Pediatric Neurosurgery

The Pediatric Neurosurgery Service at Stanford and the Lucille Packard Children's Hospital treats children with brain and spinal cord tumors, cranio-facial anomalies, hydrocephalus, trauma, and epilepsy. A pediatric neuro-oncology team evaluates, treats, and monitors children with brain cancer. Advanced techniques are employed in the treatment complex hydrocephalus and childhood seizures. A new MRI and CT imaging center designed specifically for pediatric imaging, the only facility of its kind in northern California, has just opened at the Packard Children's Hospital.



Specialties

Pediatric cerebrovascular disease

Although cerebrovascular disorders occur less often in children than in adults, recognition of stroke in children has probably increased because of the widespread application of noninvasive diagnostic studies such as magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), computed tomography (CT) and, in the neonate, cranial ultrasound studies. These studies allow confirmation of a diagnosis that in previous years would not have been suspected or at least not recognized as a vascular lesion. Also, the number of patients with cerebrovascular lesions from certain risk factors may have increased as more effective treatments for some causes of stroke have allowed patients to survive long enough to develop vascular complications. Patients with sickle cell disease or with leukemia, for example, now have a longer life-expectancy, and during this time they may have a stroke.

Probably the most fundamental difference between cerebrovascular diseases in children and adults is the wide array of risk factors seen in children versus adults. Congenital heart disease and sickle cell disease, for example, are common causes of stroke in children, while atherosclerosis is rare in children. No cause can be detected in about a fifth of the children with ischemic infarction, yet many of these children seem to do well. The recognized causes of cerebrovascular disorders in children are numerous, and the probability of identifying the cause depends on the thoroughness of the evaluation.

No randomized controlled treatment trials have been completed in children with stroke; many of the procedures increasingly used in children with cerebrovascular disease have been adapted from studies in adults. Accumulating experience with antithrombotic and anticoagulant treatment in children suggests that these agents can be safely used in children, though their efficacy and proper dose still need to be established by controlled trials. Thrombolytic agents should be as effective in children as in adults, but the safety data are inadequate for children and the timing and dosage need to be determined for children and adolescents.

Craniosynostosis

Craniosynostosis is a congenital anomaly characterized by premature closure-before the completion of brain growth-of one or more cranial sutures (the fibrous joints between the bones of the skull). The disorder results in an abnormality of the shape of the skull. The condition may be familial; a result of a chromosomal or genetic abnormality; or it may occur sporadically, with no other affected relatives. Some cases are associated with disorders such as microcephaly (abnormally small head) and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain). The first sign is an abnormal head shape. Other symptoms, which include increased intracranial pressure, developmental delay, and mental retardation, may be caused by constriction of the growing brain. Seizures and blindness may also occur.

Treatment for craniosynostosis generally consists of surgery (usually performed early in life) to relieve increased intracranial pressure, assure capacity of the skull to accommodate brain growth, and improve the appearance of the head. Prognosis varies depending on whether single or multiple sutures are involved and the presence of associated abnormalities. Prognosis is better for those with single suture involvement and no associated abnormalities.

Head and spinal cord injury

Head and spine injury - Spinal cord injury (SCI) occurs when a traumatic event results in damage to cells within the spinal cord or severs the nerve tracts that relay signals up and down the spinal cord. The most common types of SCI include contusion (bruising of the spinal cord) and compression (caused by pressure on the spinal cord). Other types of injuries include lacerations (severing or tearing of some nerve fibers, such as damage caused by a gun shot wound), and central cord syndrome (specific damage to the corticospinal tracts of the cervical region of the spinal cord). Severe SCI often causes paralysis (loss of control over voluntary movement and muscles of the body) and loss of sensation and reflex function below the point of injury, including autonomic activity such as breathing and other activities such as bowel and bladder control. Other symptoms such as pain or sensitivity to stimuli, muscle spasms, and sexual dysfunction may develop over time. SCI patients are also prone to develop secondary medical problems, such as bladder infections, lung infections, and bed sores.

Traumatic brain injury is sudden physical damage to the brain. The damage may be caused by the head forcefully hitting an object such as the dashboard of a car (closed head injury) or by something passing through the skull and piercing the brain, as in a gunshot wound (penetrating head injury). The major causes of head trauma are motor vehicle accidents. Other causes include falls, sports injuries, violent crimes, and child abuse.

The physical, behavioral, or mental changes that may result from head trauma depend on the areas of the brain that are injured. Most injuries cause focal brain damage, damage confined to a small area of the brain. The focal damage is most often at the point where the head hits an object or where an object, such as a bullet, enters the brain.

Lipomyelomengocele

Lipomyelomengocele is the growth of fatty tissue that tethers the spinal cord usually at the distal (towards the tailbone) end. Tethering of the cord leads to compromise of the spinal cord blood supply and possibly leads to progressive neurologic deficit.

Myelomengiocele

Myelomeningocele is one of the most common birth defects of the brain and spinal cord (central nervous system). The bones of the spine do not completely form, and the spinal canal is incomplete. This allows the spinal cord and meninges (the membranes covering the spinal cord) to protrude out of the child's back.

