CSF Shunts gone bad!

Brad Sobolewski, MD

Ventricular system
LATERAL LATERAL

3rd

4th

CISTERNS
Subarachnoid space

STATS Total volume 50ml Production 20ml/hr Turnover 3-4x/day

Brad Sobolewski, 2013

Hydrocephalus
Imbalance of absorption and production of CSF Estimated incidence of 1/500-1000 children 125,000+ shunts OBSTRUCTIVE: Ventricular system is blocked
Not possible to have complete obstruction

COMMUNICATING: Subarachnoid system blocked

Brad Sobolewski, 2013

Etiology
Congenital infection: Rubella, CMV, Toxo, Syphilis Acquired: Infection, trauma, tumors, head bleeds Neural tube defects: associated with Chiari or aqueductal stenosis. Linked to teratogens and deficiency of folate. Isolated: aqueductal stenosis (inflammation d/t intrauterine infection) X-Linked hydrocephalus: stenosis of aqueduct of Sylvius
Brad Sobolewski, 2013

Etiology CNS malformations

Chiari II Often accompanies NTD Brainstem and Cerebellum are displaced caudally

Brad Sobolewski, 2013

Etiology CNS malformations

Dandy-Walker
Large posterior fossa cyst continuous with 4th ventricle Abnormal cerebellar development Hydrocephalus in 70-90%

Brad Sobolewski, 2013

Obstructive Hydrocephalus
Ventricular system is blocked CSF accumulates proximally
LATERAL LATERAL

3rd

4th

CISTERNS
Subarachnoid space

Brad Sobolewski, 2013

Communicating Hydrocephalus
Subarachnoid system blocked
Results in impaired absorption Entire system is dilated

Causes
IVH/SAH Meningitis Scarring after inflammatory process

Brad Sobolewski, 2013

Pseudotumor cerebri
Isnt it due to overproduction of CSF? Pathogenesis unknown
Cerebral venous outflow abnormalities Increased CSF outflow resistance at arachnoid or lymphatic level Obesity related changes to intracranial venous pressure Altered Na and H2O retention mechanisms Abnormal Vitamin A metabolism

Brad Sobolewski, 2013

Excessive CSF Production

Rare Only really happens in cases of a functional choroid plexus papilloma

Brad Sobolewski, 2013

Symptoms of hydrocephalus
Headache Vomiting: increased ICP in the posterior fossa Behavioral changes Drowsiness: midbrain/brainstem dysfunction Visual changes: Optic Nerve compression Incoordination Loss of developmental milestones Head circumference increases rapidly Sunsetting eyes: fixed downward gaze

Pro-Tip: These symptoms obviously vary based on the age of the patient
Brad Sobolewski, 2013

Shunt Devices
Proximal portion is placed in a ventricle (usually R) Could also be in an intracranial cyst or lumbar subarachnoid space

Distal portion
Internalized: peritoneum, pleura, atrium Externalized
EVD: Acute hydrocephalus for pressure monitoring, infected shunt Ommaya reservoir: Generally for administration of drugs (antibiotics or chemo)
Brad Sobolewski, 2013

Shunt Complications
Mechanical Obstruction (Malfunction/Failure)
proximal tip is obstructed with cells, choroid plexus, or debris Kinking of the tubing Migration of the distal end

Infection Acquired Chiari I due to over draining Slit ventricle syndrome Intraventricular hemorrhage (subdural)

Brad Sobolewski, 2013

Shunt infections
Risk of 5-15% overall Sx are generally few, fever is variable Paucity of meningeal Sx as there is no communication between shunt and meninges VP shunt infections can manifest as peritonitis VA shunt infections as bacteremia/endocarditis

Brad Sobolewski, 2013

Shunt infections
Increased risk Highest in initial month after placement Risk extends up to 6 months post op Patients requiring serial revisions Intracranial hemorrhage Cranial fracture with CSF leak Craniotomy

Brad Sobolewski, 2013

Shunt infections
What are the most common infectious agents Proximal end: skin flora 50% coag negative staph, 33% S. aureus Distal end: peritonitis/intestinal perforation or hematogenous seeding Streptococci, gram negative (P. aeruginosa), anaerobes, mycobacteria, fungi

Brad Sobolewski, 2013

Shunt infections
Treatment No RCTs or prospective data Remove the device + IV antibiotics (vanc + gram negative) Decreasing risk Periop Vanc Antibiotic impregnated catheters

Brad Sobolewski, 2013

Shunt malfunctions
Mechanical failure
Majority of 1st failures due to obstruction
Shunt over drains Ventricles shrink Tip gets clogged against choroid plexus

15% due to fractured tubing

Brad Sobolewski, 2013

Shunt malfunctions
Median survival of a shunt (before need for revision) child under 2 years of age is 2 years over two years of age is 8 - 10 years Also associated with decreased survival Shunts inserted prior to first birthday Inserted when pt. weighed <3,000g
Pro-Tip: Children with NTD have longer shunt survival than children with hydrocephalus from other etiologies
Brad Sobolewski, 2013

