The scientific history of hydrocephalus and its treatment

Hydrocephalus cases were regularly described by Hippocrates, Galen, and early and medieval Arabian physicians, who believed that this disease was caused by an extracerebral accumulation of water. Operative procedures used in ancient times are neither proven by skull findings today nor clearly reported in the literature. Evacuation of superficial intracranial fluid in hydrocephalic children was first described in detail in the tenth century by Abulkassim Al Zahrawi. In 1744, LeCat published findings on a ventricular puncture. Effective therapy required aseptic surgery as well as pathophysiological knowledge--both unavailable before the late nineteenth century. In 1881, a few years after the landmark study of Key and Retzius, Wernicke inaugurated sterile ventricular puncture and external CSF drainage. These were followed in 1891 by serial lumbar punctures (Quincke) and, in 1893, by the first permanent ventriculo-subarachnoid-subgaleal shunt (Mikulicz), which was simultaneously a ventriculostomy and a drainage into an extrathecal low pressure compartment. Between 1898 and 1925, lumboperitoneal, and ventriculoperitoneal, -venous, -pleural, and -ureteral shunts were invented, but these had a high failure rate due to insufficient implant materials in most cases. Ventriculostomy without implants (Anton 1908), with implants, and plexus coagulation initially had a very high operative mortality and were seldom successful in the long term, but gradually improved over the next decades. In 1949, Nulsen and Spitz implanted a shunt successfully into the caval vein with a ball valve. Between 1955 and 1960, four independent groups invented distal slit, proximal slit, and diaphragm valves almost simultaneously. Around 1960, the combined invention of artificial valves and silicone led to a worldwide therapeutic breakthrough. After the first generation of simple differential pressure valves, which are unable to drain physiologically in all body positions, a second generation of adjustable, autoregulating, antisiphon, and gravitational valves was developed, but their use is limited due to economical restrictions and still unsolved technical problems. At the moment, at least 127 different designs are available, with historical models and prototypes bringing the number to 190 valves, but most of these are only clones. In the 1990s, there has been a renaissance of endoscopic ventriculostomy, which is widely accepted as the method of first choice in adult patients with aquired or late-onset, occlusive hydrocephalus; in other cases the preference remains controversial. Both new methods, the second generation of valves as well as ventriculostomy, show massive deficits in evaluation. There is only one randomized study and no long-term evaluation.