Stephen Cantrell DDS, MD (left), heads a team of professionals that repairs damage to the skull caused by genetic malformation or injury. He is the director of the University Craniofacial Center of New Jersey at UMDNJ-New Jersey Medical School.
Sonia was catapulted forward. She smashed her jaw, probably on the gear shift knob, and sustained extensive internal injuries. Help arrived, and the teen was quickly airlifted to the trauma center at UMDNJ-University Hospital in Newark. Her mother and father sustained back and head injuries, respectively, and were taken to the hospital by ambulance. The impact on the minivan was so great, a crumpled mass of metal that was once the rear of the van had replaced the front seat.
Trauma surgeon Kenneth Swan, MD, professor of surgery at UMDNJ-New Jersey Medical School (NJMS), met Sonia in the emergency room. Almost immediately, he whisked her into the operating room, where he spent about two hours doing exploratory surgery and stopping the internal bleeding. When she was stable, Stephen Cantrell, DDS, MD, was called to attend to her facial injuries. Cantrell is director of the University Craniofacial Center of New Jersey, headquartered in the Department of Surgery, NJMS. He is also an assistant professor in the Department of Oral and Maxillofacial Surgery at UMDNJ-New Jersey Dental School (NJDS) and clinical assistant professor in the Department of Plastic and Reconstructive Surgery at NJMS.
"Sonia had a laceration, about five inches long, on her chin and down onto her neck," he recalls. "She also had multiple fractures of the jaw. Some of the pieces of her jaw were protruding through the gash. And she had lost 10 teeth."
The first thing the surgeon did was to reconstruct Sonia's jaw. "Several large pieces were still intact, so we were able to piece it together pretty well and move forward," he says.
But that was just the start. After about four months of healing, Sonia underwent her second operation. This time Cantrell reshaped her jaw, which allowed her to have orthodontic treatment. He also removed the hardware he had used to hold the jaw together during the first operation. In September 1996, she had a third procedure. Bone from her hip was grafted onto her lower jaw, or mandible. But an infection set in, and the graft was unsuccessful. It would have to be redone. A little less than a year later, it was, and this time it was successful. A titanium implant was also put in, to replace two missing teeth. During those years, Sonia also had two operations to diminish the scars on her legs and abdomen.
Now a freshman studying graphic art at New Jersey City University, Sonia is having the last of her missing teeth replaced with the help of Hoda Aboyoussef, DMD, clinical assistant professor at NJDS. Soon, the only reminder of the ordeal will be a very vague scar under her chin.
right coronal(frontal) suture joint fused prematurely, causing the right
side of her forehead to appeaer flat and the left side to protrude. Her
right eye also appeared to be pulled back.
Craniofacial surgery is a relatively new area of surgery that primarily treats deformities and injuries of the face and skull. Specialized surgeons help trauma victims like Sonia, as well as people who develop tumors or growths. Cancer victims whose faces have become distorted, or who have lost an ear or nose, can also benefit from this form of surgery.
The majority of patients, however, are children born with birth defects that cause craniofacial deformities. Although these defects are not among the most common, neither are they extremely rare. In New Jersey, several dozen children are born with them each year.
The skull reaches about 95 percent of its adult size by the end of the second year of life
Some defects are inexplicable anomalies, while others may be caused by more than 200 syndromes. Two of the more common ones are Crouzon syndrome, which affects about 1 in 25,000 children, and Apert syndrome. Both are characterized by severe aberrations of the skull, forehead, eye sockets and the middle part of the face. Other areas of the body may also be affected. In Apert syndrome, for example, a child's fingers and/or toes may be fused.
"These two syndromes are autosomal dominant," Cantrell says. "That means if a person has one of them, approximately 50 percent of his or her offspring will be affected." He points out that the cause often has nothing to do with the parents, but is a genetic mutation that first appears in the child.
Cleft lips and palates, not associated with syndromes, remain a mystery. There is a genetic component, Cantrell says, but its mechanism is still unclear. Statistics show that those with lower incomes, especially in foreign countries, have an increased incidence, as do certain ethnic groups. "For reasons we don't know, those of Asian heritage are the hardest hit," he says. "Caucasians are somewhere in the middle and racial groups with dark skin are affected least." Cleft Lip and Palate Care at UMDNJ-University Hospital has now been incorporated with the Craniofacial Center.
