Management of Hydrocephalus Complications:

Complications

1. Obstruction due to malposition of the device or inaccurate selection of components with excessive resistance in the system. (McCullough, 1995) 2. CSF and shunt Infections: (Oram, 2006) Shunt infections can present in a number of ways: a. Meningitis. b. An indolent infection with a chronic inflammatory response leading to shunt obstruction. c. Local soft tissue infection around the shunt hardware with wound breakdown and/or purulent discharge. d. Infection within the peritoneal cavity that presents with abdominal pain, shunt obstruction and/or an accumulation of fluid within the peritoneal cavity. (Frim and Gupta, 2006) Immediate CSF infection is rare, but most septic incidents occur within the first two months of insertion or revision of a shunt. They usually manifest with obstructive symptoms, fever, cellulitis, and leukocytosis. The most reliable therapy is complete shunt removal with temporary external drainage and appropriate systemic antibiotics. A new device can be inserted after obtaining at least two consecutive negative cultures 48 hours apart (McCullough, 1995) Etiology: a. Coagulase-negative staphylococci and Staphylococcus aureus cause about 75% of shunt infections. b. Gram-negative bacilli cause 20% of shunt infections. c. The remainder is caused by Micrococcus species, Streptococcus species, fungi (usually Candida species) and anaerobes such as Propionibacterium acnes. (Oram, 2006) Cause: Contamination of the apparatus at the time of surgical placement is the most common mechanism by which shunts become infected. Only 20% of infections due to skin flora are caused by an organism present before surgery. Shunt infections are also caused by retrograde infection originating at the distal end of the shunt. The abdomen is the usual source, and infection can occur through either peritonitis resulting from penetration of the bowel by the distal end of the catheter or arising spontaneously may cause a secondary ascending infection of the shunt. Approximately 80% of shunt infections occur within 3 months of shunt placement or revision. Patients may present
1

Management of Hydrocephalus

Complications

with shunt malfunction and associated symptoms such as headache, vomiting, irritability and lethargy. Fever and meningeal signs may not be present. (Oram, 2006) Diagnosis of shunt infection Clinical: Patient presents with : 1. 2. 3. 4. 5. 6. fever or with evidence of shunt malfunction. Low grade fever between 37.5 and 38.8C. poor feeding. failure to thrive. Irritability. bulging fontanelle.

Once the cranial sutures are closed, as in older children and adults, a somewhat different spectrum of symptoms occurs, such as: 7. fever. 8. Headaches. 9. vomiting not associated with meals. 10.lethargy. 11.Abdominal pain and tenderness or evidence of local inflammatory reactions at the wound sites or along the shunt tract also provide specific clues as to the possibility and location of infections. Investigations : 1. Radiological: Patients should have a radiological evaluation like: a. anteroposterior and lateral skull, chest, abdominal x-ray. b. CT scan. 2. The Shunt Tap: a. It's performed to obtain CSF for study and to avoid contamination of the CSF or the shunt itself by skin flora. b. A mask and sterile gloves must be worn and the area over the shunt reservoir is shaved, prepped with The shunt tap. (George & Kureshi, Betadine or Hibiclens solution, 1997) then draped with sterile adhesive
2

Management of Hydrocephalus

Complications

paper drapes. c. A 23- gauge butterfly needle is passed percutaneously into the reservoir. d. Testing shunt flow dynamics will vary depending on the shunt system. The testing sequence begins with occlusion of the distal flow which allows for measurement of the CSF flow from the ventricular catheter and of intracranial pressure, along with sampling of CSF. Next, the proximal flow should be occluded and a column of fluid run distally to assess adequacy of valve function and peritoneal tubing patency. e. CSF obtained should be tested for glucose, protein, cell count and differential, and culture and antibiotic sensitivity. (George & Kureshi, 1997) Treatment: The presence of a shunt infection proven by CSF Gram stain or culture requires : a. Appropriate antibiotics: Consists of a combination of antimicrobial therapy and surgery. Initial empiric antimicrobial therapy consists of an antistaphylococcal antibiotic, such as nafcillin or vancomycin. A third-generation cephalosporin and an aminoglycoside are added if abdominal symptoms are present or the Gramstain smear of the CSF shows Gram-negative bacilli. Once the antimicrobial susceptibilities of the organism are known, empiric therapy is adjusted to target the causative organism(s). The use of intrathecal antibiotics in the treatment of CSF shunt infections is controversial. Many antibiotics, including vancomycin, have poor CSF penetration when given parenterally, even in the presence of inflamed meninges. Therefore, direct installation of the antibiotic into the meninges has been used to improve delivery. However, the pharmacokinetics of intraventricular antibiotics are not well studied, and the potential exists for neurotoxicity and chemical ventriculitis. Use of intraventricular antibiotics should be reserved for infections that have failed standard treatment. Frequent CSF cultures are used to monitor response to therapy. If the shunt was left in place and cultures remain positive after several days, removal of the shunt with EVD placement should be considered. The duration of antibiotic therapy is usually 10 to 14 days following sterilization. Several factors, such as the infecting organism, time to sterilization and presence of distal complications, should be considered when determining length of therapy. A new shunt can be placed once the CSF is sterile, but there are no data as to the optimal timing of shunt replacement. (Oram, 2006) Surgical strategy
3

