Mandibular advancement by distraction osteogenesis for tracheostomy-dependent children with severe micrognathia.

PURPOSE: The purpose of this study was to evaluate mandibular lengthening by distraction osteogenesis (DO) to achieve decannulation of micrognathic children with "permanent" tracheostomies. PATIENTS AND METHODS: Using a retrospective chart review, patients were included who had 1 ) airway compromise/tracheostomy, 2 ) micrognathia, 3 ) polysomnography-documented obstructive apnea, and 4 ) mandibular advancement using DO. Excluded were 1 ) adults, 2 ) neonates without tracheostomy, and 3 ) patients with central apnea. Patient age, past medical history, age at tracheostomy, and distraction protocol were documented. Oxygen saturation, posterior airway space (in millimeters), and sella-nasion-B point (SNB) angle were recorded. The distraction protocol consisted of a latency of 48 hours and a rate of 1 mm/day. RESULTS: There were 5 children, aged 2 to 14 years, who received a tracheostomy between ages 2 and 36 months for airway obstruction. All patients underwent bilateral mandibular distraction using semiburied, unidirectional devices. The average latency was 58 hours, the rate was 1 mm/day, the duration of fixation was 40 to 60 days, and the magnitude of advancement was 23 mm. Healing was evaluated by clinical, radiologic, and ultrasound examinations. No complications were experienced. Mean follow-up was 3.2 years. Postdistraction sleep studies demonstrated no obstructive apneic events and a mean oxygen saturation of 98% (preoperative, 76%, P < .005). Cephalometric values improved: posterior airway space 4 to 14 mm; SNB 66 degrees to 72 degrees ( P < .005 for both variables). Four of the 5 patients have been successfully decannulated to date. CONCLUSIONS: The results of this preliminary study indicate that mandibular advancement by DO is a potentially viable treatment option for tracheostomy-dependent children with upper airway obstruction secondary to micrognathia.     

Prenatal indices for mandibular retrognathia/micrognathia

Journal of Orofacial Orthopedics / Fortschritte der Kieferorthopädie - Micro- and retrognathia of mandibular origin may lead to life-threatening respiratory problems in connection with...     

Severe congenital hypoglossia and micrognathia with other multiple birth defects

Severe congenital hypoglossia with the anterior two-thirds of the tongue missing is a rare finding. This case report describes an infant 14 months of age who manifests this tongue anomaly along with other congenital malformations including an underdeveloped mandible, partial anodontia, enamel hypoplasia, situs inversus, dextrocardia, and asplenia. Only one other case similar to this case has been reported in the literature. There is no report of any case of severe congenital hypoglossia with asplenia.
The etiology of this birth defect is only speculative at this time, but the mother of our patient was exposed to radiation from routine chest films during her first trimester of pregnancy.     

Severe congenital hypoglossia and micrognathia with other multiple birth defects

Severe congenital hypoglossia with the anterior two-thirds of the tongue missing is a rare finding. This case report describes an infant 14 months of age who manifests this tongue anomaly along with other congenital malformations including an underdeveloped mandible, partial anodontia, enamel hypoplasia, situs inversus, dextrocardia, and asplenia. Only one other case similar to this case has been reported in the literature. There is no report of any case of severe congenital hypoglossia with asplenia. The etiology of this birth defect is only speculative at this time, but the mother of our patient was exposed to radiation from routine chest films during her first trimester of pregnancy.     

Distraction osteogenesis for tracheostomy dependent children with severe micrognathia

Obstructive sleep apnea (OSA) in pediatric populations is often associated with congenital craniofacial malformations resulting in decreased pharyngeal airway, which in severe cases leads to tracheostomy dependence. The purpose of this study was to use distraction osteogenesis to improve the airway and decannulate the tracheostomy. This study involved 11 OSA tracheostomy-dependent patients (age range, 4 months to 6 years) who underwent bilateral distraction in the mandibular body using extraoral distraction devices. Following a latency period of 4 days, gradual distraction at a rate of 1 mm/d was performed followed by a consolidation period of 10 weeks. Three-dimensional computed tomography reconstruction of the face and neck before and after the mandibular lengthening aided in quantitative volumetric evaluation of mandibular volume and airway volume. The results demonstrated mandibular elongation of a mean of 30 mm on each side, an increase in mandibular volume by an average of 29.19%, and increase in pharyngeal airway by an average of 70.53%. Two to 3 months following the last lengthening, all 11 patients were decannulated with improvement of signs and symptoms of OSA and elimination of oxygen requirement. Mean follow-up was 2.0 years. The oxygen saturation level rose to more than 95%, and the apnea index respiratory disturbance index was less than 2 episodes per hour for all patients. Bilateral mandibular distraction is a useful method in younger children to decannulate permanent tracheostomy expanding the hypoplastic mandible and concomitantly advance the base of tongue and hyoid bone increasing the pharyngeal airway.