eMedicine Specialties > Pediatrics: Surgery > General Surgery
Management of Spina Bifida, Hydrocephalus and Shunts
Updated: Nov 19, 2009
Introduction
Hydrocephalus is defined as excess cerebrospinal fluid (CSF) accumulation in the head caused by disturbance of formation, flow, or absorption. The term stems from the Greek hydro (water) and cephali (head).
Infantile hydrocephalus is associated with the following:
- Congenital anomalies
- Aqueductal stenosis
- Spina bifida
- Arnold-Chiari II malformation
- Less common conditions
- Dandy-Walker syndrome
- Encephaloceles
- Viral or parasitic infections
- Arachnoid cysts
- Intracranial neoplasms
- Vascular problems
- Nutritional deficiencies
- Poisonings
- Acquired conditions
- Perinatal intraventricular hemorrhage (IVH)
- Meningitis
- Trauma - Closed head injury
The lumbar region of a newborn baby with myelomeningocele. The skin is intact, and the placode-containing remnants of nervous tissue can be observed in the center of the lesion, which is filled with cerebrospinal fluid (CSF).
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Posthemorrhagic hydrocephalus is an increasingly significant contributor to overall incidence of hydrocephalus because of improved neonatal care and increasing survival rates of very low birth weight infants.
History of the Procedure
In the 1940s, before shunting was established, children with hydrocephalus had a poor prognosis. Most patients were not offered treatment, and only 20% of children who did not undergo surgery for hydrocephalus reached adulthood. Furthermore, children who survived had a 50% chance of having permanent brain damage. Outcomes improved after the introduction of valved shunt systems by Nulsen and Spitz in 1952 and after the development of silicone systems by Holter and Pudenz in the 1960s. Most children with hydrocephalus currently reach adulthood if the shunt is appropriately maintained. In a 20-year follow-up survey of children who received shunting in the 1970s, more than half of them graduated from mainstream education.
The outcome of patients with spina bifida has also improved. In a review of a cohort of patients treated in the 1970s for spina bifida aperta, 52% of the patients were alive 20 years after treatment. Most of the deaths occurred in the first year of life, mostly due to renal and respiratory problems associated with spina bifida. Only a few of the deaths were related to hydrocephalus. In a similar, but more recent, review of children treated in the 1980s, only 27% died; most of them died in the first year of life from causes related to spina bifida rather than hydrocephalus. In a recent survey of adults with spina bifida, 6% of patients died from shunt-related problems or died after craniovertebral decompression for Arnold-Chiari II malformation.
Problem
Hydrocephalus is caused by either increased production of CSF or impaired circulation and absorption. Hydrocephalus caused by impaired circulation is called obstructive hydrocephalus because CSF circulation is anatomically blocked. Hydrocephalus caused by increased production or impaired absorption of CSF is called communicating hydrocephalus because CSF circulation is not anatomically blocked. According to some authorities, all cases of hydrocephalus are obstructive (ie, patients with communicating hydrocephalus have a functional obstruction at the final stage of absorption at the arachnoid granulations).
Frequency
The incidence of infantile hydrocephalus is estimated at 3-5 cases per 1000 live births. The peak ages of presentation in this group include the first few weeks of life, age 4-8 years, and early adulthood. The latter 2 peaks represent delayed presentations of infantile hydrocephalus. An estimated 750,000 people have hydrocephalus, and 160,000 ventriculoperitoneal shunts are implanted each year worldwide. About 56,600 children and adolescents younger than 18 years have a shunt in place.
The incidence of myelomeningocele ranges from 0.2-2 per 1000 live births. The overall incidence of myelomeningocele has significantly declined in the last 2 decades because of improved maternal nutrition during pregnancy, including the addition of folic acid, a wider availability of prenatal diagnosis, and therapeutic termination of pregnancy. In a significant proportion of patients with open spina bifida, hydrocephalus is absent at birth but develops in the first few weeks or months of life. Hydrocephalus occurs in 15-25% of children with open myelomeningocele at birth; however, in most surgical series, the proportion of patients with myelomeningocele who require shunting reaches 80-90%.
In a retrospective chart review, shunt placement has been shown to vary based on the level of the lesion, with a greater number of patients with thoracic lesions requiring shunts than those with lumbar or sacral lesions. Lesions at levels of T12 and above have also been associated with increased incidence of brain abnormalities and lower scores on psychometric testing than lesions at L-1 or below.
