Nursing Care of the Hydrocephalus Patient after Surgery

The most common operations that children with hydrocephalus undergo are shunt placement, shunt revision, and endoscopic third ventriculostomy. Because these children frequently have other diseases related to the hydrocephalus they often undergo other surgeries to treat a multitude of other problems.

Neurological Assessment
The assessment must occur in a developmentally appropriate manner. The nurse must also consider what is developmentally appropriate behavior for the infant or child, based upon his age. It is vital to also consider the individual child and his baseline. Many conditions related to hydrocephalus are also associated with significant delays, and because complications of hydrocephalus may worsen such delays, these children have a wide range of developmental abnormalities. A detailed history of developmental skill and baseline function is a vital part of being able to assess the infant/child.

Parents and families are an excellent resource to provide information about their particular child’s developmental level. The signs and symptoms of increasing intracranial pressure may initially be very subtle. Hence, the child’s caretaker is a valuable resource in such assessment and may notice subtle changes before nursing and medical staff.

Neurological assessment of the child after surgery to treat the hydrocephalus needs to be done frequently. The surgeon will usually specify the frequency, but assessment should occur every 1–4 h, depending on the condition of the child. An exam that is changing subtly over time may be an indication of a failed surgical treatment or postoperative complication. The first signs of increasing intracranial pressure are usually subtle and related to mildly increasing somnolence, lack of interest in activities (feeding) or play, and subtle behavioral changes. Level of consciousness is the most important single indicator of neurological status. Altered level of consciousness may progress to confusion, disorientation, somnolence, lethargy, obtundation, stupor, and coma.

A thorough neurological assessment starts with watching the child play and interact with those around him. Assessment also includes asking the child if he has a headache. The child should be examined for his ability to answer questions appropriately and follow directions. Asking a child to move his arms and legs will also allow the examiner to assess muscle strength, tone, and movement. Vital signs should also be assessed. Bradycardia can be a sign of increased intracranial pressure. Increased blood pressure is usually not a common finding in children until late in the process of increasing intracranial pressure.

It is important to carefully examine the eyes, noting that checking pupils without further exam is never an adequate exam. The pupils are checked for equality, roundness, and reactivity to light. Dilated and nonreactive pupils are a very late sign of increased intracranial pressure. A “sun-setting” appearance to the eyes or the loss of upward gaze is an abnormal finding. The extraocular movements should be intact.

The infant’s head should be examined. The occipital frontal circumference should be measured and documented on a daily basis to determine appropriate head growth. The fontanels should be palpated with the child upright and calm. The anterior fontanel should feel soft and pulsatile. A tense or bulging fontanel is suspicious for increased intracranial pressure. The suture lines of the skull should also be examined. Normal suture lines are palpable and apposed. If they are overriding, the infant may have overdrainage of the system. If the sutures are splayed there is likely increased intracranial pressure.

Wound and Dressing Care
The child will usually come from the operating room with a dressing over the incision. The dressing is normally removed, or changed, during the first few postoperative days. If a dressing is soiled or saturated with blood, most surgeons agree that it should be replaced. If the child is likely to pick at the incision, a dressing may be left on to prevent infection. Before a child goes home, most surgeons agree the dressing should be changed and the wound inspected for any erythema, drainage, swelling, or infection.

Medications
A substantial majority of neurosurgeons will order intravenous antibiotics for 24–48 h after the surgery to prevent shunt infection. Cefazolin or nafcillin are the most commonly used antibiotics, as Gram-positive organisms demonstrate a sensitivity to them. Vancomycin may also be used.

Pain management starts with good pain assessment. Age-appropriate pain assessment scales such as CRIES (crying, requires increased oxygen administration, increased vital signs, expression, sleeplessness), the Objective Pain Scale, and Oucher may be used. There is a wide variety of pain experienced by children after surgery for hydrocephalus. Pain may be related to the cranial incision(s), the abdominal incision, the amount of intra-abdominal manipulation, and the tunneling of the distal catheter through the subcutaneous tissue. Other factors influencing pain may include the age of the child, the child and/or family’s prior experience with pain, and the child and family’s anxiety. Pain is usually managed with medications, although other techniques may be helpful. The first drug of choice is usually acetaminophen. It should be adequately dosed at 15 mg/kg/dose and can be given orally or rectally. Nonsteriodal anti-inflammatory drugs (NSAIDS) may be used, but they can inhibit platelet aggregation and prolong bleeding time. For this reason, some neurosurgeons do not use NSAIDS during the immediate postoperative period.

If the child needs additional medication for pain, the surgeon’s beliefs about pain control in neurosurgical patients will be a factor. Some neurosurgeons will order opiates such as morphine sulfate, oxycodone, and codeine. Other surgeons do not want to alter the patient’s neurological exam with these drugs. The nurse should not administer these drugs if there is concern that the pain is due to increasing intracranial pressure or the neurological exam is changing. Other modalities to relieve pain may include age-appropriate relaxation techniques, play therapy, music therapy, massage, distraction, and acupuncture or acupressure.

