Glanzmann's thrombasthenia: A nightmare for hernia surgeons.

Area of Science:

• Hematology
• Surgical Oncology

Background:

• Glanzmann's thrombasthenia is a rare inherited bleeding disorder affecting platelet aggregation due to a defective platelet membrane receptor complex (αIIb/βIIIa).
• Managing hemostasis in patients with Glanzmann's thrombasthenia undergoing surgery presents significant challenges due to impaired platelet function.

Purpose of the Study:

• To report the first successful perioperative hemostatic management of a Glanzmann's thrombasthenia patient undergoing elective laparoscopic hernia repair.
• To demonstrate the feasibility of minimally invasive surgery in patients with rare bleeding disorders with appropriate medical support.

Main Methods:

• The patient with Glanzmann's thrombasthenia underwent an elective laparoscopic inguinal hernia repair using the extended-view totally extraperitoneal technique.
• Perioperative hemostatic management was achieved with recombinant activated factor VIIa (rFVIIa) support.
• Close collaboration between hematologists, anesthesiologists, and surgeons was crucial for successful outcomes.

Main Results:

• The laparoscopic hernia repair was successfully completed without any intraoperative or postoperative complications.
• The patient experienced an uneventful recovery and showed no signs of bleeding during the follow-up period.
• rFVIIa effectively обеспечил hemostasis throughout the surgical procedure.

Conclusions:

• Elective laparoscopic procedures are feasible in patients with Glanzmann's thrombasthenia.
• Careful perioperative hematological consultation and coordination between surgical and anesthesia teams are essential.
• Recombinant activated factor VIIa provides effective hemostatic support for managing Glanzmann's thrombasthenia during elective minimally invasive surgery.