The surgeon explains how craniofacial deformities occur. There are areas on a newborn's skull known as suture joints. The soft spot on a baby's head, for example, is part of that system. These soft junctures allow the skull and brain to continue growing with the child. When the skull reaches about 90 to 95 percent of its adult size - which usually happens by the end of the second year of life - the bones begin the slow process of fusing and becoming solid.
Sometimes, however, they solidify prematurely. If that happens at only one suture joint, the brain compensates by growing abnormally prominent somewhere else. "The skull may grossly protrude over one eye, for example, or out one side of the head," Cantrell says. "But the brain can still grow, so it is not harmed. In those cases, surgery is done for cosmetic and psychological reasons." He says deformed children, some not even old enough to speak, know they look different. "The mother of a 2-year-old patient told me her daughter covered the distorted part of her face with her hands whenever they were in public."
The abnormal growth may have a devastating effect on self-esteem, but the condition is not grave. It's when more than one suture joint fuses prematurely that the situation is critical. Because the skull and brain can't grow, the increased pressure can cause blindness, retardation, and in extreme cases even death.
"If caught early, many of the horrific outcomes are preventable, especially blindness," Cantrell notes. He adds that it's usually a "sharp pediatrician" or the child's mother who first spots the trouble. A pediatrician may notice that a child's head is not growing at the proper rate. Mothers often realize there is something wrong while shampooing a baby's hair. When the hair is wet, the shape of the head is much more distinct. It's then that a mother may notice a bulge or a flat spot.
Facial deformities have been described by physicians for hundreds of years. They were seen mostly as a source of morbid fascination, however, rather than a medical condition. As long ago as the 1600s, some physicians took a scientific approach to the problem, but only in the last 30 years have the necessary advances made the surgery feasible.
A French plastic surgeon, Paul Tessier, is considered the "grandaddy"of the specialty. He worked with monkeys in the late 1960s to develop the basic technique. In 1971, he rocked the medical world when he published a journal article saying he had successfully performed craniofacial surgery on a human.
A member of Tessier's team, it turns out, was Cantrell's mentor at the Australian Craniofacial Unit in Adelaide. It was there - at one of the busiest and most well-established centers in the world - that Cantrell did a year of intense training. But first, he completed dental school and medical school and did an oral and maxillofacial surgery residency. He is one of only 200 or so craniofacial surgeons practicing in the United States.
Exactly how is the surgery done? In stages, Cantrell says, using an entire team of health care professionals. There may be as many as 20 specialists involved besides the craniofacial surgeon: an orthopedic surgeon, an audiologist, a pediatric dentist, an otolaryngologist (ear, nose and throat physician), an orthodontist, an oral and maxillofacial surgeon, and a speech pathologist, to name just a few. "No one of us could do it alone," Cantrell says.
Nancy Schneider, MA, CCC-A, a senior audiologist at UMDNJ-University Hospital, helps Cantrell coordinate craniofacial clinic days. Children with craniofacial deformities often need to see as many as seven or eight specialists.
"What we've started doing is setting up appointments for these kids with all of the professionals they need to see in one day," Schneider says. "Besides saving everyone an incredible amount of time, the kids don't miss as much school. We all get a much fuller appreciation of the patient's needs." Sometimes, she says, the treatment plan for an individual will change as the specialists talk.
There are some 70 different professionals in various capacities who participate in the center at one time or another. "It truly is a team effort," Schneider says. "And what a team it is. The level of enthusiasm is unbelievable. Everyone gives their all and then some. It's great."
One professional who is often involved from the start is a behavioral psychologist, to support the patient and family. "It's not easy for parents to cope with a birth defect," says Cantrell. "They often feel guilty, believing somehow it was their fault. I had one mother ask me if the one and only cigarette she smoked during her pregnancy could have caused the deformity."
Accident victims, too, may need immediate counseling. When Sonia saw her face for the first time after it was injured, she was devastated. "All I could do was cry," she says. And she had no recollection of the accident at all. "You wake up and have all these painful injuries and you don't even know how you got them," she says. "It dominates your life."