Management of Hydrocephalus Shunt infections are manifested by three scenarios:

Complications

1. Catheter contamination with a mild or fulminant ventriculitis: the entire shunt hardware should be removed, an EVD placed for the duration of antibiotic therapy, and then replacement of the shunt. If there is a fulminant (George & Kureshi, 1997) ventriculitis (i.e., intraventricular purulence or Gram-negative infection), the EVD should be set up so that irrigation of the ventricular system can be performed. This will allow for intraventricular antibiotic therapy and/or continuous irrigation to debride purulent material or help wash out bacteria when CSF production is diminished in the face of Gram negative infection. 2. Superficial wound infection with or without CSF contamination: Superficial wound infections should be treated by removal of the shunt hardware and placement of an EVD. If the CSF cultures are negative after 3 days of antibiotics, (George & Kureshi, 1997) the shunt can be replaced in a new location. However, if the CSF cultures are positive, then the usual 10-day antibiotic course is given and the shunt replaced after the infection is resolved. If there is only a suture abcess, the suture can be removed and the wound treated locally with topical antibiotics. 3. Peritoneal infection manifested as a loculated cyst or frank peritonitis with or without CSF infection: in the presence of an abdominal infection, the
4

(George & Kureshi, 1997)

Management of Hydrocephalus

Complications

shunt can be removed from the abdomen and left with the distal end externalized when the CSF studies are negative. Once again, if the CSF studies are positive, removal of the entire shunt system and placement of an EVD is necessary. Generally, we prefer to treat all infections by the prompt removal of the infected hardware, placement of a temporary external ventricular drain (EVD), and then delayed replacement of the shunt after the infection is resolved. Placement of an external ventricular drain The placement of an EVD is performed with the patient under general anesthesia (Figure 3). Induction is accomplished using intravenous thiopental, and anesthesia is maintained using opiates and inhalation agents such as forane. Close monitoring of body temperature is maintained and hypothermic measures are instituted to treat pyrexia; warming lights are used if hypothermia is present. The patient is positioned so that the total shunt system as well as a new site for the ventricular catheter are accessible. The key to the procedure is to remove all original hardware that can be safely removed. The site of the previous cranial incision is reopened and the shunt hardware exposed. The ventricular catheter should be disconnected from the distal tubing, and then the valve and peritoneal catheter removed. When the distal system has been in place for several years, the tubing may become frail and prone to breakage, making necessary the use of one or two small additional incisions to remove the hardware. Next, the original ventricular catheter is removed. When ventricular catheters are adherent and difficult to remove, we use the 1.2-mm ventriculoscope to visualize the lumen of the tubing and cauterize adherent choroid plexus in order to avoid intraventricular bleeding (Figure 4). Usually, a soft passing technique can be utilized to place the new 25-cm ventricular drainage catheter when the old tract is used. If a new site is needed, the preferred ventricular catheter placement is via a frontal approach. The entry point on the skull should generally be 2 cm anterior to the coronal suture and 2 cm lateral to the midline. The catheter tip trajectory should be Placement of the EVD from a aimed at the ipsilateral medial canthus and frontal or a posterior approach. The anterior to the tragus. Typically, a depth of 4 to 6 tubing is tunneled to a separate exit cm will place the catheter in the frontal horn, just site and connected to a closed drainage system. (George & Kureshi, 1997)

