Cerebrospinal Fluid Diversion Procedures in the Pediatric Population


Hydrocephalus

Definition

The term hydrocephalus represents a condition defined as an imbalance between the production and absorption of the cerebrospinal fluid (CSF), resulting in increased intracranial pressure (ICP). Ventricular dilation may or may not occur, because ventriculomegaly can be present without signs of elevated ICP (i.e., without hydrocephalus). Congenital hydrocephalus is a term referring to hydrocephalus that is associated with congenital malformations such as myelomeningocele or congenital aqueductal stenosis. Acquired hydrocephalus occurs as a complication of a separate, primary process such as cerebral or intraventricular hemorrhage, infection, or neoplasm.

The treatment of hydrocephalus dates back to the dawn of civilization, where different methods were described to establish CSF diversion for hydrocephalus. Initially, the procedures resembled the currently used endoscopic third ventriculostomy (ETV), whereas in the second half of the 20th century, shunts became more widely available for and implanted in patients. Early 20th century attempts at achieving closed ventricular drainage included gold, glass, silver, or rubber tubes, as well as catgut and linen threads passed from the ventricle to the subdural space. Similar techniques were used to connect the lumbar thecal sac to the peritoneum or renal pelvis. , Attempts at third ventriculostomy were made by Dandy and Mixter, and choroid plexectomy was performed by Dandy too. Shunts from the lateral ventricle to the cisterna magna (Torkildsen shunts) and shunts from the lumbar spine to the ureter came into more widespread use as well. , Nulsen and Spitz reported the successful use of a ventriculojugular shunt in 1952 using a spring and stainless steel ball valves. However, occlusion of the venous catheter by blood clots was a frequent complication. Holter was the first to use a silicone catheter with a multislit valve in his son who had hydrocephalus. Pudenz, at about the same time, used silicone to develop shunt tubing for ventriculoatrial shunts.

Epidemiology

The incidence of congenital hydrocephalus has been estimated to be 0.48 cases per 1000 live births. The incidence of neonatal hydrocephalus is 3 to 5 cases per 1000 live births. An estimated 33,000 shunts are placed in patients of all ages annually in the United States, with an estimated shunt prevalence of more than 56,000 in children younger than 18 years. There is a significant variety in the prevalence of hydrocephalus across different regions of the world, where the prevalence of pediatric hydrocephalus is almost twofold higher in Africa compared with North America. Shunt placement is the most common solution for treating hydrocephalus. Although the popularity of endoscopic procedures for treating hydrocephalus is growing rapidly, shunts are still the favorable solution in most cases. In the United States, shunt placement accounts for 38,200 to 39,900 hospital admissions and 3.1% of all pediatric hospital charges ($1.4 to $2.0 billion).

Pathology and Pathophysiology

The traditional theory of CSF production in the choroid plexus of the lateral, third, and fourth ventricles and its reabsorption in the arachnoid granulations has recently been called into question. Modern CSF radionuclide cisternography and magnetic resonance imaging (MRI) did not show adequate CSF flow through the arachnoid granulations, and animal models demonstrated that nasal lymphatics may be the primary absorptive mechanism at normal pressure. , A current model suggests that the choroid plexus is the driving force for the circulation of CSF, and the absorption occurs in the Virchow-Robin spaces around blood vessels. Regional changes in the capillary bed caliber and permeability may affect the absorptive mechanism. For example, when an obstruction develops, the absorptive capacity is reduced and a higher hydrostatic pressure is required to reach equilibrium between absorption and production. , A model from mice described a brain-wide pathway for fluid transport, which includes the para-arterial influx of subarachnoid CSF into the brain interstitium, followed by the clearance of interstitial fluid along large-caliber draining veins. The transport of water from different compartments is facilitated, as in other organs, by different types of aquaporins (AQPs). For example, AQP1 is related to the transfer of water through the choroid plexus, whereas AQP4 is believed to be involved in the glymphatic pathway, where it allows for exchange of water and metabolites between the perivascular CSF and the brain. ,

The current hypothesis is that CSF follows the following pathway:

  • Production occurs mainly in the choroid plexus, and CSF passes through AQP1 and accumulates in the ventricular system.

