To determine whether the use of mandibular distraction osteogenesis (DOG) can help to avoid tracheotomy or achieve decannulation in patients with mandibular hypoplasia and severe upper airway obstruction.Design: Retrospective medical record review (spanning a 27-month period).Setting: Tertiary care children's hospital. Subjects: Group A (n=8) was composed of infants with Pierre Robin sequence and no tracheotomy (mean age, 2.5 months); group B (n = 6), older nontracheotomized micrognathic children with obstructive sleep apnea (OSA) (mean age, 69 months); and group C (n = 12), tracheotomized children with complex congenital syndromes (mean age, 33 months).Intervention: Bilateral mandibular DOG with endoscopic (n = 24) and/or radiographic (n = 17) airway evaluation (mean follow-up, 16 months [range, 2-42 months]).Outcome Measures: Group A, tracheotomy avoidance; group B, resolution of OSA (clinically or on polysomnography); and group C, decannulation.Results: Group A, 7 patients (88%) successfully avoided tracheotomy; group B, 5 patients (83%) had resolution of OSA; and group C, 2 patients (17%) underwent decannulation.Conclusions: Mandibular DOG (1) allows tracheotomy avoidance in infants with isolated Pierre Robin sequence and (2) relieves OSA in older micrognathic children without tracheotomy. However, mandibular DOG does not frequently lead to decannulation in tracheotomized patients with complex congenital syndromes.

Tracheostomy for management of neonatal airway obstruction may be life saving but is associated with complications and developmental problems. As an alternative, the effectiveness of internal mandibular distraction osteogenesis was investigated in select neonatal patients with micrognathia and upper airway obstruction. Preoperative tests (sleep study, direct laryngobronchoscopy, and "milk scan" for GI reflux) were used to select appropriate candidates for the procedure. Excluded were patients with 1) central apnea, 2) severe reflux, 3) other airway lesions, and 4) mild to moderate obstruction controlled by positioning. Of 44 newborns (aged <3 weeks) with upper airway obstruction and micrognathia seen in the neonatal intensive care unit, 19 underwent tracheostomy, 10 were discharged with home monitoring and positional instructions, and 15 underwent bilateral mandibular lengthening with microdistractors. Of those who underwent mandibular distraction, a tracheostomy was avoided in 14 of 15 patients. Relative improvement in the posterior airway space was seen on 3D CT scans, cephalograms, and laryngobronchoscopies obtained preoperatively, postoperatively, and during follow-up evaluation. One of these 15 patients required a tracheostomy for postoperative central apnea. In an average of just 4.5 days following completion of distraction, patients were discharged home with improved oral feeding and no feeding tube. This study suggests that for selected newborns, the use of internal microdistractors allows for avoidance of a tracheostomy and improved oral feeding.

An epignathus is an oropharyngeal teratoma composed of cells from ectodermal, mesodermal, and endodermal layers. Epignathi that arise from the palate or pharynx and protrude from the mouth result in life-threatening airway obstruction and usually cause asphyxiation shortly after birth. In our reported case, an antenatal ultrasound diagnosis allowed for preparation of an ex utero intrapartum treatment (EXIT) procedure. A tracheostomy was performed at birth with maternal-fetal blood flow still intact through the umbilical cord. Debulking of the large extraoral portion of the tumor, followed by complete intraoral resection, was performed. Masseteric function and swallowing slowly improved over several weeks. Cleft palate repair is planned at 10 months of age. In the following report, the histology, classification, and pathogenesis of these "parasitic" tumors are reviewed.

Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight ‘cornrow’ hair braiding. Cranial vault expansion was successfully performed. Ophthalmological problems have stabilized but have not reversed. Late presentation of craniosynostosis and the pathophysiology of secondary optic atrophy are discussed.