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Laryngo-tracheo-bronchial stenosis in a patient with primary pulmonary amyloidosis: a case report and brief review

[Une sténose laryngo-trachéo-bronchique chez un patient atteint d’amylose pulmonaire primaire : une étude de cas et une brève revue]

From the Departments of Anaesthesia and Surgery, Vancouver General Hospital, Vancouver, British Columbia, Canada.

Address correspondence to: Dr. Sean Minogue, Department of Anaesthesia, BC Children & Women’s Hospital, Room 1-L7 - 4480 Oak Street, Vancouver, B.C. V6H 3V4, Canada: Phone: 604-875-2711; Fax: 604-875-3221; E-mail: minogues{at}indigo.ie

Purpose: To report a case of lower respiratory tract obstruction occurring in a patient with primary pulmonary amyloidosis and discuss anesthetic management.

Clinical features: A 53-yr-old man was referred to our institution for microlaryngoscopy and laser treatment of the larynx. He presented with a five-year history of primary laryngo-tracheo-bronchial amyloidosis and symptoms consistent with narrowing of the conducting airways. General anesthesia was induced with iv propofol 150 mg and remifentanil 50 µg. Mivacurium 20 mg provided muscle relaxation for endotracheal intubation. Following endotracheal intubation, the airway became obstructed and patient ventilation impossible. The endotracheal tube was removed and a Dedo laryngoscope inserted. Gas exchange was maintained using supraglottic jet ventilation via a distal port of the laryngoscope. Rigid bronchoscopy showed tissue partially obstructing the lumen of the lower trachea. This was removed and the airway appeared patent. At the end of the case, a further episode of lower airway obstruction occurred requiring reinsertion of the laryngoscope and resumption of jet ventilation. Extensive debridement through the bronchoscope was required before adequate ventilation could be restored. Some days later when the patient’s condition deteriorated again and he required further debridement of the trachea and insertion of a tracheostomy, guide wires were positioned in the femoral vessels in the event that cardiopulmonary bypass was required for gas exchange.

Conclusions: Primary laryngo-tracheo-bronchial amyloidosis is a recurrent disease, requiring repetitive surgical procedures. Airway compromise can be a persistent problem. Awareness of this uncommon disease process and its presentation may serve to caution the anesthesiologist presented with this type of case.