Etiology
The simplistic distinction of obstructive and communicating hydrocephalus is historically related to different conditions that cause or are associated with impairment of CSF circulation. Examples of conditions associated with obstructive hydrocephalus include congenital aqueductal stenosis, tumors of the ventricular system (eg, colloid cyst of the third ventricle, astrocytoma of the third ventricle), and tumors of the posterior cranial fossa (eg, cerebellar astrocytoma, medulloblastoma). An example of communicating hydrocephalus caused by CSF overproduction is the presence of choroid plexus papilloma in one of the ventricles. Examples of conditions with communicating hydrocephalus caused by impaired CSF absorption include IVH, meningitis, and head injury. Occasionally, obstructive and communicating hydrocephalus coexist and therefore cannot be differentiated.
Pathophysiology
Production of CSF is an active process that occurs at a rate of 0.35 mL/min. Absorption of CSF at the arachnoid granulations is also an active process and requires at least 6.8 mm of water pressure to overcome the venous blood pressure inside the sagittal sinus. During a 24-hour period, a total of 500 mL and 250 mL of CSF is produced and absorbed in adults and in children, respectively. At any given time, a total of 140 mL and 70 mL of CSF is present in the head in adults and in children, respectively. Normal CSF pressure inside the ventricles is 110 mm of water pressure. When CSF circulation is impaired, the resulting CSF accumulation leads to ventricular enlargement and a rise in intraventricular (and, hence, intracranial) pressure. In infants with open fontanelles, some of this rise in pressure is counteracted with enlargement of the head. When the maximum capacity for head enlargement has been exceeded, rapid deterioration follows because of raised intracranial pressure.
In children with aqueductal stenosis, the aqueduct of Sylvius is narrower than usual or is completely occluded and the CSF flow is obstructed. Some believe that aqueductal stenosis is the primary deformity and leads to ventriculomegaly. Others believe that ventriculomegaly is the primary deformity (because of altered abnormal ventricular wall compliance) and leads to secondary aqueductal stenosis caused by continuing compression of the midbrain.
In children with postmeningitic or posthemorrhagic hydrocephalus, protein (meningitis) or blood degradation products (IVH) are hypothesized to occlude arachnoid granulations, rendering CSF absorption relatively ineffective.
Several factors are implicated in the pathogenesis of hydrocephalus in children with myelomeningocele; these include the Arnold-Chiari II malformation, a degree of aqueductal stenosis, anomalous venous drainage in the posterior fossa caused by compression of the sigmoid sinuses, open myelomeningocele, and the presence of other CNS malformations.
Extensive deformity of the posterior fossa and its structures is associated with Arnold-Chiari II malformations. The brain stem has abnormal disposition with respect to the midbrain and the tentorial hiatus, the posterior fossa has smaller capacity than usual, the fourth ventricle is displaced caudally, and the cerebellar tonsils through the foramen magnum are significantly prolapsed. These anatomic factors all contribute to the impairment of CSF circulation.
Although the development of hindbrain herniation during gestation in myelomeningocele was once thought to be the result of cord tethering “pulling” on the brain tissue, hindbrain herniation is now believed to be caused by the progressive caudal migration of the hindbrain in association with the low-pressure conditions created in the spine by the open myelomeningocele. Continued loss of CSF soon after birth exacerbates the hindbrain hernia and the associated hydrocephalus. This can lead to acute neurologic deterioration caused by a combination of raised intracranial pressure related to the ventriculomegaly and acute bulbar dysfunction caused by compression of the brain stem in the foramen magnum region. The neurologic state usually improves after ventricular shunting. In most patients, ventriculomegaly gradually develops within the first few weeks or months of life.
Hydrocephalus development can be temporally related to the closure of the myelomeningocele. In a small group of patients with open myelomeningocele, dramatic deterioration occurs after closure of the defect. The impaction of the hindbrain hernia plays a significant role in this acute deterioration.
