Glanzmann's thrombasthenia: A nightmare for hernia surgeons.

Abstract

Abstract: Glanzmann's thrombasthenia is a rare inherited disorder affecting one in one million. It is characterised by a lack of platelet aggregation due to a defect in the platelet membrane receptor complex (αIIb/βIIIa), which mediates the aggregation of platelets at the site of vessel injury. We report here the first case of successful perioperative haemostatic management of a male patient with Glanzmann's thrombasthenia, who underwent an elective laparoscopic hernia repair. The patient was posted for elective surgery considering the availability of expertise in minimally invasive surgery and accessibility to recombinant activated factor VII. The patient was operated using the extended-view totally extraperitoneal technique for inguinal hernia repair. The patient recovered successfully and reported no complication during follow-up. We conclude that with proper perioperative haematological consultation, and careful coordination between anaesthetists and surgeons, elective laparoscopic procedure can be done in patients with Glanzmann's thrombasthenia, with only recombinant factor VIIa support.