Diagnosis of Hydrocephalus by Imaging Studies

The three major techniques used for diagnosis and evaluation of hydrocephalus are ultrasonography (US), CT, and MRI.

Ultrasonography
Prenatal US can be highly reliable and accurate in diagnosing hydrocephalus. Hydrocephalus can be detected in a fetus as early as the later part of the first trimester of pregnancy, although abnormal dilation of the fetus’s ventricles are more clearly detectable after 20–24 weeks gestation. Although prenatal US can detect abnormal CSF collection, it may not show the precise site or cause of the obstruction. Amniocentesis can often detect the presence of open neural tube defects, such as myelomeningocele, chromosome abnormalities, and in-utero infections, and may also help indicate the overall health of the fetus. In general, the first trimester development of significant hydrocephalus can be a poor prognostic sign for infant mortality and developmental progress. In some cases, mild ventricular dilation identified by US will resolve by the third trimester.

Cranial US is useful in infants and young children while the anterior fontanel is still open, usually under the age of 18 months. Through the fontanel, it can demonstrate lateral ventricular morphology and intraventricular clots. It is less accurate in its ability to look at the third and fourth ventricles and subarachnoid spaces. For this reason, the precise diagnosis and cause of hydrocephalus is rarely made by US alone. It is particularly useful, however, for follow-up screening of infants with untreated and treated hydrocephalus. The equipment is portable, involves no radiation, does not require sedation, and is considerably less expensive than CT/MRI.

Computed Tomography
Since the advent of CT scanning in 1976, it continues to be the most commonly used radiologic technique for the diagnosis and follow-up of hydrocephalus. CT images can accurately demonstrate the ventricular size and shape, the presence of blood and calcifications, cysts, and shunt hardware. In hydrocephalus, an enlarged ventricular system is usually seen, and is usually first seen in the lateral ventricles. CT images can also accurately reflect signs of increased intracranial pressure, such as compressed cerebral sulci, absent subarachnoid spaces over the convexity, and transependymal reabsorption of CSF in the white matter. When contrast enhancement is used, tumors, abscesses, and some vascular malformations can be visualized. It is currently the most rapid diagnostic screening tool, taking only a few minutes, and few children need to be sedated for the procedure. Despite the fact that it uses low-level radiation, little is known about the longterm effects of multiple scans. CT scanning has a lower resolution than MRI and is usually only performed in the axial plane.

Magnetic Resonance Imaging
Commercially available MRI was introduced in 1986 and is the examination of choice for revealing the underlying cause of hydrocephalus. It allows anatomical visualization in the axial, coronal, and sagittal planes, providing detailed information regarding the anatomy, and the position and extent of lesions. Subtle findings, such as white matter pathology, dysmorphic anatomy, and characteristics of lesions can be readily demonstrated. In addition, the aqueduct of Sylvius can be visualized, as well as membranes and loculated ventricular systems. With the addition of gadolinium (an intravenous contrast medium), some neoplasms and vascular lesions can be better visualized. CSF flow dynamics can be visualized through the use of phase-contrast cine MRI. This sequence takes only a few extra seconds and allows for real-time flow measurements
that are demonstrated on the sagittal plane of the MRI. Furthermore, constructive interference in the steady state (CISS) sequence MRI may be used. This sequence provides great detail of the ventricular system, basal cisterns, and may show membranes not otherwise seen on conventional MRI. Both phasecontrast cine MRI and CISS sequence MRI can be very helpful in determining the underlying cause of hydrocephalus. They can also provide valuable preoperative information related to the potential success of endoscopic third ventriculostomy, as well as postoperative information by being able to visualize the CSF flow pattern. MRI takes approximately 45 min or longer and, therefore, young children need to be sedated for the exam. Typically, children with normal development over the age of 5 or 6 years can often do the exam without sedation.

Signs and Symptoms of Hydrocephalus

The signs and symptoms of hydrocephalus in infants and children vary depending on their age, the degree of hydrocephalus at presentation, the primary etiology, and the time over which the hydrocephalus develops. Because of the plasticity of the infant brain and the ability of the cranium to expand, ventriculomegaly can progress without obvious signs of increased intracranial pressure. In premature infants, in which hydrocephalus is caused predominately by an IVH, there is a general correlation between the severity of hemorrhage and the degree of hydrocephalus. Infants with PHH may have minimal symptoms, or may exhibit increasing spells of apnea and bradycardia. They may also have hypotonia, sunsetting eyes, ophthalmoplegia and seizures. As the ventriculomegaly progresses, the fontanel will bulge, become tense and nonpulsatile, and the cranial sutures become splayed. In a healthy premature infant, the head circumference generally increases about 1 cm a week. In premature infants with progressive ventriculomegaly, the head circumference may increase more rapidly than normal (when charted on the head growth chart), but may not accurately reflect the rate of increase in ventricular size.

