Idiopathic Subglottic Tracheal Stenosis in Identical Twin Sisters

Idiopathic subglottic tracheal stenosis is a rare inflammatory disease of the trachea; most commonly affects females within the age range of 20-50 years. No etiologic factor has yet been identified for this rare tracheal disease and therefore it should be diagnosed after the exclusion of other inflammatory, traumatic, and autoimmune diseases of the trachea. The familial or genetic predisposition to this disease is still unknown although one published report in the literature showed some familial predisposition.

A 41-year old woman presented with progressive dyspnea and stridor. The bronchoscopic evaluation revealed subglottic tracheal stenosis; however, there was no significant etiology of this disease after complete evaluations. Therefore, the idiopathic subglottic stenosis was the final diagnosis. After two years, her identical twin sister presented with the same signs and symptoms. There was also no etiology for her tracheal stenosis. The first patient was managed surgically through cricotracheal resection. However, the second sister didn't need surgical resection due to the mild to moderate tracheal stenosis.

The obtained results of our cases along with the previously reported family cases can potentiate the hypothesis that there is some genetic predisposition to the development of this disease.