Management of Hydrocephalus

Complications

anterior to the foramen of Monro. CSF samples should be sent for routine studies. The excess tubing is then tunneled subcutaneously at least 4 to 5 cm toward a frontal exit site for ease of nursing care and to avoid having the patient lie on the tubing. A sterile Becker or Connell closed drainage system is connected. The wound should be irrigated with copious amounts of bacitracin solution and a meticulous two-layer closure is performed with 3-0 or 4-0 Vicryl and 3-0 or 4-0 nylon. An adherent occlusive dressing is applied. An EVD should not be routinely changed. In the event that the drain becomes secondarily contaminated, a new EVD should be placed as described above. Externalization of distal shunt tubing The procedure can be performed in a clean treatment room or preferably in the operating room. Depending on the age of the patient, the local anesthesia using 0.5% to 1% lidocaine/epinephrine mixture with or without a standby sedation protocol is used. The area around the clavicle should be prepped with Betadine or Hibiclens and draped with a sterile adhesive drape. The shunt tubing is palpated as it courses over the clavicle and the skin is then infiltrated with the local anesthetic.

Management of Hydrocephalus

Complications

Externalizing a VP shunt: A, ultrasound of abdomen revealing an infected peritoneal pseudocyst at the distal tip of the peritoneal catheter and the site of externalization below the clavicle. B, dissection exposing the shunt tubing. C, sectioning of shunt tubing and aspiration of peritoneal contents using the distal tubing. D, the proximal shunt tubing is connected to a closed sterile drainage system. (George & Kureshi,

1997) A small 1- to 2-cm transverse incision is made down through the epidermis. The tubing is located again by palpation, and gentle blunt dissection is continued to fully expose the tubing. We prefer to use monopolar cautery to perform dissection since the shunt tubing is resistant to damage by the coagulation current. Approximately 3 or 4 cm of the distal portion of tubing is removed and then sectioned once exposed (Figure 5B). Prior to removal of the distal tubing, aspiration of any peritoneal fluid or cyst fluid should be attempted by using a 10- or 20-cc syringe connected to a 19-gauge blunt needle, and the aspirated fluid sent for culture (Figure 5C). This maneuver provides a diagnostic test while treating a cyst and removing the foreign body. The proximal tubing is connected to a Luer connector, which is then connected to a closed disposable external drainage system (Becker and Connell have systems available) in which the level of drainage can be measured and drainage bags changed in a sterile fashion (Figure 5D). Usually, a single layer closure will suffice using an interrupted nylon suture. Prevention of ventriculoperitoneal shunt infections
7

Management of Hydrocephalus 1. 2. 3. 4. 5.

Complications

A diligent skin preparation with Betadine or Hibiclens solution protocol. The use of iodine-impregnated sterile adhesive paper drapes. The gentle handling of tissues. Meticulous hemostasis. Use preoperative antibiotics aimed principally to cover staphylococcal species. It's given 1 hour prior to skin incision. 6. Shunt components are removed from the sterile packaging following complete skin preparation and draping and are kept in a bacitracin solution until used. Contact of the shunt tubing with the skin is avoided and tubing exposed to the surgical drapes is wrapped in bacitracin-soaked sponges. (George & Kureshi, 1997) 3. Injury to an abdominal organ occurs in 0.4% of patients. This often presents as an infectious-obstructive episode, but extrusion of the catheter via the intestinal tract has been observed. (McCullough, 1995) 4. Mechanical failure: If the volume of drained CSF is inadequate the problem of overdraining or underdraining occurs. It results from an inappropriate opening pressure of the shunt system for the individual patient (Trojanowski , 2009): a. Overdrainage: It may cause : i. Subdural haematomas may occur as a direct result of overdrainage, causing further difficulties in treatment. ii. Slit ventricle syndrome (SVS) describes a longer term complication of overdrainage that occurs in approximately 1% of shunted paediatric patients. The aetiology is unclear but has been attributed to a. b. c. d. Overshunting. Intracranial hypertension. Periodic shunt malfunction. Decreased intracranial compliance. The ventricles are small on CT scan, but the pressure within them can be very high. The child complains of headaches and vomiting.