  • CSF then exits the ventricular system in two potential ways: (1) travel through foramina of Magendie and Luschka to reach the subarachnoid space and from there to the deep white matter to reach the periarterial spaces in a centripetal manner, or (2) travel into the brain parenchyma through the transependymal route to reach the deep periarterial spaces in a centrifugal manner.

  • CSF is then transferred to the brain parenchyma along perivascular spaces surrounding penetrating arteries, mainly through AQP4; this causes the transfer of water from the parenchyma toward the perivenous spaces surrounding large-caliber veins. , ,

  • It is still unclear how the CSF is resorbed, and it might be occurring in more than one mechanism. The two most discussed CSF resorption pathways are the lymphatic outflow pathway and the venous outflow pathway.

Common causes of hydrocephalus and a rough estimate of their frequency in pediatric patients are outlined in Table 80.1 .

Table 80.1
Common Causes of Hydrocephalus in 344 Pediatric Patients
Cause Patients
Intraventricular hemorrhage 24.1%
Myelomeningocele 21.2%
Tumor 9.0%
Aqueduct stenosis 7.0%
CSF infection 5.2%
Head injury 1.5%
Other 11.3%
Unknown 11.0%
Two or more causes 8.7%
Total patients 99.0% (344)
CSF, Cerebrospinal fluid.

Clinical and Radiologic Diagnosis

The diagnosis of hydrocephalus is based on clinical symptoms, radiologic appearances, and, sometimes, invasive ICP recordings. Evidence of ventriculomegaly is not sufficient for the diagnosis of hydrocephalus and does not mandate any treatment by CSF diversion methods. Common signs and symptoms of pediatric hydrocephalus are listed in Table 80.2 . Children more commonly present with symptoms of irritability, delayed development, and vomiting. Examination reveals increasing head circumference, bulging fontanelle, and loss of upward gaze. Papilledema is highly suggestive of raised ICP; however, it has been shown to be absent in 86% of patients with shunt obstruction. Sixth nerve palsy or loss of upward gaze may be a sign of raised ICP.

Table 80.2
Presenting Clinical Features of Hydrocephalus in Pediatric Patients
Symptoms Children
Irritability 26.6%
Delayed developmental milestones 19.8%
Nausea or vomiting 19.0%
Headache 17.5%
Lethargy 17.5%
New seizures or change in seizure pattern 6.6%
Diplopia 5.8%
Worsening school performance 4.2%
Fever 2.6%

Signs Infants
Increasing head circumference 81.3%
Bulging fontanelle 70.6%
Delayed developmental milestones 20.9%
Loss of upward gaze 15.8%
Decreased level of consciousness 12.6%
Other focal neurologic deficits 12.4%
Papilledema 12.0%
Sixth nerve palsy 4.6%
Hemiparesis 3.8%
Nuchal rigidity 1.8%

Imaging commonly used in the primary assessment of a child with suspected hydrocephalus includes ultrasound, computed tomography (CT), and MRI. Ultrasound performed through the open anterior fontanelle is useful in critically ill premature infants with intraventricular hemorrhage. MRI may help in determining the etiology of hydrocephalus. The risk of radiation from CT has prompted increased interest in limited or fast sequence MRI. T2-weighted sequences such as fast imaging employing steady-state acquisition (FIESTA) and time-spatial labeling inversion pulse sequences provide information about fluid movement within the ventricles. In patients with mild ventricular enlargement, evidence of transependymal flow of CSF usually suggests that the process is acute. Enlargement of the temporal horns, dilation of the third ventricle, and effacement of the sulci are suggestive of progressive hydrocephalus; however, these findings are not specific to an acute or subacute process. Comparison with previous imaging in a patient with suspected hydrocephalus due to shunt malfunction is essential. In addition to evaluating the brain and ventricles, radiographs of the shunt tubing (a.k.a. “shunt series”) should be obtained when evaluating a patient for possible shunt malfunction. A shunt series typically includes two views of the head, neck, chest, and abdomen so that the entire shunt system is interrogated and deemed continuous without breaks or disconnections. Radionuclide CSF scintigraphy can also be performed for evaluation of shunt patency. The shunt reservoir or valve is accessed and 0.25 to 3 millicurie in 0.3 to 0.5 mL of technetium 99m DTPA is injected, with the distal end briefly occluded to allow the material to partially enter into the proximal end and then flow through the system along with the CSF. Images are then obtained using a 30-second frame rate. If the shunt is patent, the radionuclide activity is uniformly observed in the entire shunt system with rapid spillage into the distal draining body cavity, usually within the first 20 minutes. Absent or delayed activity at the distal end, focal activity at the distal end without spillage, and/or absence of ventricular reflux indicate obstruction. ,

