The management of thoracic inlet syndrome associated with Hurler's syndrome: a novel surgical technique.
Ahsan, Rauf M Early, Sarah A O'Meara, Anne Nolke, Lars
Ahsan, Rauf M
Early, Sarah A
O'Meara, Anne
Nolke, Lars
Advisors
Editors
Other Contributors
Date
2012-02-01T10:24:47Z
Date Submitted
Keywords
Other Subjects
Subject Mesh
Airway Obstruction/etiology
Bone Transplantation/methods
Constriction, Pathologic/complications/surgery
Decompression, Surgical/methods
Humans
Jugular Veins/pathology/*surgery
Macroglossia/etiology
Male
Manubrium/surgery
Mucopolysaccharidosis I/*surgery
Thoracic Outlet Syndrome/*surgery
Young Adult
Bone Transplantation/methods
Constriction, Pathologic/complications/surgery
Decompression, Surgical/methods
Humans
Jugular Veins/pathology/*surgery
Macroglossia/etiology
Male
Manubrium/surgery
Mucopolysaccharidosis I/*surgery
Thoracic Outlet Syndrome/*surgery
Young Adult
Planned Date
Start Date
Collaborators
Principal Investigators
Alternative Titles
Publisher
Abstract
A 21-year-old male developed significant swelling of his tongue after a respiratory arrest. The patient had a history of Hurler's syndrome. Magnetic resonance imaging (MRI) angiogram delineated that the swelling was due to compression of his internal jugular veins at the level of the first rib, resulting in thoracic inlet obstruction. The standard surgical treatment of thoracic inlet obstruction was not suitable in this patient's case due to his short thick neck and his characteristic Hurler's syndrome body habitus. Therefore, a novel surgical strategy was used to decompress his head and neck vessels. The manubrium was widened using an iliac crest bone graft, stabilised using internal fixation plates and reconstructed with a pectoral muscle flap.
Language
eng
ISSN
1873-734X (Electronic)
1010-7940 (Linking)
1010-7940 (Linking)
eISSN
ISBN
DOI
10.1016/j.ejcts.2009.05.055
PMID
19864154