The reconstruction process begins with planning a surgical strategy. "Usually the craniofacial surgeon and the neurosurgeon consult," says Cantrell. "We use the computer to reformat the patient's CT scans into a three-dimensional simulation of the skull. We look for anatomical landmarks, both normal and abnormal, and plan what modifications we'll make. By making these decisions ahead of time, we can save as much as two hours of surgery."
Hemwattie Papana's jaw was significantly longer on one side than on the other. To correct this problem, a relatively new technique know as distraction osteogenesis was used. It saved the 21-year-old from having a more complicated surgical procedure.
The surgery itself begins with one member of the team making an incision across the crown of the head, from ear to ear. "Using this technique, we can expose virtually any bone of the skull without leaving a scar on the face," says Cantrell. The scalp is then brought forward, and a cut is made in the frontal bone, or forehead. The surgeons are now able to work on the thin bone between the brain and the eye. The procedure of opening the head is similar to that used to remove a brain tumor.
"We can change the bones above the eye, for example, by fusing new bone onto what's there or onto a spot where it is missing," he says. "We can reshape by changing the angles, the thickness and the curves. It never ceases to amaze me what can be done." The bones are put back into position and the skull is reconstructed to near-adult size. "The child will grow into his larger head very quickly," Cantrell says, "and if the bones fuse again, there won't be a problem."
A very new procedure, now being performed at the center, uses a technique known as distraction osteogenesis. "Distraction means 'to pull apart,'" Cantrell explains, "osteo means 'bone' and genesis means 'new.' We use it to grow new bone." The instrument, known as an osteodistractor, is V-shaped with a universal joint at the base of the V. When the joint is rotated, each end of the V moves at a specified angle.
Hemwattie Papana, 21, a patient of Cantrell's, recently had her jaw reshaped using the technique. One side of her jaw was significantly longer than the other, somewhat resembling the Nike symbol. Hemwattie could only open her mouth about a half inch, making it difficult to eat, drink, and talk. Cantrell says she probably wasn't born with the abnormality, but perhaps sustained an injury or got an infection in the jaw that went undiagnosed.
For the procedure, a cut was made in Papana's jaw and the instrument inserted. Each day it was turned one millimeter, separating the existing bone. As the bone was slowly pulled apart, new bone grew in the space that was created. "At first she came in once or twice a week so we could check her. After that, she moved it herself at home," the surgeon says. "It saved her from having a more complicated surgical procedure."
Before having it done, Papana says she was very self-conscious about her crooked jaw. "I would try to cover that side of my face with my hair," she says. "I was always embarrassed, in school and everywhere. It was very difficult."
She wore the appliance for about three months. "At first it was a little painful," she admits. "But after a few weeks, it didn't hurt at all." She could eat only soft foods and sleeping was sometimes uncomfortable. But she says it was worth it. "I feel so much better about myself now," she says. "I have two tiny scars, but I cover them with makeup." She recently had surgery to bring her teeth into perfect alignment and to diminish the two scars.
While each patient is unique and presents a challenge of his or her own, Cantrell recently found himself in a situation he never faced before. A partially bald, male patient needed surgery on his eye socket, or orbit, but did not want a scar on his face or across the crown of his head.
"We decided to make the cut under his remaining hair, around the bottom of his head," he says. "As far as we know that had never been done before, so we formulated a back-up plan. But it worked very well."
Cantrell points out that while craniofacial surgery can dramatically change lives for the better, the road to complete reconstruction is usually a very long one.
"Families need to understand the commitment they're taking on. They need to know what will be done when the child is, say, 6 months old, and then what will be done when he is 2 years old, 5 years old, and so forth. But once again, the whole team guides them through the maze."
As Sonia nears the end of the tunnel, she admits it wasn't easy. "Sometimes I just wanted to give up," she says.
"For so long, I had to deal with the physical pain. Now I'm dealing with the emotional end of it."
She was "pretty nervous" when she first started to drive last summer, especially when merging onto a major highway. But now, she says, she feels more comfortable behind the wheel.
Her self-confidence is returning, too. She finally did compete in the Princess of Portugal pageant two years ago. "I was hesitant at first, but Dr. Cantrell encouraged me," she says. "He told me I'd do fine." And she did. Along with being third runner-up, Sonia was voted the most photogenic. A pretty face to be sure. But not just another one.
1999 Table of Contents
The magazine of the University of Medicine and Dentistry of New Jersey