Hydrocephalus: Tackling a global health problem

Benjamin Warf, MD, director of Neonatal and Congenital Anomalies Neurosurgery at Children’s Hospital Boston, developed a new treatment for infant hydrocephalus, or “water on the brain,” while a medical missionary in Africa, where hydrocephalus is common and usually untreated. His innovation, which has saved the lives of thousands of children, is minimally invasive, relatively inexpensive and has been taught to other surgeons in developing countries. The post below is adapted from Warf’s testimony last week before the House Subcommittee on Africa, Global Health and Human Rights (viewable on C-SPAN; jump to 17:54). John Mugamba, MD, whom Warf trained and who is currently medical director at CURE Children’s Hospital of Uganda, gave testimony in video form.

In 2000, my family and I moved to Uganda as medical missionaries to help start a specialty hospital for pediatric neurosurgery, the CURE Children’s Hospital of Uganda. At the time, there were no pediatric neurosurgical hospitals and few trained neurosurgeons in all of Africa.

From its opening, our hospital was inundated with a steady stream of mothers seeking treatment for their infants with hydrocephalus, a condition in which the cerebrospinal fluid made in the cavities of the brain is unable to circulate out of the brain and be absorbed normally. This leads to mounting pressure, rapid expansion of the infant’s head, progressive damage to the developing brain and usually death if untreated.

The burden of hydrocephalus in Africa is arresting. We conservatively estimate that there are 100,000 to 375,000 new cases of infant hydrocephalus each year in sub-Saharan Africa, with an annual economic burden as high as $1.4 to $56 billion. This burden is comparable to that of more common medical conditions in Africa such as malignancies, perinatal conditions, congenital anomalies, and cataracts and glaucoma. Yet, we are the first to highlight infant hydrocephalus as a serious health burden in any region of the developing world.

In the U.S., most infant hydrocephalus is either congenital or related to brain hemorrhage in very premature newborns. In marked contrast, 60 percent of the Ugandan cases are caused by infections – mostly within the first month of life. The infections are characterized by a febrile illness, usually accompanied by seizures, followed by rapid enlargement of the infant’s head. In addition to the hydrocephalus, the brains of these children contain frank pus and blood and substantial destruction of tissue.

The average U.S. patient has two to three operations for shunt failure during childhood, but in rural Africa accessing emergency neurosurgical care is impossible.

We could successfully save the vast majority of these children by treating the hydrocephalus, but the primary brain injury from the original infection is often devastating. Of the children we treated for post-infectious hydrocephalus, we found that one third had died by five years, while one third of the survivors had severe disabilities. The importance of prevention or early treatment of these infections is obvious.

Infant hydrocephalus is almost always treated by implanting a tube called a shunt, which drains the fluid from the brain into the abdomen. In the U.S., half of these shunts fail and need revision within two years, and 80 to 90 percent fail at least once in the first decade.

Shunt failure is a life-threatening emergency in children. In the U.S., the average patient has two to three operations for shunt failure during childhood, but in rural Africa accessing emergency neurosurgical care is impossible. This led us to develop a new endoscopic treatment for hydrocephalus, avoiding shunt dependence in more than half of these babies, including those with post-infectious hydrocephalus. The operation combines two procedures: endoscopic third ventriculostomy (ETV), which makes a new pathway for the fluid to escape the cavities (ventricles) of the brain, and endoscopic choroid plexus cauterization (CPC) which cauterizes part of the tissue that makes the fluid thus decreasing its rate of production.

The above video by my colleague John Mugamba, now medical director at CURE Children’s Hospital of Uganda, demonstrates this technique; we have since taught it to many other surgeons. Detailed economic analysis estimates a lifetime treatment cost of around $90 per disability-adjusted life year averted, with a minimum benefit-to-cost ratio of 7:1. This cost compares very favorably to the few other surgical interventions that have been studied in developing countries.

But our work isn’t done. Post-infectious hydrocephalus, even when treated, leads to premature death or severe disability in the majority of infants. It is imperative that we identify the organisms that infect these babies so that public health strategies for prevention can be constructed and millions of lives saved.

Hydrocephalus has never been a public health priority in developing countries. Most infants in Africa receive no treatment. Training and equipping centers in an evidence-based treatment paradigm is essential. That is the challenge that lies before us.