The Evolution of Cerebrospinal Fluid Shunts: Advances in Technology and Technique.

Over the past few centuries, there have been many advances in shunt technology, from the evolution of shunt tubing materials, to advances in valves for regulating the flow of cerebrospinal fluid (CSF). Additional medical advances have enabled the antibiotic impregnation of catheters. Finally, advances in technology have expanded the options for minimally invasive techniques and improved the management of complicated cases of hydrocephalus. The evolution of technology and technique in the management of hydrocephalus with CSF shunts will be discussed here.

Robert H. Pudenz (1911-1998) and Ventriculoatrial Shunt: Historical Perspective.

Robert H. Pudenz was a renowned neurosurgeon in North America in the 20th century, famous for his contributions in the evolution of the shunt valve and ventriculoatrial shunt surgery. With his innovative idea and help from Heyer, in 1955, he demonstrated that a venous catheter worked best when in the right atrium and that the slit valve should be located at the most distal portion of the shunt system to prevent retrograde filling and thrombosis. He also contributed to various experimental studies on the brain, especially the electrical response of different neural structures. This historical vignette focuses on the work of Robert Pudenz and the evolution of the ventriculoatrial shunt.

Is there an ideal shunt? A panoramic view of 110 years in CSF diversions and shunt systems used for the treatment of hydrocephalus: from historical events to current trends.

OBJECT: The goal of this study is to evaluate whether an "ideal shunt" exists. METHODS: This is a retrospective analysis based on original papers in the field of Hydrocephalus and Shunts. Patients of all age groups, who had hydrocephalus, and underwent some form of CSF diversion were included. The study has been divided into four stages: from 1900 to 1949, 1950 to 1974, 1975 to 1999, and from 2000 to 2010. RESULTS: In stage 1 (historical era): Saphenous vein grafts, rubber conduits, and other materials were used in CSF diversions. In 1949, the first implantable shunt tube was developed by Nulsen. In stage 2 (experimental stage): the Holter valve was developed. Newer innovations were developed in relation to the ventriculo-atrial shunt, which was the preferred CSF diversion. In stage 3 (developmental stage), a large number of different design shunt systems were developed, with the aim of reducing complications. The ventriculo-peritoneal shunt had become the preferred CSF diversion. Also, the programmable valve was born. In stage 4 (era of programmable valve, there is a preference for the use of programmable shunt systems. However, shunt failure rate at 1 year being around 25 to 40%, and shunt survival at 1 and 2 years are 50-70 and 47-53% in most series. CONCLUSION: Every shunt is an ideal shunt provided the choice of the shunt used should be made by the matching performance of the shunt system to the altered profile of CSF dynamics of a given patient. The most important factor being the opening pressure.

The truth and coherence behind the concept of overdrainage of cerebrospinal fluid in hydrocephalic patients.

INTRODUCTION: Overdrainage, siphoning, and slit-ventricle syndrome are well-documented complications of shunting in hydrocephalic patients. Despite the prevalence of these conditions, their mechanisms are still not fully understood. In this paper, the authors trace the concept of overdrainage and the related phenomena of siphoning and slit-ventricle syndrome. PURPOSE: To provide a historical overview of overdrainage and to reignite discussion of a topic that has been settled. METHODS AND RESULTS: A medical literature search and review were performed via Google Scholar. Of 565 publications, 3 primary papers were identified and a timeline was developed demonstrating the convergence of the aforementioned concepts. From the primary papers, 25 relevant publications were selected and further analyzed searching for hypothesis, evidence, and conclusions. CONCLUSION: Overdrainage, siphoning, and slit-ventricle syndrome are associated concepts that have converged into a pathophysiological theory where siphoning of CSF leads to overdrainage, which is then hypothesized to cause slit-ventricle syndrome in a small subset of patients. Our data suggests that while there have been numerous reports regarding overdrainage and its consequences, the evidence is not as robust as currently presumed and this subject requires prospective exploration.

The history of external ventricular drainage.

External ventricular drainage (EVD) is one of the most commonly performed neurosurgical procedures. It was first performed as early as 1744 by Claude-Nicholas Le Cat. Since then, there have been numerous changes in technique, materials used, indications for the procedure, and safety. The history of EVD is best appreciated in 4 eras of progress: development of the technique (1850-1908), technological advancements (1927-1950), expansion of indications (1960-1995), and accuracy, training, and infection control (1995-present). While EVD was first attempted in the 18th century, it was not until 1890 that the first thorough report of EVD technique and outcomes was published by William Williams Keen. He was followed by H. Tillmanns, who described the technique that would be used for many years. Following this, many improvements were made to the EVD apparatus itself, including the addition of manometry by Adson and Lillie in 1927, and continued experimentation in cannulation/drainage materials. Technological advancements allowed a great expansion of indications for EVD, sparked by Nils Lundberg, who published a thorough analysis of the use of intracranial pressure (ICP) monitoring in patients with brain tumors in 1960. This led to the application of EVD and ICP monitoring in subarachnoid hemorrhage, Reye syndrome, and traumatic brain injury. Recent research in EVD has focused on improving the overall safety of the procedure, which has included the development of guidance-based systems, virtual reality simulators for trainees, and antibiotic-impregnated catheters.

