Re-building baby jaws: New surgery for PRS

A mysterious birth defect that often comes with a perfectly normal pregnancy is taking parents by surprise, and the condition can kill up to 30 percent of children who are born with it.

Pierre Robin Syndrone (PRS) affects one out of every nine thousand babies. Their jaws are severely undersized which cause their tongues to fall back and block their throats.

Micah Cercy had PRS and had life-changing surgery just six days after he was born.

Mark Cercy, Micah’s dad, reflects on the terrifying experience. "It was just absolutely crushing…you thought you'd prepared for everything."

Traditional treatment for PRS is a tracheostomy to help breathing or external hardware to extend the jaw. However, at Wolfson Children's Hospital, Dr. Saswata Roy is doing something different.

Dr. Roy describes their treatment method. “To lengthen the jaw and open the airway, metal hardware is placed on the bone on both sides, and then gradually extended over several weeks as new bone grows around it. As they lay down new bone, we keep extending the fracture farther and farther forward. In effect, we can reconstruct a normal shaped jaw in these babies.”

Cindy Cercy, Micah’s mom, is thrilled with the results. She says, “It just astounds you how much of a difference it is."

Now, thanks to the surgery, Micah is an active little boy who can eat and breathe normally

Dr. Roy says not only is this procedure safer and more effective for babies born with Pierre Robin Syndrome, the long term costs are less than treating babies with tracheostomy.

The causes of the defect are mainly unknown but doctors believe it is a genetic disorder.

BACKGROUND: The exact causes of Pierre Robin syndrome are unknown. It may be part of many genetic syndromes. The lower jaw develops slowly before birth, but speeds up during the first year of life. Infants with this condition should NOT be put on their back, to prevent the tongue from falling back into the airway. In moderate cases, the patient will need to have a tube placed through the nose and into the airways to avoid airway blockage. In severe cases, surgery is needed to prevent a blockage in the upper airway. Some patients need surgery to make a hole in the windpipe (tracheostomy). Feeding must be done very carefully to avoid choking and breathing liquids into the airways. The child may need to be fed through a tube occasionally to prevent choking.

SYMPTOMS: The symptoms of Pierre Robin Syndrome include a cleft soft palate or high-arched palate; Jaw that is very small with receding chin; jaw placed unusually far back in the throat; large-appearing tongue compared to jaw; natal teeth (teeth appearing when the baby is born); recurrent ear infections; and/or a small opening in the roof of the mouth, which causes choking.

COMPLICATIONS: Complications that may arise if the disorder is not treated include; choking episodes; feeding difficulties; breathing difficulties, especially when the child sleeps; low blood oxygen and brain damage (due to difficulty breathing); pulmonary hypertension; congestive heart failure and death.
NEW TREATMENT: A newer procedure for rebuilding the jaw of an infant with micrognathia, which relieves the blockage permanently, is being performed at Wolfson Children's Hospital by pediatric craniofacial surgeon Saswata Roy, MD, from Nemours Children's Clinic, Jacksonville.
Called neonatal distraction osteogenesis, the procedure involves performing surgery on the baby within days of birth, before he leaves the neonatal intensive care unit (NICU). Through a 2-cm incision, Dr. Roy creates a small fracture in the lower jaw (called an osteotomy) and attaches a device known as a distractor to both sides of the bone on both sides of the cut. The distractor keeps the fracture open and is slowly turned over weeks or days to stretch the break in the abnormal bone; then, new tissue fills the space and creates new bone. This method, which is usually not painful to infants, forms a longer bone from a shorter one in a period of about six weeks. Once the new bone is strong enough, the surgeon removes the distractor during a short second operation.

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