Authors
Christopher O'Loughlin, Kirsten Fitzgerald, Emer Fitzpatrick, Andras Kelecsenyi
Published in
BMJ case reports. Volume 19. Issue 9. Sep 05, 2026. Epub Sep 05, 2026.
Abstract
X-linked erythropoietic protoporphyria (XLEPP) is a rare non-acute porphyria associated with severe photosensitivity and, in some patients progressive hepatobiliary disease. We describe the perioperative management of a middle-childhood-aged female with XLEPP, end-stage liver disease, portal hypertension-related thrombocytopenia and coagulopathy who required urgent dental extractions to facilitate transplant preparation. Key anaesthetic considerations included avoidance of phototoxic light exposure, safe drug selection, altered pharmacology in liver disease and bleeding risk. Surgery was performed under propofol-remifentanil total intravenous anaesthesia using the hospital's iLED 7 (Baxter Medical Systems GmbH + Co. KG, Saalfeld, Germany) light-emitting diode (LED) theatre lighting system, without additional optical filters or intraoperative light dosimetry. Additional precautions included avoidance of sunlight and fluorescent lighting, theatre-blind closure, occlusive coverings during transfer and recovery, coagulation optimisation, tranexamic acid and meticulous local haemostasis. The patient had no perioperative phototoxic injury, haemodynamic instability or red cell transfusion requirement and was discharged home the following day. This case highlights multidisciplinary planning in rare paediatric photosensitive liver disease.
PMID:
42700979
Bibliographic data and abstract were imported from PubMed on 06 Sep 2026.
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