Cerebral shunt

Cerebral shunts are commonly used to treat hydrocephalus, the swelling of the brain due to excess buildup of cerebrospinal fluid (CSF). If left unchecked, the cerebral spinal fluid can build up leading to an increase in intracranial pressure (ICP) which can lead to intracranial hematoma, cerebral edema, crushed brain tissue or herniation.^[1] The cerebral shunt can be used to alleviate or prevent these problems in patients who suffer from hydrocephalus or other related diseases. Shunts can come in a variety of forms but all of them consist of a pump or drain connected to a long catheter, the end of which is usually placed in the peritoneal cavity. The main differences between shunts are usually in the materials used to construct them, the types of pumps used, and whether the pump is programmable or not.^[2]

Types of valves

Valve type	Description
Delta	Designed to prevent overdrainage. Remains closed until ICP reaches a predetermined level. Leaves shunted ventricle larger than the non-shunted ventricles. [3][4]
Medium pressure cylindrical	Can lead to uneven drainage of ventricles.[3]
Nulsen and Spitz	Contains two ball-valve units connected with a spring. Does not have an adjustable pressure setting. First mass produced valve used to treat hydrocephalus in 1956.[5]
Spitz-Holter	Uses slits in silicone to avoid mechanical failure.[6][7]
Anti-siphon	Prevents over drainage by preventing the siphon effect. The device closes when the pressure within the valve becomes negative relative to the ambient pressure. Prevents overdrainage that might occur when a patient sits, stands or rapidly changes posture.[8]
Sigma	The Sigma valve operates on a flow-control mechanism as opposed to the pressure-control system of other valves. The device can regulate CSF flow changes without being programmed or surgically changed. The first iteration was introduced in 1987. Valve operated in three stages to prevent over and under drainage.[9]

Shunt location

The location of the shunt is determined by the neurosurgeon based on the type and location of the blockage causing hydrocephalus. All brain ventricles are candidates for shunting. The catheter is most commonly placed in the abdomen but other locations include the heart and lungs.^[10] Shunts can often be named after the route used by the neurosurgeon. The distal end of the catheter can be located in just about any tissue with enough epithelial cells to absorb the incoming CSF. Below are some common routing plans for cerebral shunts.

Shunt routing

Route	Location of Fluid Drain
Ventriculo-peritoneal shunt (VP shunt)	Peritoneal cavity
Ventriculo-atrial shunt (VA shunt)	Right atrium of the heart
Ventriculo-pleural shunt (VPL shunt)	Pleural cavity
Lumbar-peritoneal shunt (LP shunt)	Peritoneal cavity

In addition to these four common locations for shunt drainage, the gallbladder and ureter can be suitable locations as well.

Complications

There are a number of complications associated with shunt placement. Many of these complications occur during childhood and cease once the patient has reached adulthood. Many of the complications seen in patients require immediate shunt revision (the replacement or reprogramming of the already existing shunt). The common symptoms often resemble the new onset of hydrocephalus such as headaches, nausea, vomiting, double-vision, and an alteration of consciousness.^[10] Furthermore the shunt failure rate 2 years after implantation has been estimated to be as high as 50%.^[11]

Infection

Infection is a common complication that normally affects pediatric patients because they have not yet built up immunities to a number of different diseases. Normally the incidence of infection decreases as the patient grow older, and the body gains immunity to various infectious agents.^[10] Shunt infection is a common problem and can occur in up to 27% of patients with a shunt. Infection can lead to long term cognitive defects, neurological problems and in some cases death. Common microbial agents for shunt infection include Staphylococcus epidermidis, Staphylococcus aureus, and Candida albicans. Further factors leading to shunt infection include shunt insertion at a young age (<6 months old) and the type of hydrocephalus being treated. There is no strong correlation between infection and shunt type.^[12] The symptoms of a shunt infection are very similar to the symptoms seen in hydrocephalus but can also include fever and elevated white blood cell counts.^[13]

Obstruction

Another leading cause of shunt failure is the blockage of the shunt at either the proximal or distal end. At the proximal end the shunt valve can become blocked due to the buildup of excess protein in the CSF. The extra protein will collect at the point of drainage and slowly clog the valve. The shunt can also become blocked at the distal end if the shunt is pulled out of the abdominal cavity (in the case of VP shunts), or from similar protein buildup. Other causes of blockage are overdrainage and slit ventricle syndrome.^[10]

Over drainage

Over drainage occurs when a shunt has not been adequately designed for the particular patient. Overdrainage can lead to a number of different complications some of which are highlighted below.

