Necessity Spurs Invention

MUSC Children’s Health offers a new shuntless technique, developed in Africa, to address hydrocephalus

By Vitria Adisetiyo

Since arriving at MUSC Children’s Health in 2014, pediatric neurosurgeon Ramin Eskandari, M.D., has been offering a new shuntless procedure for treating hydrocephalus — endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) — and training residents and neurosurgery colleagues on the technique.

As chief resident at the University of Utah, Eskandari was one of the first residents in the U.S. to travel to Uganda, where the technique was developed, for training. While a fellow at Stanford University, he trained his colleagues and mentors to perform the procedure.

Traveling to a developing country for advanced medical training might seem counterintuitive, but necessity spurs innovation, and the urgent need to address the high shunt infection rates in Africa led to the development of the novel ETV/CPC technique that is now being adopted in more developed countries.

Hydrocephalus is a common disorder in which build-up of cerebrospinal fluid (CSF) in brain ventricles creates harmful pressure on neural tissue. If untreated, hydrocephalus leads to abnormal brain, cognitive and physical development, and eventually death. Traditionally, a ventriculoperitoneal shunt is implanted to drain excess CSF from the brain to a peripheral part of the body, where it can be readily absorbed. Since the 1960s, this approach has proven to be an effective treatment, albeit an imperfect one that requires life-long maintenance. Patients face high infection rates from the hardware implant along with a high likelihood of reoperation due to shunt malfunction.

In Uganda, as in other sub-Saharan countries, hydrocephalus is widespread due to high rates of post-meningitis and post-ventriculitis infections. Despite shunt treatment, the survival rate for hydrocephalus has been abysmal because of high infection rates and limited access to medical care.

In 2000, Benjamin C. Warf, M.D., an American neurosurgeon working in Uganda, began to look for a solution. The advent of the flexible endoscope led Warf to revisit an old surgical technique wherein a hole is created in the floor of the third ventricle to reroute excess CSF followed by shrinkage of the choroid plexus to temporarily reduce CSF production. This approach allows the brain to gradually reabsorb the extra CSF and establish a new equilibrium. While historically the technique was performed as an open brain surgery, Warf advanced the method into a minimally invasive procedure by conducting the surgery with a flexible endoscope through a small hole in the skull.

Warf published outcomes for hundreds of patients treated with ETV, demonstrating that the procedure was effective and that, when combined with CPC, it could be used successfully even in children younger than one year.¹ Moreover, he showed that, even if the ETV/CPC procedure fails, the shunt option remains available,² whereas ETV/CPC care can rarely be done after a shunt is placed. Today, the ETV/CPC method is the mainstay for hydrocephalus treatment in sub-Saharan Africa.

As word spread, more neurosurgeons in developed countries sought training for the ETV/CPC procedure. Capitalizing on sophisticated neuroimaging, neuropsychological, and big-data technology available in developed countries, a multi-center Hydrocephalus Clinical Research Network (HCRN) has been established to rigorously study ETV/CPC treatment outcomes.

“In the developed world, we are well beyond mere survival as a benchmark of success,” says Eskandari. “Families are rightly demanding that medicine and science strive to produce durable treatments for their children that allow them not just to survive but also to thrive in terms of their physical and cognitive abilities.”

Collective data thus far suggest the ideal patients for the procedure are those with an anatomical obstruction that prevents correct CSF flow and those without a history of infection or post-hemorrhagic hydrocephalus. However, in infants aged three to nine months, even those with post-hemorrhagic hydrocephalus have a 25 to 45 percent chance of success. Patients who lack CSF absorption capacity, such as premature babies, do better with shunt treatment.³

As for the future of hydrocephalus treatment at MUSC Children’s Health, Eskandari’s goal is to establish a large pediatric center for hydrocephalus and to propose a study to the HCRN, for which the center would serve as a site.

“Long-term data are needed to truly establish the value of the technique. If we can show that the outcome of the ETV/CPC surgery is the same as shunting at age five, age ten, and during the college years, then that really supports continuing to perform this procedure in this patient population,” says Eskandari. “I would love to see us continue down this evidence-based path because it really is the best care we can offer our patients.”

To watch a video of Dr. Eskandari discussing the technique and narrating surgical footage from a case, visit the MUSC Health Medical Video Center and select pediatrics from the dropdown menu of specialties.

References

¹ Warf BC. J Neurosurg. 2005 Jan;102(1 Suppl):1-15.

² Warf BC, et al. J Neurosurg Pediatr. 2012 Dec;10(6):463-470.

³ Kulkarni AV, et al, for the Hydrocephalus Clinical Research Network. J Neurosurg Pediatr. 2016 Oct;18(4):423-429.