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

Yuki Takemoto; Shoichiro Mukai; Tetsuya Mochizuki; Masatoshi Kochi; Hiroyuki Egi; Hideki Ohdan

doi:10.1016/j.ijscr.2019.03.048

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

Int J Surg Case Rep. 2019:60:191-195. doi: 10.1016/j.ijscr.2019.03.048. Epub 2019 Apr 5.

Authors

Yuki Takemoto¹, Shoichiro Mukai², Tetsuya Mochizuki¹, Masatoshi Kochi¹, Hiroyuki Egi¹, Hideki Ohdan¹

Affiliations

¹ Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8551, Japan.
² Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8551, Japan. Electronic address: mukais@hiroshima-u.ac.jp.

Abstract

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.

Keywords: Anesthesia; Erythropoietic protoporphyria; Hemin; Surgery.