Table : Signs and symptoms of hydrocephalus in children
Premature infants	Full-term infants	Toddlers and older
Apnea	Macrocephaly	Headache
Bradycardia	Rapid head growth	Nausea
Hypotonia	Decreased feeding	Vomiting
Acidosis	Increased drowsiness	Irritability
Seizures	Tense fontanel	Lethargy
Rapid head growth	Vomiting	Delayed development
Tense fontanel	Distended scalp veins	Decreased school performance
Splayed cranial sutures	Splayed cranial sutures	Behavioral disturbance
Vomiting	Poor head control	Papilledema
Sunsetting eyes	Parinaud’s sign	Parinaud’s sign
	Sunsetting eyes	Sunsetting eyes
	Frontal bossing	Bradycardia
		Hypertension
		Irregular breathing patterns

Posthemorrhagic Hydrocephalus of Prematurity

The most common cause of hydrocephalus in the premature infant is a germinal matrix hemorrhage. The germinal matrix is a very vascular area in the fetal brain, in the subependymal region located at the level of the foramen of Monro. It is from the very thinwalled germinal matrix vessels that the bleeding is thought to occur in preterm infants. Bleeding can spread, most often to the adjacent ventricles and into the surrounding parenchyma. The germinal matrix gradually involutes after 34 weeks gestation and nearly disappears by 40 weeks. A grading system has been devised to describe the severity of the bleeding – grades I–IV.

Premature infants of less than 34 weeks gestation with very low birth weight (<1500 g) are at greatest risk for developing IVH. With current management, 20% of these preterm infants will develop an IVH. The risk of developing posthemorrhagic hydrocephalus (PHH) is related directly to the extent of the hemorrhage. Hydrocephalus develops in 20–74% of infants with IVH [3]. Infants with a grade I or II bleed do not have hydrocephalus by definition; 55% of infants with a grade III hemorrhage and 80% of those with a grade IV bleed develop hydrocephalus. PHH may develop as a result of the accumulation of blood and hemorrhagic debris within the ventricles and subarachnoid spaces. Obstruction of the aqueduct of Sylvius or foramen of Monro may occur. The breakdown of blood may also render the arachnoid villi unable to reabsorb the CSF. Multiloculated hydrocephalus may occur after IVH due to ventriculitis. Ventricular septations develop causing isolated compartments of fluid within the ventricles.

Many premature infants require surgical intervention to treat the hydrocephalus until it is resolved. About 20–30% will require permanent shunting.

Classifications of Types of Hydrocephalus

Hydrocephalus is subdivided into several different categories. Communicating and noncommunicating are the most common categories. These terms were previously used interchangeably with obstructive and nonobstructive. The latter terms have fallen from use, as it is believed that in almost all cases of hydrocephalus there is some obstruction of CSF reabsorption; the exception is the rare state of overproduction of CSF. Hydrocephalus is also subdivided into congenital versus acquired, and internal versus external (see table). Other categories include normal pressure hydrocephalus and ex vacuo hydrocephalus.

Table. Classifications of hydrocephalus

Communicating

Congenital Achondroplasia Arachnoid cyst Dandy-Walker malformation Associated with craniofacial syndromes Acquired Posthemorrhagic: intraventricular or subarachnoid Choroid plexus papilloma or choroid plexus carcinoma Venous obstruction as in superior vena cava syndrome Postinfectious

Noncommunicating

Congenital Aqueductal stenosis Congenital lesions (vein of Galen malformation, congenital tumors) Arachnoid cyst Chiari malformations either withor without myelomeningocele X-linked hydrocephalus Dandy-Walker malformation Acquired Aqueductal gliosis (posthemorrhagic or postinfectious) Space-occupying lesions such as tumors or cysts Head injuries

Hydrocephalus : History of Hydrocephalus

Hydrocephalus is a condition resulting from an imbalance between the production and absorption of cerebral spinal fluid (CSF). This imbalance results in an increased volume of spinal fluid, dilation of the ventricular system, and often increased intracranial pressure. Hydrocephalus can be acute and occur over hours or days. It may also be chronic and occur over months or years. Hydrocephalus can occur as an isolated condition or one associated with numerous other neurological conditions and diseases.

The term hydrocephalus is derived from the Greek words “hydro” meaning water, and “cephalus” meaning head. The description and treatment of hydrocephalus dates back to the era of Hippocrates and Galen. Galen (AD 130–200) identified the ventricles. He believed that the soul was purified through the pituitary gland, and that waste was discharged via the nose as “pituita.” During the Renaissance, Vesalius (1514–1564) described the ventricular system in his original text on human anatomy. A century later, Franciscus Sylvius (1614–1672) described the cerebral aqueduct. Morgagni (1682–1771) described the pathology of hydrocephalus, and Monro (1733–1817) named the intraventricular foramen. In 1786, Whytt distinguished the internal and external hydrocephalus.

