Abstract

The Bernard-Soulier syndrome also known as hemorrhagioparous thrombocytic dystrophy, is a rare autosomal recessive bleeding disorder. Macrothrombocytopenia and increased bleeding time are the two main features of this syndrome. There is a reduced adherence of giant platelets to the subendothelium due to a deficiency of glycoprotein GPIb-V-IX complex. Routine dental extraction in a patient with a bleeding disorder can lead to life-threatening complications and there are very few specific protocols for the same. Here, we present a case report describing haemostasis management during dental extraction in a 7-year-old female child, suffering from this syndrome. It was observed that the preoperative and postoperative human leucocyte antigen-matched platelet transfusion, systemic intraoperative tranexamic acid and minimally traumatic procedure improved bleeding control and management.