Abstract
BACKGROUND: Tracheobronchomalacia (TBM) is a severe life-threatening perioperative complication. It is a rare condition caused by congenital and developmental anomalies of the trachea and/or the bronchus. It is often difficult to diagnose TBM before surgery as this congenital disease presents very few symptoms preoperatively and most often appears postoperatively. CASE PRESENTATION AND REVIEW: The study describes a case of cleft-lip-plate (CL/P) in a 7-month-old Japanese female with TBM and Tetralogy of Fallot syndrome. Before undergoing cleft-lip surgery, her TBM was not fully elucidated by preoperative examinations, and the operation was completed uneventfully. After the surgery, however, she started showing severe respiratory distress and developed hypoxia and bradycardia in the operating room. CPR was performed successfully, but a bronchoscopy revealed a severely collapsed airway, and the pathological condition was diagnosed as TBM occurred postoperatively. Eight months later, she died of sudden respiratory failure similar to that of the postoperative event caused by TBM. A literature review was conducted on the complications of CL/P from 1990 to 2017 in Japan. CONCLUSIONS: It was hypothesized that CL/P with congenital heart disease (CHD) and TBM with CHD may crossover in relatively high rates. Currently, there are very few solutions available to treat severe airway obstruction related to TBM. This highlights the need for preoperative diagnosis of TBM as an important step in overcoming severe airway complications.