Abstract
Proteus syndrome is a rare disorder of patchy or mosaic postnatal overgrowth, with only about 100 cases having been reported to date. We describe a case of progressive tracheobronchial stenosis induced by thoracic deformity and scoliosis which were caused by progressive overgrowth of vertebrae and back muscles with Proteus syndrome. An 11-year-old boy suddenly developed severe dyspnea during hospitalization for treatment for asthma, then he was intubated and on mechanical ventilation. Chest CT revealed severe stenosis of trachea and bilateral bronchi. Despite medical treatment he could not be weaned from ventilator. He underwent tracheotomy and additional intervention to the thoracic deformity, the Nuss procedure, to push the sternum ante-ward with a curved steel bar, and episternectomy. Although he could not be taken off the ventilator postoperatively, reduced support was feasible and he was transferred to the pediatric ward. Chest CT showed amelioration of the tracheobronchial compression. Approximately 2 months later, however, the thoracic deformity worsened and the bronchi became stenotic again. He ultimately died of pneumonia. Although tracheobronchial stenosis was improved by the surgical intervention, its effect did not last long.