Overview of Treatment Options

Shunting

Fundamentally, a shunt consists of a ventricular or proximal catheter, a valve, and a distal catheter. In some cases an additional reservoir is used to connect the ventricular catheter to the valve. The purpose of the shunt is to divert CSF from the ventricle to another site that can absorb the excess fluid. The valve is required to maintain a one-way flow and prevent reflux into the ventricular system. Valves may also serve to avoid overdrainage of CSF and gravity-dependent shifts in ICP (see section “Valve Selection”). The most common locations for distal catheter insertion include the peritoneum, the pleura, and the right atrium.

There are several factors to consider when planning for the installation of a shunt:

  • Site of ventricular catheter insertion

    • Most catheters are inserted via a frontal or parieto-occipital burr hole on the right or left side.

    • Underlying pathology may dictate laterality or position.

    • Meticulous planning of the entry site should be taken into consideration in special cases. For example, in patients with brain tumors, the surgeon should avoid inserting the ventricular catheter in a potential area that may be operated on in a future surgery or an area that underwent an extensive surgery before.

  • Site of distal catheter insertion

    • In general, the preferred location for the distal catheter is the peritoneum, followed by the pleura and the right atrium.

    • The peritoneum is always used unless there is evidence that distal shunt insertion into the peritoneal cavity is highly likely to result in CSF malabsorption, infection, or abdominal content damage.

    • The pleura is usually not selected in very young children because of the high likelihood of developing significant pleural effusion that may lead to respiratory failure. Infants are more likely to develop a significant effusion temporarily. Some recommend a preoperative chest x-ray and pulmonary function testing prior to placing a pleural shunt.

    • In patients who suffer from chronic cardiac failure or pulmonary hypertension, a ventriculoatrial shunt should be considered very carefully, and it is preferred to avoid the use of the right atrium as the distal site in these patients.

  • Type of valve to implant

    • Younger children require valves that will drain at a lower pressure, whereas older children often receive medium pressure valves. In young children with severe hydrocephalus and extreme ventriculomegaly, a programable valve is usually preferred over a fixed-pressure valve to allow for slow and gradual drainage; the ability of changing the opening pressure over time is also an advantage in programable shunts.

    • In general, it is recommended to try and set the valves at the highest possible setting (higher opening pressure) and try not to end up with a collapsed ventricular system.

    • Variable-pressure valves are often reserved for patients with complex shunt problems, in whom the optimal intraventricular pressure remains unclear.

    • The same type of valve should be reimplanted if the patient had no issues with their settings before dysfunction.

  • Inspection of the site of insertion of the ventricular and peritoneal catheter as well as the trajectory of the distal catheter

    • Tunneling underneath scar tissue, lines or tubes should be avoided whenever possible.

    • Skin thickness or quality should be evaluated because changes may occur due to prior radiation, age of the patient, and prior revisions.

  • Evidence of concurrent illness

    • Shunt placement is usually not recommended if there is evidence of systemic infection.

    • In critically ill patients, external ventricular drainage should be considered until the patient is stable enough to tolerate general anesthesia for internal shunt placement.

  • Details of prior shunt placements

    • It is important to know what kind of valve and shunt tubing were placed previously.

    • Redundant tubing that is left in place should be mentioned in the operative note.

    • Prior operative reports may give insight about any difficulties experienced during the last surgery.

  • Need for general surgery assistance for distal catheter placement

    • History of extensive bowel surgery or difficulty in accessing the peritoneum during a prior surgery may necessitate a general surgery consultation.

    • Some neurosurgeons advocate for the use of general surgery in all ventriculoperitoneal shunting cases.

Ventriculoperitoneal Shunts

There are many variations in the technique of installing a ventriculoperitoneal shunt. Meticulous attention to detail and thorough planning of the procedure are likely to reduce shunt-related complications, the vast majority of which are related to blockage or infection.