In addition to the presumed effect of the low-pressure leak during gestation on development of Arnold-Chiari II malformation described above, the abnormal and exposed spinal tissue is theorized to sustain damage in utero through trauma and exposure to neurotoxic substances in amniotic fluid. This is thought to lead to neurologic deficits that are worse than if the exposure did not occur. Most recently, several surgical centers have attempted to lessen the effect of Arnold-Chiari II malformation, neurologic impairment, and need for shunting through the use of in utero surgical repair of the myelomeningocele. The first such repair was performed in 1997, after several animal models demonstrated benefit of the procedure. Studies of the outcomes of these surgeries have been somewhat promising, although still inconclusive.
A reduction in shunt-dependent hydrocephalus has been shown in children who underwent surgical correction at less than 25 weeks’ gestation, who had ventricular measurements of less than 17 mm at time of surgery, and who had an anatomic level lower than L3. An approximately 50% reduction in the need for shunts was reported in this select group. However, long-term follow-up was lacking. One study demonstrated a decrease in hindbrain herniation with improvement on serial fetal scans. However, another study failed to demonstrate any difference in progression of ventriculomegaly in patients who underwent intrauterine repair when compared with controls.
Several small studies have investigated leg and neurologic function and have not provided clear evidence that in utero repair improves either function. A study involving 30 children (43% of whom required a shunt) investigated neurodevelopmental outcome at 2 years following treatment. The study revealed that 87% of the children had normal or mildly delayed cognitive language and personal-social skills.
In utero repair is certainly not without risk to both the mother and the fetus. In fetuses, a 4% risk of mortality and an 11% risk of morbidity (primarily from prematurity) is associated with the surgery. In mothers, uterine dehiscence, uterine rupture, and hysterectomy are also risks. Development of minimally invasive techniques may improve these outcomes, but they have not yet been successful in this procedure. Because the risks and benefits are not clear, a randomized control trial (the Management of Myelomeningocele Study [MOMS]) funded by the National Institute of Child Health and Human Development is currently underway at 3 US centers in an attempt to definitively evaluate the risks and benefits of in utero repair of myelomeningocele.
Presentation
Infants with hydrocephalus develop an enlarging head with bulging fontanelle, enlarged scalp veins, macrocrania, suture diastasis, and positive Macewen (ie, cracked pot) sign. If the hydrocephalus is not treated, these infants develop sunset eyes, recurrent vomiting, and, later, respiratory arrest. Persistent CSF leak from the repaired spinal wound almost invariably indicates active hydrocephalus, even if the ventricular size is only modestly enlarged and the anterior fontanelle is not bulging.
The particular concern in children with myelomeningocele is the presence of hindbrain hernia in the context of the Arnold-Chiari II malformation, which can cause early clinical symptoms of bulbar palsy due to compression of the brainstem and can remain unnoticed by inexperienced observers or be confused with symptoms of shunt malfunction or untreated hydrocephalus.
Poor feeding, recurrent vomiting, poor sucking, generally subdued behavior with poor crying, high-pitched cry or stridor caused by vocal cord paralysis (a predictor of poor outcome), episodes of apnea, extremity weakness in older children, and recurrent aspiration (often manifesting as recurrent pneumonia) can all be manifestations of brain stem dysfunction caused by hindbrain hernia and aggravated by ventricular dilatation. Approximately 20% of children with myelomeningocele who also have an Arnold-Chiari II malformation develop brainstem symptoms. Myelomeningocele is also a contributor to mortality and morbidity in the first 2 decades of life.
Older children with closed fontanelles develop clinical signs of intracranial hypertension without progressive head enlargement. They develop headaches, blurred vision, decline in intellectual performance, and gradual drowsiness, which, if left untreated, lead to coma and death due to respiratory arrest.
Indications
Children with a clinical picture of active hydrocephalus and significant ventriculomegaly (often with evidence of periventricular lucency indicating raised cerebrospinal fluid (CSF) pressure in the ventricular system) require treatment early in life. However, children with mild or moderate ventriculomegaly and a head circumference within the reference range may not require initial treatment. Watchful waiting may be adopted during the first few months of life, and head circumference monitoring and repeated ultrasonography, CT scanning, or MRI are helpful in deciding whether shunting is required.
Age, prematurity status, and weight are significant considerations for the timing of treatment in children. In general, shunting is avoided, if possible, in children younger than 6 months because they have an increased risk of infection. For the same reason, shunting should be deferred, if possible, in premature babies who weigh less than 1.5 kg until they have gained weight. These considerations often arise in children with posthemorrhagic hydrocephalus because they are often premature and small for age.