Usually one of two types of overdrainage can occur. First when the CSF drains too rapidly, a condition known as extra-axial fluid collection can occur. In this condition the brain collapses on itself resulting in the collection of CSF or blood around the brain. This can cause severe brain damage by compressing the brain. Furthermore a subdural hematoma may develop. Extra-axial fluid collection can be treated in three different ways depending on the severity of the condition. Usually the shunt will be replaced or reprogrammed to release less CSF and the fluid collected around the brain will be drained. The second condition known as slit ventricle syndrome occurs when CSF slowly overdrains, over several years. More information on slit ventricle syndrome appears below.^[10][14]

Chiari I malformation

Recent studies have shown that over drainage of CSF due to shunting can lead to acquired Chiari I Malformation.^[15] It was previously thought that Chiari I Malformation was a result of a congenital defect but new studies have shown that overdrainage of Cysto-peritoneal shunts used to treat arachnoid cysts can lead to the development of posterior fossa overcrowding and tonsillar herniation, the latter of which is the classic definition of Chiari Malformation I. Common symptoms include major headaches, hearing loss, fatigue, muscle weakness and loss of cerebellum function.

Slit ventricle syndrome

Slit ventricle syndrome is an uncommon disorder associated with shunted patients, but results in a large number of shunt revisions. The condition usually occurs several years after shunt implantation. The most common symptoms are similar to normal shunt malfunction, but there are several key differences. First the symptoms are often cyclical and will appear and then subside several times over a lifetime. Second, the symptoms can be alleviated by lying prone. In the case of shunt malfunction neither time nor postural position will affect the symptoms.

The condition is often thought to occur during a period where overdrainage and brain growth occur simultaneously. In this case the brain fills the intraventricular space, leaving the ventricles collapsed. Furthermore, the compliance of the brain will decrease, which prevents the ventricles from enlarging, thus reducing the chance for curing the syndrome. The collapsed ventricles can also block the shunt valve, leading to obstruction. Since the effects of slit ventricle syndrome are irreversible, constant care in managing the condition is needed.^[14][15]

Intraventricular hemorrhage

An intraventricular hemorrhage can occur at any time during or after a shunt insertion or revision. The hemorrhage can cause an impairment in shunt function which can lead to severe neurological deficiencies.^[16] Studies have shown that intraventricular hemorrhage can occur in nearly 31% of shunt revisions. ^[17]

Conditions requiring shunting

Below is a short list of known complications that can lead to hydrocephalus requiring shunting.

Diagnoses	Description	Incidence
Congenital hydrocephalus	A wide range of genetic abnormailities that could lead to hydrocephalus at birth.	0.04-0.08%[18]
Tumor	A number of different tumors can lead to CSF blockage if they are located in certain areas. Some of these areas include the lateral ventricles, the posterior fossa, and intraspinal tumors. The tumors may be malignant or benign.[19]	Unknown
Spina bifida	Specifically spina bifida myelomeningocele can cause the development of hydrocephalus because the cerebellum will block the flow of CSF in a development of Chiari Malformation II.	.125%[20]
Congenital aqueductal stenosis	A genetic disorder which can cause deformations of the nervous system. The defect is commonly associated with mental retardation, abducted thumbs and spastic paraplegia.[18]	.003%[18]
Craniosynostosis	Craniosynostosis occurs when the sutures of the skull close too early. The result of multiple sutures fusing before the brain stops growing is an increase in ICP leading to hydrocephalus.[21]	0.05%[21]
Dandy-Walker syndrome	Patients usually present with a cystic deformity of the fourth ventricle, hypoplasia of the cerebellar vermis, and an enlarged posterior fossa. The condition is a genetically heritable disease.[22]	0.003%[23]
Arachnoid cyst	A genetic defect caused when CSF rushes into ruptured arachnoid mater. The resulting cyst can then block the normal flow of CSF from the brain resulting in hydrocephalus as well as other defects. The most common locations for an arachnoid cyst are the middle fossa and the posterior fossa. The most common symptoms are nausea and vertigo.[24]	0.05%[25]

Idiopathic Intracranial Hypertension A rare neurological disorder affecting approximately 1 in 100, 000 people, most of whom are women of child baring age. IIH result in a raised intracranial pressure and can lead to permanent loss of vision.

