Johns Hopkins’ Division of Pediatric Neurosurgery is performing innovative studies in hopes of solving some of neurosurgery’s most pressing problems, including those involving hydrocephalus and Chiari malformation.

Hydrocephalus has long been treated by inserting shunts that reroute cerebrospinal fluid away from the brain. This fix can lead to common and lifelong complications, such as shunt malfunctions and infections.

Babies born before 32 weeks gestation are at increased risk to develop hydrocephalus due to brain bleeds that can cause lasting anatomical changes, but some recover successfully without intervention, says Johns Hopkins pediatric neurosurgeon Shenandoah Robinson.

“It suggests that endogenous restoration mechanisms could enhance repair and prevent hydrocephalus,” she says.

Funded by a grant from the Eunice Kennedy Shriver National Institute of Child Health and Human Development, Robinson and collaborator Lauren Jantzie, Ph.D., are working with colleagues at Johns Hopkins’ neurosciences intensive care nursery on a study to test the safety of administering a combination of melatonin and erythropoietin, two hormones that they found prevent progression of brain bleeds to hydrocephalus in a preclinical model.

In other research, funded by the Department of Defense, Jantzie, Robinson and their colleagues are using preclinical models to study whether existing medications can prevent hydrocephalus from traumatic brain injury, and whether a combination of other medications can treat hydrocephalus from other causes, such as genetic mutations and hemorrhage.

Ongoing work led by Robinson’s pediatric neurosurgery colleague Eric Jackson, M.D., also focuses on hydrocephalus. Through Johns Hopkins’ membership in the Hydrocephalus Clinical Research Network, a collaborative network of pediatric neurosurgery centers in North America working to improve hydrocephalus care, Jackson and his colleagues are involved in several studies to innovate treatment for this condition. For example, Johns Hopkins is participating in a large, randomized clinical trial with other in-network hospitals to compare shunting with an endoscopic surgical technique for treating hydrocephalus. The trial is funded by the National Institutes of Health.

Jackson and his colleagues also maintain a database of all patients with hydrocephalus who sought care in Johns Hopkins’ Department of Emergency Medicine for suspected shunt malfunction. Findings derived from this database avoid the bias present in most literature on this population, which centers on surgical treatment. The aim is to eventually use this data to create a scoring system to better individualize work-ups for patients with shunts to optimize outcomes and minimize unnecessary tests.

Jackson’s research also includes several active studies on Chiari malformation. As a member of the Park-Reeves Syringomyelia Research Consortium, he and his colleagues are involved in multiple retrospective investigations, and they recently completed a randomized clinical trial comparing outcomes from posterior fossa decompression with those of posterior fossa decompression with duraplasty.

Johns Hopkins has also joined the Chiari Surgical Success Scale study, a multi-institutional cohort that aims to predict the best outcomes for surgical treatment of Chiari malformation. Because the study only includes surgical patients, Jackson and his colleagues are developing an internal registry to investigate all patients with the condition, including those not treated surgically. A different retrospective database at Johns Hopkins for patients who are surgically treated will help Jackson and other researchers answer questions about treatments and more in future studies. In addition, he and colleagues are collaborating with researchers at other institutions to investigate the genetic aspects of Chiari malformation.

“Together," Jackson says, “all these efforts will improve care both for patients at our institution and beyond.”