Spina bifida includes any congenital defect involving insufficient closure of the spine. Myelomeningocele accounts for about 75% of all cases of spina bifida and may affect as many as 1 out of every 800 infants. The rest of the cases are most commonly spina bifida occulta (where the bones of the spine do not close, the spinal cord and meninges remain in place, and skin usually covers the defect) and meningoceles (where the meninges protrude through the vertebral defect but the spinal cord remains in place).

The cause of myelomeningocele is unknown. However, folic acid deficiency is thought to play a part in neural tube defects such as myelomeningocele. The disorder appears to be more common in families. If a child is born with myelomeningocele, subsequent children in that family have a higher risk than the general population.

A viral cause or trigger has been theorized because there is a higher incidence of the defect in children born in the early winter months. Research also indicates possible environmental factors such as radiation.

Protrusion of the spinal cord and meninges damages the spinal cord and nerve roots, causing a decrease or lack of function of body areas controlled at or below the defect. Symptoms are related to the anatomic level of the defect. Most defects occur in the lower lumbar or sacral areas of the back (the lowest areas of the back) because this area is normally the last part of the spine to close.

Symptoms include partial or complete paralysis of the legs, with partial or complete lack of sensation, and may include loss of bladder or bowel control. The exposed spinal cord is susceptible to infection (meningitis).

Other congenital disorders may also be present in the child, usually disorders of the spinal cord or the musculoskeletal system such as hydrocephalus (which may affect as many as 90% of children with myelomeningocele), syringomyelia, hip dislocation, or similar disorders.

Pediatric brain tumor

Pediatric brain tumors differ considerably from their adult counterparts. A child's developing bodies and brains require special care and consideration. With only about 2,200 children diagnosed with a brain tumor in the United States each year, it is a rare disease of childhood. The mostcommon presenting symptoms are a history of flu-like symptoms; frequent headaches that are worse in the morning and are associated with nausea or vomiting; a wobbly walk; a decline in school performance; and changes in vision. Symptoms that don't improve after a reasonable period of time should be investigated.

More brain tumors are found in children age 7 and younger than in older children, and they tend to be more common in boys than girls. Childhood brain tumors are classified by their biological characteristics and their location in the brain. About half of all pediatric brain tumors are infratentorial (they occur in the structures below the tentorium). This area, also called the posterior fossa, contains the fourth ventricle, the brain stem and the cerebellum.

Pediatric epilepsy

Pediatric epilepsy is the tendency of children to experience seizures in. Seizures can be of several types. The most familiar type is the generalized tonic clonic (Grand Mal) convulsion, in which there is a loss of consciousness and the whole body shakes rhythmically. An absence (Petit Mal) seizure involves a brief loss of awareness, which can be accompanied by blinking or mouth twitching. Petit Mal seizures have a very characteristic appearance on an electroencephalogram (EEG). Partial seizures occur when the convulsion starts in one area of the brain. Simple partial seizures do not cause an alteration of consciousness, whereas complex partial seizures involve some alteration of consciousness. The manifestations, or symptoms, of the seizure depend on where in the brain it starts. For example, a partial seizure originating in the area of the brain that controls hand movements will result in hand twitching. A partial seizure that starts in the area of the brain controlling emotion may result in a fearful feeling. Sometimes, it is difficult to distinguish a partial seizure from a petit mal seizure, but they can be differentiated on an EEG.

Pediatric head and spine trauma

Pediatri head and spine trauma - Spinal cord injury (SCI) occurs when a traumatic event results in damage to cells within the spinal cord or severs the nerve tracts that relay signals up and down the spinal cord. The most common types of SCI include contusion (bruising of the spinal cord) and compression (caused by pressure on the spinal cord). Other types of injuries include lacerations (severing or tearing of some nerve fibers, such as damage caused by a gun shot wound), and central cord syndrome (specific damage to the corticospinal tracts of the cervical region of the spinal cord). Severe SCI often causes paralysis (loss of control over voluntary movement and muscles of the body) and loss of sensation and reflex function below the point of injury, including autonomic activity such as breathing and other activities such as bowel and bladder control. Other symptoms such as pain or sensitivity to stimuli, muscle spasms, and sexual dysfunction may develop over time. SCI patients are also prone to develop secondary medical problems, such as bladder infections, lung infections, and bed sores.

Traumatic brain injury is sudden physical damage to the brain. The damage may be caused by the head forcefully hitting an object such as the dashboard of a car (closed head injury) or by something passing through the skull and piercing the brain, as in a gunshot wound (penetrating head injury). The major causes of head trauma are motor vehicle accidents. Other causes include falls, sports injuries, violent crimes, and child abuse.

The physical, behavioral, or mental changes that may result from head trauma depend on the areas of the brain that are injured. Most injuries cause focal brain damage, damage confined to a small area of the brain. The focal damage is most often at the point where the head hits an object or where an object, such as a bullet, enters the brain.