The patient is placed under general endotracheal anesthesia, with the administration of intravenous antibiotics on induction. Antibiotic selection provides coverage for typical organisms of surgical site infections such as staphylococci, with additional coverage if methicillin-resistant Staphylococcus aureus (MRSA) have tested positive preoperatively or there is suspicion of MRSA colonization (i.e. frequent hospitalizations, prior MRSA infection). The bladder should be emptied at the start of the case with either straight catheterization or Foley catheter insertion.

Various techniques have been described to determine the proper location for the burr hole. Many surgeons measure distances from either the ear or the midline to approximate the site of the burr hole. It is important to correlate the projected burr hole location with the optimal location on the preoperative imaging. Measurements made from CT scan images are probably more accurate than choosing the site of the burr hole based on arbitrary surface landmarks. The use of neuronavigation optimizes the burr hole location without any guesswork but may increase the cost of the procedure and the time under anesthesia. Some neuronavigation techniques such as electromagnetic-based systems eliminate the requirement for head fixation in pins, thus allowing for easier positioning and tunneling.

The hair is shaved in the operating room to prevent it from twisting into the wound. A horseshoe headrest is typically used with a bolster placed under the shoulder that is ipsilateral to the ventricular catheter insertion site. In younger children, the head can be positioned on a donut with a small towel rolled underneath the shoulders. For the parieto-occipital burr hole, the head is rotated to the contralateral side and slightly flexed. This position is optimal for tunneling of the distal catheter because it provides a straight line from the cranial incision to the abdominal incision and allows good access to both the cranial and abdominal incisions. For a frontal burr hole, the patient is positioned supine on the horseshoe with the head tilted to the contralateral side ( Fig. 80.1A ). The burr hole is situated approximately 2 to 3 cm from the midline in the midpupillary line just anterior to the coronal suture. A small incision just posterior to the ear is usually required to tunnel through the subcutaneous tissue from the frontal region to the abdomen. The site of the burr hole and abdominal incisions should be selected and marked before draping. For frontal shunts, the postauricular incision should also be marked and prepped. The cranial incision should be made so that the implanted hardware is not placed directly under the incision. The skin is carefully prepped with an antiseptic solution such as povidone-iodine or chlorhexidine. Disposable, adhesive drapes are used to cover the patient and the entire operating table except for a small band of skin from the burr hole site to the abdomen, under which the tunneling will be performed (see Fig. 80.1B ). Iodine-impregnated transparent adhesive drapes are typically used; however, there is no evidence to suggest that shunt infection rates are reduced with this technique. Shunt protocols have been implemented at various institutions with improvements in shunt infection rates. The protocols are variable in terms of surgical preparation, use of antibiotics, draping, gloving, etc. The use of a standardized protocol has been shown to reduce shunt infection rate by several groups with different protocols. The Hydrocephalus Clinical Research Network (HCRN) group found that the implementation of a protocol significantly reduced shunt infection rates across their network. , Other groups with different protocols found similar results, where a unified checklist was strictly followed and led to significant reduction in infection rates.

FIGURE 80.1, Sequential steps on shunt insertion. (A) Patient positioning and marking of incisions. (B) Draping. (C) Making a small incision that will not cross over shunt equipment. (D) Passing a blunt dissector into the peritoneal cavity. (E) Using an abdominal trocar. (F) A tunneling device.

A time-out is performed immediately prior to incision and includes verification of the patient, surgical side, administration of prophylactic antibiotics, and availability of necessary radiographic imaging and equipment. We typically infiltrate the wound with a local anesthetic mixed with epinephrine for improved hemostasis and pain relief at the surgical site. Before incision, all equipment and material needed for surgery are brought into the operating room to prevent traffic in and out of the room during the procedure; a sign requesting not to enter the room is placed on all entry doors.

A curved incision (see Fig. 80.1C ) is typically used with its pedicle in the direction of tunneling. It is important to keep the pedicle wide enough so that no shunt hardware lies under the incision and to ensure adequate blood supply to the wound area. A subgaleal pocket is created to secure the valve and the reservoir. The size of the burr hole is made large enough to insert the ventricular catheter, with consideration of the possible need to change the angle of entry if initial insertion attempts were not successful. The dural incision is minimized as much as possible to allow for the passage of the ventricular catheter only; this decreases the risk of CSF extravasation into the subgaleal space. The pia is opened using bipolar electrocautery at low voltage.