Harvey Cushing's early management of hydrocephalus: an historical picture of the conundrum of hydrocephalus until modern shunts after WWII.

PURPOSE: Throughout his early career, Cushing proposed a variety of methods for temporary and permanent drainage and diversion of CSF in his patients, and acknowledged that certain techniques were more suited to particular subsets of hydrocephalus. METHODS: Following IRB approval, and through the courtesy of the Alan Mason Chesney Archives, the surgical records of the Johns Hopkins Hospital, from 1896 to 1912, were reviewed. Patients operated upon by Harvey Cushing were selected for further analysis. Within this cohort, we recovered all available records for a single patient with hydrocephalus and spina bifida, who was treated with a ventriculosubgaleal shunt prior to repair of the spina bifida. RESULTS: A 3 month-old infant presented with hydrocephalus associated with spina bifida. Cushing performed serial lumbar and ventricular punctures. Following this, Cushing took the patient to the operating room for placement of a ventriculosubgaleal shunt. The patient subsequently underwent excision of the myelomeningocele sac, with post-operative mortality due to unspecified causes. CONCLUSIONS: Cushing's publications document a preference for translumbar-peritoneal drainage in patients with congenital hydrocephalus, particularly those with spina bifida. Although the placement of ventriculosubgaleal shunts has become an accepted practice for contemporary neurosurgeons, this case illustrates the challenges that early neurosurgeons faced in developing operative approaches for the treatment of congenital hydrocephalus.

Theories of cerebrospinal fluid dynamics and hydrocephalus: historical trend.

According to the CSF bulk flow theory, hydrocephalus is caused by an imbalance between CSF formation and absorption, or a block at various locations in the major CSF pathway. New theories, however, have been proposed in which minor CSF pathways may play a significant role in the development of congenital hydrocephalus. The authors review major contributions to the literature and analyze the evolution of theories of CSF dynamics in relation to hydrocephalus, dividing their development into 4 stages on the basis of historical trends. In Stage I (prior to 1950), 2 systems of classifying hydrocephalus were proposed, namely Dandy's classifications of communicating and noncommunicating hydrocephalus and Russell's nonobstructive and obstructive hydrocephalus. In Stage II (1950-1974), based on these theories of major CSF pathway dynamics, treatment focused on ventriculostomy as an alternative to reduction of CSF production by choroid plexus coagulation. In Stage III (1975-1999), some of the specific forms of hydrocephalus, especially in premature infants, were found to be unsuitable for ventriculostomy. In Stage IV (2000-2008), selection of treatment modalities evolved further, with a focus on analysis of the chronological changes in CSF dynamics and the differences in absorption pathways in the developing and mature brains. The authors focus on "minor pathway hydrocephalus" in the immature brain, differentiating it from the conventional classification of obstructive and nonobstructive "major pathway hydrocephalus."

Marvelous medicine: the untold story of the Wade-Dahl-Till valve.

On December 5, 1960, 4-month-old Theo Dahl, the only son of best-selling author Roald Dahl (1916-1990), had his skull shattered in a horrific traffic accident. What began as a personal tragedy for the Dahl family would soon evolve into a dogged crusade by Dahl to expand upon preexisting valve technology with the goal of developing a shunt that would not become obstructed. Based upon exclusive access to private archives of the Dahl estate, as well as interviews with those involved, this article tells the intricate tale of one famous father's drive to significantly alter the natural history of pediatric hydrocephalus. Dahl's collaboration with British toymaker Stanley Wade and pioneering pediatric neurosurgeons Joseph Ransohoff, Kenneth Shulman, and Kenneth Till to create the Wade-Dahl-Till (WDT) valve is examined in detail. The ensuing rift between the American and British contingents, the valve's multiple design revisions, and the goal of creating an affordable shunt for children in developing countries are among the issues addressed. The development of the WDT valve marked a significant turning point in the surgical management of pediatric hydrocephalus in general and in shunt valve technology in particular.

Overview of the CSF dual outflow system.

It was firmly established in the mid-twentieth century that the arachnoid villi represented an open pathway between the subarachnoid space and the dural venous sinuses. Intracellular and extracellular pathways within the villous structure provided the conduit for cerebrospinal fluid (CSF) and particulate matter. The importance of the lymphatic system was established by the demonstration of CSF tracers entering the nasal lymphatic system via the perineural subarachnoid space enveloping the olfactory nerve rootlets. It appears that because of the late development of the arachnoid villus (AV) system, the lymphatic outflow system is the more dominant one in the young animal, but in the mature animal the importance of both systems appears equal. In general, the lymphatic system in lower animals appears dominant, but in the case of primates, this may not be the case. The global outflow system has a definite opening pressure of ca. 50-70 mm of water, and the balance between production of CSF and absorption occurs at a resting pressure of ca. 115 mm water. The bicompartmental CSF outflow curves obtained from hydrocephalic patients support the presence of a dual outflow system utilized in normal CSF drainage.

