Anaesthetic management of a patient with erythropoietic protoporphyria for ventricular septal defect closure.
Erythropoietic protoporphyria (EPP) is due to a deficiency in ferrochelatase required for haem synthesis. We describe the anaesthetic management of a seven-year-old with EPP undergoing closure of a haemodynamically significant ventricular septal defect. Photosensitivity in EPP patients is triggered at wavelengths near 400 nm and light-excited porphyrins generate free radicals and singlet oxygen that lead to erythrocyte deformity and haemolysis. Stimuli that could trigger a porphyric crisis during anaesthesia and surgery were reduced by avoiding exposure to the sensitive 400 nm spectrum and using light sources covered with yellow acrylate filters in the operating room. Anaesthetic agents not previously associated with porphyric crisis were chosen. Whole blood priming of the extracorporeal circuit was performed to ensure adequate haemoglobin concentrations during the perioperative period.
Department of Anesthesiology, Rush Medical College at Rush-Presbyterian-St. Luke's Medical Center, Chicago, IL 60612, USA.
B
B
Asokumar
C
C
Kierney
T W
TW
James
J
J
Amato
K J
KJ
Tumanmd
D000768:Anesthesia, General; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D018686:Anesthetics, Intravenous / Q000008:administration & dosage; D002315:Cardiopulmonary Bypass; D002648:Child; D004913:Erythrocytes, Abnormal / Q000473:pathology; D005283:Fentanyl / Q000008:administration & dosage; D005374:Filtration; D005609:Free Radicals / Q000378:metabolism; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006454:Hemoglobins / Q000032:analysis; D006461:Hemolysis; D006801:Humans; D007530:Isoflurane / Q000008:administration & dosage; D008029:Lighting / Q000009:adverse effects; D008297:Male; D010787:Photosensitivity Disorders / Q000209:etiology / Q000378:metabolism; D017121:Porphyria, Hepatoerythropoietic / Q000150:complications*; D046351:Protoporphyria, Erythropoietic; D017382:Reactive Oxygen Species / Q000378:metabolism
D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D005609:Free Radicals; D006454:Hemoglobins; D017382:Reactive Oxygen Species; D007530:Isoflurane; D005283:Fentanyl