UB pediatric surgeon performs life-changing procedure on newborn

BUFFALO, N.Y. —  The newborn was struggling to breathe.

Born in Tel Aviv, Israel, in July with Pierre Robin Sequence, a rare craniofacial condition that consists of upper airway obstruction, the 3-day-old baby girl was in danger. A tracheostomy looked like the only solution.

The hospital treating her, Sheba Medical Center, is ranked as a top medical facility in the world. However, the standard of care in Israel is to perform a tracheostomy and then mandibular distraction, or lengthening of the jaw, later in life. This contrasts to the United States, where mandibular distraction is often offered in the first few weeks of life with the goal of avoiding a tracheostomy.

At the urging of the baby’s parents, the medical center connected with Michael Markiewicz, DDS, MD, MPH, professor and Feagans Endowed Chair of the Department of Oral and Maxillofacial Surgery at the University at Buffalo School of Dental Medicine, who is a leading expert in mandibular distraction.

“At birth, the baby was not breathing well on her own,” Markiewicz said. “They have a wonderful surgical team at Sheba, but they don’t do this procedure for children this young. When trying to help them find a surgeon, I learned that no hospital in Israel does.”

A specialist in pediatric craniomaxillofacial surgery who also serves as the clinical co-director of the Laurence C. Wright Craniofacial Center at John R. Oishei Children’s Hospital in Buffalo, and as an attending surgeon in the Department of Head and Neck/Plastic and Reconstructive Surgery at Roswell Park Comprehensive Cancer Center, Markiewicz has performed the procedure numerous times locally and elsewhere.

Robin Sequence is characterized by a small, underdeveloped jaw and a tongue positioned back in the mouth. It affects an infant’s ability to breathe, eat and swallow.

The mandibular distraction procedure involves small cuts to the lower jaw and insertion of a device that lengthens the jaw up to 3 centimeters. This enables the muscles associated with the lower jaw to come forward, opening up the airway and relieving upper airway obstruction, Markiewicz explained.

“In the majority of cases, we can get these patients to breathe on their own,” he said.

Fortunately, that was the case with this baby girl.

“The baby did well,” he said. “Just a few days after surgery, the breathing tube was removed and she began eating, drinking and breathing on her own.”

Parents lobbied for procedure

When the hospital suggested the traditional tracheostomy procedure for the infant, her parents pushed for the more progressive procedure. They spoke with the head of the hospital’s craniofacial surgery team, Tal Yoffe, DMD, MD, who connected with Markiewicz through UB colleague Laurie Sadler, MD, clinical associate professor of pediatrics in the Jacobs School of Medicine and Biomedical Sciences, a geneticist and co-director of the craniofacial team in Buffalo, who has relatives and friends in Tel Aviv.

“Colleagues at the hospital said that mandibular distraction is not the standard of care option for children this young with Robin Sequence, but they would like to learn how to perform it on a very young patient,” Markiewicz said. “They were receptive to collaborating.”

They asked Markiewicz if he would fly to Tel Aviv and lead the procedure. He requested that his fellow and clinical assistant professor of oral and maxillofacial surgery, Jessica Li, DDS, accompany him to assist, and they agreed. With fast-tracked paperwork, including immigration documents, hospital credentials and malpractice insurance, the two were able to leave for Israel the following week.

Time of the essence

“The hospital staff had intubated [placed a breathing tube] the baby, and the situation was getting bad. With the potential for long-term complications, we didn’t want to keep the baby intubated for long,” said Markiewicz, who made the 12-hour flight with Li on July 24, arriving in Tel Aviv on July 25 and heading into surgery the following day.

“They sent an ambulance to our hotel to pick us up,” he said. “The surgeons, anesthesiologists and nursing team there were excellent and very welcoming. The collaboration with the team was a wonderful experience.”

The three-hour surgery included going through two small incisions on each side of the neck and inserting hardware onto the baby’s lower jaw, which Sheba physicians removed in November. The baby was also born with a cleft palate, as is often the case with Robin Sequence, but it will be addressed at a later date by the surgeons  in Israel.

“We don’t normally repair cleft palates until a child is 12 to 18 months old,” Markiewicz said. “The timing of palate repair is based on speech development, so there is no rush. The baby can feed with a special bottle in the meantime.”

Powerful procedure

Markiewicz has performed dozens of mandibular distractions, as well as written and lectured on the topic.

“It’s a very powerful procedure,” he said. “My hope is that the success of this surgery changes the paradigm in Israel, and they’ll start doing mandibular distraction on young infants just like we do in the U.S.”

While Markiewicz had completed pediatric surgeries in other parts of the world, this was his first surgery in Israel. Overall, he said it was an incredibly rewarding experience. After making sure the baby was stable, he returned to the U.S. two days after the surgery.

“At a time when the world is going through a lot of change and turmoil,” he said, “it was a special experience to go over there and work with a team of surgeons with the collective goal of just making the baby better.”