Harvey Cushing's experience with treating childhood hydrocephalus: in his own words.

PURPOSE: As the founder of modern neurosurgery, Harvey Cushing is known for his innovations and treatment for many neurosurgical disorders. However, few are aware that Cushing treated childhood hydrocephalus. Examples of these treatments include the placement of ventriculo-subgaleal shunts, ventriculo-jugular shunts, ventriculo-superior sagittal sinus shunts, and at least 12 lumbar cistern-to-retroperitoneal shunts that, as he put it, had "a considerable measure of success." The authors wish to provide insight into Cushing's treatment of childhood hydrocephalus. METHODS: The authors review Cushing's writings on this topic. RESULTS: The authors provide a window into this pioneer's early thinking on the physiology and production of cerebrospinal fluid. CONCLUSIONS: Although decades went by before reliable treatments for hydrocephalus were established, Cushing pioneered both the thought processes and surgical options for this disease.

Salomón Hakim and the discovery of normal-pressure hydrocephalus.

INTRODUCTION: Normal-pressure hydrocephalus (NPH) is a chronic neurological disorder characterized by enlarged ventricles and a triad of clinical symptoms affecting gait, cognition, and urinary continence. Salomón Hakim first identified the syndrome in 1957 at the Hospital San Juan de Dios in Bogotá, Colombia. Even after decades of international focus and thousands of publications on his disorder, Hakim's story remains largely untold. METHODS: In this historical review, we explore the discovery of NPH through a series of personal interviews with Professor Hakim and his family, discussions with former colleagues, and review of the relevant medical literature. RESULTS: Professor Hakim first published his thesis in 1964 and 6 case reports of NPH in The New England Journal of Medicine and the Journal of the Neurological Sciences in 1965. Hakim rose to the forefront of academic medicine as he described a newfound ability to reverse symptoms of "neurodegeneration" that had long been considered irreversible. CONCLUSIONS: As we learn more about NPH, the fascinating story of Professor Hakim, the father of NPH, is of both historical relevance and current interest.

Harvey Cushing's use of a transplanted human vein to treat hydrocephalus in an infant in the early 1900s. Historical vignette.

A review of the Johns Hopkins Hospital surgical records from 1896 to 1912 revealed a case from 1908 wherein Dr. Harvey Cushing attempted to treat hydrocephalus in a 4-month-old infant by constructing a shunt for which he used a venous segment harvested from the patient's father. Prior to this procedure, surgeons used shunts constructed from various often highly immunogenic materials. In addition to addressing the limitations of these materials, Cushing's technique allowed the inclusion of valves within the shunt, preventing the retrograde flow of CSF. Despite the success of this procedure in canine models, the child's postoperative death prevented an assessment of its success in a human. It is possible that Cushing's approach would meet with more success today, given the modern benefits of human leukocyte antigen tissue typing and immunosuppressant agents.

Father of neurosurgery in Hong Kong.

Neurosurgery in Hong Kong had its origins as a division of General Surgery and became a subspecialty only 46 years ago with the arrival of Hsiang-Lai Wen. For well over a decade, Wen would be the only neurosurgeon in the colony. His contributions to neurosurgery included the ventriculosuperior sagittal sinus shunt and the application of acupuncture in anesthesia, pain ablation, and drug detoxification. A pilot with the China National Aviation Corporation during World War II, he played an active part in the Allied war effort. As a diplomate of the American Board of Neurological Surgery, Wen sought to improve the standard of neurosurgery in Hong Kong and southern China with the establishment of the Hong Kong Neurosurgical Society in 1981 and the Research Institute of Neurosciences in Guangzhou in 1988. Wen was acknowledged as Hong Kong's "father of neurosurgery," and his work paved the way for the development of modern neurosurgery in the region.

The first description of a device for repeated external ventricular drainage in the treatment of congenital hydrocephalus, invented in 1744 by Claude-Nicolas Le Cat.

An 18th century report of a device for repeated extracranial drainage of cerebrospinal fluid in the treatment of congenital hydrocephalus is reviewed. On 15th October 1744, the French surgeon Claude-Nicolas Le Cat (1700-1768) introduced a specially invented canula into the lateral ventricle of a newborn boy with hydrocephalus. The canula was used as a tap and was left in place for 5 days, until the death of the child. This procedure should be seen as the first documented description of a device for repeated ventricular taps in the treatment of hydrocephalus.

Development of the Spitz-Holter valve in Philadelphia.

The history of the treatment for hydrocephalus dates back to the Fertile Crescent thousands of years ago. Despite three millennia of management, significant advances in the surgical treatment of the disease have been infrequent. During the 1950s, a milestone occurred at the Children's Hospital of Philadelphia, with the successful development of the first working shunt valve for the treatment of hydrocephalus. In this historical vignette, based on recent interviews with John Holter, D.Sc. (Hon) and Eugene Spitz. M.D., and on a review of the available literature, the authors narrate the exciting story of the development of the Spitz-Holter valve, which took place in Philadelphia during the early 1950s.