Glanzmann thrombasthenia and its perioperative management in head trauma: A rare and challenging situation

Neeraj Kumar; Abdul A. Khan; Nilesh Kumar; Ashish Bindra; Charu Mahajan; Keshav Goyal

doi:10.4103/2348-0548.173235

Journal of Neuroanaesthesiology and Critical Care, Table of Contents

CC-BY-NC-ND 4.0 · J Neuroanaesth Crit Care 2016; 03(01): 052-055
DOI: 10.4103/2348-0548.173235

Case Report

Thieme Medical and Scientific Publishers Private Ltd.

Glanzmann thrombasthenia and its perioperative management in head trauma: A rare and challenging situation

Neeraj Kumar

Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences and Associated Jai Prakash Narain Apex Trauma Centre, New Delhi, India

,

Abdul A. Khan

Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences and Associated Jai Prakash Narain Apex Trauma Centre, New Delhi, India

,

Nilesh Kumar

¹Department of Anaesthesiology, All India Institute of Medical Sciences and Associated Jai Prakash Narain Apex Trauma Centre, New Delhi, India

,

Ashish Bindra

Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences and Associated Jai Prakash Narain Apex Trauma Centre, New Delhi, India

,

Charu Mahajan

Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences and Associated Jai Prakash Narain Apex Trauma Centre, New Delhi, India

,

Keshav Goyal

Department of Neuroanaesthesiology and Critical Care, All India Institute of Medical Sciences and Associated Jai Prakash Narain Apex Trauma Centre, New Delhi, India

› Author Affiliations

Abstract

Glanzmann thrombasthenia is a rare inherited coagulation disorder, manifesting itself in early childhood as bleeding episodes. The patients with this disease may present with uncontrollable bleeding following trauma. We report a patient of Glanzmann syndrome with head injury, who presented to us following fall from stairs. Platelet transfusion is the mainstay of treatment in emergency situations. As patients generally have a history of platelet transfusion they may be refractory to the same. Leuko reduced or human leukocyte antigen matched platelets should be used. Recombinant factor VIIa can be used to control bleeding in refractory cases and in patients with antibodies against platelets. Anti-fibrinolytics can also be used as adjuvants. There is need of advanced perioperative coagulation screening spot tests in emergency trauma settings as routine coagulation tests may not be able to pick up the abnormality. Thromboelastography/thromboelastometry can be a great tool for perioperative management of such coagulation disorders.

Keywords

Anaesthesia - glanzmann thrombasthenia - head injury - perioperative - platelets - thromboelastography

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References

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