Treatment: The low-pressure valve will consequently be changed to a medium or high-pressure resistance valve, or an antisyphon device. In extreme situations a subtemporal decompression craniectomy has been performed, with mixed results. (May, 2001)
8

Management of Hydrocephalus

Complications

5.

6.

7. 8.

9.

b. Underdrainage: This results in enlarged ventricles and necessitates changing to a lower pressure valve. (May, 2001) Obstructive complications will inevitably occur. The majority involve the distal portion of the device. Patients with high CSF protein values at the initiation of therapy may require several shunt revisions during the first postoperative year. After about the sixth year, patients treated in infancy may present with obstruction due to axial somatic growth and relative shortening of the peritoneal catheter. Obstruction may also result from rarely encountered peritoneal pseudocysts, ascites due to peritoneal absorptive failure, peritoneal adhesions, internal debris, or breakage and disconnection of shunt components. The use of modern kink-resistant silicone tubing with avoidance of older, delicate, spring-reinforced peritoneal catheters has virtually ended the occurrence of disconnection and catheter migration. A small group of pediatric and adolescent patients with CSF diversion receives considerable attention because of frequent bouts of vomiting, headache, and deterioration of consciousness. The patients tend to have small ventricles, diminished extraventricular CSF spaces, and a thick skull. In my experience less than 4% of treated patients were suspected of having this syndrome. The majority of these actually had true proximal shunt obstruction solved by appropriate catheter replacement in the frontal horn. In the remainder, corrective procedures such as augmenting valve resistance and subtemporal craniectomy may be required to manage symptoms and prevent decompensation. (McCullough, 1995) Shunt failure is mostly due to suboptimal proximal catheter placement. Occasionally, distal catheters fail. Suspect infection if the distal catheter is obstructed with debris. Abdominal pseudocysts are synonymous with low-grade shunt infection.( Sahrakar, 2002) Shunt disconnection: the technique of securing all connections with a nonabsorbable suture tied across the connector can help prevent disconnection. (Klinge, 2008) Aspiration: The patients either have a full stomach or have been vomiting and are at risk for aspiration. A modified RSI (Rapid Sequence Induction and Intubation ) should be considered for tracheal intubation. To avoid further increase in ICP, blunting the stimulus of laryngoscopy and intubation will be necessary. Wise use of opioids, lidocaine, and/or intravenous anesthetics can be considered. However overadministration of these agents can cause a decrease in BP, leading to inadequate cerebral perfusion pressure. (Aliason & Koh, 2012) Latex Allergy: Children with a history of myelomeningocele are at significantly increased risk for latex allergy. Many children with myelomeningocele also have hydrocephalus. It's reported that the prevalence of sensitization to latex may be as high as 64% in children with spina bifida. Allergy to latex is a type 1 IgE-mediated reaction clinically manifested as urticaria, angioedema, bronchospasm, and anaphylactic shock. The best way to prevent latex allergy is to prevent latex exposure.
9

Management of Hydrocephalus

Complications

Cause: Patient may expose to latex in latex gloves, tape, medication vials, blood pressure cuffs or other latex-containing products. Treatment is through: Removing all latex agents, Secure the airway, FIO 2 100% Epinephrine as needed, Reduce or discontinue volatile, anesthetics as dictated by, hemodynamics, IV fluid boluses to support blood pressure, Corticosteroids, Antihistamines. (Aliason & Koh, 2012) 10. Surgical Trauma: May be direct or indirect. Rapid surgical decompression of hydrocephalus can lead to rupture of the cortical bridging veins and SDH, or upward herniation of the brainstem causing bradycardia, irregular respirations, or ECG changes. (Aliason & Koh, 2012) 11. Subdural hematoma/upward herniation Rapid surgical decompression. Rapid lowering of open EVD. (Aliason & Koh, 2012) 12. Intrathoracic trauma (heart, lung, and great vessels) can occur when the passer sheath is tunneled across the chest wall and toward the abdomen. Cause: VP shunt passer sheath inadvertently tunneled into chest cavity. Treatment: Depending on which organ has been damaged, the staff must be prepared to treat hemorrhage, tamponade, and pneumothorax. (Aliason & Koh, 2012) 13. Venous Air Embolism (VAE) VAE can occur at any time, especially during placement of a ventriculoatrial shunt. To help prevent VAE, the anesthesiologist can keep the surgical site lower than the level of the heart (if possible), mechanically ventilate the patient, maintain high venous pressure, and remove air from IV tubing and solutions. Treatment: FIO 2 100%, Reduce or discontinue volatile anesthetics as dictated by hemodynamics, Aspirate air if central line is in situ, IVFs/vasopressors to maintain BP, Valsalva, Surgical site below the heart and LLD, if possible, CPR if cardiac arrest. (Aliason & Koh, 2012) Complications associated with ventriculostomy. Endoscopic ventriculostomies carry risks that are mainly related to damage of structures near the floor of the third ventricle. Endoscopic ventriculostomy can be acutely complicated by arrhythmias, asystole, hypertension, hemorrhage and bradycardia. (Aliason & Koh, 2012) 1. Injury to the basilar artery or its branches. Hemorrhage, stroke, or false aneurysmal formation may be seen following injury to the basilar artery. Treatment:
10

