eMedicine Specialties > Pediatrics: Surgery > General Surgery
Management of Spina Bifida, Hydrocephalus and Shunts: Treatment
Updated: Nov 19, 2009
Treatment
Medical Therapy
In general, medical therapy of hydrocephalus is far inferior to surgical management of the condition. Medical therapy has been used with limited success in an attempt to avoid shunting in patients with posthemorrhagic hydrocephalus. Prenatal administration of antenatal steroids may modestly reduce the incidence of intraventricular hemorrhage (IVH) in premature infants. Postnatal use of indomethacin has been shown to reduce severe IVH in some studies, although improvement in cognitive functioning has not been documented. Diuretic therapy, such as the use of acetazolamide and furosemide, was invalidated as a therapy in one recent study of 177 infants. In this study, the medications did not affect rates of shunt placement and may have impaired neurologic outcome. Fibrinolytic therapy is currently under investigation.
Serial lumbar puncture has been frequently performed after IVH to prevent or manage developing hydrocephalus, but no clear guidelines indicate when to initiate treatment and no explicit evidence of effectiveness has been reported. Early use of lumbar puncture (prior to evidence of head expansion) has been shown by meta-analysis to have no benefit.
Surgical Therapy
In most cases, surgical treatment of hydrocephalus consists of ventricular shunt insertion. The shunt is an artificial device, made mostly of plastic (although some parts may be metal), that includes a catheter inserted in the ventricle of the brain (a one-way valve that allows the unidirectional flow of cerebrospinal fluid [CSF] out of the brain) and a distal catheter that drains the CSF to an extracranial location in the body. The most preferred distal site remains the peritoneum. However, other sites are available (eg, right atrium, pleura, gall bladder, ureter, bladder, sagittal sinus) in patients with other coexisting abdominal problems. In current practice, the overwhelming majority of shunts are ventriculoperitoneal.
All shunts are designed to maintain normal intracranial pressure. More than a dozen different commercial shunts are currently available. The design of the valve is controversial. Essentially, 2 types of shunts are available: the pressure-regulating shunt and the flow-regulating shunt (as well as 2 brands of programmable shunt valves). The pressure-regulating shunts are designed to maintain a difference of pressure between their inlet and outlet and allow flow of CSF once the preset pressure has been reached. The flow-regulating shunts are designed to allow a constant flow of CSF, simulating the normal flow. Despite different designs, large randomized trials have been unable to demonstrate differences between the various types. Different types of valves are seemingly associated with different types of complications. For example, the pressure-regulating valves are more prone to cause overdrainage complications, whereas the flow-regulating valves are more prone to cause valve obstruction.
Endoscopic third ventriculostomy (ETV) was first performed by Walter Dandy in the 1910s with moderate success and has recently experienced resurgence. The endoscopic equipment has improved, which has resulted in increased use of the procedure. ETV has a success rate of 70% when used in patients with aqueductal stenosis and is regarded by many as the procedure of choice in these patients. Endoscopic cyst fenestration can be used in the presence of arachnoid cysts in various locations (ie, suprasellar, interhemispheric, posterior fossa) with variable success.
Third ventriculostomy has been recently performed to treat hydrocephalus in children with myelomeningocele. However, the reported success rates are only approximately 30-40%. One possible explanation for the low success rate of third ventriculostomy is that most patients are infants or neonates when they receive initial treatment and do not have fully developed subarachnoid spaces. A frontal ventricular catheter attached to a blind reservoir or an Ommaya reservoir can be left in place and can be converted to a ventriculoperitoneal shunt if the third ventriculostomy fails. ETV can be used in children who have already received shunting and who present with shunt malfunction at an older age.
The reported success rate is approximately 50%. In such patients, an external ventricular drain should be used for the first few days following third ventriculostomy (especially if the shunt has been removed) to allow emergency decompression if the third ventriculostomy does not function adequately and the patient’s condition rapidly deteriorates.
