Ten minutes after Natalia Semper was born in a local hospital, it was obvious something was wrong. As the nurses bathed the listless, 8-pound-7-ounce newborn, they noticed she was turning blue and had difficulty breathing.
Natalia was soon transferred to Children’s Hospital Central California’s Regional Level III Neonatal Intensive Care Unit (NICU). She was diagnosed with Pierre Robin Sequence, a birth defect characterized by an abnormally small lower jaw. The condition causes the tongue to “ball up” and fall backward toward the throat and block the airway, making it hard to breathe or feed. About one in every 5,000 children is born with this type of facial anomaly. Some of those affected may also have a cleft palate, like Natalia.
“It was tough,” recalled Natalia’s mother, Andrea Castillo, as she rocked her then 5-month-old daughter in her arms in their Fresno home. “She couldn’t gain weight because she was spending more calories trying to breathe than she could take in. She had to have a nasogastric feeding tube and be kept on her stomach to breathe better.”
Natalia remained at Children’s Hospital for the next three weeks. During that time, Dr. Angela Rodriguez, of the Hospital’s multidisciplinary plastic surgery team, performed “mandibular distraction,” a procedure to correct the breathing and feeding problem. “No child with a craniofacial issue should need to go outside the Valley to get treated – we have everything here,” said Dr. Rodriguez.
The one to three-hour surgery involves inserting small pins into the jaw bone. A device called a distractor is inserted either externally or internally and attaches to the pins. The distractor is slowly lengthened by turning the pins 1 mm each day for about two weeks. Space created by the distraction quickly fills in with new bone called “regenerate.” After the bone solidifies, the distractor can be removed six weeks later in a short, outpatient surgery. The jaw will continue to grow as the child grows and no additional surgery is required. Slight scars along the jawline typically fade over time.
“It sounds painful, but the child experiences little discomfort because it’s done gradually,” said Dr. Rodriguez, whose internationally-known mentor, Stephen Schendel, MD, DDS, invented the internal method she used for Natalia.
The result? “Now Natalia breathes and sleeps normally and she’s so active,” said Castillo as she spoon-fed the smiling infant mashed pears and sweet potatoes. “Many kids who have this condition need a tube in their trachea and their stomach to survive. This procedure avoided that.”
Next Dr. Rodriguez will repair Natalia’s cleft palate in September when she is 10 months old, the time when a child begins developing speech. Without the mandibular distraction, Natalia would have had to wait until she was 18 to 24 months old to do the cleft palate surgery.
“Doing the procedure sooner reduces the possibility of Natalia having to unlearn any bad speech habits she otherwise may have developed,” said Dr. Rodriguez.
Doctors are not sure what causes Pierre Robin but do not think it is genetic. It is called a sequence because it results from a series of events that take place as the fetus forms in early pregnancy.
Castillo, a massage therapist, said she and her husband, Mark Semper, owner of Semper Speed and Marina, appreciate Children’s Hospital. “Dr. Rodriguez and all of the NICU nurses were wonderful,” said Castillo. “Thanks to the help we received, Natalia won’t have long-term effects from this. We’re very blessed.”