HISTORY

John Holters Shunt

Joshua S Baru, BA, David A Bloom, MD, FACS, Karin Muraszko, MD, C Everett Koop, MD, FACS
Although healers had been aware of the nature of hydrocephalus since the earliest days of recorded medical practice, effective therapy was lacking until the mid-20th century. In 1956 a son, Casey, was born to John and Mary Holter. The infant had lumbar myelodysplasia, and hydrocephalus developed in infancy. John, an enterprising machinist, was astonished and challenged by the inability of medical technology to correct what appeared to be a simple hydraulic problem and in his workshop at home he created the first dependable and effective ventricular shunt. Holter went on to devote his life to biomedical technology. The original valve design is still in wide use. Hydrocephalus has challenged healers since the earliest days of recorded medical practice when Hippocrates described cerebral ventricular puncture.1 Since then physicians have attempted ingenious techniques for relieving or counteracting the rises in pressure that cause hydrocephalus and its damaging effects. Hydrocephalus is present in approximately 1 to 3 per 1,000 births and most commonly occurs with myelodysplasia and Arnold Chiari malformation. Hydrocephalus is characterized by an accumulation of cerebrospinal fluid (CSF) in the ventricles of the brain. Dilation of the ventricles compresses the brain, replacing parenchymal volume with CSF. Obstruction of the CSF pathway is the most common cause of hydrocephalus. Although the pathophysiology of hydrocephalus has been understood since the late 19th century,2 effective treatment was limited by surgical and material technology until 1956, when John Holter, a machinist, developed an effective cerebrospinal shunt for his hydrocephalic son.
Hydrocephalus and its therapy

Infants with hydrocephalus, from antiquity until recently, had a hopeless prognosis and usually died
No competing interests declared.

This work was supported by the Babcock Foundation of the Section of Urology, Department of Surgery, University of Michigan. Received May 18, 2000; Revised June 12, 2000; Accepted June 29, 2000. From the Department of Surgery, The University of Michigan Medical Center, Ann Arbor, MI. Correspondence address: David A Bloom, MD, 1500 East Medical Center Dr, Ann Arbor, MI 48109-0330.

quickly. As anatomic knowledge of the brain and the cerebrospinal pathway accrued in the past few centuries, the pathophysiology of hydrocephalus became better understood and therapeutic efforts expanded modestly, although most infants were still left to the grim natural history of the disease. Head wraps and other measures attempted to counteract the expansion of the skull. At the start of the 20th century, with developing pediatric expertise and the increasing sophistication of surgical techniques, physicians applied new tools to hydrocephalus.3,4 In the 1910s, Walter Dandy and KD Blackfan5 at the Johns Hopkins Hospital in Baltimore induced hydrocephalus in dogs by placing cotton-filled gelatin capsules in the aqueduct of Sylvius to block the drainage of CSF from the third and lateral ventricles. They then injected dyes into the ventricles. If the dye did not make its way to the spinal cord in 30 minutes they inferred that blockage was complete and the hydrocephalus was termed obstructive or noncommunicative. If the blockage was partial and some dye reached the spinal cord, the condition was termed communicative. Severity of the blockage was judged by the rate of dye migration.6 Although Dandy and Blackfans experiments implied that drainage techniques might bypass the blockages, VD Lespinasse, a urologist in Chicago, attempted an alternate approach in 1910. He applied his endoscopic skills to hydrocephalus, cauterizing the choroid plexus of two hydrocephalic patients through a cystoscope in an attempt to minimize CSF production.7,8 Dandy in 19189 tried to excise the choroid plexus. Through two bone flaps at the top of the skull over the parietal eminence he incised the cortex, exposed the lateral ventricles, and excised the choroid plexus. Dandys technique had a high mortality rate, did not arrest the production of CSF or the increase in intracranial pressure (ICP), and was successful only in a few patients. Later cauterizing the choroid plexus, he was only a little more successful and he eventually abandoned both methods.10 By the mid-20th century therapeutic methods for hydrocephalus remained unsatisfactory. Ingraham and coworkers11 wrote:

2001 by the American College of Surgeons Published by Elsevier Science Inc.

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The treatment of hydrocephalus in infancy remains one of the most unsatisfactory and discouraging problems the neurological surgeon is called upon to treat. Penfields statement made in 1935 that all surgeons who continue to face the hydrocephalus problem require the support of fortified optimism, is just as pertinent today.

decompression by ventricular-venous shunts will fail and should not be submitted to clinical trial.

