Published: Oct. 28, 2007
Updated: May 27, 2010
An Angel Team to Treat Craniosynostosis Patient DJ McLean
Waking up to the late-night cries of your six-month-old baby is nothing to be alarmed about. But one night, when Seneca McLean reached her son DJ’s crib, she found her baby with a badly protruding eye.
“Somehow his upper and lower eyelid were stuck behind his eyeball,” she says. “We later found out that this came from the pressure of his skull on his brain.”
DJ was tested and cleared for spina bifida, but then a fairly minor fall triggered dangerous bleeding under his skull, and DJ was rushed to Duke for emergency surgery.
His pediatric neurosurgeon discovered that DJ had craniosynostosis -- a condition in which one or more of the joints in the skull fuses prematurely, constricting the infant’s growing brain and leading to skull and face deformities. In DJ’s case, the building pressure from his brain caused his skull to become thin, making his fall more harmful.
Craniosynostosis is a rare condition, and optimal treatment requires highly complex surgery. But for the vast majority of these children, growth and development proceed normally after treatment.
Duke Children's Hospital is home to one of the nation’s few dedicated pediatric craniofacial surgery teams, and in 2004 DJ underwent a special surgery at Duke to allow his facial features to develop normally.
Plastic surgeon Jeffery R. Marcus, MD, led a team that fitted DJ with a therapeutic halo called a distraction device. With the help of pediatric neurosurgeon Herbert Fuchs, MD, and pediatric dentist Martha Ann Keels, DDS, PhD, a halo headframe -- designed to fit DJ -- was connected to surgical wires that were implanted at various points around his skull, teeth, and facial bones. DJ’s distraction device slowly pulled his midface and teeth forward, by about 1 millimeter every day.
DJ began to wear the device that July -- his only complaint, says his mother, was that he could not have a hamburger -- and by September his face was transformed.
“I can remember one incident when we went to visit our family,” says McLean. “DJ’s young cousin spotted him first from behind, and when DJ turned around to greet him, the boy stopped dead in his tracks because he thought he had the wrong person.”
“Treatment of craniosynostosis by distraction osteogenesis is the biggest advance in our field in the last 25 years,” says Marcus. He notes that the technical challenges of the surgery require not only specially trained surgeons and anesthesiologists, but also a top-notch nursing and surgical staff.
“The surgery can be high risk,” he says, “and the results of the surgery must be good enough to last a lifetime.”
In DJ’s case, the surgical team was joined by Keels, as well as pediatric anesthesiologist and airway expert Warwick Ames, MD, during the procedure. Social workers helped DJ and his family cope with the day-to-day challenges of life under a halo. Duke speech pathologists helped DJ adjust to feeding and communication.
“It really takes a team of very talented individuals working in harmony to make the treatment a success,” Marcus says.
DJ, now a smart and witty 11-year-old, is proof of that harmony. McLean reports that her son’s only medical problem is some trouble with his sinuses.
“Our son is so confident -- he’s never met a stranger,” she says. “DJ loves science and often tries mixing different substances to create chemical reactions. His newest love is wrestling and body-building -- my husband and I see more of his bare chest now than when he was a baby. We have to beg him to put a shirt on.”
Marcus says Duke has seen nearly a two-fold increase in craniosynostosis referrals since this treatment became available, and these patients come from a wide area.
“Duke is one of the busiest centers in the country for treating this condition.” While conditions like craniosynostosis aren’t necessarily fatal, treating them can have a profound effect on these children’s quality of life. That’s what Marcus says is so gratifying.
“The surgeries we perform aren’t necessarily life-saving, but they are certainly life-altering.”