Helping Children with a Rare Form of Hydrocephaly

Posted on Jan 31, 2017

Damian Bell was born with an extremely large head: The circumference was nearly 20 inches compared with an average of 13.4 inches for newborn boys.

This rare condition known as extreme macrocephalic hydrocephaly usually would not be repaired until middle childhood.

Damian and his PhysiciansBut Johns Hopkins All Children’s Hospital pediatric neurosurgeon Gerald Tuite, M.D., and his colleagues pioneered a new approach that offered earlier correction, with fewer complications and improved appearance.

In hydrocephaly, excess cerebrospinal fluid accumulates within the brain, raising intracranial pressure, which leads to increased head size. The more typical form is usually treated by inserting a shunt (tube) that drains the excess fluid into the abdomen.

For extreme cases of hydrocephaly like Damian’s, patients traditionally have surgery at age 5 or 6, sometimes even as late as age 9 or 10, Tuite explains. Because the bony plates of the skull harden and fuse over the years, the resulting head shape can be startlingly abnormal. Surgical repair at that point could be extremely difficult and dangerous. Tuite’s technique avoids these issues by taking action on newborns such as Damian, whose skulls haven’t begun to harden.

As part of a clinical research study that began in 2011, Tuite’s approach called for Damian to have surgery during the first week of his life. First, a programmable shunt slowly drained some of the fluid. A few days later, the surgeons removed a portion of Damian’s skull and also stabilized it with the help of an absorbable synthetic plate. Immediately, the head size was reduced by 49 percent and the bones were held firmly in place. With a smaller head and bones that no longer moved with every change in position, Damian’s care was much easier for his parents. After six weeks in the neonatal intensive care unit, Damian was able to head home with his parents. Initially there were clinic visits every other month with Tuite for follow-up care; now Damian returns just once a year.

Tuite and his colleagues described the new surgical technique and the successful outcome in a technical note published in 2013 in the Journal of Neurosurgery: Pediatrics.

“We are really thankful for all that Dr. Tuite has done” - Damian's mother Ashley

Damian, now 5, and several other patients who benefited from this surgery recently returned to the hospital to help with the next part of the clinical research study. Tuite and the team took careful measurements, both digitally and by hand, in order to carefully study the head shape of the five children who had the procedure in the past six years.  It was an unusual clinic visit with Tuite and the research team, which includes Lisa Tetreault, R.N., a nurse and clinical research coordinator, Rajiv Iyer, M.D., a pediatric neurosurgery resident at the Johns Hopkins Hospital, and Nir Shimony, M.D., an Israeli surgeon who is the first international pediatric neurosurgery fellow at Johns Hopkins All Children’s.
Iyer chose to spend six months with the pediatric neurosurgeons at the Johns Hopkins All Children’s Institute for Brain Protection Sciences as he completes the fifth year of his seven-year neurosurgical residency. “It’s been great to be part of this study and to see the positive outcomes. There are great clinical research opportunities at Johns Hopkins All Children’s, and I’ve also gained new perspectives by working with each of the surgeons in the operating room and clinic.”

Damian is doing better than his parents could have imagined. “We are really thankful for all that Dr. Tuite has done,” says his mother, Ashley.

“In pediatric neurosurgery, a lot of rewarding research is clinically focused research that is derived from technical innovation,” Tuite says. “Our team of four pediatric neurosurgeons works closely with our residents and fellows to help advance our field and the care of our patients on several fronts. We have a great team. We and our patients are fortunate to have the vast resources available through Johns Hopkins All Children’s Hospital.”

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