The abdominal incision is made simultaneously by a second operator whenever possible to decrease the operative time. There is no evidence that any specific incision location on the abdomen results in reduced complications. We typically perform an incision at approximately 2 to 3 cm to the right of the midline at a level that is few centimeters superior to the umbilicus. Some surgeons slide the dissector through the peritoneal opening to confirm entry (see Fig. 80.1D ), but this maneuver should be performed with great vigilance so that visceral structures are not injured. A mini-laparotomy or a trocar (see Fig. 80.1E ) may be used to access the peritoneal cavity. Various methods can be used to confirm the opening of and easy access through the peritoneal layer—an important step that should not be overlooked—such as visualization of intraperitoneal contents, flooding the field with warm saline, and watching the fluid as it drains into the peritoneum, and intraoperative lateral fluoroscopy. If general surgery assistance is required due to the presence of abdominal adhesions, direct visualization of entry into the peritoneum can be confirmed via laparoscopy.

Tunneling can either be performed cranial to caudal or vice versa, and it is made easier if the patient is positioned such that the mastoid, clavicle, and xyphoid are co-planar. The goal of tunneling is to stay deep within the subcutaneous tissue but superficial to the thoracic cavity, peritoneum, great vessels of the neck, and skull base. A curved snap may be used to dissect the soft tissue before the insertion of the tunneling device (see Fig. 80.1F ), which may be challenging upon the initial attempts. The device should be palpable below the skin at all times and should be carefully directed superficial to the ribs and clavicle. A common site of resistance to tunneling is the deep cervical fascia of the neck, and a separate incision may be necessary for better control of the tunneling process. The anesthetist should be made aware of the tunneling step due to the increased stimulation and possible changes in ventilation while tunneling along the chest. The peritoneal tubing is then passed through the tunneling sheath; attaching a suction device to the distal end and continuous irrigation can assist in the passage of the tubing.

The ventricular catheter trajectory is determined according to external landmarks or by using image guidance such as neuronavigation or ultrasound (see Fig. 80.1G and 80.1H ). From a frontal burr hole, the traditional landmarks for the foramen of Monro are the intersection of the plane through the midline and the plane just anterior to the external auditory meatus. If the patient’s head is tilted, these landmarks can be difficult to appreciate, thus electrocardiogram (ECG) electrodes placed at the nasion and external auditory meatus prior to draping may help to orient the surgeon. The optimal position for the tip of the catheter is anterior and superior to the foramen of Monro with a catheter length of 5 to 5.5 cm. From an occipital burr hole, an ECG electrode placed at the midpoint of the forehead at the hairline is used to direct the catheter to the frontal horn as opposed to the temporal horn. The catheter length is approximated at 5.5 to 6 cm, and the optimal tip position is the atrium of the lateral ventricle, which causes a “pop” feeling when the ependyma is penetrated, followed by an initial spurt of CSF. There is no evidence that multiple catheter passages affect outcome; however, minimizing the number of passages through the brain parenchyma is likely to decrease the rate of complications such as hemorrhage or future seizures due to gliosis. Endoscopic shunt placement (see Fig. 80.1I ) allows for visualization of the ventricular catheter in real time and can assist in guiding the tip of the catheter away from the ventricular walls or choroid plexus. Ultrasound guidance can identify entry of the catheter into the ventricle and therefore reduce the number of required passages.

After preparing a subgaleal pocket (see Fig. 80.1J ), the ventricular catheter is connected to the valve or valve/reservoir system, and ties are placed along all connections (see Fig. 80.1K ). Placing the suture knot on the underside of the connection sites can help in preventing skin erosions, especially in infants with thin cutaneous tissue. The valve system is positioned deep inside the subgaleal pocket (see Fig. 80.1L ), and the valve is firmly sutured to the pericranium (see Fig. 80.1M ). The peritoneal catheter should be checked for spontaneous CSF flow, and the surgeon should not initiate closure until the shunt is clearly draining CSF. The distal catheter is then inserted into the peritoneum. If the catheter does not cross easily, it may be coiling in the preperitoneal space or there may be abdominal adhesions. If a mini-laparotomy is performed, the abdominal fascia is closed followed by the deep subcutaneous tissue and the dermal layer to prevent the development of herniation through the abdominal wall.