The laboratory at the Childrens Hospital in Boston responded to these frustrations in 1947 when Donald Matson and Eben Alexander developed a reproducible hydrocephalic canine model with cellophane in the CSF pathway.12 This allowed systematic, controlled studies of various techniques that, until then, had only existed in theory or been tested sporadically on patients. The laboratory investigated various shunting destinations by inserting biocompatible polyethylene tubing between the subarachnoid spaces and various low-pressure spaces capable of absorbing or excreting the excess CSF. These spaces included the sagittal sinus, the vena cava, and the veins of the neck (external jugular and maxillary). This concept had been considered as early as 1896, when Gartner suggested diversion of CSF from the dysfunctional CSF pathway and into the venous and lymphatic systems.4 Ventricularvenous shunts fulfilled the basic requirements for effective treatment, provided significant drainage of CSF in control conditions, were technically feasible, and approximated normal human physiology. These short shunts also minimized the influence of the patients growth and required fewer subsequent operations for adjustment. Ingrahams team revealed the substantial downside of venous shunts: vigorous exertion, such as barking in the dogs, elevated venous pressure and caused reflux of blood into the shunt. If the blood did not clear quickly enough it clotted, clogged the tube, and permitted ventricular pressure to rise. Vannevar Bush, a physicist at the Massachusetts Institute of Technology, worked with the Ingraham laboratory to develop a magnetic valve in 1946.1,4 Used in patients, it apparently was not successful enough to warrant a published report or widespread use. In 1948 the Ingraham group11 concluded:
. . .unless an extremely sensitive but completely competent artificial valve can be devised and introduced between the cerebrospinal fluid pathways and the venous system in such fashion as to permit only unidirectional flow of fluid no matter what the relative hydrostatic pressures may be, all attempts to achieve ventricular

In 1952 Frank Nulsen and Eugene Spitz,13 residents in neurosurgery at the Hospital of the University of Pennsylvania, reported clinical experience with a valve. This ball and spring valve was made by the Johnson Foundation, a subsidiary of Johnson & Johnson. It was placed in the internal jugular with the tip protruding slightly into the atrium of the heart and it was equipped with a pump that could be digitally manipulated to flush the tube, but the valve was inconsistent and clotted periodically. Donald Matson at Duke University in 194810 developed a procedure that obviated the problematic clotting. He performed a lumbarureteral shunt on an 8-year-old girl with hydrocephalus secondary to a head injury and meningitis, removing a kidney and placing a polyethylene tube from the lumbar subarachnoid space to the ureter. The technique worked well and remained popular until the development of a better valve. Ureteral drainage was useful only for communicating hydrocephalus and it carried added risk in female patients because short urethras increased the likelihood of bladder infection and, subsequently, meningitis. Matson reported on the state of treatment of infantile hydrocephalus in 1956,13 commenting: weary of many disappointments, I am still optimistic enough to believe that the next decade will see considerably more progress in this field than has occurred in the last. His optimism was rewarded.15
John Holter

John Holter was born in 1916 to Favian Erskine and Charles Robert Holter. Growing up in Woodbury, NJ during the Depression, without much money for toys, John became a gadgeteer, spending much of his time, energy, and money building and repairing various devices. After graduating from high school in 1934, Holter worked as a mechanic in garages and auto dealerships in New Jersey, doing anything [he] could to learn, and be involved in the automotive world, (personal communication, 1998). In 1938, he applied for work at the Socony Vacuum and Oil Co in Paulsborough, NJ, and began an apprenticeship to be an automotive machinist. Holter said of his job testing fuels and oils (personal communication, 1998):
That was a wonderful opportunity because they gave me the opportunity to work in the machine shop, the electric shop, and the instrument shop. I learned a lot of

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basics on a lot of these things that the average kid does not have a chance to know. I was working with engineers. They were very helpful and, because I was so enthusiastic, they took the time to talk to me and work with me on a lot of these problems. I was trying to figure out why things happened. So I had a good indoctrination there.

Holters work at Socony was interrupted when he was drafted into a New Jersey Army National Guard unit in April 1941. After basic training he went to the Fort Knox Tracked Vehicle School. In August of 1942 he was sent to England and then North Africa. Eighteen months later he was mobilized to Sicily and southern Italy. He took part in the Cote dAzur invasion of southern France moving up into Alsace, Germany, and, finally, Austria at the wars end. He served as the crew chief of a 20-person maintenance group for a mechanized cavalry reconnaissance unit. According to him (personal communication, 1998), a lot of these kids (who I was working with) came in, all younger than I, but theyd been selling shoes or groceries and had no idea of mechanics. They were from the cities: Newark, Jersey City, New York. Most of my time I was teaching these fellas what and how to do the maintenance on the vehicles. After the war Holter rejoined the Socony Vacuum and Oil Research Laboratory. He remained there until 1950, leaving with the intent to buy a boatyard, but when the Korean War broke out the deal fell through. In 1951 he found a job as a technician at the Yale and Towne hydraulics research laboratory in King of Prussia, PA. His testing work at Yale and Towne gave him the skills and knowledge that would prove essential in his development of the CSF shunt. THE SHUNT Holter and his wife Mary waited seven years for a child. Charles Case Holter (Fig. 1) was born on November 7, 1955. Casey had a lumbo-sacral myelomeningocele about the size of a half a lemon, in addition to Arnold Chiari malformation. After Casey acquired meningitis in early December, Spitz and the team at Childrens Hospital of Philadelphia (CHOP) closed the myelomeningocele. Caseys head started to expand a few weeks later because of hydrocephalus. The Holters, living in Gulph Mills, PA, took their son back to Spitz, who tried to maintain normal ventricular pressures by tapping through the fontanel to remove CSF once or twice a day. Concerned about the risk of infection, Holter asked if

