Floating Hospital the only Massachusetts hospital offering a surgery that can spare newborns from feeding tubes or tracheotomies
When Anthony Hill was born six weeks premature in July 2011, he had micrognathia, a facial malformation characterized by mandibular hypoplasia and a small, receding chin. More than just a cosmetic issue, the degree of his deformity made it impossible for him to maintain his tongue in a forward position, leading to potentially life-threatening feeding and breathing problems.
Anthony’s pre-adoptive parents were faced with three surgical options to correct the problem. The first was tongue-lip adhesion in which the tip of the baby’s tongue is sewn to the lower lip; this aids breathing by pulling the tongue forward over the course of a baby’s first year. Swallowing remains a problem during this time, however, so a gastrostomy tube is often required for feeding, along with home nursing for g-tube management and suctioning. Option two was to bypass the tongue entirely with a tracheotomy; this would require long-term home nursing care, suctioning, cardiopulmonary monitoring and a high probability of language delays. The third option was a procedure called mandibular distraction osteogenesis, which permanently corrects micrognathia over the course of a two-week hospital stay, and without the need for after-care.
Anthony’s family opted for mandibular distraction osteogenesis, performed by Andrew Scott, MD, pediatric otolaryngologist and facial plastic surgeon at Floating Hospital for Children. Fellowship trained at the University of Minnesota – which was among the first institutions to pioneer the procedure– Scott is the only surgeon in Massachusetts who performs this procedure on newborns. Other hospitals in the Bay State currently offer only tongue-lip adhesion and tracheotomy procedures to correct micrognathia.
“At other institutions in Boston, surgeons feel that tongue-lip adhesion does the job of preventing the need for tracheotomy,” Scott says. “And in the right hands, it does deliver an adequate airway result. But even in the best surgical hands, kids with tongue-lip adhesion are unable to eat normally for the duration of treatment, so they frequently require supplemental feeding through a tube.”
“Recently I performed mandibular distraction osteogenesis on a child who had had an adhesion procedure elsewhere and it failed,” he notes. “This child had never had a taste of food and he was eight months old by the time I saw him.”
“What’s nice about jaw distraction is that over 90 percent of patients do not require any type of feeding tube long term,” he adds. “They can eat and breathe normally within two weeks after the procedure.”
What, exactly, does the procedure entail?
“During the initial surgery, which takes about three hours, a careful break is made in the jaw bone on either side, then hardware is attached behind and in front of the break,” Scott explains. “After a couple of days of healing, the jaw is slowly brought forward and the gap is spread apart. Since it’s done at a slow pace, the gap in the jaw fills in with bone over time. Once the desired length of the jaw is reached, which takes about two weeks, the child is able to breathe normally and can begin feeding by mouth. After this point, the child can return home and we simply wait for the bone to harden. The incision leaves a small scar under the chin.”
“Most patients are discharged two weeks after the procedure which, in general, is faster than with tongue-lip adhesion or tracheotomy,” he notes. “Most important, the infant goes home breathing and feeding normally, and without the need for nursing services or monitoring.”
“After about 6 weeks the bone sets, and the hardware is no longer necessary so we remove it,” he continues. “This involves a second procedure that’s performed on an outpatient basis with brief sedation. The pins we placed in the jaw are simply pulled out and band-aids left in place; there are no incisions or blood loss.”
Scott has performed nearly 20 of these complex procedures on newborns, eight of them since arriving at Floating Hospital for Children last year. His youngest patient was five days old; most are about three weeks of age.
“It’s not a common procedure, and whenever possible we use alternate therapies,” he says. “Whenever we can avoid surgery for a child we do; we only say yes in cases where the child absolutely needs this surgical intervention.”
“We don’t perform the jaw distraction procedure simply because an infant has a small jaw that is a cosmetic issue, but only when the infant has a small jaw and can’t eat or breathe normally,” Scott stresses. “If the baby has no problems breathing and can feed normally, the small jaw can be fixed later as more of a cosmetic procedure.”
Saving and improving lives
For the one in three babies born with micrognathia so severe that it compromises feeding or breathing, the procedure not only saves lives but confers tremendous quality-of-life benefits.
“Without fixing it early, these newborns will end up with a tracheotomy or tongue-lip adhesion and a feeding tube,” he says. “For infants with a lot of airway issues and/or moderate to severe jawbone deformity, jaw distraction is an effective, less invasive and more successful way to treat micrognathia. In our long-term follow-up with these young patients, the majority don’t need any other jaw surgery. This is it.”
The surgery may affect tooth growth and damage wisdom teeth, so many children will require braces later on. Most children with this condition require braces no matter what treatment they receive; it is a reality of micrognathia.
“When parents fully understand the treatment options, we find they will invariably opt for a small scar under their child’s chin versus having the child live with a feeding tube or tracheotomy,” Scott adds. “The mandibular distraction procedure gives them this choice.”