ETV may be more effective if it is combined with choroid plexus cauterization. Improved outcomes were reported in a recent study of select patients younger than one year. However, cauterization is not routinely performed and remains a controversial option; further study is needed.
Kulkarni et al have developed and validated a model to predict the probability of success of ETV in the treatment of hydrocephalus.1 Analysis of 618 ETVs performed consecutively on children at 12 international institutions identified predictors of ETV success at 6 months. A multivariable logistic regression model was developed on 70% of the dataset and was validated on 30% (validation set). The model contained patient age, cause of hydrocephalus, and previous cerebrospinal fluid shunt. ETVs were successful and the model maintained good fit (Hosmer-Lemeshow, P = .45), discrimination (C statistic = 0.68), and calibration (calibration slope = 0.88) on 64.4% of the validation set. Kulkarni et al conclude that this model can be used to identify children in whom ETV is most likely to succeed and who can thus be spared the long-term complications of CSF shunting.
Premature infants with posthemorrhagic hydrocephalus may benefit from placement of a subgaleal ventricular reservoir (an Ommaya reservoir), subgaleal shunt (which drains into a subgaleal pocket created during surgery), serial lumbar punctures, or external ventricular drainage as a temporizing measure. Placement of a permanent shunt may be avoided in as many as 25% of these children.
Preoperative Details
Anesthetic factors must be considered, particularly those relative to respiratory function and reserve. Many of these patients are premature neonates who have poor respiratory reserve and may be experiencing physiologic jaundice when surgery is required. Because the magnitude of the surgery is not extensive, the circulating blood volume is usually not problematic, and a blood transfusion is likely to be unnecessary during shunt insertion or endoscopic ventriculostomy, unless a preexisting problem is present.
Intraoperative Details
The importance of avoiding hypothermia and excessive blood loss in pediatric patients cannot be overemphasized; especially in neonates. Also, having a dedicated pediatric anesthesia team and operating room team are equally important. In addition, limiting operating room traffic during these procedures can be helpful in decreasing infections.
In most cases, shunt insertion involves making a posterior parietal or frontal burr hole through a small linear or curved skin incision. The peritoneal cavity is entered through a small linear incision either in the upper midline epigastric region or in the right upper quadrant. The distal tube is advanced from the cranial to the abdominal wound using a purpose-designed tubular dissector advanced in the subcutaneous fat. In typical cases, if a posterior parietal burr hole is used, the shunt valve is situated behind the ear, avoiding the need for a step incision, and is usually easily palpable by the patient and parents. Administering prophylactic antibiotics, usually cephalosporin or vancomycin, is common at the commencement of the operation to lessen the likelihood of shunt infection.
In children born with open spina bifida who undergo simultaneous shunting and myelomeningocele closure, additional precautions should be taken to maintain sterility of the surgical fields. Most neurosurgeons prefer to close the myelomeningocele with the child prone and subsequently turn the child on his or her back for the shunt placement while adequately protecting the newly repaired spinal wound with ample padding.
ETV is traditionally performed through a frontal burr hole situated just anteriorly to the coronal suture. A rigid or flexible endoscope is preferred. The third ventricle floor is perforated using a purpose-designed monopolar diathermy with retractable tip or another similar purpose-designed dissector. After formation, the stoma is commonly dilated using some kind of purpose-designed balloon dilator.
Perforation of the third ventricle floor is the most delicate and important phase because perforation of the adjacent basilar artery is a risk. ETV can be particularly difficult in children with myelomeningocele because the ventricular anatomy is often abnormal, the third ventricle floor is thicker and more difficult to penetrate, the size of the third ventricle is smaller in these children than in those with aqueductal stenosis, or the septum pellucidum is absent, which can lead to disorientation in the inexperienced operator. In general, inexperienced operators should avoid ETV in children with hydrocephalus caused by myelomeningocele. Apart from damage to the basilar artery, another potential source of intraoperative difficulty is damage to the choroid plexus, which can lead to hemorrhage that clouds the operative field. Most nonarterial bleeding stops with gentle warm irrigation.