Figure 1. John and Mary Holter with their son Casey.

anything else could be done to treat the hydrocephalus and Spitz recommended the Johnson Foundations ball and spring valve. Holter recollected the unfortunate turn of events (personal communication, 1998):
It was a spring-loaded ball, a scaled-down model of the typical pressure relief valve that automobile engines have in them. It didnt work very well, and they also used a polyethylene distal catheter down into the heart. They told me that it would be a jugular shunt, but actually the thing went down into the atrium of the heart. Now, the tiny heart of an infant is precious small, and its a moving organ, and its very sensitive to any foreign body. [The shunt] caused an irritation, it probably touched the SA node, and it stopped his heart. His heart was [stopped] for 30 minutes. Of course it caused irreparable damage to the brain, a terrible insult to him. He wasnt in fit condition for further surgery for quite a while.

After this procedure Holter asked Spitz technical details of the problem, particularly the nature of the CSF fluid and the volumes and pressures concerned. Spitz replied that someone needed to develop a competent one-way valve that would be stable in the human body. Holter went home and constructed a model valve in his garage that night (Fig. 2). Holters approach was inspired by his observation of a nurse puncturing a clogged intravenous line to irrigate it. The puncture site closed spontaneously after the nurse removed the needle and Holter reasoned that a slitlike opening would have the same valvular effect, with minimal resistance to the ex-

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Figure 2. Holter in his garage, working on the shunt.

iting fluid. Holters prototype valve had two rubber condoms with slits in the top inserted at either end of a flexible piece of tubing. The valves acted like infant bottles: when pressure was applied the slit opened and fluid flowed out, but once the pressure was relieved the slit closed. Holter recalls the details (personal communication, 2000):
The prototype valve had two tiny condom-shaped sections with one longitudinal slit in each. They were arranged in tandem with a connecting tube that could be depressed with finger action to displace liquid contents downstream distally. Each compression displaced about 1/5 cc. Various slit lengths were made, five in all, to accommodate the various needs of patient and surgeon.

to legal ramifications that they wouldnt give me any information whatsoever. But, one of the rubber companies in Philadelphia referred me to their parent company, the Lee Rubber and Tire Co in Conshohocken, PA. The man at the head of the research laboratory said that he thought that he could help me. So, I went there and we discussed this problem of shrinkage of natural latex rubber. He said Why dont you try silicone?

Holters model looked promising to Spitz but was far too large and needed to be made of a biocompatible substance that would withstand the heat and pressures of an autoclave. Holter made several other versions of his slit valve (personal communication, 1999):
Latex and any number of synthetic vinyls did close, but when put through the autoclave, the material would shrink a little bit, and the slit would not close, which resulted in reflux. I spent about two days going through the phone book, calling every plastic and rubber company I could think of. Of course, they were so sensitive

Dow Chemical in Midland, MI, in 1955 had developed Silastic, a silicone derivative, but this was little more than a laboratory curiosity when Holter learned of its existence. An unusually stable, long-chain polymer, Silastic tolerated extreme temperatures and Holter had reason to believe that it would be stable in the body. He ran a series of tests on a sample of Silastic tubing and became confident that it would work. Three weeks after the conversation with Spitz, Holter had a valve that met all of the size, pressure, and chemical requirements. He made all of this on his own with equipment at the Yale and Towne research laboratory (the laboratory director permitted after-hours usage of the facilities) and tested the valve with an autoclave he had built on his kitchen stove. In March 1956, when Holter completed the valve, Casey was still too weak for another operation, so the first valve was successfully installed in a ventriculoatrial shunt in another child at CHOP. Casey recovered suffi-

