Etiology of Airway Diseases Requiring Reconstruction and the Registry of Patients with Intractable Diseases

Abstract

Airway diseases can be life-threatening and lower the quality of life of the patients. Airway reconstruction after resection of malignancies or stenotic lesions remains a difficult surgical challenge. The etiologies of airway diseases, including congenital, traumatic, inflammatory and tumorous lesions, are described in this article. At present, congenital subglottic stenosis/tracheal stenosis are officially considered as intractable diseases in Japan. Conventionally, reconstruction for large airway defects is performed using several kinds of cartilage grafts. In most cases, repeated surgeries at multiple sites are necessary, making it considerably invasive for the patients. Moreover, even with such invasive treatment, the tracheal stoma may have to remain in several patients because of unstable grafts or airway re-stenosis. As a new perspective, in situ tissue regeneration-inducing artificial trachea may significantly contribute to effective airway reconstruction.

The diagnosis procedure combination（DPC）database was utilized for estimating the number of patients who undergo tracheoplasty and related procedures. In the database, thyroid cancer with tracheal invasion accounts for the highest proportion of patients undergoing these operative procedures.

The Rare Disease Data Registry of Japan (RADDAR-J) has constructed a system to establish a sustainable framework that provides an environment where participation is significantly beneficial for all individuals concerned with and involved in the treatment of intractable diseases, such as researchers, physicians, and patients.

A pharyngeal, laryngeal and tracheal stenosis patient registry is under construction, based on RADDAR-J. This will serve as a reference source for the various evidences of rare diseases.