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.