Distraction osteogenesis can be very effective in improving upper airway obstruction in children with micrognathia, said a pediatric otolaryngologist during the session focused on sleep medicine, held at the Triological Society Combined Sections Meeting. The session also included a review of oral appliances designed to help with obstructive sleep apnea (OSA) and surgical options other than uvulopalatopharyngoplasty.
Micrognathia comes in three forms—“isolated” and not associated with a syndrome; “syndromic,” caused by a syndrome such as Trisomy 13; and Pierre Robin Sequence, a combination of micrognathia, glossoptosis, and cleft palate, said Andrew Scott, MD, assistant professor, pediatric otolaryngologist, and pediatric facial plastic surgeon at Tufts University School of Medicine in Boston.
In distraction osteogenesis, a mandiblular osteotomy is made and a distractor device is attached to either side of the gap. The bone is allowed to heal for several days, after which the bone segments are pulled apart by approximately 1 mm to 2 mm a day as new bone grows in the gap. Typically, only one out of three children with Pierre Robin Sequence needs the surgery.
The procedure might seem “barbaric,” Dr. Scott added, “but the outcomes are incredible once you’ve seen it.” After 14 days in the hospital, patients generally spend four to six weeks at home with the device. When it is removed, the patient generally experiences dramatic improvements in breathing, feeding, and overall facial structure. There is some puckering at the incisions when an external device is used, but these scars tend to fade with time, said Dr. Scott.
An internal, curvilinear device can be used in older children, and even performed trans-orally in adolescents. One 11-year-old patient with Treacher Collins syndrome had severe sleep apnea for more than 10 years and was taking three psychiatric medications because of problems with attention and emotional instability at school. After distraction osteogenesis, her OSA was completely resolved, with an apnea-hypopnea index (AHI) down to 0.0. “She was able to come off all her psych meds, her grades and school performance have improved, and she is thriving socially,” Dr. Scott said. “Her parents think it’s a miracle.”
M. Boyd Gillespie, MD, MSc, professor and director of the Snoring Clinic at the Medical University of South Carolina in Charleston and ENTtoday editorial board member, said that oral appliances (OA) are worth considering for OSA, particularly as part of combination therapy.
There are many device types, and otolaryngologists and dentists should work together to determine which is best for a given patient, he said, but they generally work by moving the mandible forward during sleep to help prevent airway collapse. “You’ll become a believer in oral appliances if you actually look at what these things do,” Dr. Gillespie said.
An analysis of 11 randomized controlled trials comparing OA to continuous positive airway pressure (CPAP) found that CPAP, which has high rates of nonadherence, typically gets the better results in terms of AHI, but OA yields better results in patient preference and usage (J Clin Sleep Med. 2014;10:215-227). One study found that after uvulopalatopharyngoplasty (UPPP), AHI improved at first but then regressed after three years. In cases in which an OA was also used, however, that regression tended to be prevented (Ir J Med Sci. 2015;184:329-334).
“If you look at any sleep apnea treatment across the board, it seems about two-thirds of people have a good response: Two-thirds respond well to surgery; two-thirds respond well to CPAP; two-thirds respond well to oral appliance,” Dr. Gillespie said. “Therefore, I think a combination will allow you to get a higher response.”
Erica R. Thaler, MD, professor of otorhinolaryngology-head and neck surgery at the University of Pennsylvania in Philadelphia, said that transoral robotic base-of-tongue surgery (TORS) for OSA can produce good results above and beyond previous surgery. Her indications for TORS are an AHI score higher than 20, with no maximum; no body-mass index cut-off, but with BMI considered when assessing a patient’s candidacy; and evidence of hypopharyngeal obstruction that is contributing to OSA.
According to data from her center recently published in The Laryngoscope, patients who had received a prior UPPP had a pre-surgery AHI of 40.3 and an AHI of 29.8 after TORS. Dr. Thaler said TORS should be a consideration (Laryngoscope. 2016;126:266-269).
Hypoglossal nerve stimulation is another option beyond UPPP, she said. The first eight patients in whom she has implanted the device have had impressive results. She presented AHI scores on the day of the implant—one with the device off, the other with it on. One patient’s index fell from 60 to less than five. Another dropped from more than 80 to approximately 10. A third patient’s index decreased from more than 100 to less than five, and Dr. Thaler acknowledged that while such an AHI would have been too high for the implant, the patient’s AHI had previously been 68.
Most of the patients had undergone some prior surgical procedure, she said, adding that because there are so many additional surgical options beyond UPPP, otolaryngologists may need to change the paradigm of how they manage patients surgically to a model in which they talk to patients up front about all possible avenues of surgical treatment.
Thomas Collins is a freelance medical writer based in Florida.