ABSTRACT

Robin sequence (RS), previously known as Pierre Robin syndrome and Pierre Robin anomalad, consists of the following 3 essential components:

•       Micrognathia or retrognathia

•       Cleft palate

•       Glossoptosis

Upper airway obstruction and feeding difficulties are the main concerns related to the pathology. Mandibular distraction should be considered a treatment option (when inadequate results with other treatment are delivered).

MATERIAL AND METHODS

Two neonates of the ages of 2 weeks and 2 months with severe micrognathia and airway obstruction were treated with extraoral Mandibular Distraction Osteogenesis (MDO).

The patients underwent fibroscopic examination of the upper airway and a radiographic imaging and/or computed tomography scans to detect malformations and to confirm that the obstruction was caused by posterior tongue displacement. All patients were evaluated by a multidisciplinary team. Indications for surgery included frequent apneic episodes with severe desaturation (70%). Gavage therapy was employed in all patients since oral feeding was not possible. The one tracheotomy patient was 2 months old, and the distraction procedure was performed after the other therapies had failed. Both the neonates were treated with bilateral external mandibular distraction.

RESULTS

The resolution of symptoms was obtained in both the patients.  Patients were discharged when the endpoint was obtained: symptoms and signs of airway obstruction were resolved, and maxillomandibular relationship improved. During the follow-up, no injury to the inferior alveolar nerve was noted. However, scarring was significant as they were treated with external devices.

CONCLUSION

Mandibular Distraction Osteogenesis is a good treatment option in solving respiratory distress when other procedures are failed in neonatal/paediatric patients with severe micrognatia.