Symptoms associated with shunt malfunctions

PEC, 2008
647 visits to the ED 78% younger than age 1 at time of insertion of shunt 38% failure rate at 3 years, 8.5% by infection Built a decision tree model
+LR 44.6 13.7 11.1 6.02 4.28 -LR 1.84 1.75 1.58 1.86 1.22
Brad Sobolewski, 2013

Sign/Symptom Bulging fontanel Irritability Nausea/Vomiting Accelerated head growth Headache

Shunt series
Radiographs of the skull, neck, chest, and abdomen Look for mechanical breaks, kinks, and disconnections in the shunt Utility Pitetti, PEC, 2007 Retro review of 291 kids (461 ED visits) 78% had a shunt series 15% (71/291) Dx with malfunction 22 of these 71 had a normal head CT 6 of these 22 had an abnormal shunt series
Brad Sobolewski, 2013

Neuroimaging
Head CT Not always diagnostic, even if ventricles are bigger Cumulative radiation is a concern Iskandar Pediatrics, 1998 1/3 of patients Dx with shunt malfunction were not supported by CT findings Rapid sequence MRI is now being explored
Brad Sobolewski, 2013

Imaging test characteristics

Zorc, PEC, 2002
60/233 reviewed retrospectively had a shunt malfunction

Brad Sobolewski, 2013

Management of shunt malfunctions

Replacement or externalization
If infected the EVD is preferred Otherwise it is up to the surgeon No comparison studies in kids

Bedside EVD
Kakarla Neurosurg, 2008 retro review of 346 adults that had bedside EVD Analyzed success of placement, ideal ipsilateral frontal horn or 3rd ventricle Highest success in cases of IVH and trauma Midline shift decr success Caveat: Not studied in shunted patients
Brad Sobolewski, 2013

Shunt tap
Indications
Diagnostic
Suspected shunt blockage, infection or meningitis

Therapeutic
Severely raised ICP in the presence of a VP shunt

Contraindications
Skin infection over shunt site Coagulopathy Lack of shunt imaging/info
Brad Sobolewski, 2013

Shunt tap
Procedure
23 or 25G butterfly needle Aspiration can suck choroid plexus into the tube = bad

Utility
Opening pressure >25cm H2O associated with distal obstruction in 90% Poor flow associated with proximal shunt in >90%
Brad Sobolewski, 2013

Shunt tap
When should a shunt tap be performed?
Miller J. Neurosurg Peds, 2008 Retro review of 155 patients Low utility overall, doesnt often contribute to Dx Risks
Infection Changes in flow dynamics post shunt tap can cause a partially working shunt to malfunction

Brad Sobolewski, 2013

Miller, J. Neurosurg Pediatrics 2008

Brad Sobolewski, 2013

Management of a suspected shunt malfunction

Treatments for elevated ICP in shunt malfunctions

Do they work? Answer: Probably No literature on hypertonic/osmotic therapies General pearls are still useful prior to definitive management

Brad Sobolewski, 2013

ICP Management
In shunt malfunctions NONE of these are as important as a trip to the OR Positioning Head midline, elevated 30o Maintain homeostasis Treat hypoxia (sats >95%), hypercarbia , hypotension, and hypoglycemia Temperature control Therapeutic cooling (fever incr metabolism and CBF) Mild sedation (dont cause hypotension) Control severe shivering w/ paralytics Prophylactic fosphenytoin to patients at risk for seizures Parenchymal abnormalities, depressed skull fractures, and TBI No definitive evidence in children
Brad Sobolewski, 2013

ICP Management
In shunt malfunctions NONE of these are as important as a trip to the OR

Intubate if: Respiratory failure Loss of airway protective reflexes Refractory hypoxia GCS <8 Acute herniation needing hyperventilation

Brad Sobolewski, 2013

ICP Management
In shunt malfunctions NONE of these are as important as a trip to the OR

In the intubated patient Avoid high pressures (decr venous return by incr intrathoracic pressure) Hyperventilation: though it can lower ICP (if you get ETCO2 25-30), aggressive hyperventilation leads to cerebral vasoconstriction and decr CBF Reserved for patients herniating or at imminent risk

Brad Sobolewski, 2013

ICP Management
In shunt malfunctions NONE of these are as important as a trip to the OR

Experimental therapies Hypothermia Indomethacin Stuff that doesnt help Steroids (unless swelling from a tumor or abscess)

Brad Sobolewski, 2013

ICP Management
In shunt malfunctions NONE of these are as important as a trip to the OR

Hypertonic 3% saline 6-10ml/kg over 5-10 min

Generates an osmotic gradient between the intravascular space and cerebral tissue Effective plasma volume expander in multiple trauma patients May have beneficial effects on cerebrovascular regulation Effective to a serum osmo of 360

Brad Sobolewski, 2013

ICP Management
In shunt malfunctions NONE of these are as important as a trip to the OR

Mannitol 20% solution 0.25-1g/kg over 10-20 min

An osmotic diuretic Effective mainly around the lesion, where blood brain barrier integrity is impaired It may also reduce CSF production Hypovolemia is a real concern & pts will start diuresing in 20-30 minutes (in the scanner) Not effective above a serum osmo of 320

Brad Sobolewski, 2013