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ciently by April and a Holter valve was implanted by Spitz at Lankenau Hospital in the suburbs of Philadelphia. Casey did well but never fully recovered from the brain damage caused by his previous cardiac arrest. Five years later, during one of his periodic seizures, Casey aspirated gastric fluid and died (J Holter, personal communication, 1998). After Holters shunt proved successful, he left Yale and Towne. He could make only a few shunts a week with the obsolete machinery that had been donated to him by Yale and Towne. Holter filed a patent October 2, 1956, describing his shunt as a Device for draining ventricular fluid in cases of hydrocephalus (Application No. 2,969,066; Serial No. 613,549; Fig. 3). A filing fee of $30 was submitted. The device was approved January 24, 1961, after a final fee of $30 was paid. No other government intervention or regulation occurred. Sales were limited because Spitz was less than generous to his colleagues and profession with Holters valve. Spitz did not publish his method and also demanded that any surgeon who wanted to use the shunt had to come to CHOP and personally be instructed in the technique. The first publication did not come until 1959 when Ken Carrington16 reported on 50 patients who received Holter shunts at the University of Michigan. The success rate was 86% and no patient had a problem with the valve mechanism. Considering the advantages of internal shunting and the success of the new valve, Carrington concluded: this type of shunt is probably the best available for all types of hydrocephalus.16 Holter recalls that this paper broke the ice and he felt free to send (the shunts) to any and everybody (personal communication, 1998). As demand increased, Holter hired two people to help production at home and established the Holter Company. After the house became too crowded, he rented a building in Bridgeport, PA. The Holter Company grew and at its peak employed 30 people. The company not only made shunts, but in time also built heart valves, artificial hearts, bilateral roller pumps for kidney dialysis, Hunter artificial tendons, and other biomedical devices. Ten years after its conception, Holter sold his company to Extra Corporeal Specialties in Bridgeport, PA, a prominent producer of artificial kidneys. Holter worked as a consultant for Extra Corporeal, but the company did not want to become involved with new valve developments. Johnson & Johnsons Codman division eventually acquired Extra Corporeal Specialties, which still makes the Holter valves. Holter continued to consult for Codman and devel-

Figure 3A. Patent title for the Holter shunt.

oped a new cruciform valve design. He collaborated with one of his former engineers, John Hausner, to found Holter-Hausner International and produce the new valve. Rather than a slit in the end of a rubber tube, this valve had a slit in the side. The new design was more compact and had a stronger closing force than the old one to prevent overdrainage. A revised model had a cruciform slit at the distal end and seemed to provide better ICP. Holter spent most of his time promoting the company and left the management to Hausner. The company grew, rendering Holter a minority shareholder. He recalls that he ended up with little input in the companys administration; his previous standard of 100% testing was decreased to 10%, and quality control deteriorated to such an extent that the federal government threatened to close the company. In 1986, facing a lawsuit and the possibility of having the business shut down, Holter closed the company, bought the assets, and formed Phoenix Bioengineering. Phoenix Bioengineering flourished and was bought by Charles Hokanson in 1993, who renamed the company Phoenix Biomedical. It continues to make the Holter cruciform valve today.17
In Conclusion

Although John Holters basic concepts culminated in a competent one-way valve, nuances of pressure regulation, growth, and surgical complications inspired additional refinement. Because of differences in intracranial pressures among patients, manufacturers soon began producing shunts that responded to different pressure

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Figure 3B. Shunt illustration.

levels, but pressures varied widely within the same patient. When a patient rose from a prone or sitting position to a standing position, gravity and simple hydrodynamics in devices resulted in increased cranial outflow

causing headaches, dizziness, and other complications. This required great experience in selection of valve types for each patient. Newer antisiphon mechanisms slow CSF flow during these periods of pressure change. The

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problems arising from patient growth and surgical complications were effectively solved by a simple change in procedure first publicly advocated by RH Ames in 1967.18 Ames reported a greater complication rate with ventriculoatrial shunts than ventriculoperitoneal shunts, the heart being a more problematic location for a prosthetic tube. Although long lengths of tubing can coil in the peritoneal cavity without complications, only the tip of the shunt can extend into the atrium. The ventriculoperitoneal shunt requires fewer revisions during growth, leaves more room for error in placement, and has replaced the ventriculoatrial shunt as the most popular method of treating hydrocephalus. Approximately 150,000 shunts are used annually worldwide, 40,000 of which are placed in the United States. Of these almost 15,000 use the slit and the cruciform valves developed by John Holter. Although specific procedures have changed and technology has advanced, the valve mechanisms developed by John Holter have stood the test of time.
Acknowledgment: We are grateful to John Holter and to Casey Holter, who inspired the shunt. John Holter provided comments and illustrations. Thanks also to Charles Hokanson of Phoenix Biomedical who put us in contact with Holter and provided information about CSF shunts in present-day medicine.

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