Glanzmann’s thrombasthenia: A nightmare for hernia surgeons

Author:

Gandhi Jignesh1,Gajjar Aarsh2,Shinde Pravin1,Takalkar Yogesh1

Affiliation:

1. Department of General Surgery, Seth GSMC and KEM Hospital, Mumbai, Maharashtra, India

2. Intern Doctor, Seth GSMC and KEM Hospital, Mumbai, Maharashtra, India

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.

Publisher

Medknow

Reference11 articles.

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3. Simultaneous occurrence of intussusception and duodenal haematoma in a patient with Glanzmann's thrombasthenia;Jan;Afr J Paediatr Surg,2016

4. Poon M-C Successful Use of Recombinant Factor VIIA (rFVIIa) For Surgical Hemostasis in A Boy with Glanzmann Thrombasthenia;Wu;Pediatr,1999

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