INTRODUCTION


Hydrocephalus

Hydrocephalus, which, in Greek, translates as water (hydro) and head (cephalus), is an abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles (cavities) in the brain. CSF is produced in the ventricles, circulates through the ventricular system and is then absorbed into the bloodstream. CSF protects the brain and spinal cord, contains nutrients and proteins that nourish the brain, and carries away waste. Hydrocephalus occurs when there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed. As the CSF builds up, it causes the ventricles to enlarge and increases pressure in the head. It occurs in about 2 out of 1,000 births. The incidence of adult-onset hydrocephalus is not known. Hydrocephalus that is congenital (present at birth) is caused by a complex interaction of environmental and genetic factors, such as spina bifida. Acquired hydrocephalus may be caused by intraventricular hemorrhage, meningitis, head trauma, tumors or cysts.

The most effective and common treatment for hydrocephalus is surgical insertion of a shunt, although an endoscopic third ventriculostomy (ETV) is also an increasingly common treatment. A shunt is a flexible tube that diverts the flow of CSF from the ventricular system into another region of the body, such as the peritoneal (abdominal) cavity, where it can be absorbed. The surgical placement of a shunt is a relatively short and uncomplicated procedure. Endoscopic third ventriculostomy (ETV) surgery involves making a hole in the floor of the third ventricle to allow CSF fluid to flow into the basal cisterns where it can be absorbed without putting increased pressure on the brain.

Myelomeningocele (Spina Bifida)

The spinal cord injury associated with the myelomeningocele can result in paraplegia, bowel and bladder incontinence, hydrocephalus and sexual dysfunction. Our pediatric neurosurgeon participates in the NIH-sponsored clinical trials to assess the role of intrauterine closure of myelomeningocele defect in maintaining neurological function, correction of Chiari II malformation and prevention of hydrocephalus. 

Chiari Malformation (Arnold Chiari Malformation)

Also known as Arnold Chiari Malformation, a Chiari Malformation is a benign structural problem affecting the cerebellum and is relatively common. Most children with these malformations who do not have spina bifida will have the form known as type I, which usually has less severe symptoms than type II. Their cerebellum blocks or restricts the outlet of the brainstem/spinal cord from the skull on its way to the spinal canal. This blockage or restriction usually leads to headaches, neck pain, funny feelings in the arms and/or legs, stiffness, and, sometimes, will cause difficulties with swallowing or gagging. A MRI is usually used to diagnose the problem. Chairi malformations are extremely difficult to see on CT scans and impossible to see on plain x-rays. Sometimes these malformations can be made worse by, or can cause hydrocephalus. In addition they can often lead to fluid filled cavities in the spinal cord known as syrinxes (syringohydromyelia). Untreated, the chronic crowding of the brainstem and spinal cord can lead to serious consequences including paralysis.

There are many ways to treat Chiari malformations, but all require surgery. The basic operation is one of unblocking the area at the base of the cerebellum where it is pushing against the brainstem and spinal cord. This is done by removing a small portion of bone at the base of the skull and, sometimes, part of the first and occasionally additional spinal column segments. Most children who have the surgery do quite well and have a significant improvement in their symptoms.

Epilepsy

Epilepsy is an intermittent derangement of the nervous system due to a sudden, excessive, disorderly electrical discharge of cerebral neurons. More than two-thirds of all epilepsy begins in childhood, when seizures can have drastic and devastating results on the child's psyche and development. Surgical treatment is highly effective in the treatment of many of the epilepsies that have not responded to medical therapy. Nearly 40 percent of children with partial epilepsy are candidates for surgical therapy.