Idiopathic Subglottic Tracheal Stenosis in Identical Twin Sisters

Abbasi Dezfuli, Azizollah; Shadmehr, Mohammad Behgam; Sheikhy, Kambiz

doi:10.22038/ijorl.2018.31113.2061

Idiopathic Subglottic Tracheal Stenosis in Identical Twin Sisters

Document Type : Case Report

Authors

¹ Lung Transplantation Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.

² Tracheal Diseases Research Center (TDRC), National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.

10.22038/ijorl.2018.31113.2061

Abstract

Introduction:
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.

Case Report:
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.

Conclusion:
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.

Keywords

Main Subjects

Larynx

References

1. Brandenburg JH. Idiopathic subglottic stenosis. Trans Am Acad Ophthalmol Otolaryngol. 1972; 76(5):1402-6.

2. Walner DL, Stern Y, Gerber ME, Rudolph C, Baldwin CY, Cotton RT. Gastroesophageal reflux in patients with subglottic stenosis. Arch Otolaryngol Head Neck Surg. 1998 May; 124(5): 551-5

3. Damrose EJ. On the development of idiopathic subglottic stenosis. Med Hypotheses. 2008; 71(1):122.

4. Dumoulin E, Stather DR, Gelfand G, Maranda B, Maceachern P, Tremblay A. Idiopathic subglottic stenosis: a familial predisposition. Ann Thorac Surg. 2013 Mar; 95(3):1084-6.

5. Shabani S, Hoffman MR1, Brand WT1, Dailey SH. Endoscopic Management of Idiopathic Subglottic Stenosis. Ann Otol Rhinol Laryngol. 2017 Feb; 126(2):96-102.

6. Grillo HC, Mathisen DJ, Ashiku SK, Wright CD, Wain JC. Successful treatment of idiopathic laryngotracheal stenosis by resection and primary anastomosisAnn Otol Rhinol Laryngol. 2003 Sep; 112(9 Pt 1):798-800.

7. Costantino CL, Mathisen DJ. Idiopathic laryngotracheal stenosis. J Thorac Dis. 2016 Mar; 8(Suppl 2): S204-9.