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Cardiopulmonary bypass in a child with erythropoietic protoporphyria
Lydia Pathman1, Martin Bennett1, Yves d'Udekem1,2
1The Royal Children's Hospital, Melbourne, Australia.
Insights
Children with erythropoietic porphyria (EP) face surgical risks from photosensitivity. This case study highlights using exchange transfusion and other methods to prevent photosensitivity-induced hemolysis during major surgery in an EP patient.
Area of Science:
- Pediatric Dermatology
- Hematology
- Metabolic Disorders
Background:
- Erythropoietic porphyria (EP) is a rare genetic disorder characterized by extreme photosensitivity.
- Children with EP typically receive care from pediatric dermatologists.
- Major surgery in EP patients poses risks of photosensitivity-induced complications, particularly hemolysis.
Observation:
- A pediatric patient diagnosed with erythropoietic porphyria (EP) required open heart surgery.
- The surgical team sought dermatological advice to manage photosensitivity risks.
- Standard anesthetic and surgical protocols needed modification to mitigate light exposure.
Findings:
- The case involved implementing an exchange transfusion prior to surgery.
- Additional strategies were employed to minimize light exposure during the procedure.
- These interventions aimed to prevent hemolysis triggered by photosensitivity.
Implications:
- This approach demonstrates a viable strategy for managing EP patients undergoing major surgery.
- It underscores the importance of multidisciplinary collaboration between dermatology, hematology, and surgical teams.
- Successful management can reduce the risk of severe complications in EP patients requiring surgical intervention.
Abstract:
Children with erythropoietic porphyria are generally under the care of paediatric dermatologists. When these children undergo major surgery, they are at risk of unusual complications due to their photosensitivity. Dermatologists may be consulted prior to surgery for advice. We describe a case of a child with erythropoietic porphyria undergoing open heart surgery, utilising an exchange transfusion alongside other strategies to minimise the risk of photosensitivity-induced haemolysis.
