Airway obstruction in the pierre robin sequence

Abstract

Airway obstruction and feeding difficulties vary among patients with Pierre Robin sequence (PRS). Treatment is challenging and the appropriate management may not be readily identified, leading to delay in securing the airway. A retrospective review of 90 children with PRS was done to identify subgroups at a higher risk of developing severe airway obstruction using oxygen and apnea monitoring, sleep studies, and response to treatment. Patients with isolated PRS (group I, 27 patients) and Stickler syndrome (group II, 32 patients) do not suffer from debilitating airway and feeding difficulties when compared to those with unique syndromes (group III, 16 patients) and recognized named syndromes (group IV, 15 patients). Feeding difficulties were universal with the severity proportional to airway obstruction. Aggressive intervention should be considered early in group III and IV patients.

Functional outcomes of mandibular distraction for the relief of severe airway obstruction and feeding difficulties in neonates with Pierre Robin sequence

“…Leading surgical interventions to prevent glossoptosis are tongue-lip adhesion 1 and release of suprahyoid muscles 14 . In extreme cases to provide long-term support and relieve airway obstruction tracheotomy procedure is inevitable 15 .…”

Mandibular Distraction Osteogenesis in Low-Weight Neonates with Robin Sequence

“…[20][21][22][23][24][25] An extended ICU stay increases risk to nosocomial infection and increases the economic burden to the healthcare system. Alternatively, there can be significant risk associated with early MDO intervention in neonates during their first days of life.…”

Pierre Robin syndrome: a case report

“…Subsequent complications of chronic hypoxia are chronic carbon dioxide retention, elevated pulmonary vascular resistance, cor pulmonale, right heart failure, and cerebral hypoxia. [6][7][8] Most of the patients with PRS gets relieved by conservative measures however patients with pronounced micrognathia, failure to thrive, prolonged use of nasopharyngeal airway or prolonged endotracheal intubation needs surgical correction. As in our case conservative measures were sufficient to manage the symptoms of neonate.…”