Early treatments included bleeding, purging, surgical release of the fluid, puncturing the ventricles to drain the fluid, injection of iodine or potassium hydriodate into the ventricles, binding of the head, application of a plaster of herbs to the head, application cold wraps to the head, lumbar puncture, and diuretics. Confusion about hydrocephalus persisted into the 1800s. It was thought to be caused by fevers, rheumatism, pulmonary consumption, and worms; however, treatment did not change.

The earliest attempts at surgery occurred during the late 1800s. The first shunts diverted CSF from the ventricles to the subcutaneous or subdural spaces. During the early 1900s, other surgical procedures were attempted to treat the condition. These procedures included surgical removal of the choroid plexus, diversion of spinal fluid through a third ventriculostomy, and continued attempts at shunting, including attempts to shunt into the vascular space. Most of these patients did poorly, and either suffered the consequences of prolonged increased intracranial pressure or died. Many institutions cared for these disabled children with very large heads, small bodies, and severe mental retardation.

Modern shunting procedures began in the 1950s with the introduction of the antireflux valve. The first valves developed by Nulson and Spitz in 1952, used a spring and steel ball valve. Holter then developed the first slit valve. He was particularly interested in shunt development, as he had a son with a myelomeningocele and hydrocephalus. These first modernized shunts diverted CSF from the ventricles to the right atrium of the heart. The ventricular-to-peritoneal shunt became the preferred shunt in the 1970s because it allowed for the child to grow, and not outgrow the length of the shunt tubing. This has remained the preferred shunt procedure among modern neurosurgeons. Neurosurgeons have also placed shunts leading from the ventricle to the pleural space, gall bladder, ureter, or fallopian tube if the abdominal cavity is not an appropriate place to terminate the shunt. Numerous improvements in shunt hardware have occurred in the last four decades.

A genetic understanding of hydrocephalus and the diseases associated with hydrocephalus has occurred in the last decade. Such knowledge of genetics has allowed for improved prenatal diagnosis and genetic counseling.

Growth and Developmental Tasks by Age - Developmental Assessment

Knowledge of human growth parameters and normal developmental landmarks is critical to the assessment of each age group. Growth is defined as changes in the values given certain measurements of maturity; where as development may encompass other aspects of differentiation of form or function, including those emotional or social changes preeminently shaped by interaction with the environment.

Serial measurements can indicate the normal or abnormal dynamics of the child’s growth. One key growth measurement important to the neurological assessment of the child is the head circumference. The measurement is taken around the most prominent frontal and occipital bones which offer the maximal circumference. How rapidly the head circumference accelerates or decelerates away from the percentile curve can determine whether the underlying cause of the growth change is more benign or serious. An example of a benign finding is the presence of extra-axial fluid collections of infancy, which often present with an accelerating head circumference. Generally, the infant with this finding is observed over time, but no intervention is warranted. On the other hand, an accelerating head circumference can also be a sign of increasing intracranial pressure in uncompensated hydrocephalus, which would require immediate evaluation and treatment.

Development is the essential distinguishing feature of pediatric nursing. Normal development is a function of the integrity and maturation of the nervous system. Only with a working knowledge of agerelated developmental standards can the examiner be sensitive to the deviations that indicate slight or early impairment of development and an abnormal neurological assessment. An abnormality in development from birth suggests an intrauterine or perinatal cause. Slowing of the rate of acquisition of skills later in infancy or childhood may imply an acquired abnormality of the nervous system. A loss of skills (regression) over time strongly suggests an underlying degenerative disease of the central nervous system.

Voluntary motor skills generally develop in a cephalocaudal and proximodistal progression, as it parallels the process of myelination. First the head, then the trunk, arms, hands, pelvis, legs, bowel, and bladder are brought under voluntary control. Early in life, motor activity is largely reflexive, and generalized movements predominate. Patterns emerge from the general to the specific; for example, a newborn’s totalbody response to a stimulus is contrasted with the older child, who responds through simply a smile or words. So, as the neuromuscular system matures, movement gradually becomes more purposeful and coordinated. The sequence of development is the same for all children, but the rate of development varies from child to child.

Finally, also important to a complete neurological exam is an assessment of the child’s cognitive and emotional development. These abilities impact directly on expectations of the child’s behavioral, social, and functional capabilities. The younger the child, the more developmental history is needed from the parents. Accurate identification of the child’s mastery of cognitive and emotional developmental milestones, as it relates to chronological age, is necessary for a comprehensive neurological assessment.