Solution for Newborns with “Small Jaw Syndrome” Superior to Traditional Therapy

When Kieli Rauert was born last April she couldnt breathe or eat like other babies. Kieli is one of nearly 8,000 American infants born each year with a birth defect called Pierre Robin Syndrome.

“At one month we could tell she had no chin or jaw line on her lower mandible,” said Tina Rauert, Kielis mom.

Small jaw, or micrognathia, and cleft palate are two of the defining characteristics of Pierre Robin Syndrome. The structural abnormalities can cause the infant’s tongue to be pushed so far back in the throat the baby can’t swallow, or even breathe, without assistance.

In the most severe cases, the infant requires a tracheostomy, or tube placed in the neck to allow unimpeded breathing.

Kieli avoided a tracheostomy, thanks to Michael Miloro, D.M.D., M.D., section chief and program director for Oral and Maxillofacial Surgery at University of Nebraska Medical Center in Omaha. Dr. Miloro is one of just a handful of surgeons nationwide performing a specialized and novel surgery on infants with “small jaw syndrome.”

The treatment is called jaw distraction osteogenesis.

“Jaw distraction is a permanent solution to micrognathia, and it has several advantages over a tracheostomy, which is a riskier option, and does nothing to correct the small jaw,” Dr. Miloro said.

“A tracheostomy carries a mortality rate of up to 5 percent, even at major centers,” he said. “And while it creates a temporary solution to the infants breathing problem, it has to remain in place for up to two years or more. During that time the baby must be fed by a gastric tube and experiences delays in speech and language development.”

During jaw distraction surgery, Dr. Miloro makes a partial cut through the jawbone, behind the last developing baby tooth. A portion of the bone marrow and blood vessels in this bone are preserved to support new bone growth that will occur during the distraction process.

Two titanium rods about the size of pencil lead are surgically placed through the babys upper and lower mandible, and then tiny distraction devices are attached to the rods during the surgical procedure. These external distraction devices are turned a few millimeters each day to gradually lengthen the porous jawbone in the area where the cut was made in the bone. Jaw distraction not only removes the need for tracheostomy, it lengthens the jawbone to normal size within weeks.

Kielis jawbone was lengthened 4 centimeters over approximately 10 days. It made a significant difference for the little girl whose birth defect caused her tongue to block her airway and sent her to Omaha by medical helicopter just hours after being born. Prior to surgery, Kieli was on oxygen and an apnea monitor, and had to be tube fed. At the age of ten weeks, Kieli underwent a one-hour jaw distraction surgery at The Nebraska Medical Center.

“Within a day or two after surgery there was a huge difference in her appearance,” Tina Rauert said. “We have never once regretted our decision. It was heartbreaking, but her father and I knew in the long run shed turn out healthier.”

Dr. Miloro has performed jaw distraction osteogenesis on infants as young as 5 days old. He has trained health professionals and surgeons in Mexico, and donated the expensive distraction devices to Mexican physicians, and treated about a dozen patients throughout Mexico.

Dr. Miloro also has performed several dozen cases of distraction osteogenesis for babies in the neonatal period to avoid or remove a tracheostomy which had already been placed for severe airway obstruction. In every case, he said, successful prevention or removal of the tracheostomy has been achieved and the babies have not had any airway difficulties after surgery. In addition, their feeding tubes were discontinued and the patients were using a bottle within days to weeks after surgery.

Because the technology and surgical expertise for distraction osteogenesis has been available only for the past five years and only a few surgeons perform the surgery nationwide, Dr. Miloro said there are questions about the long-term prognosis for “normal” growth of the lower jaw following a jaw distraction procedure performed in the neonatal period.

“Its not an easy question to answer,” he said.

Provided by ArmMed Media
Revision date: July 4, 2011
Last revised: by Dave R. Roger, M.D.