The purpose of this study was to measure changes in the airway cross-sectional area of pediatric patients with micrognathia and obstructive airway symptoms after treatment by mandibular distraction. The measurements obtained were correlated with the clinical outcomes.Ten patients, ranging in age from 3 months to 8 years, underwent measurement and distraction. Eight patients were under 30 months of age. Six were diagnosed with Pierre Robin sequence, two with Treacher Collins syndrome, and two with Nager syndrome. All patients had retrognathia of greater than 8 mm and obstructive airway symptoms while awake that had resulted in tracheostomy (3), repeated apnea monitor triggering (5), or abnormal sleep study (2). Cephalometric analysis was performed pretreatment and posttreatment by distraction. The effective airway space was defined with the following boundaries: a horizontal line from the tip of the odontoid to the velum, the uvula tip to the tongue base along the shortest line, the tongue base down to the base of the epiglottis, and the horizontal line to the posterior pharynx. These lines were traced for each cephalogram, the outline was digitized, and the area was calculated by computer. An analysis of the square area change was done by paired t test. The range of distraction was 8 to 22 mm; the mean effective airway increase was 67.5 percent, with a range of 26 to 120 percent. Measurable airway increase occurred in all patients who underwent distraction, and all patients showed clinical improvement. Six patients with Pierre Robin sequence became asymptomatic, with normal sleep, feeding, and weight gain. Two patients with Nager syndrome and tracheostomies were decannulated and were asymptomatic postdistraction. One patient with Treacher Collins syndrome without tracheostomy became asymptomatic after mandibular distraction; one patient failed to distract because of premature consolidation and continued to require a cannula. Mandibular distraction seems to provide a consistent change in tongue base position that improves obstructive airway symptoms by increasing measured effective airway space. The potential for mandibular distraction exceeds the simple correction of malocclusion also by eliminating soft-tissue obstruction of the micrognathic airway. Airway improvement is independent of the syndrome diagnosed. Mandibular distraction osteogenesis may be useful to avoid or decannulate existing tracheostomy in infants with micrognathia.
Over the past 5 years, the authors developed an application of mandibular distraction osteogenesis to eliminate existing tracheostomy. That experience led the authors to attempt mandibular distraction osteogenesis in neonates as an alternative before tracheostomy. Success with this approach using supporting objective airway measurements has been reported previously. This report includes six neonates diagnosed with Pierre Robin sequence. Of the six, five neonates ranging in age from 6 to 26 days (mean, 14.5 days) were treated by the authors with mandibular distraction over a 22-month period. The sixth neonate was treated with tracheostomy, because of other airway abnormalities. Findings included retrognathia, glossoptosis, incomplete cleft palate, and airway obstruction in each patient. Birth weights ranged from 2.8 to 3.2 kg. All patients were unable to control their airway during feeding, as evidenced by repeated episodes of choking and obstruction. Resting oxygen saturations were in the 70 to 80 percent range in all patients, with further deterioration during attempted feeding. Bronchoscopy was performed in all patients under anesthesia before distraction. Recurrent near-complete and intermittent complete airway obstruction were present in all patients at the level of the tongue base. There was a consensus by a pediatric intensivist, a pediatric anesthesiologist, and a pediatric otolaryngologist in all cases. Each patient met all criteria requiring ventilation for life support. Tracheostomy would be required if mandibular distraction osteogenesis was not performed, or if it failed. Patients with other airway abnormalities were not considered for treatment. Maxillomandibular disharmony measured at the midline ranged from 8 to 15 mm (mean, 11.2 mm). Active distraction was performed at the rate of 1 to 2 mm a day, with a consolidation period of 4 weeks. Total time of treatment was less than 6 weeks in all cases. All patients were extubated by the completion of active distraction. Distraction distance ranged from 8 to 15 mm (mean, 12.4 mm). All patients were discharged to home on apnea monitors, the use of which was discontinued after 90 days with no further apneic events. Weight gains met or exceeded the average 500 g a month after distraction. Bronchoscopy at the time of distractor removal showed correction of airway obstruction at the tongue base. Radiographs showed bilateral ossification of the distraction sites. Tracheostomy was avoided in all cases selected for treatment by distraction. Patient follow-up range was 9 to 22 months. In selected Pierre Robin sequence patients with tongue base airway obstruction, mandibular distraction osteogenesis can successfully avoid the need for and the associated mortality and morbidity of indwelling tracheostomy.
Tongue-lip adhesion can be effective in relieving tongue-based airway obstruction in the neonatal period. However, longitudinal evaluation of these patients demonstrates they require additional procedures for ultimate control of their airway, feeding, and orthognathic relationship. Charts of patients with Pierre Robin sequence who underwent unsuccessful nonoperative treatment and tongue-lip adhesion procedures by the senior author between 1989 to 1999 were reviewed. In particular, secondary interventions were quantified and qualified to determine if tongue-lip adhesion was a definitive treatment of the pathology caused by this sequence. Eleven patients with ages ranging from 2 to 6 weeks underwent initial tongue-lip adhesion. Seven patients were syndromic (3 Stickler's, 1 Goldenhar's, 1 Fragile X, 1 Miller's, 1 chromosome 15 rearrangement), and four had isolated Pierre Robin sequence. Two patients had dehiscence of their initial repair within 2 weeks, for a primary surgical success rate of 82%, consistent with other published reports. A detailed follow-up of these patients revealed that 10 of 11 (91%) required additional intervention for airway or feeding issues. Of those requiring secondary intervention, an additional 1.9 secondary procedures were performed per patient to achieve control of the airway, feeding, and orthognathic problems. More than half of the patients needed gastrostomy tubes to overcome feeding difficulties. Five patients necessitated secondary surgery for recurrent airway obstruction within 4 months of the initial surgical treatment (four distractions and one repeat tongue-lip adhesion). Four others required distraction at an older age for orthognathic indications. Two patients were successfully treated with tongue-lip adhesion only. Tongue-lip adhesion has a high initial success rate for correction of neonatal airway obstruction. However, long-term follow-up indicates a high incidence of secondary intervention requirements. In retrospect, perhaps tongue-lip adhesion should be considered a temporizing procedure for most patients with Pierre Robin sequence.
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