Anesthetic management of a patient with dyskeratosis congenita undergoing head and neck tumour dissection
Vol 5 | Issue 2 | May-Aug 2019 | page: 16-19 | M.J. Wong, C. Wong, B. Macaulay
Authors: M.J. Wong , C. Wong , B. Macaulay 
 Department of Anesthesia, Pain Management & Perioperative Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.
Address of Correspondence
Dr. Mike Wong,
Department of Anesthesia, Pain Management & Perioperative Medicine, Dalhousie University,
Victoria General Hospital, 1276 South Park (10th Floor), Halifax, Nova Scotia, Canada, B3H 2Y9
Introduction: We present a patient with dyskeratosis congenita presenting for resection of a tongue base tumour. Dyskeratosis congenita is a rare genetic disorder caused by abnormal maintenance of chromosome telomere regions and is associated with multi-organ dysfunction. These considerations may impact peri-operative care, including pre-operative optimization, airway management, and choice of anaesthetic. There is very scant literature focused on anaesthetic management of this rare condition, and this is the first case report describing anaesthetic care for a patient with dyskeratosis congenita undergoing head and neck tumour resection.
Case presentation: A 54-year-old Caucasian male presented for resection of a locally-advanced oral tumor.He had liver disease and bone marrow failure as complications of dyskeratosis congenita. We managed his care with a multidisciplinary approach, and he recovered with an uncomplicated perioperative course.
Conclusion: Successful execution of this case depended on careful planning. There are only four case reports regarding anaesthetic management of patients with dyskeratosis congenita, and most of them only described ambulatory dental procedures.This case report further characterizes the preparation required in caring for these patients in the context of major surgery. Given the miniscule literature concerning perioperative management of patients with dyskeratosis congenita, we have also reviewed and summarized the anaesthetic considerations for this rare disease and its multi-system manifestations.
Keywords: Dyskeratosis Congenita; Anesthesia, General; Perioperative Care.
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|How to Cite this Article: Wong M J, Wong C, Macaulay B. Anesthetic management of a patient with dyskeratosis congenita undergoing head and neck tumour dissection. Journal of Anaesthesia and Critical Care Case Reports May-Aug 2019; 5(2): 16-19.|
.Tags: Dyskeratosis Congenita; Anesthesia, General; Perioperative Care.