Management of Hydrocephalus Call for help FIO 2 100% Decrease or D/C volatile anesthesia IVFs and vasopressors as needed Transfusion of blood products as needed

Complications

Manage elevated ICP until craniotomy(Aliason & Koh, 2012) 2. Subdural hematoma from ventricular decompression. (figure 2) 3. Meningitis with or without CSF leakage. 4. Arrhythmias or bradycardia/asystole Cause: Stimulation of the floor of the third ventricle by the endoscope High speed irrigation fluid Rapidly increasing ICP Vagal response to surgical or other manipulation (i.e., pressure on eye) Venous air embolism

Treatment:

Fig. 2. Coronal T2W MRI sequence showing bilateral subdural hygromas and subcutaneous collection post endoscopic 3rd ventriculostomy for hydrocephalus. The stoma later blocked and a shunt was placed. (Najjar & Turkmani, 2011)

Stop stimulating floor of 3rd ventricle and to stop irrigation fluid. If arrhythmia does not resolve , treat supportively according to rhythm disturbance. FIO 2 100%.(Aliason & Koh, 2012) 5. Hypertension: Cause: Catecholamine release due to surgical stimulation or Local surgical effects on third ventricle floor. Treatment: Repeat BP measurement to verify accuracy Deepen anesthesia if needed Stop stimulating floor of third ventricle and irrigation fluid Raised ICP may require mannitol, furosemide, and/or hyperventilation Urinary catheter if bladder is distended(Aliason & Koh, 2012)
11

Management of Hydrocephalus 6. Complications due to trauma of Hypothalamus: (fig:3) it may lead to SIADH or DI, Temperature regulation problems (can be mistaken for malignant hyperthermia), Post-op trance-like state (differential postop agitation/delirium). (Aliason & Koh, 2012) 7. Complications due to trauma of Cranial nerves: 3rd and 6th nerve palsies have been reported. (Aliason & Koh, 2012)

Complications

8. fever. Due to an increase CSF temperatures due to the use of a cold light source and a monopolar coagulation in the confined volume of the third ventricle. 9. Short-term memory loss: Since the procedure may affect the hypothalamus and the areas of the mamillary body, which are responsible for memory. However, given time, an individual usually recovers from any short-term memory loss following endoscopic third ventriculostomy. (Hydrocephalus Association, 1997)

Fig. 3. Coronal T1W MRI sequence showing a small left thalamic lesion with hydrocephalus. The child underwent endoscopic 3rd ventriculostomy and biopsy. Symptomatic hydrocephalus was thus treated and the low grade tumor proven at biopsy was stable and followed with imaging. (Najjar & Turkmani, 2011)

10. How to avoid complications :1. Fenestration should be performed at the most transparent portion of the floor. 2. Fenestration should be performed in the midline of the patient. 3. Blunt perforation is preferable to cautery or the use of sharp instruments. (Crone, 2006)

(Tsang & Leung 2012)

Complications of EVD include hemorrhage, misplacement, dislodgement, disconnection, blockage, and, most significantly, infection. External ventricular drain infection EVD-related infections may lead to further serious complications such as ventriculitis, meningitis, cerebritis, brain abscess and subdural empyema. An infected EVD contraindicates immediate permanent shunting and may therefore delay definitive CSF
12

