. 32 (3):171-5. [Medline].

Eide PK, Sorteberg W. Outcome of Surgery for Idiopathic Normal Pressure Hydrocephalus: Role of Preoperative Static and Pulsatile Intracranial Pressure. World Neurosurg. 2016 Feb. 86:186-193.e1. [Medline].

Espay AJ, Da Prat GA, Dwivedi AK, Rodriguez-Porcel F, Vaughan JE, Rosso M, et al. Deconstructing normal pressure hydrocephalus: Ventriculomegaly as early sign of neurodegeneration. Ann Neurol. 2017 Sep 11. [Medline].

Tuniz F, Vescovi MC, Bagatto D, Drigo D, De Colle MC, Maieron M, et al. The role of perfusion and diffusion MRI in the assessment of patients affected by probable idiopathic normal pressure hydrocephalus. A cohort-prospective preliminary study. Fluids Barriers CNS. 2017 Sep 12. 14 (1):24. [Medline].

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].

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 Dec. 12 (6):565-74. [Medline].

Janson CG, Romanova LG, Rudser KD, Haines SJ. Improvement in clinical outcomes following optimal targeting of brain ventricular catheters with intraoperative imaging. J Neurosurg. 2014 Mar. 120 (3):684-96. [Medline].

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):593-9. [Medline].

Nocuń 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].

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].

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

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].

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].

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].

Herbert H Engelhard, III, MD, PhD, FACS, FAANS Professor of Clinical Neurosurgery and Bioengineering, Chief, Division of Neuro-Oncology, Medical Director, UIC Neurosurgery Clinic, Department of Neurosurgery, University of Illinois at Chicago College of Medicine

Herbert H Engelhard, III, MD, PhD, FACS, FAANS 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, Chicago Neurological Society, Congress of Neurological Surgeons, Illinois State Medical Society, North American Spine Society, Society for Neuro-Oncology, Society for Neuroscience

Disclosure: Nothing to disclose.

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.

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.

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, American Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons, International Parkinson and Movement Disorder Society, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from Abbott Neuromodulation for consulting.

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.

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

Neurosurgery for Hydrocephalus

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