History

Michael J. Fritsch

3.1 Early History

Hydrocephalus, which is derived from the Greek words hydro (water) and cephalon (head, brain), is a condition that has been known for more than 2,000 years.

Hippocrates (ca. 460–370 BC) gave one of the earliest scientific descriptions of hydrocephalus. In the Corpus Hippocraticum, which is a collection of around 70 medical works from ancient Greece (probably written not only by Hippocrates but also by his followers and students), the term hydrocephalus was first used. The term referred to “fluid collection” in and around the brain. The disease was explained by liquefaction of the brain caused by epileptic seizures. The symptoms correlated to hydrocephalus were described as headaches, vomiting, and visual disturbances.¹

Galen of Pergamon (ca. 129–199) acquired anatomic knowledge by dissecting living (vivisection) and dead animals. The law at that time prohibited the dissection of human cadavers. Galen gave descriptions of ventricular anatomy, the choroid plexus, as well as of the cerebrospinal fluid (CSF) as watery clear liquid.¹ Galen also described the difference between motor and sensory nerves, the concept of muscle tone, and the concept of muscle agonists and antagonists.

Leonardo da Vinci (1452–1519) completed the first detailed drawing of the ventricular system in 1510. New discoveries in human anatomy and physiology became possible when dissection of the human body was tolerated and later legalized.¹ As an artist, da Vinci was given permission to dissect human bodies at the Hospital of Santa Maria Nuova in Florence, Italy. Based on these studies, Leonardo created illustrations of the human skeleton, muscles, heart, and vascular system, and a fetus in utero. Da Vinci’s illustrations depicted anatomical details in a then-unknown realism.

Andreas Vesalius (1514–1564), a Flemish anatomist and physician, was the author of one of the most influential books of his time, entitled De Humani Corporis Fabrica (On the Structure of the Human Body). Vesalius is often referred to as the founder of modern anatomy. He acquired his knowledge through the anatomical dissection of human bodies.

Vesalius gave the first scientific description of hydrocephalus based on clinical and anatomical data. He observed the disease in a 2-year-old girl with a growing head circumference. Vesalius examined the patient while she was still alive; after her death, he found that the head enlargement came from fluid collection inside the ventricles (9 lb [4.08 kg] of water) rather than from fluid accumulation around the brain. This opened the way for further thoughts and studies about CSF, its pathway, and the related disease, hydrocephalus.¹

Thomas Willis (1621–1675), an English anatomist, neurologist, and psychiatrist, is today best known for the “Circle of Willis.” He was the first to number the order of the cranial nerves in the way in which they are numbered today. Based on the studies of his coworker Richard Lower (1631–1691), who had shown that the cribriform plate is watertight and, therefore, cannot be the exit of CSF from the brain, Willis postulated that the circulation of CSF must occur within the brain. He described the communication of the surface of the brain (sulci, subarachnoid space) with the cavities beneath the fornix (lateral ventricles).

Antonio Pacchioni (1655–1726) was an Italian anatomist who described the arachnoid granulations that are now named after him. However, he assumed that they were sites of CSF secretion.¹

Claude-Nicolas Le Cat (1700–1768), a French surgeon, on October 15, 1744 introduced a specially invented cannula into the lateral ventricle of a newborn with hydrocephalus. The cannula was used as a tap and was left in place for 5 days until the child died. Le Cat had developed the device for the purpose of repeatedly draining extracranial CSF to treat congenital hydrocephalus. The procedure is considered to be the first use of a device for the repeated therapeutic removal of CSF from the ventricular system, inaugurating the concept of external ventricular drainage.^2,3

Robert Whytt (1714–1766) performed scientific clinical studies on patients, especially in children with suspected hydrocephalus. For example, Whytt described the difference in the clinical course depending on whether an infant had open or closed cranial sutures.

Francois Magendie (1783–1855) described the caudal opening of the fourth ventricle and postulated that an occlusion of the CSF pathway may cause hydrocephalus. Magendie inaugurated CSF pressure measurement by performing a suboccipital puncture in a dog in 1841.¹

3.2 Hydrocephalus and its Treatment in the 19th and 20th Centuries

Axel Hendrick Key (1832–1901) and Magnus Gustav Retzius (1842–1919) developed and proved a new modern concept of CSF physiology of their time, which, for the most part, is still valid today. They proposed that CSF is produced in the choroid plexus, flows through and out of the ventricular system, and is reabsorbed through the subarachnoid villi.¹

Emil Theodor Kocher (1841–1917) was a Swiss physician and is best known for his work in thyroid surgery. He received the Nobel Prize for Physiology and Medicine in 1909. A number of instruments and surgical approaches are named after him; one of these is the frontal precoronal bur-hole approach for ventricular puncture.

Heinrich Irenaeus Quincke (1842–1922) was a German internist and surgeon. He worked at the universities of Vienna, Berlin (Charité), Bern, and Kiel. His main contribution to today’s understanding of hydrocephalus was the lumbar puncture (at the time, it was called “Quincke puncture”), which he performed for diagnostic and therapeutic purposes. His main interest was the diagnosis and treatment of meningitis and multiple sclerosis. In 1893, he described a condition of “increased intracranial pressure,” a disease he called “meningitis serosa,” which is now referred to as pseudotumor cerebri.

Carl Wernicke (1848–1905) was a German psychiatrist and neuropathologist. In 1873, he examined a patient who had experienced a stroke. Although the man was able to speak and his hearing was unimpaired, he could not understand what was said to him. After the patient died, Wernicke dissected his brain and found a lesion in the left temporal parietal region. Wernicke concluded that this region was an important part of speech comprehension and named the syndrome “sensory aphasia.”

With regard to hydrocephalus, Wernicke is known for performing punctures of the ventricular trigone by using a trocar from a lateral approach, under aseptic conditions.⁴ Until recently, the trigonal approach had certain applications and was mainly used by pediatric surgeons and neurosurgeons for shunt placement in infant hydrocephalus.

Jan Mikulicz-Radecki (1850–1905) was a Polish surgeon who worked in Krakau, Königsberg, and Breslau. Mikulicz contributed mainly to the field of abdominal surgery. In 1893, he implanted a wick made of glass wool into the lateral ventricle of an infant aged 6 months; the wick extended into the subarachnoid and subgaleal spaces. This is considered to be the first permanently implanted CSF drainage. The clinical course of the patient was documented for the following 2 years and displayed a long-lasting reduction of head circumference.¹

Erwin Payr

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