Management of Hydrocephalus

Complications

diversion. It significantly prolongs hospital stay and increases cost. Patients in the infected group also suffered from more severe neurological damages . Empirical antibiotics with good CSF penetration should be given to cover the common offending organisms. The commonly used agents are cephalosporins and rifampicin. Intraventricular vancomycin may be used for resistant organisms. Revision of the EVD at a different site should be considered if CSF diversion or ICP monitoring is still required. Causative organisms: 1. Coagulase-negative Staphylococcus is consistently reported to be the most common bacteria isolated in patients with EVD-related infections, accounting for up to 47% of infected cases. 2. Enterococcus. 3. Enterobacter. 4. Staphylococcus aureus. 5. Gram-positive organisms are classically associated with ventriculitis Risk factors 1. Subarachnoid hemorrhage (SAH) and Intraventricular hemorrhage (IVH): This has been postulated to be the result of frequent manipulations of the drainage system for flushing blocked EVD, the infusion of fibrinolytic agents, and the higher chance of EVD revision in these patient with hemorrhages. The risk of infection was found to be 6 to 10% higher in patients with hemorrhages. 2. Craniotomy and other neurosurgical procedures: The conduction of craniotomies or other neurosurgical procedures were found to be a risk factor for EVD-related infections when compared with patients who had received EVD alone. 3. Venue of insertion and skill level of surgeon: Many authorities advocated the operating theatre as a preferred venue for EVD insertion. However, some surgeons demonstrated separately that inserting EVDs in the intensive care units, emergency rooms or neurosurgical wards was not inferior in terms of infection risks or other complications. The location of insertion did not appear to affect the risk of infection provided that strict aseptic technique was used. There was also no significant difference amongst EVDs that were performed by neurosurgical trainees, consultants or neuro-intensivists. (Tsang & Leung 2012) 4. Subcutaneous catheter tunnel: Friedman & Vries, 1980 have devised a new ventriculostomy technique that involves tunneling the ventricular catheter through the scalp, between the dermis and the galea. The average duration of drainage was 6.2 days. There were no infections subsequent to the insertion of the ventricular catheter in patients. (Friedman & Vries, 1980). 5. Duration of drainage: The literature was very much divided on the issue of whether and how the duration of external CSF drainage may affect the risk of infection. The recent researches demonstrated no association between the risk of infection and the
13

Management of Hydrocephalus

Complications

duration of drainage. The timing of EVD related infections appeared to follow a normal distribution during the first five postoperative days, and the majority of infections occurred between day two and day 11. So early infections may arise from initial inoculations, which developed into detectable infections after variable incubation periods of around five days. A delayed peak of infection has also been observed after day 20, but the small number of reported cases rendered confirmation difficult. Based on the belief that the risk of infection would increase with prolonged EVD, some authorities have advocated elective revisions of EVD after a fixed interval of, say, five days. However, the review by Park et al on 595 patients with EVD insertions found that the daily infection rates would plateau after day 4 postinsertion, and remain steady beyond day 10. 6. Manipulation of the EVD system: Manipulations and opening of the otherwise closed EVD system for CSF sampling or flushing may introduce microorganisms and potentially cause infection. 7. Prophylactic antibiotics: There is no consensus as to what and for how long it should be given. Responders in Europe favored a single dose of antibiotics given immediately before the operation, while those from Asia and North America tend to cover also the whole period of post-operative drainage. The use of prophylactic antibiotics was found to significantly reduce the risk of EVD- related infections 8. Coated ventricular catheter: The underlying rationale is that coating the surface of the catheter with special materials or antibiotics may decrease bacterial colonization and thus prevent infection. The findings were controversial depending on the coating material used. Antibiotics-impregnated catheter is an important development. (Tsang
& Leung 2012)

How to avoid complications Complications are unavoidable but we can reduce their frequency. To achieve this goal reliable diagnosis of NPH should be made, appropriate selection of patients with high likelihood of effectiveness of shunting. Meticulous execution of implantation plays an important role in complication avoidance. Too long or too short ventricular catheter, placed sub optimally in the ventricular system, mechanical damage of the shunt system by inadequate handling or bad instruments, too long operation time, violation of strictly aseptic technique increase the risk of shunt dysfunction or infection. (Trojanowski , 2009)

14

Management of Hydrocephalus

Complications

15