A Case of Acute Appendicitis with Erythropoietic Protoporphyria in which Elective Surgery was Performed Using a Special Film

Abstract

Erythropoietic protoporphyria (EPP) is a rare inherited disorder of heme synthesis. We report a case of acute appendicitis in a 21-year-old female patient with EPP. She was diagnosed with EPP in early childhood ; it was associated with photosensitivity of the skin and a high risk of causing visceral damage. Thereafter she had been followed up. Recently she presented with upper abdominal pain and was diagnosed with acute appendicitis. Following conservative treatment for several days, she was admitted to our hospital for the purpose of undergoing surgery. In the perioperative period, we performed light wavelength measurements to avoid the wavelength that might cause phototoxic reactions. During appendectomy under laparotomy, we covered surgical lights and headlights with a special film to prevent the harmful wavelength causing photosensitivity. After the surgery, no emerging complications happened. She was discharged on the third hospital day. Meticulous perioperative management using a special film ensured a safe surgery for the patient with EPP.