Failure to perforate the Liliequist membrane may also result in ETV failure. Preoperative MRI is very important because it reveals the bowing of the third ventricle floor and its relationship to the basilar artery. Bowing of the third ventricle floor correlates with a pressure gradient between the ventricular system and the extraventricular CSF spaces. If the third ventricle floor is not bowed, the success rate of ETV is significantly decreased.
In cases of shunt revision or shunt removal after successful ventriculostomy, rupture of the choroid plexus during retrieval of the ventricular catheter is common and can lead to life-threatening hemorrhage. Different techniques can be used to avoid this complication; the most common of these techniques involves insertion of a stylet into the catheter lumen, allowing for coagulation with the diathermy before the catheter is retrieved. However, if the ventricular catheter is not easily removed, it should be left in place and an additional catheter should be placed. Image guidance can also be very helpful in ventricular catheter placement, especially in patients with loculated hydrocephalus and cannulating complex cysts.
Spina bifida is a midline defect in the mesenchymal-derived tissues and is classified as closed or open neural tube defects (NTDs). Closed NTDs do not involve exposed neural tissue and do not leak CSF. Open NTDs are subclassified into myelomeningocele (most common), myeloschisis, or hemimyelomeningocele (most rare). The goals of surgery are to preserve neural function, to prevent infection, and to prevent long-term complications such as an epidermal or dermal inclusion cyst and cord tethering.
Operative correction starts with anesthesia and positioning. Heat and blood loss are of vital importance in pediatric operative correction because both account for significant morbidity and mortality. The anesthetic can be modified if electrophysiologic monitoring and intraoperative nerve testing are desired.
The patient is usually placed prone on the operating room table, with sufficient padding of all pressure points. Open NTDs are cleansed with either sterile normal saline or lactated ringers; Betadine and other neurotoxic agents should be avoided. The wound is prepared and draped in a sterile fashion, and CSF is obtained for culture.
The neural placode is circumferentially incised, and every attempt is made to identify and separate all layers to allow for a multilayered closure that can be performed with fine running absorbable sutures. Inclusion of the epidermal tissue in the deep layers of the closure should be avoided because this can lead to late formation of a dermal inclusion cyst, an epidermal inclusion cyst, or both. The dura mater should be widely mobilized and closed without constricting the intradural contents, which can increase the likelihood of arachnoid adhesions and tethering.
Tissue should be conserved and trimmed only after it is definitively not needed for the closure. Working in conjunction with a plastic surgeon is helpful when myofascial flaps, rotational skin flaps, partial-thickness skin grafts, or flank-relaxing incisions are needed to close the defect. Closure in a transverse direction is preferable to a horizontal direction because the former offers better cosmesis and keeps the wound farther from the anus. In patients with severe hydrocephalus, placement of a ventriculoperitoneal shunt first theoretically decreases the risk of shunt contamination.
Postoperative Details
After successful completion of shunt insertion or revision in patients in whom a differential-pressure valve has been implanted, elevation into the upright position is commonly avoided to prevent shunt overdrainage and subdural hematomata formation. In contrast, patients in whom a flow-regulating shunt has been implanted are commonly elevated to 30° or more the day after the implantation to promote CSF drainage. The efficacy of these practices is unclear. Feeding of very young babies can commence soon after surgery.
Patients who undergo shunt revision tend to recover quickly, and they are usually discharged home a few days after surgery.
Follow-up
Postoperative follow-up a few weeks after shunt placement is usually necessary to ensure that the wound is healing well and that the head circumference is decreasing accordingly. Performing a CT scan and shunt series before discharge is customary to verify the position of the ventricular tube and to serve as future reference in case of possible shunt malfunction.
The issue of repeat scanning in the months after shunt insertion or ventriculostomy remains controversial. Certainly, satisfactory shunt function should be verified with at least one scan during the first year. In patients who undergo third ventriculostomy, cine phase-contrast MRI is mandatory to verify patency of the ETV stoma. Customarily, some surgeons perform yearly scanning, but the use of such routines is not universally accepted.
