Modern Marvel

When Monica Schaffer of Paso Robles, Calif., underwent a three-dimensional ultrasound during the 30th week of her pregnancy, she and her husband, Jon, were startled to learn that their daughter suffered from a severe birth defect. Their tiny girl had an abnormally small lower jaw and no chin. Without a fully developed lower jaw for support, the soft tissues of her middle airway had “balled-up” – effectively sealing off her throat. This left her unable to swallow and would, once she was born, render her unable to breathe.On August 6, 2002, Ashlynn was born with a severe form of micrognathia, a rare condition affecting 1 in 30,000 births, or approximately 70 children, in America every year. Despite their rarity, craniofacial conditions such as Ashlynn’s can have devastating lifelong effects and craniofacial surgeons are working to improve the treatments for these and other facial defects. As with all cutting-edge procedures, however, there exists an element of trial and error, as surgeons are constantly trying to improve upon their techniques.According to Dr. Thomas Chang, a pediatric plastic surgeon at Kaiser Permanente in Kensington, Md., treating patients when they are younger is often crucial, as correcting the problem early can have an impact on how a child’s face develops as he gets older. But it’s a double-edged sword, he said, because when children are younger they are still growing, so there’s always a question about when and how to best intervene. “We want patients to have positive outcomes in the short term as well as in the long term,” said Dr. Chang. “And with some procedures it’s still too early to know all the long-term effects.”It took the hospital’s critical care doctors 45 minutes to surgically open an airway for Ashlynn – by cutting into her windpipe and inserting a tube, a procedure known as a tracheotomy. During those 45 minutes, Ashlynn was without oxygen and the Schaffers were told that, as a result, she wasn’t likely to survive, and that if she did, she’d be destined to live life in a vegetative state. Ignoring advice to consider removing Ashlynn from life support, the Schaffers insisted that the doctors do more. Despite her grim prognosis, Ashlynn clung to life in Loma Linda Medical Center’s neonatal intensive care unit.After two months, Ashlynn was transferred to Mattel Children’s Hospital at the University of California in Los Angeles, where the family looked deeper into “distraction osteogenesis.” The process, based on the ideas of mid 20th-century Siberian orthopedic surgeon Gavriel Ilizarov, takes advantage of the human body’s natural tendency to heal itself. Ilizarov discovered that when the bones of the leg are gradually pulled apart vertically – or “distracted” – the body generates new bone in the empty space, thereby lengthening the limbs. In time, doctors came to realize that the distraction process had applications beyond lengthening long bones. The boost in the development of distraction techniques to combat facial deformities came when doctors and manufacturers teamed up to develop miniature forms of the tool used for distraction. Known as “distractors,” the metal devices advance small bone fragments like those in the jaw, and the soft tissues associated with them. In 1990, the first lower jaw distraction was performed by Dr. Joseph McCarthy and his colleagues at Manhattan’s New York University Medical Center.At UCLA, the Schaffers met with pediatric craniofacial surgeon Dr. James Bradley and decided to move forward. “The other options,” Ms. Schaffer said, “were to do nothing right away and revisit the idea of distraction three or four years down the road, or do nothing at all and just allow her jaw to grow on its own.” In both instances, however, Ashlynn would have no hope of living without the breathing tube.In the following days, a three-dimensional CAT scan revealed that Ashlynn had enough of a lower jawbone to serve as an anchor for the distraction devices, and that she also had the joints necessary for moving her jaw. But though Ashlynn was technically a candidate for distraction, Dr. Bradley said that her case of micrognathia is the most extreme he’s ever seen. “We definitely weren’t sure if we could technically do it or if it would work,” he said, “but based on similar cases we thought so. And it was the best shot she had.”When Ashlynn was 16 months old, Dr. Bradley and his surgical team made the first cut into her lower jaw, broke her jawbone in one place on each side, and anchored the distractor into the bones, which was in turn secured by a bulky external device resembling scaffolding. For the next week, doctors waited while the inflammation at the sites picked up, initiating the formation of new bone. Dr. Bradley then turned each of the two pins once, with a specially designed screwdriver no bigger than a pencil.Within days, Ashlynn was released from Mattel Children’s Hospital. Once home, her mother turned the pins every day – gradually pulling apart and lengthening her daughter’s jaw. “I was scared to do it at first,” Ms. Schaffer said, “but then the doctors told me that it feels kind of like having your braces tightened.” After 42 days of turning the pins, Ms. Schaffer had lengthened Ashlynn’s jaw more than 2.5 inches.Nine months later, Dr. Bradley feels hopeful, and expects that Ashlynn’s tracheostomy will be removed within the next year. “Ashlynn’s well on her way to a normal life,” he said. “By the time she’s a teenager, she’ll look, eat, and talk just like other girls her age.” But he points out that her treatment is a long process. “Because her face will continue growing,” he added, “we don’t want to overdo it now. She will need another distraction procedure in five or six years and a final one when her facial growth is complete – at age 15 or 16.”Today, because of the tracheostomy, Ashlynn needs to be fed through a tube in her stomach and her family has still never heard the sound of their little girl’s voice. They have been told by several neurologists that having her breathing tube removed will be difficult because the lack of oxygen she experienced at birth has left her with developmental delays. “They’ve told us she might have problems coordinating all of the muscles she’ll need to breathe through her nose, her mouth, and swallow,” Ms. Schaffer said. “She didn’t do those things at birth – so she’s going to have to learn and some neurologists say it might not happen.”But Ms. Schaffer says that she and her husband choose not to believe the experts. Just last week, she said, “I was washing dishes and caught sight of Ashlynn out of the corner of my eye. She had stood up and slid something off of the kitchen counter. A few seconds later Jon asked me if I had given her a sucker. I said, ‘No,’ but then I turned to look at Ashlynn and she had red sucker all over her mouth. She was going to town on that huge thing and it was pure heaven to see that happen. When she was born I couldn’t even fit my pinkie in there. Modern technology,” she added, “it’s a wonderful, beautiful thing.”

Modern Marvel

Modern Marvel

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