Ventriculopleural Shunts

The ventricular catheter placement and tunneling steps of implanting a ventriculopleural shunt are identical to that of a ventriculoperitoneal shunt. A 3-cm transverse incision is placed along the anterior axillary line in the fourth-to-sixth right intercostal spaces or along the midclavicular line at the second intercostal space, and a muscle-splitting approach through the pectoralis major and intercostal muscles is made along the upper border of the rib to avoid the neurovascular structures. The parietal pleura is identified as a translucent thin membrane covering the lung, which appears to move with ventilation. The anesthetist deflates the lungs, and the pleura is sharply incised for around 2 to 4 mm; the lung retracts from the chest wall as the pleura is cut. After tunneling from the cranial incision, the distal catheter is approximated and cut to a smaller length and adequate CSF flow through the distal end is confirmed. The catheter is then introduced into the pleural space and guided along the chest wall away from the lung parenchyma. Note that, unlike in the peritoneum and to avoid damage to the lung parenchyma, excess catheter is not placed in the pleural space; an intrathoracic length of 30 cm should be sufficient to prevent potential future displacement. A Valsalva maneuver inflates the lung, and under continuous irrigation until air bubbles cease to escape from the pleural space, the site is closed in layers.

Lateral and anteroposterior chest radiographs are performed to confirm the catheter location and usually demonstrate a small pneumothorax as well as a small pleural effusion due to accumulation of CSF. The intrapleural fluid will typically disappear over the next several weeks. However, in select patients the pleural fluid may progressively accumulate and lead to respiratory distress requiring percutaneous drainage of the effusion.

Ventriculoatrial Shunts

The same steps of placing the ventricular catheter for a ventriculoperitoneal or ventriculopleural shunt are used for the ventriculoatrial shunt installation technique. Although some surgeons may prefer to place the distal catheter prior to inserting the ventricular catheter during a ventriculoperitoneal shunt surgery—due to risk of displacement, it is recommended to place the ventricular catheter before the distal one during ventriculoatrial shunting to prevent backflow and clotting of venous blood inside the distal end or dislodgement from the recipient vessel.

Depending on the chosen technique, different vessels can be used as access points to the right atrium. During the open approach, neck dissection is performed until the internal jugular vein (IJV) is exposed. After temporary blockade of the descending venous blood by pulling a rubber band placed around the proximal IJV, a purse-string suture is placed on the anterior part of the distal IJV, followed by a small incision, direct insertion of the catheter into the vein, and tight closure of the suture around the catheter. Alternatively, the transverse facial vein may be sacrificed, and its distal part is used to insert the distal catheter directly into the IJV. One or two sutures may be used to firmly secure the vein around the distal catheter after confirming adequate CSF flow. A minimally invasive technique, the percutaneous approach, is quite similar to installing a central catheter and may also be performed under ultrasonography. A 20/22-gauge cannulating needle is used to cannulate the IJV (1 to 3 cm above the clavicle between the sternal and clavicular heads of the sternocleidomastoid muscle) or the subclavian vein (below the clavicular bone at the junction of the middle and lateral third of the clavicle). This is followed by the passage of a guidewire inside the cannula, preferably under fluoroscopy, until it reaches the superior vena cava or right atrium. The cannula is removed, a nick incision is performed, and the peel-away sheath, with the dilator stacked inside it, is introduced into the vessel along the guide wire. The dilator and the guidewire are then removed, and the distal catheter is passed down the peel-away sheath as far as possible in the right atrium; the peel-away sheath is then removed. Under fluoroscopic guidance, the distal catheter is pulled back to its final position at the middle-to-lower segments of the right atrium (approximated to the T6 vertebral body level in healthy individuals). If the catheter tip is not visualized, injection of a small amount of radiopaque material will be helpful to pull back the catheter into its optimal position. Finally, the distal catheter is flushed with heparinized saline and connected to the valve or directly to the ventricular catheter using a straight connector after verifying adequate CSF flow from the tip of the ventricular catheter.

Hardware Selection

The shunt hardware should be selected considering individual patient characteristics. The primary components include a ventricular catheter, a valve with or without a reservoir, and a distal catheter.

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