A Rambam surgical team working to seperate the congenitally fused jaws of an infant..
(photo credit: PIOTR FLITR FOR RAMBAM)
Surgeons at Haifa’s Rambam Medical Center have successfully operated on a two-month-old baby born with a very rare defect in which his jaws were fused shut. Worldwide, there have been only about 50 such cases.
When the infant from the center of the country, identified only as N., was born about two months ago, his parents discovered that he had a cleft palate and another congenital defect, congenital maxillomandibular fusion, in which his jaws were connected by soft tissues so he was unable to open his mouth completely. It was the first such diagnosis in Israel.
A few days ago, Rambam doctors performed an operation to remove the soft tissue to disconnect his jaws.
Congenital maxillomandibular fusion is associated with a number of genetic syndromes, but due to the few cases reported worldwide, the cause of such congenital malformations is still unknown.
In some cases, the connection between the jaws develops through bone augmentation, and in other cases through the tissue, as in this case. When the baby was born, the side parts of his jaw were attached to one another at the gums, but at the center of his mouth there was a narrow opening that allowed him to drink milk.
In consultation with his parents about the continuation of his treatment, they were referred to Rambam’s department of oral and maxillofacial surgery under the direction of Prof. Adi Rahmiel, who recommended that the operation to separate the tissues and open the jaws be performed as soon as possible, despite the baby’s tender age, as leaving the defect could cause serious problems in his development.
Although the operation was never carried out in Israel, the Rambam team approached the task and assembled a multidisciplinary team from the departments of orthodontics, anesthesiology and oral and maxillofacial surgery.
The team of surgeons included Dr. Omri Amoudi, a senior physician in the maxillofacial department, who headed the team together with Dr. Boaz Frankel, a senior maxillofacial surgeon, and Dr. Amit Lehavi, a senior pediatric anesthesiologist. Dr. Yair Israel and Dr. Shimrit Arbel-Prati, specialists in the field of oral and maxillofacial surgery, also took part along with Prof. Dror Aizenbud, director of the orthodontics department who is an expert in congenital malformations.
During the operation, the doctors gently cut the soft tissues that connected the upper and lower jaw and then cauterized the separated areas to stop the bleeding and allow the damaged tissue to recover optimally. Measurements were taken to make an orthodontic plate to separate the upper and lower jaw and prevent the connection from joining again.
At the end of the operation the baby was transferred to a recovery room and, only a day after the surgery, discharged in good condition. According to the doctors, the child’s parents will have to repeat the process of separating the gums with their finger to prevent the tissue from reforming until the area has completely recovered. Following the operation, the baby’s jaws are expected to develop properly and without any restriction.
“We are very pleased with the results,” commented the baby’s relieved father. “In the near future, we will return to Rambam for further follow-up, but the difficult part is behind us.”
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