Hepatectomy in a patient with Erythropoietic Protoporphyria

Hepatectomy in a patient with Erythropoietic Protoporphyria

Published: 13 Nov 2017

© Anaesthesia Cases / 2017-0208 / ISSN 2396-8397

Authors

Dr Laura Peltola [1]
Dr Derek Amoako [2]
  • [1] Senior Registrar, Anaesthesia, Kings College Hospital, London, United Kingdom
  • [2] Consultant, Anaesthesia, Kings College Hospital, London, United Kingdom

Summary

We describe what we believe to be the first reported case of a modified left hepatectomy in a patient with the rare genetic condition, erythropoietic protoporphyria (EPP). Our patient was particularly vulnerable to photosensitivity, burns and liver damage secondary to EPP, further compounded by a complex and prolonged hepatic surgical procedure. Measures were taken to reduce the patient’s light exposure, including a single layer of yellow film omitting wavelengths <400nm secured to all theatre lights. Despite the surgery taking more than 8hrs, the patient did not sustain any photosensitivity, burns or motor neuropathies. Blood transfusions were used to reduce the drive for haem synthesis. Ursodeoxycholic acid was continued to increase biliary secretion of protoporphyrin, and hepatotoxic drugs were avoided. Liver function recovered well postoperatively and the patient was discharged home after nine days. 

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