Severe Multilevel Tracheal Stenosis with Significant Twisting in a Patient with Spondylometaphyseal Dysplasia: A Case Report

Author:

Stefko Jordan M.1,Oommen Abraham2,Jones Judith2,Arai Lynda2,Dicindio Sabina2,Theroux Mary2

Affiliation:

1. Department of Anesthesiology, Sidney Kimmel Medical College at Thomas Jefferson University Hospital, Philadelphia, Pennsylvania

2. Department of Anesthesiology, Nemours Children’s Health, Wilmington, Delaware.

Abstract

Spondylometaphyseal dysplasia (SMD) is a rare genetic disorder affecting skeletal growth and development presenting anesthesiologists with many perioperative challenges. We present a case of a patient found to have multilevel tracheal stenosis due to twisting and folding of his trachea. This was discovered on imaging during a research review of SMD cases at our institution. Structural and functional abnormalities of the trachea have not been reported in SMD. This is the first description of a patient with SMD with severe multilevel tracheal disease requiring tracheal reconstructive surgery.

Publisher

Ovid Technologies (Wolters Kluwer Health)

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