Rambam physicians successfully operated on a two-month-old infant born with a rare birth defect—a fused jaw. Only 50 cases have cases of infants born with a fused jaw have been reported worldwide.
Doctors during the surgical procedure
performed for the first time ever,
in Israel, at Rambam.
Photo Credit: Pioter Fliter
When N, an infant from the central region of the country, was born about two months ago, his parents discovered that he was suffering from a cleft palate and an additional phenomenon which occurs in only the rarest of circumstances: the child was born with a fused jaw, by way of the soft tissue, in a way that prevented him from fully opening his mouth. This birth defect, which to date has only been described in 50 cases in all of the world's medical literature, has never been diagnosed in Israel. Just a few days ago, Rambam physicians performed a successful surgery to separate the two sections of the fused jaw.
The development of a fused jaw, known in English as Congenital maxillomandibular fusion, appears to be related to a number of genetic syndromes. The cause of this rare birth defect is unknown due to the small number of reported cases. Fusion of the jaw may result from either the joining together of the bone, or, as in this case, from the connection of soft tissue. At birth, the sides of N’s jaw were connected at the gums, but a small opening remained at the center of his mouth, which enabled him to drink milk. Seeking how to best care for him, N's parents were directed to the Department of Oral and Maxillofacial Surgery at Rambam, directed by Professor Adi Rachmiel. They were advised to have surgery as quickly as possible to separate the tissue, despite the child’s young age. According to the professional evaluation of the physicians, delaying surgery placed the infant’s development at risk.
Although such a surgery had never been performed in Israel, Ramba’s staff rose to the occasion. The multidisciplinary surgical team included experts in orthodontics, anesthesiology, and maxillofacial surgery. The lead surgeons were Dr. Omri Emodi, Senior Surgeon and Deputy Director of the Department of Oral and Maxillofacial Surgery and Dr. Boaz Frenkel, Senior Surgeon of the Department of Oral Maxillofacial Surgery. Also on the team were Dr. Amit Lehavi, Senior Anesthesiologist specializing in children; Dr. Yair Israel and Dr. Shimrit Arbel-Parati, residents in oral and maxillofacial surgery; and Professor Dror Aizenbud, Director of the Orthodontics Unit and an expert in craniofacial anomalies.
During the operation, a delicate incision was made in the tissue connecting the infant’s upper and lower jaws. The separated tissue was then cauterized so as to limit bleeding and to allow the tissue to heal in an optimal fashion. Measurements were also taken for an orthodontic plate that would be subsequently implanted, to separate N’s upper and lower jaws and prevent them from fusing together again.
Following the operation, N was taken to a recovery room and only twenty-four hours later, he was released to his home in good condition. According to the physicians, the parents will need to perform regular exercises involving the use of their fingers to keep N's gums from growing together again, until the tissue is fully healed. The baby's jaws are expected to develop normally, without any limitations.
"We are very pleased with the results" stated N's father. "In the near future, we will return to Rambam for continuing treatment for our child, but the most difficult part is now behind us."