Pediatric hydrocephalus

Hydrocephalus is excess cerebrospinal fluid (CSF) in the head. This often begins at birth with blockage of one of the CSF drainage pathways, such as in aqueductal stenosis. It may also occur through impairment of absorption of the CSF following bleeding into the brain either in infancy or after a ruptured aneurysm. Often the fluid back up will cause symptoms such as headache, lethargy or vomiting. In the infant it may cause excessive enlargement of the head. In the elderly, hydrocephalus may cause difficulties with memory, bladder control or gait. The treatment often includes placement of a ventriculoperitoneal shunt to drain the fluid from the ventricles to the peritoneal cavity where it can be reabsorbed into the blood stream. Shunts may develop problems such as blockage or infection and thus they may need further surgery for repair. In some situations the ventriculoscope may be used to open a new pathway to bypass the block and thus treat the hydrocephalus without a shunt.

Pediatric spinal cord tumor

Pediatric spinal cord tumor - A spinal tumor may be caused by the growth of abnormal cancer cells that have traveled to the spinal cord (metastasis). It could also be caused by rapid, uncontrolled division (proliferation) of normal cells of the area.

Spinal tumors are abnormal growths. They may occur in the spinal cord (intramedullary), within the meninges (membranes) covering the spinal cord (extramedullary, intradural), between the meninges and the bones of the spine (extradural) or may overlap in other locations. Most spinal tumors are extradural. They may be primary tumors originating in the spine, or secondary tumors that are the result of spread of cancer (metastasis) from other locations, primarily the lung, breast, prostate, kidney, or thyroid gland.

Any type of tumor may occur in the spine, including lymphoma, leukemic tumors, myelomas, and others. A small percentage of spinal tumors occur within the spinal cord, most often consisting of ependymomas and other gliomas. The cause of primary spinal tumors is unknown. Some primary spinal tumors are associated with congenital or hereditary tendencies. Spinal tumor is much less common than primary brain tumor.

The tumor affects the spinal-cord cells, nerve roots, meninges, blood vessels, or the bones of the spine. It causes symptoms because of compression of the spinal cord or nerve roots (similar to spinal cord trauma), invasion of normal cells by the tumor, or ischemia (lack of oxygen) that results from obstruction of blood vessels.

Courtesy National Institutes of Health

Pediatric tethered spinal cord

Tethered spinal cord syndrome is a neurological disorder caused by an abnormal stretching of the spinal cord. The course of the disorder is progressive. In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. Tethered spinal cord syndrome may go undiagnosed until adulthood, when symptoms such as sensory and motor problems and loss of bowel and bladder control emerge. This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time. Tethered spinal cord syndrome appears to be the result of improper development of the neural tube, and is closely linked with spina bifida.

Spina bifida

Spina bifida (SB) is a neural tube defect (a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings) caused by the failure of the fetus's spine to close properly during the first month of pregnancy. Infants born with SB

sometimes have an open lesion on their spine where significant damage to the nerves and spinal cord has occurred. Although the spinal opening can be surgically repaired shortly after birth, the nerve damage is permanent, resulting in varying degrees of paralysis of

the lower limbs. Even when there is no lesion present there may be improperly formed or missing vertebrae and accompanying nerve damage. In addition to physical and mobility difficulties, most individuals have some form of learning disability. The three most common

types of SB are: myelomeningocele, the severest form, in which the spinal cord and its protective covering (the meninges) protrude from an opening in the spine; meningocele in which the spinal cord develops normally but the meninges protrude from a spinal

opening; and occulta, the mildest form, in which one or more vertebrae are malformed and covered by a layer of skin. SB may also cause bowel and bladder complications, and many children with SB have hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain).

Stem Cells in Pediatric Brain Diseases

The rapid growth of cells in malignant tumors is suggestive of the self-renewal capacity of stem cells (cells that can self-renew and may become one of several types of cell). Neurosurgery researchers are investigating whether stem-like cells participate in brain tumor growth.

Preliminary findings show that stem-like cells, including phenotypic neural stem cells, can be found in several types of pediatric brain tumors. The oncogenic potential of these cells is being studied in animal models in collaboration with pioneering stem cell researcher Dr. Irving Weissman.

Dr. Huhn, in collaboration with the Palo Alto biotech firm StemCells, Inc., will be launching within in the next year the first clinical trial using neural stem cells for treatment of Batten's Disease, the most common form of a disorder called Neuronal Ceroid Lipofuscinosis (or NCL), which is marked by the accumulation of lipopigments in the body's tissues, and results in progressive loss of cognitive function, vision, and motor skills, and eventually death. This will be one of the first of its kind to explore treatment of metabolic diseases of the brain with neural stem cell transplantation.

Regional Pediatric Neurosurgery

We are expanding our Pediatric Neurosurgery program to advance our commitment to translational medicine, with the aim of developing and introducing innovations that will improve the treatment of brain diseases in children. In this effort we are proud to announce both the establishment of our program in Regional Pediatric Neurosurgery, and the hiring of its Director, Dr. Michael S.B. Edwards.