Aorto-Tracheopexy in the Treatment of Hunter Syndrome

Whitney L Quong; Mazyar Kanani; Martin J Elliott

doi:10.1016/j.athoracsur.2014.08.048

Aorto-Tracheopexy in the Treatment of Hunter Syndrome

Ann Thorac Surg. 2015 Jun;99(6):2190-2. doi: 10.1016/j.athoracsur.2014.08.048.

Authors

Whitney L Quong¹, Mazyar Kanani², Martin J Elliott³

Affiliations

¹ The National Service for Severe Tracheal Disease in Children, The Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
² The National Service for Severe Tracheal Disease in Children, The Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
³ The National Service for Severe Tracheal Disease in Children, The Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom. Electronic address: martin.elliott@gosh.nhs.uk.

PMID: 26046872
DOI: 10.1016/j.athoracsur.2014.08.048

Abstract

Hunter syndrome is a multisystem genetic disease in which a significant proportion of morbidity and mortality arise from respiratory dysfunction. Notably, tracheal abnormalities in Hunter syndrome can compromise clinical stability, and management is primarily supportive. We describe here the first successful implementation of aorto-tracheopexy in a 19-year-old patient as a surgical strategy to resolve progressive respiratory deterioration.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Aorta, Thoracic / surgery*
Bronchoscopy
Humans
Male
Mucopolysaccharidosis II / diagnosis
Mucopolysaccharidosis II / surgery*
Plastic Surgery Procedures / methods*
Sternotomy / methods*
Tomography, X-Ray Computed
Trachea / surgery*
Vascular Surgical Procedures / methods*
Young Adult