Pancreaticoduodenectomy in a patient with protein C deficiency

Abstract

Protein C (PC) deficiency is a rare disease with an incidence of 0.2 % in the general population. Because PC deficiency is likely to be thrombogenic, particularly in the venous system, continuous anticoagulant therapy is required, and when undergoing surgical treatment, such patients are considered a high-risk group. To our knowledge, the current case is the first report of successful pancreaticoduodenectomy for a patient with PC deficiency. A 46-year-old man with PC deficiency was referred to our hospital for cancer of the head of the pancreas. He had been managed with oral warfarin and baby-strength aspirin to prevent thrombotic disorders. A right adrenal pheochromocytoma was also diagnosed. Laparoscopic adrenalectomy was first performed. In spite of strict perioperative management with administration of heparin, deep venous thrombosis developed in the right femoral vein on postoperative day 8. On elective pancreaticoduodenectomy 19 days after the adrenalectomy, taking into account the postoperative course after the urological surgery, perioperative management of anticoagulant therapy was performed as follows: (1) preoperative intravenous administration of heparin up to 6 h before surgery; (2) on the day of the surgery, the heparin administration was discontinued, and dried concentrated human activated PC formulation (APC) was started intravenously from 6 h before the surgery at a dose of 210 U/kg/day. (3) Postoperatively, after the confirmation of hemostasis, the heparin administration was resumed on day 2. APC administration was discontinued as soon as the ongoing administration of APC was completed on day 2. During operation, no abnormal findings pertaining to hemostasis or thrombosis were observed. On his postoperative course, no new thrombus formation was identified. For patients with PC deficiency, there has been no clear standardized regimen for antithrombotic prophylaxis in surgical procedures. The prophylactic administration of APC might ensure a higher level of safety than that offered by surgery with the intraoperative administration of heparin or without the intraoperative administration of anticoagulants.