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Orthopaedic Manifestation of Bernard Soulier Disease and Management.
Case Report | Volume 4 | Issue 2 | JOCR April-June 2014 | Page 38-41 | Bisland SK, Smith FC. DOI: 10.13107/jocr.2250-0685.165
Authors: Bisland SK, Smith FC
 Division of Orthopaedic Surgery, McMaster University, Hamilton, Ontario L8L 2X2 Canada.
 Division of Orthopaedic Surgery, Juravinski Hospital, Hamilton, Ontario L8L 2X2 Canada.
Address of Correspondence:
Dr Stuart K Bisland, Division of Orthopaedic Surgery, McMaster University, Hamilton, Ontario L8L 2X2 Canada. Tel: 905 577 0639. Email: email@example.com
Introduction: The management of patients with coagulopathic disorders undergoing orthopaedic surgery requires a dedicated, multi-disciplinary team with detailed perioperative planning. Bernard-Soulier Syndrome (BSS) is an extremely rare disorder, affecting 1 in 1 million individuals worldwide. It is caused by a deficiency in glycoprotein 1b-V-IX which is required for normal platelet-mediated clot formation. The deficiency results in prolonged bleeding time with high risk of spontaneous bleeds. Few reports exist in the clinical literature of BSS patients undergoing major surgery.
Case Report: A 40 year old, female with known BSS and developmental dysplasia of her left hip (DDH) was referred to us for consideration of left total hip arthroplasty (THA). Consultation with her Haematologist for pre-operative optimization of platelets and related clotting times together with detailed discussions of her intended anaesthesia protocol and surgery resulted in a successful operation with less than anticipated blood loss. She entered our rehabilitation program just one week after surgery.
Conclusion: BSS is an extremely rare bleeding disorder that puts patients at very high risk of blood loss following surgery. This is the first report that we are aware of describing a BSS patient undergoing a THA. A cohesive, highly specialized, multi-disciplinary team is crucial to the success of these patients.
Keywords: Orthopaedic, Surgery, hip arthroplasty, Bernard-Soulier syndrome, platelets, coagulopathy, tranexamic acid.
|How to Cite This Article: Bisland SK, Smith FC. Total Hip Arthroplasty in A Young Patient with Bernard-Soulier Syndrome. Journal of Orthopaedic Case Reports 2014 April-June;4(2): 38-41. Available from: https://www.jocr.co.in/wp/2014/01/11/2250-0685-147-fulltext/|
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