Removing shunts

Though there have been many cases of patients reaching "shunt independence", there is no common accord in which doctors can agree in which a patient might survive without a shunt. Another problem with shunt removal is that it is very difficult to discern when a patient might be shunt independent without very specific conditions. Overall shunt removal is a rare but not unheard of procedure.^[26]

References

1. ^ Hlatky, R., Valadka, A. B., Robertson, C. S.; Intracranial Hypertension and Cerebral Ischemia After Severe Traumatic Brain Injury; Neurosurg Focus; 14:1-4, 2003
2. ^ Bradley, W. G., Bahl, G., Alksne, J. F.; Idiopathic Normal Pressure Hydrocephalus May be a "Two Hit" Disease: Benign External Hydrocephalus in Infancy Followed by Deep White Matter Ischemia in Late Adulthood; Journal of Magnetic Resonance Imaging; 24:747-755 (2006)
3. ^ ^{a b} Jain, H., Natarajan, K., Sgouros, S.; Influence of the Shunt Type in the Difference in Reduction of Volume Between the Two Lateral Ventricles in Shunted Hydrocephalic Children; Childs Nerv Syst; 21:552-558 (2005)
4. ^ (2008). http://www.medtronic.com/neurosurgery/valves.html, Retrieved November 30, 2009
5. ^ Boockvar, J. A., Loudon, W., Sutton, L. N.; Development of the Spitz-Holter valve in Philadelphia; J Neurosurg; 95:145-147, 2001
6. ^ http://www.uh.edu/engines/epi2582.htm
7. ^ http://www.medterms.com/script/main/art.asp?articlekey=26245
8. ^ Anti-Siphon Device; Integra Neurosciences; Label 2002
9. ^ http://www.integra-ls.com/products/?product=47, Retrieved November 30, 2009
10. ^ ^{a b c d e} Interview with Dr. Gary R. Gropper; Piedmont Neurosurgery; October 15, 2009
11. ^ Drake, J. M., Kestle, J. R. W., Tuli, S.; CSF Shunts 50 Years On – Past, Present and Future; Child’s Nerv Syst; 16:800-804, 2000
12. ^ Enger P. O., Swendsen, F., Wester, K.; CSF Shunt Infections in Children: Experiences From a Population-Based Study; Acta Neurochir; 145:243-248, 2003
13. ^ Brook, I.; Meningitis and Shunt Infection Caused by Anaerobic Bacteria in Children; Pediatric Neurology; 26:99-105, 2002
14. ^ ^{a b} Browd, S. R., Gottfrie, O. N., Ragel, B. T., Kestle, J. R. W.; Failure of Cerebrospinal Fluid Shunts: Part II: Overdrainage, Loculation, and Abdominal Complications; Pediatric Neurology; 34:171-176, 2006
15. ^ ^{a b} Martinez-Lage, J. F., Ruiz-Espejo, A. M., Almagro, M., Alfaro, R., Felipe-Murcia, M., Lopez-Guerrero, A. L.; CSF Overdrainage in Shunted Intracranial arachnoid Cysts: A Series and Review; Childs Nerv Syst; 25:1061-1069, 2009
16. ^ Martinez-Lage, J. F., Almagro, M., Ruiz-Espejo, A., Leon. M., Garcia-Martinez, S., Moralo, S.; Keeping CSF Valve Function With Urokinase in Children With Intra-Ventricular Hemorrhage and CSF Shunts; Childs Nerv Syst; 25:981-986 (2009)
17. ^ Brownlee R. D., Dold O.N.R., Myles S.T.; Intraventricular hemorrhage complicating ventricular catheter revision: incidence and effect on shunt survival; Pediatr Neurosurg; 22:315–320, 1995
18. ^ ^{a b c} Chrander-Stumpel C., Fryns, J. P.; Congenital Hydrocephalus: Nosology and Guidelines for Clinical Approach and Genetic Counseling; Eur J Pediatr; 157:355-362, 1998
19. ^ Zuccaro, G., Sosa, F., Cuccia, V., Lubieniecky, F., Monges, J.; Lateral Ventricle Tumors in Children: A Series of 54 Cases; Child's Nerv Syst; 15:774-785, 1999
20. ^ http://www.nlm.nih.gov/medlineplus/ency/article/001558.htm
21. ^ ^{a b} http://www.chw.org/display/PPF/DocID/21810/router.asp
22. ^ Tal, Y., Freigang, B., Dunn, H. G., Durity, F. A., Moyes, P. D.; Dandy-Walker Syndrome: Analysis of 21 Cases; Develop. Med. Child Neurol.; 22:189-201, 1980
23. ^ http://emedicine.medscape.com/article/408059-overview
24. ^ Samii M., Carvallho, G. A., Schuhmann, M. U., Mattthies, C.; Arachnoid Cysts of the Posterior Fossa; Surgical Neurology; 51:376-382, 1999
25. ^ http://www.wrongdiagnosis.com/a/arachnoid_cysts/prevalence.htm
26. ^ Iannelli, A., Rea, G., Rocco, C. D.; CSF Shunt Removal in Children with Hydrocephalus; Acta Neurchir (Wien); 147:503-507 (2005)

External links

• MeSH Cerebrospinal+Fluid+Shunts