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.
- dentistry and oral medicine
- congenital disorders
- haematology (incl blood transfusion)
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Contributors RKC and RK allotted the patient, of a rare disease after diagnosis to SS. The treatment procedure was performed by SS under the guidance of RKC and RK.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
Patient consent for publication Parental/guardian consent obtained.
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