Perioperative management of a bleeding jejunal tumor in a patient with erythropoietic protoporphyria: A case report and literature review

INTRODUCTION: Erythropoietic protoporphyria (EPP) is a rare disorder caused by reduced ferrochelatase activity and shows incomplete autosomal dominant inheritance. Meticulous perioperative management can avoid characteristic complications. This report describes a case of a bleeding jejunal tumor in a patient with EPP. PRESENTATION OF CASE: A 49-year-old man with a history of EPP was admitted to our department with abdominal distention and severe anemia. Contrast-enhanced computed tomography revealed an abdominal tumor measuring 5 cm, originating from the small bowel wall or mesentery. Tumor resection was planned after correction of anemia. Red blood cell transfusion restored his hemoglobin to acceptable levels; however, his liver function worsened. Institution of liver support therapy achieved gradual reduction in his elevated liver enzymes; however, hyperbilirubinemia persisted. He underwent tumor resection on the 12(th) day of hospitalization. Yellow filters were used to avoid operating room light-induced tissue injury. The tumor was located in the jejunum 30 cm from the Treitz ligament toward the anal aspect. The histopathological diagnosis was desmoid-type fibromatosis of the jejunum. Postoperatively, his hemoglobin levels were stabilized; however, his serum bilirubin level remained high. His serum bilirubin level gradually decreased following hemin injections (150 mg/day). DISCUSSION: Reducing heme synthesis and minimizing protoporphyrin generation are important perioperatively. Additionally, preventing operating room light-induced tissue burns and selecting appropriate anesthestic agents are important during surgery. CONCLUSION: The institution of appropriate treatment and adequate intra- and perioperative measures can ensure safe surgery in patients with EPP even under emergency conditions.