Congenital Tracheal Stenosis with Unilateral Pulmonary Agenesis

Abstract

Cogenital tracheal stenosis with unilateral pulmonary agenesis is a rate and frequent fetal combination. In an 8-year period, 5 infants (ages 2 to 6 months) with these anomalies were treated. The presenting signs and symptoms consisted of wheezing, stridor, and tachypnea and included frank respiratory failure requiring emergency therapy in several patients. The operative repair consisted of segmental resection and anastomosis in one patient, and rib-cartilage tracheoplasty in the other four. Two infants died, one of cerebral hypoxia, and the other of aortotracheal fistula. Long-term follow-up in the three survivors is satisfactory.

Surgical outcome of slide tracheoplasty in patients with long congenital segment tracheal stenosis and single lung

“…The presence of LCSTS and single lung or severe hypoplasia of one lung is a rare association connected with a high mortality (18-65%) [6][7][8]. Backer and associates reviewed the literature and reported a total of 28 patients with functional single lung [8], forming 10-20% of the total population of patients with LCSTS undergoing surgical repair.…”

“…The association of single lung with LCSTS and other anomalies, such as cardiovascular, gastrointestinal or genetic, is not uncommon [5,8,[10][11].…”

“…One such extreme variant is LSCTS associated with agenesis or severe hypoplasia of one lung [6]. Very few articles have documented this association, and these report a mortality ranging from 18% to 65% [7][8][9].…”

Perioperative extracorporeal membrane oxygenation support for critical pediatric airway surgery

“…For pediatric critical airway stenosis, fiberoptic bronchoscopy may cause acute ventilation failure because it might completely obstruct the very narrow airway or induce bronchospasm [13]. Additionally, to facilitate the procedure, sedatives or muscle relaxants are sometimes needed, but both cause respiratory depression.…”

Extracorporeal Membrane Oxygenation, Extubation, and Lung-Recruitment Maneuvers as Rescue Therapy in a Patient With Tracheal Dehiscence Following Slide Tracheoplasty

“…4 Combined, unilateral lung agenesis and long-segment congenital tracheal stenosis is a rare condition with a high mortality rate. 5,6 Long-segment congenital tracheal stenosis requires surgical repair; the options include pericardial tracheoplasty, tracheal autograft, tracheal resection, and/or slide tracheoplasty. Slide tracheoplasty is advantageous because it avoids the use of graft material, has a high early-extubation success rate, and is associated with better long-term out-comes.…”