Prenatal pioneer arrives
Immediately following his birth, the VUMC team modified the shunt implanted in little James Neal Borkowski while he was still in his mother's womb. The shunt helped drain fluid that built up due to congenital hydrocephalus. (photo by Anne Rayner)
Taking part in last week's news conference were (from left) Dr. Joseph Bruner, Dr. Noel Tulipan, and the Borkowski family – father James, mother Susan and Baby Neal. (photo by Donna Jones Bailey)
A first-of-its kind fetal brain surgery procedure to repair hydrocephalus has been performed on a Knoxville infant by an open fetal surgery team at Vanderbilt University Medical Center.
James Neal Borkowski, born May 12 at Vanderbilt University Hospital, was diagnosed with congenital hydrocephalus (excessive fluid on the brain) during a routine ultrasound at 20 weeks gestation.
His parents, James and Susan Borkowski, were simply hoping to find out their baby's gender and a definite due date when they were given the news in Knoxville. Congenital hydrocephalus, seen in about one in 2,000 deliveries, is a developmental defect of the brain in which an excessive accumulation of fluid dilates the cerebral ventricles. Babies born with hydrocephalus are usually severely brain damaged.
Four weeks after the discovery, VUMC's Dr. Joseph P. Bruner, associate professor of Obstetrics and Gynecology, and Dr. Noel B. Tulipan, associate professor of Neurological Surgery, performed the groundbreaking procedure. This week, the Bruner-Tulipan team performed its 50th open fetal surgery procedure to repair spina bifida lesions on fetuses still nestled in their mother's wombs. They began peforming that procedure two years ago.
Neal, delivered five weeks early and weighing four pounds, seven ounces, was born with no signs of hydrocephalus. His parents – James, 34, a graduate teaching assistant at the University of Tennessee, and Susan, 29, say they are hopeful.
The baby, whose due date was Monday, is "alert, feeding well and doing all the things that babies should do," his father said at a press conference held last Friday. He was discharged from the hospital last week and will soon be going home to Knoxville, where he will be monitored closely.
Still, the ultimate measure of the procedure's success will be the child's IQ as he develops – which won't be known for some time.
"There is no doubt that we prevented the continuation of hydrocephalus in this baby. But we still don't know what the outcome will be," Bruner said. "There's still the chicken and egg-type questions – does hydrocephalus cause neurological injury or is hydrocephalus a result of the neurological injury?
"Most babies with hydrocephalus suffer severe brain damage by the time they are delivered so the only way to find out the answer for sure is to shunt the babies early and see if their outcome is improved."
Tulipan said physicians will be monitoring Neal's progress closely by watching the baby's coordination and vision.
"We really have to wait and see how he does and slowly feel our way," he said. "This procedure worked perfectly as far as the shunt goes. Now the rest is up to the baby."
In the procedure, Borkowski's uterus was opened by Bruner, then Tulipan made a tiny incision in Neal's skull. He placed a shunt, a small tube that's about 2 mm in diameter, into the fluid space in the brain. The opposite end of the shunt is hooked to a small valve (about inches long) that controls the flow of fluid that escapes through the top of the shunt. The valve is connected to another long, thin tube that is tunneled through the baby's skin between the shoulder blades. The fluid drains into the amniotic fluid.
A second surgery was performed a few hours after Neal was born. In this procedure the distal tube (the tube coming through the skin, between the shoulder blades) was exchanged for a tube that goes into Neal's abdominal cavity where the fluid can drain permanently.
Tulipan said the shunt will likely be replaced at least once during Neal's life.
Bruner said the surgical team is looking at two outcomes of the procedure. The short term outcome is whether the hydrocephalus was prevented.
"Based on this first procedure the answer to this is an unqualified yes," he said.
"But there are long-term questions as well. Will this result in a better outcome or is hydrocephalus a much more complicated issue than anybody ever expected?"
James Borokowski learned of the fetal brain surgery procedure almost by accident. His secretary mentioned a story she had seen on a television news magazine show about "some type of fetal surgery." Borokowski searched the Internet and for all the news magazine shows he could think of, including 60 minutes and 20/20. On his last attempt, with Dateline NBC, he found what he was looking for – a story about Vanderbilt's open fetal surgery program. He contacted Bruner and the Borokowskis were at Vanderbilt the next week.