Perioperative management of a child with X-linked erythropoietic protoporphyria undergoing dental surgery in preparation for liver transplantation.
Christopher O'Loughlin, Kirsten Fitzgerald, Emer Fitzpatrick et al.
✦ AI-curated · Sources linked
Case in brief
A middle-childhood-aged female with X-linked erythropoietic protoporphyria (XLEPP) and end-stage liver disease required urgent dental extractions for liver transplantation preparation. Key perioperative management included avoiding phototoxic light exposure and addressing coagulopathy. The procedure was successfully performed under total intravenous anaesthesia with careful monitoring, resulting in no complications, and the patient was discharged the next day.
What made this case unusual
The case emphasizes the unique challenges of managing a patient with XLEPP undergoing surgery, particularly the need for specialized lighting and meticulous haemostasis to prevent complications.
Diagnostic / clinical pearl
Clinicians should prioritize multidisciplinary planning and specific environmental controls when managing patients with photosensitivity during surgical procedures.
Why it matters
This case underscores the importance of tailored anaesthetic strategies in rare conditions, enhancing safety and outcomes in complex surgical scenarios.