A case of perioperative management in a patient with congenital protein C deficiency and repeating purpura fulminans since the neonatal period

Abstract

Severe congenital protein C (PC) deficiency, one of the autosomal dominant diseases, causes purpura fulminans (PF) during the neonatal period. These patients usually receive long-term anticoagulant therapy to prevent thrombosis, therefore we should pay attention to both thrombotic tendency and hemorrhagic diathesis when performing surgical treatment. We report a case of perioperative management of a tooth extraction in a patient with severe congenital PC deficiency who has repeatedly developed PF since the neonatal period. A 20-year-old woman, previously diagnosed with severe congenital PC deficiency, was referred to our department for extraction of the left mandibular first and second molars with severe caries. We planned to carry out the tooth extraction without suspending anticoagulant therapy, with the attending physician in charge of hematology. The patient underwent the tooth extraction with sufficient local hemostasis. She left hospital 4 days after surgery without any complications. However, she was readmitted two days after discharge because of post-extraction bleeding. The bleeding was easily stopped with local hemostatic treatment, however purpura developed on her right leg. It was treated with antithrombotic therapy and gradually disappeared without necrosis.