Currently, shunt malfunctions cannot be detected before they manifest clinically. Attempts to visualize CSF flow using ultrasonography or other imaging techniques have not been met with universal acceptance because they are associated with some false-negative and false-positive results.
Spina bifida requires ongoing follow-up for life because problems unrelated to hydrocephalus appear at various stages and necessitate treatment. Patients with spina bifida have continuing urologic problems due to neuropathic bladder. Neurogenic bowel can cause debilitating social problems. Although neurogenic bowel and bladder are initially managed medically with medication, retrograde scheduled enemas, and routine clean intermittent catheterization, some patients and physicians opt for surgical treatment such as the Malone procedure for antegrade colonic enemas (MACE) and the Mitrofanoff procedure for ease of catheterization.
- In the Malone procedure, a section of bowel (typically the appendix) is made into a conduit from the colon to the abdominal wall. This is then used for daily antegrade enemas to maximize bowel continence.
- The Mitrofanoff procedure is similar in that a section of bowel (again, usually the appendix) is made into a conduit from the bladder to the abdominal wall for daily catheterizations.
These procedures are performed in the hope of increasing independence and quality of life in patients with spina bifida. Although the specific indications and quantitative benefits of these procedures have not been well documented, improvements in health and well being have been suggested.
Another option for bowel continence is the use of a cecostomy, wherein a synthetic tube is inserted into the cecum and becomes the conduit for antegrade enemas. This may be used in patients without an appendix or in patients who have undergone a Mitrofanoff procedure in which the appendix was used.
Retethering of the cord occurs in 15-20% patients with myelomeningocele and is characterized by progressive weakness of one or both legs, onset or progression of scoliosis, change in gait, change in bowel or bladder control or function, or lower back pain. This condition may be confused with shunt malfunction.
Orthopedic problems, such as scoliosis and foot deformities, also require careful follow-up because they are likely to necessitate surgical treatment. A consideration particular to children with myelomeningocele is the relationship between hydrocephalus and scoliosis, which is present in a very high proportion of these patients. Scoliosis deteriorates in the presence of untreated hydrocephalus and improves following successful shunting. Active hydrocephalus is postulated to exacerbate the compressive effect of the hindbrain hernia on the descending pathways at the craniovertebral junction, inducing neuromuscular imbalance.
Another consideration specific to hydrocephalus related to myelomeningocele is the high incidence of precocious puberty among female patients (as many as 16%). The mechanism may be hypothalamic dysfunction caused by congenital deformity of the midbrain. Precocious puberty does not appear to affect intellectual development.
For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education article Spina Bifida.
Complications
The patient and the doctor must have an ongoing commitment to manage the complications associated with shunting. Shunt complications can be divided into 3 categories: mechanical, infective, and overdrainage-related. As many as 80% of shunts develop mechanical complications at some stage, and one third to one half of these complications occur within the first year of shunt placement. An additional 15% of shunts fail in the second year, and 1-7% shunts per year fail after the second year. On average, each patient is likely to undergo 2-3 operations throughout childhood for shunt revision. The mortality rate associated with initial insertion is approximately 0.1%; mortality due to shunt failure ranges from 1-4%.
Infective complications occur in 5-10% of all shunt operations and are more common in younger patients, especially in those younger than 6 months. Most shunt infections manifest in the first 3 months after insertion, and almost all present within the first 6 months. Staphylococcus is the most common offending organism. The use of shunts impregnated with antistaphylococcal antibiotics may reduce the incidence of shunt infection.
Symptoms of shunt infection include redness and swelling along the surgical incision site, tenderness over the reservoir, swelling or drainage, nuchal rigidity, or abdominal pain. During the 1970s, when ventriculoatrial shunts were commonly used, an appreciable number of patients experienced bacterial endocarditis and shunt nephritis caused by direct bacteremia due to bacterial colonization of the shunt lumen. The change from ventriculoatrial to ventriculoperitoneal shunts substantially decreased complications of shunting.
