Abstract

Abstract Protein C deficiency is a rare genetic disease that predisposes to thrombotic disease, which is characterized by tendency to form thrombus due to hypercoagulation. The incidence of protein C deficiency with clinical symptoms has been reported to be estimated at 1 in 20,000. Only a few cases of surgery for gastrointestinal cancer with protein C deficiency have been reported, however, there have been no reports regarding the perioperative management in detail for those patients. Methods We present. We assess perioperative management for the case of esophagectomy for an esophageal cancer accompanied with protein C deficiency. Results A 63-year-old male with protein C deficiency underwent thoracoscopic esophagectomy and digestive reconstruction using gastric tube for thoracic esophageal cancer whose clinical stage was T1bN0M0. On postoperative day 3, his gastric tube was removed due to gastric tube necrosis. Digestive reconstruction using free jejunal graft has been attempted after 140 days from first surgery, however, thrombus formation in the jejunal artery and vein caused the reconstruction to failure. Ten days after this surgery, digestive reconstruction using colon was performed with anticoagulation control by administrating intraoperative heparin. The patient went fine without thrombus formation afterward. Conclusion During digestive reconstruction in patients with conditions predisposing to thrombus formation as typified by protein C deficiency, the perioperative management should be done with careful attention to prevent thrombus formation. In particular, because there is likely to develop necrosis of reconstructed organ due to thrombus formation, the appropriate anticoagulation control such as intraoperative heparin administration is recommended in patients with protein C deficiency.

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