The Anesthetic Management of a Patient with Hereditary Coproporphyria

Abstract

Introduction: The anesthetic management of a patient with hereditary coproporphyria (HCP) is complicated because many anesthetic drugs have the potential to induce an acute attack of porphyria. Acute attacks of HCP exhibit a wide variety of metabolic defects that may result in life-threatening reactions, such as severe autonomic dysfunction and blood pressure (BP) lability. The anesthesia provider must be knowledgeable of concomitant triggers as well as appropriate treatment of porphyric crises. Since anesthetic-linked porphyric crises are understudied, a lack of agreement on triggering anesthetic agents dominates the literature, although there is unanimity among commonly administered drugs such as barbiturates, etomidate, and ketamine.

Case presentation: The case involved a 63-year-old male with a diagnosis of right intracranial glioblastoma undergoing a right parietal temporal craniotomy for a tumor resection under general endotracheal anesthesia (GETA). The unique concerning issue was management of the patient’s anesthetic despite his underlying history of HCP and its known anesthetic risks.

Discussion: HCP is an inborn error of metabolism that causes accumulation of porphyrins and porphyrin precursors, which can potentially lead to neurotoxicity and acute crisis with introduction of a triggering agent. Many anesthetic drugs have been labeled porphyrinogenic, therefore safe anesthetic management of patients with HCP demands understanding of the disease process. Although most of the current clinical reports are anecdotal and/or outdated, there is consensus that unsafe agents include: barbiturates, ketamine, and etomidate; and safe agents include: propofol, nitrous oxide, volatile agents (excluding sevoflurane), all neuromuscular blocking agents, all reversal agents, commonly used narcotics, antiemetics (excluding metoclopramide), and sedatives. Further investigation of anesthetic management of the patient with HCP is indicated, given that most current clinical reports are anecdotal and/or outdated. The anesthesia provider should refer to the American Porphyria Foundation website for the most up-to-date information on porphyria and up-to-date drug database for healthcare professionals caring for porphyria patients.