Perioperative management of porphyria: a case report

Abstract

The porphyrias are rare inherited meta-bolic disorders of the heme biosynthesis pathway. Acute intermittent porphyria is the most common form that may result in acute porphyric crises with abdominal pain, vomiting, hemodynamic distur-bances, autonomic dysfunction, pyrexia and neurological deterioration. Provoca-tive factors include hormonal fluctuations, fasting, dehydration, smoking, excessive alcohol or illegal drugs intake and stress from illness or surgery. However, the most frequent triggers are cytochrome P450-in-ducing drugs, especially in relation to an-aesthesia. We report a case of a 63-year-old female with acute intermittent porphyria and severe hemodynamic instability dur-ing and after liver resection taken for hepa-tocellular carcinoma. The procedure was predominantly characterized by unusual hemodynamic instability with refractory hypertension, despite adequate analgesia and depth of anaesthesia. Several differ-ent treatments failed to reduce high blood pressure. There is a possibility that some drugs used in the perioperative period caused acute porphyric crises, which was manifested by severe hemodynamic insta-bility. Autonomic neuropathy might have caused labile blood pressure as well. A pre-cise etiology of hemodynamic instability in the presented case is difficult to assess, since other provocative factors like fasting, dehydration and stress from surgery were also present. A careful anaesthetic plan and treatment and postoperative surveil-lance in the ICU are cornerstones in the management of patients with porphyria subjected to major surgical procedures.