FATAL TRACHEOESOPHAGEAL COMPRESSION VASCU LAR AND NEUROFIBROMATOUS INORIGIN

Background. - Vascular rings are a classical cause of tracheal and eso phagus compression. We report the case of such an abnormality in an in fant with neurofibromatosis. Case report. - A 1 week-old male infant w ith a familial neurofibromatosis presented a strider with severe respi ratory distress. A vascular ring was demonstrated and operated on. The strider persisted after surgery. A postoperative oesophagogram and tr acheobronchoscopy shelved an irregular compression of the oesophageal lumen, thought to be due to a residual entrinsic compression. Because the postoperative echocardiogram showed an extensive tumoral infiltrat ion of both auricles, it was decided to not operate again the child. T he postmortem examination revealed a disseminated neurofibromatosis in filtrating trachea, bronchi and also the wall of esophagus. Conclusion , - Persisting strider and oesotracheal compression postoperatively re quires search for another cause. Association of vascular ring and neur ofibromatosis is probably not fortuitous.