If feasible, shunts should be placed when the myelomeningocele is closed because it appears to protect patients from CSF leak from the spinal wound, which can lead to shunt infection, and improves the chances for better development by reducing intracranial hypertension early. One of the signs of ongoing hydrocephalus after closure of myelomeningocele is persistent CSF leak. Children with open myelomeningocele in whom closure of the defect has been delayed may already have CSF infection. In such circumstances, CSF microbiological testing should be performed; if CSF infection is present, external ventricular drainage should be performed for 7-10 days in conjunction with antibiotic treatment, until CSF infection is controlled and a shunt can be inserted.
Although children with IVH may have active hydrocephalus early in life, shunting is often difficult to consider because the CSF is heavily blood stained, the protein content is too high (>1 g/dL), or both. In such cases, the traditional approach is to insert an external ventricular drain until the CSF clears and a shunt can be inserted. Injection of tissue plasminogen activator (TPA) into the ventricles has been attempted in an effort to accelerate blood clearance from the CSF, with moderate success. This therapeutic maneuver has not yet gained universal acceptance.
An issue that merits attention is the need to decide whether shunting is indicated in older children or young adults who have myelomeningocele and untreated ventriculomegaly. Some patients have the typically shaped ventricles of hydrocephalus that are caused by spina bifida but do not appear to have tension, with no periventricular flow of CSF as seen on T2-weighted MRI and no symptoms (eg, headache, drowsiness, diplopia, bulbar features) that suggest active hydrocephalus. If these patients never receive shunting, they should be serially monitored with intelligence and psychometric testing.
If a patient has no clinical symptoms and his or her psychometric test results indicate stability, shunting based solely on radiologic appearance should be discouraged because the risks outweigh the benefits, and serial follow-up should be continued. Similarly, any intervention on the shunt should be considered cautiously in patients who already have received shunting. Because shunts may be disconnected or may appear to have long since stopped working, regarding the situation as compensated hydrocephalus and choosing to remove the shunt, especially if it is causing local discomfort in the neck, is tempting. However, shunts that have been implanted for years acquire a tube of strong fibrous tissue that surrounds them along their entire length.
Although the tube may appear fractured on radiographs, CSF is bridging the gap guided by the encircling fibrous tube. Such shunts are actually functioning, and any attempt to remove them without instituting any alternative means of CSF drainage (eg, third ventriculostomy) may cause neurologic deterioration. However, if the patient has subtle symptoms or a declining intelligence based on psychometric testing, treatment should be offered. In such cases, patients who have not received shunting should receive them; in those with shunts, the shunt systems should be evaluated and explored and revised if necessary.
Relevant Anatomy
Cerebrospinal fluid (CSF) is typically produced by the choroid plexus of the ventricles and circulates in one direction from the lateral ventricles to the third ventricle and through the aqueduct of Sylvius to the fourth ventricle. From the fourth ventricle, CSF exits the brain through 3 separate openings: one in the midline (foramen Magendie) and one on either side (foramina Luschka). It enters the subarachnoid space at the foramen magnum, circulates down to the spine, and then circulates up again to the surface of the brain, where it is absorbed at the arachnoid granulations. These are sievelike structures where the CSF enters the venous circulation, leading to the sagittal sinus.
In children with hydrocephalus, ventricular dilatation affects the part of the ventricular system that is before the level of the obstruction, with respect to CSF circulation. Thus, in aqueductal stenosis, the lateral and third ventricles are dilated, but the fourth ventricles are not. In contrast, in postmeningitic or posthemorrhagic hydrocephalus, all ventricles are dilated because the obstruction is at the level of the arachnoid granulations and at the end of the intracranial CSF circulation conduit.
Contraindications
Relative contraindications to treatment of infantile hydrocephalus include severe CNS malformations that are regarded as incompatible with normal development, such as some of the congenital neurodevelopmental syndromes associated with severe malformation of a large part of cerebral substance (ie, anencephaly); in these cases, neonatologists prefer to counsel parents against treatment of hydrocephalus. The same considerations are applied to severe cases of intraventricular hemorrhage (IVH) in which radiologic investigations clearly indicate that the hemorrhage has damaged significant large parts of the brain.
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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
