Glanzmann’s thrombasthenia: A nightmare for hernia surgeons

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.

INTRODUCTION

Glanzmann’s thrombasthenia (GT) is a rare inherited disorder, affecting one in one million,[1] and 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.[2] After doing an extensive literature review, we found only a few reports concerning laparoscopy in patients with GT[3,4] and only two regarding inguinal hernia repair in patients with GT.[1,5] There have been no previous reports of laparoscopic inguinal repair in patients with GT, making this report the first of its kind. Preoperative multidisciplinary team approach between anesthetist, hematologist and surgeon is essential, and a meticulous approach to intraoperative hemostasis is mandatory. These patients may require an extended postoperative stay in spite of the minimal invasive approach to ensure return to normal physiology.

CASE REPORT

We present here a report of successful perioperative haemostatic management of a male patient with GT, who underwent an elective laparoscopic hernia repair. A 49-year-old male patient who was a known case of GT presented to our department, diagnosed with a right-sided inguinal hernia. He had a history of recurrent bleeding from gums, and his last episode of unprovoked bleeding was 7 years ago. The patient has a history of receiving 4 units of random donor platelets in the past. He had previously undergone septoplasty 15 years back, with profuse bleeding during surgery, which was managed with nasal packing.

Pre-operative laboratory investigations were within normal limits. Our pre-operative protocol for the case, in conjunct with haematology experts in our institute, was as follows:

1. Infusion of one unit of human leucocyte antigen (HLA)-compatible platelet concentration on arrival to the operating room. The risk of the development of platelet antibodies with platelet concentrate treatment was taken into consideration

2. A single intravenous (i.v.) bolus of 4 mg recombinant activated factor VII (rFVIIa) (NovoSeven; Novo Nordisk A/S, Bagsværd, Denmark) was given just before intubation, as per haematology guidance

3. Thromboprophylactic medications were avoided.

General anaesthesia was used for the surgical procedure. The patient was operated on using the extended-view totally extraperitoneal (eTEP) technique for inguinal hernia repair, as proposed by Professor Jorge Daes.[6] Intraoperatively, keeping in mind the anatomical landmarks and planes, dissection was continued between the two layers of fascia transversalis as it is an avascular plane. Care was taken to not disturb the cover of fat on the iliac and inferior epigastric vessels. Adequate haemostasis, whenever necessary, was achieved using a bipolar electrosurgical unit. Novel use of neurosurgical patties was done for blunt dissection and to achieve haemostasis. Patties are more effective than gauze to achieve haemostasis, and because they are smaller and softer, they can be easily introduced through the 5-mm working ports, which avoid the need for a 10-mm port and thus reduce skin incision size, which is preferable to avoid bleeding complications. The surgical procedure was uneventful, with minimal intraoperative blood loss.

Postoperatively, the hernia site was given an external compression with gamjee roll. The patient had an uneventful recovery but was admitted for a prolonged period for observation.

DISCUSSION

A meticulous review of the literature reveals a lack of consensus regarding the optimal protocol for surgery in patients with GT.[7] The use of platelet-rich concentrates (PRCs) and rFVIIa for achieving haemostasis has been described, but treatment protocols vary concerning optimal dose, duration and timing of administration. By far, the standard therapy for surgical support in GT is platelet transfusion. However, recurrent platelet transfusions are associated with the development of antibodies to GPIIb-IIIa and/or HLA, leading to refractoriness to platelet transfusion.[3] This has to be taken into account in emergency treatment. In such cases, the proposed guidelines are to do the procedure under rFVIIa and PRC.[8] rFVIIa promotes haemostasis by increasing platelet function via induction of thrombin, thus bypassing factor VIIa and IXa, thus making it useful in patients with coagulation cascade defects (such as haemophilia) and platelet disorders such as GT and Bernard–Soulier syndrome.[9]

Elective inguinal hernia repair is recommended, even for asymptomatic hernias, to prevent visceral incarceration and subsequently ischaemia (strangulation). It is considered a safe procedure with a low morbidity and mortality rate compared to emergency situations.[10,11] The utility of prosthetic placement in emergency surgeries is also debatable. In literature, open repairs of inguinal hernia were found.[1,5] However, the patient insisted on laparoscopic repair, and since we had the availability of expertise in minimally invasive surgery as well as haematology, laparoscopic repair of the hernia was done. Pre-operative clinical evaluation showed the distance between the umbilicus and the pubic symphysis to be 10 cm, thus eTEP was preferred over the TEP approach. With expertise, high-definition cameras and a larger field of vision due to the eTEP technique, better confirmation of haemostasis is achieved. Minimally invasive procedures in patients with GT, apart from well-known benefits such as reduced post-operative pain and fewer complications, also have the added benefit of minimal bleeding.

CONCLUSION

With proper perioperative haematological consultation, we tried laparoscopic surgery on this patient with GT. No complications were observed, intraoperatively as well as postoperatively; however, a large volume study is required to confirm the acceptability of laparoscopic surgeries in patients with GT.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.