[Fatal double tracheo-esophageal vascular compression and neurofibromatosis].

BACKGROUND

Vascular rings are a classical cause of tracheal and esophagus compression. We report the case of such an abnormality in an infant with neurofibromatosis.

CASE REPORT: A 1 week-old male infant with a familial neurofibromatosis presented a stridor with severe respiratory distress. A vascular ring was demonstrated and operated on. The stridor persisted after surgery. A postoperative oesophagogram and tracheobronchoscopy showed an irregular compression of the oesophageal lumen, thought to be due to a residual extrinsic compression. Because the postoperative echocardiogram showed an extensive tumoral infiltration of both auricles, it was decided to not operate again the child. The postmortem examination revealed a disseminated neurofibromatosis infiltrating trachea, bronchi and also the wall of esophagus.

CONCLUSION

Persisting stridor and oesotracheal compression postoperatively requires search for another cause. Association of vascular ring and neurofibromatosis is probably not fortuitous.