The management of thoracic inlet syndrome associated with Hurler's syndrome: a novel surgical technique.
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.
Department of Cardiothoracic Surgery, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
Rauf M
RM
Ahsan
Sarah A
SA
Early
Anne
A
O'Meara
Lars
L
Nölke
D000402:Airway Obstruction / Q000209:etiology; D016025:Bone Transplantation / Q000379:methods; D003251:Constriction, Pathologic / Q000150:complications / Q000601:surgery; D019299:Decompression, Surgical / Q000379:methods; D006801:Humans; D007601:Jugular Veins / Q000473:pathology / Q000601:surgery*; D008260:Macroglossia / Q000209:etiology; D008297:Male; D008371:Manubrium / Q000601:surgery; D008059:Mucopolysaccharidosis I / Q000601:surgery*; D013901:Thoracic Outlet Syndrome / Q000601:surgery*; D055815:Young Adult