Late development of airway obstruction in the Robin anomalad (Pierre Robin syndrome) in the newborn

Abstract

A survey of the records of 26 consecutive cases of the Robin anomalad, admitted in the first year of life, was undertaken to review the time at which airway obstruction was first noted to occur after birth. Sixteen cases required active treatment for episodes of airway obstruction associated with cyanosis. Of these, 11 developed obstruction on the first day of life, but five did not obstruct until later (3-21 days). Two infants, both from the early presentation group, died as a result of airway obstruction at 13 and 95 days of age. Management techniques varied over the 11 year period studied, and included prone nursing, body frame, naso-tracheal intubation, nasopharyngeal intubation, and lip tongue anastamosis. Tracheostomy was not performed on any case. There is a significant risk of major airway embarrassment in this disorder (16 of 25, 62%) even if the infant seems initially well (five of 26, 18%). Early management of infants with the Robin anomalad should, therefore, be undertaken at centres where skilled airway support is available.