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

Abstract

P Pu ur rp po os se e: : To report a case of lower respiratory tract obstruction occurring in a patient with primary pulmonary amyloidosis and discuss anesthetic management.C Cl li in ni ic ca al l f fe ea at tu ur re es s: : 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.

Laryngotracheobronchial amyloidosis: A case report

“…The clinical presentation of amyloidosis can vary widely depending on the organs involved [ 8 ]. Patients with tracheobronchial tree or laryngotracheal involvement often present with hoarseness, stridor, choking, or dyspnea on exertion [ 10 ].…”

“…Currently, no specific treatment exists for this disease [ 2 ]. The treatment of symptomatic laryngeal and tracheobronchial amyloidosis includes resolving narrowed airway segments by stenting, the piecemeal resection of tumor masses, open thoracotomy, or laser ablation therapy [ 10 ]. Other therapy options include chemotherapy and oral dexamethasone [ 13 ].…”

Undiagnosed light chain systemic amyloidosis: does it matter to anesthesiologists? -a case report-

“…Tracheobronchial infiltration may cause hoarseness, stridor, airway obstruction, and dysphagia. One case report described the extensive involvement in the entire laryngo-tracheo-bronchial tree [2]. In the report, a 53-year-old man had been followed up for five years for systemic amyloidosis involving the larynx, trachea, and bronchi.…”

“…AL amyloidosis is the most common subtype and is caused by deposition of protein derived from immunoglobulin light chain fragments. Laryngeal or pulmonary involvement requires attention by the anesthetist to airway management in the perioperative period [2,3]. Therefore, diagnosis of amyloidosis for any surgical patient should prompt an evaluation of the involvement of other organs, including the airway, before anesthetic induction and complete preparation for difficult airway management.…”

Pressures Available for Transtracheal Jet Ventilation from Anesthesia Machines and Wall-Mounted Oxygen Flowmeters

“…Although there can be life-threatening complications with jet ventilation, 8,9 it can, however, be lifesaving during attempts to establish an adequate airway. 10,11 We were interested in determining whether jet ventilation could be used outside the OR environment and whether sufficient working pressures could be obtained using equipment readily available outside the OR, such as oxygen flowmeters. Furthermore, we tested whether the anesthesia machines available in our clinical environment could act as a "high-pressure oxygen source" at sufficiently high pressures to drive a jet ventilator.…”