{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T13:11:36Z","timestamp":1788613896520,"version":"build-2803163510"},"reference-count":10,"publisher":"BMJ","issue":"9","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,8,18]]},"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:p>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\u2013remifentanil total intravenous anaesthesia using the hospital\u2019s 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.<\/jats:p>","DOI":"10.1136\/bcr-2026-272843","type":"journal-article","created":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T12:15:19Z","timestamp":1788610519000},"page":"e272843","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Perioperative management of a child with X-linked erythropoietic protoporphyria undergoing dental surgery in preparation for liver transplantation"],"prefix":"10.1136","volume":"19","author":[{"given":"Christopher","family":"O\u2019Loughlin","sequence":"first","affiliation":[{"name":"Department of Anaesthesia, Children\u2019s Health Ireland at Crumlin Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirsten","family":"Fitzgerald","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Childrens Health Ireland at Crumlin Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emer","family":"Fitzpatrick","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology, Childrens Health Ireland at Crumlin Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andras","family":"Kelecsenyi","sequence":"additional","affiliation":[{"name":"Department of Anaesthesia, Children\u2019s Health Ireland at Crumlin Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,9,5]]},"reference":[{"key":"2026090505150921000_19.9.e272843.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1608634"},{"key":"2026090505150921000_19.9.e272843.2","doi-asserted-by":"crossref","DOI":"10.12688\/f1000research.10101.1","article-title":"An overview of the cutaneous porphyrias","volume":"6","author":"Dawe","year":"2017","journal-title":"F1000Res"},{"key":"2026090505150921000_19.9.e272843.3","unstructured":"Balwani M , Desnick R . Porphyrias Consortium of the NIH-Sponsored Rare Diseases Clinical Research Network. X-Linked Protoporphyria. GeneReviews [Internet]. Seattle; 1993\u20132026. Seattle (WA): University of Washington, 2019."},{"key":"2026090505150921000_19.9.e272843.4","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v16.i36.4526"},{"key":"2026090505150921000_19.9.e272843.5","doi-asserted-by":"publisher","DOI":"10.1046\/j.1460-9592.1999.00338.x"},{"key":"2026090505150921000_19.9.e272843.6","doi-asserted-by":"crossref","first-page":"566","DOI":"10.1111\/ced.12188","article-title":"Development of acute phototoxic reaction during surgery in a patient with erythropoietic protoporphyria","volume":"38","author":"Graham-Brown","year":"2013","journal-title":"Clin Exp Dermatol"},{"key":"2026090505150921000_19.9.e272843.7","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1016\/j.transproceed.2019.12.004","article-title":"Successful Liver Transplantation for Liver Failure With Erythropoietic Protoporphyria by Covering the Operating Theater Lights With Polyimide Film: A Case Report","volume":"52","author":"Hanaki","year":"2020","journal-title":"Transplant Proc"},{"key":"2026090505150921000_19.9.e272843.8","doi-asserted-by":"publisher","DOI":"10.1002\/lt.21527"},{"key":"2026090505150921000_19.9.e272843.9","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1016\/j.jaad.2022.08.036","article-title":"Evidence-based consensus guidelines for the diagnosis and management of erythropoietic protoporphyria and X-linked protoporphyria","volume":"89","author":"Dickey","year":"2023","journal-title":"J Am Acad Dermatol"},{"key":"2026090505150921000_19.9.e272843.10","doi-asserted-by":"publisher","DOI":"10.1093\/bjaceaccp\/mkp040"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2026-272843","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T12:15:20Z","timestamp":1788610520000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2026-272843"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9]]},"references-count":10,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2026,9,5]]},"published-print":{"date-parts":[[2026,9]]}},"alternative-id":["10.1136\/bcr-2026-272843"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2026-272843","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9]]}}}