Most shunt obstructions are related to obstruction of the ventricular catheter by glioependymal tissue, which grows into the lumen from the ventricular wall through the draining holes. In many shunt revision operations, as many as 30% of intraventricular hemorrhages occur during removal of the old catheter because of rupture of the choroid plexus, which has grown into the shunt lumen. Symptoms of acute shunt obstruction include headache, nausea and vomiting, papilledema, cranial nerve VI palsy, change in personality, and the setting sun sign (lack of upward gaze) in infants. Chronic failure may be heralded by accelerated head growth, loss of milestones, papilledema, optic atrophy, and change in seizure frequency.
A significant late complication is fracture or destruction of the shunt tube due to material degradation and fatigue (see Media file 7).
Damaged shunt valve removed during shunt revision from a 22-year-old woman with hydrocephalus and spina bifida. The material of the valve has dramatically disintegrated.
Common locations for distal tube fracture include the occipitocervical junction, the root of the neck, and the junction between the inferior border of the ribs and the abdominal wall. These are points of maximal mechanical stress where the material is degraded most.
Overdrainage of cerebrospinal fluid (CSF) is another significant shunt complication that is difficult to counteract. Early overdrainage leads to formation of subdural hematomas, which are difficult to treat, and ligation of the shunt is sometimes necessary. Late chronic overdrainage leads to the development of slit ventricles and mostly affects patients with differential pressure valves, which drain excess CSF when the patients assume the upright position, because of the siphoning effect of the column of fluid in the distal tube. Chronic overdrainage leads to collapse of the ventricles and intermittent shunt obstruction.
Siphoning is due to a pressure gradient between the proximal and distal ends and is a factor of the height multiplied by the mass of CSF multiplied by the acceleration of gravity. Siphoning usually becomes apparent when patients come out of recumbency. Antisiphon devices of different types have been developed to overcome overdrainage and are incorporated in many shunt systems, with variable success. Over the years, technical aspects of the shunt valves and the development of flow-regulating valves have improved the frequency of adverse effects related to overdrainage and mechanical complications.
Endoscopic treatment of hydrocephalus carries the risk of complications similar to those of intraoperative and immediate postoperative shunt insertion (ie, a 10% risk of infection or hemorrhage, basilar artery injury, and hypothalamic or pituitary dysfunction) but does not carry the long-term problems and complications of shunts and is not associated with overdrainage. However, a rare complication of late rapid deterioration can occur up to 7 years after the surgery and is not well understood but is thought to be a result of stoma scarring. This can result in coma and possible death due to obstructive hydrocephalus.
Leaving an Ommaya reservoir in place can be helpful, especially in patients who are in areas where access to neurosurgical care is limited. Tapping the Ommaya reservoir and aspirating CSF can serve as a bridge to definitive treatment. An element of underdrainage is present even in successful cases because the ventricles remain larger than in shunted patients. The mortality rate of ETV is approximately 1%.
Some adolescents with myelomeningocele and shunted hydrocephalus develop focal discomfort at the shunt valve or along the distal catheter in the posterior triangle of the neck. This had been termed shuntalgia and is characterized by tenderness with a palpable firm fibrotic sheath of scar tissue in the area of pain. This may be related to the adolescent growth spurt and, although resistant to nonsteroidal anti-inflammatory drugs (NSAIDs), is usually self-limiting.
Lastly, 20-40% of children with myelomeningocele have an allergy to latex. Care must be exercised to avoid contact with latex products during surgery and postoperative hospitalization to minimize the risk of anaphylactic reactions.
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Further Reading
Keywords
hydrocephalus, infantile hydrocephalus, hydrocephaly, ventriculomegaly, aqueductal stenosis, intraventricular hemorrhage, spina bifida aperta, spina bifida occulta, hydrocele spinalis, schistorrhachis, myelomeningocele, Arnold-Chiari II malformation, Dandy-Walker syndrome
