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Educated Hand Publishing LLC 
“The Science Behind the Art” 
Volume 9 - No.1 2021 

 Anesthesia eJournal - Online
ISSN 2333-2611

Page 1

Anesthetic Management of a Patient with Hereditary Coproporphyria
Audrey F. Fisher, BSN, RN, RRNA

Affiliation:
Texas Christian University

Grant/Financial Support: 
None

Biographical data:  
Audrey F. Fisher is a Registered Nurse pursuing her DNP in Nurse Anesthesia at Texas Christian University in Fort Worth, Texas. 

KEYWORDS:  Hereditary Coproporphyria, Porphyria, Triggering agents, Acute Attacks

Abstract
Hereditary coproporphyria (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outdated, there is consensus that unsafe agents include: barbiturates, ketamine, and etomidate; and safe agents include: 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, all neuromuscular blocking agents, all reversal agents, commonly used narcotics, 
antiemetics (excluding metoclopramide), and sedatives. 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. 
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reports are anecdotal and/or outdated.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

AEJ



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Introduction
• HCP is a rare (1:1,000,000) heme biosynthesis disorder 

inherited from either parent via autosomal dominance. 
• HCP causes accumulation of porphyrins and porphyrin 

precursors which lead to neurotoxicity and subsequent 
acute attack in the presence of known triggering agents.

• Acute attacks can be life threatening and involve severe 
autonomic instability, respiratory failure, and paralysis.

• Triggering agents include many anesthetic agents that 
have been labeled porphyrinogenic; see Table 1.

• Safe anesthetic management of patients with HCP 
demands understanding of this disease process. 

Purpose
• This case report describes the pathophysiology and 

anesthetic management of a patient with hereditary 
coproporphyria (HCP). 

Discussion
• The patient was at increased risk of an attack of acute 

porphyria due to PMH of HCP combined with the necessity 
to undergo general anesthesia.

• The decision to substitute the planned sevoflurane with 
isoflurane was an attempt to prevent an exacerbation of 
porphyria.

• The patient did not have any porphyrinogenic-related 
reactions to the anesthetic agents administered 
intraoperatively.

Key Points
• Identifying drugs as either safe or unsafe is critical.
• Anxiolysis with benzodiazepines is recommended. 
• Safe anticonvulsant therapy includes levetiracetam, 

clonazepam, gabapentin, and/or vigabatrin for seizure 
prevention.

• Correction or prevention of hyponatremia and electrolyte 
imbalances is recommended. 

• IV hydration with glucose containing fluids (not to exceed 
300 g/day) to downregulate aminolaevulinic acid synthetase 
(ALAS) and to minimize caloric restriction and dehydration.

• General and regional anesthesia are both possible and 
equally efficacious if initiating factors are avoided.

• Arterial line for hemodynamic monitoring is recommended 
due to likelihood of autonomic dysfunction.

• If a severe attack is suspected, IV heme (Panhematin) 
should be administered expeditiously.

Case Critique
• Although the patient was not anxious or agitated 

preoperatively, administration of an anxiolytic to reduce 
stress, a known trigger of porphyria, is indicated.

• The patient received dexamethasone despite many studies 
claiming it to be unsafe.

• It is widely established that sevoflurane is safe. 

Anesthetic Management of a Patient with
Hereditary Coproporphyria

Audrey F. Fisher, BSN, RN, CCRN, Texas Christian University

Case Description
• A 63-year-old, 101 kg, 180 cm, male presented for a 

right parietal temporal craniotomy for a glioblastoma 
tumor resection after suffering frequent left sided falls 
at home and subsequent loss of consciousness. 

Pre-Anesthetic Evaluation
• Medical History: coronary artery disease, carotid 

stenosis, tobacco use, gastroparesis, pemphigus 
vulgaris, schizophrenia, and HCP. 

• Surgical History: cataract surgery and heart 
catheterization. 

• Current Medication Regimen: metoprolol, vitamin C, 
amlodipine, labetalol, nicotine patch, levetiracetam, 
famotidine, dexamethasone, risperidone, clonazepam, 
benztropine, and aspirin. 

• Diagnostic Testing: MRI diagnosed 7.5 cm right temporal 
lobe mass with a left midline shift of 7 mm, abnormal 
labs WBC 17.6 and glucose 151. 

Intraoperative Anesthetic Management
• Pre-induction vital signs: pulse 81 in NSR, BP 125/78 

mm Hg, SpO2 100%, RR 20, and temperature 35.1 °C. 
• General anesthetic induction:  fentanyl 100 mcg IV, 

lidocaine 100 mg IV, propofol 100 mg IV, and 
succinylcholine 140 mg IV. 

• 8.0 ETT placed followed by Isoflurane titrated to 0.5 
MAC.

• Remifentanil and phenylephrine infusions.
• Arterial line and internal jugular central line placed.
• Patient placed in prone position with head in Mayfield 

Keep skull pins which were verified and secured by the 
surgeon. 

Intraoperative Course
• Preoperatively patient calm, pleasantly confused and 

denied discomfort.
• No signs of acute attack of porphyria.
Postoperative Course
• Patient transported to neuro ICU and remained 

intubated on mechanical ventilator and fentanyl, 
propofol, and nicardipine infusions.

References
1. American Porphyria Foundation website. Updated 2021. Accessed February 13, 2021. 

https://porphyriafoundation.org. 

2. Jensen NF, Fiddler D S, Striepe V. Anesthetic considerations in porphyrias. Anes Analg.1995;80(3). 591-599. 

3. Genetic and Rare Diseases Information Center website. Updated April 9, 2019. Accessed February 15, 2021. 
https://rarediseases.info.nih.gov.

4. Rapp HJ, James M, Bonkovsky. Anaesthesia recommendations for patients suffering from porphyria. Orphan 
Anesthesia website. Updated December 2014. Accessed February 15, 2021. 
https://www.orpha.net/data/patho/Ans/en/Porphyria_EN.pdf. 

5. Hines RL, Marschall KE. Chapter 19: Inborn errors of metabolism. In: Tantawy H, Tao J. Stoelting’s
Anesthesia and Co-Existing Disease. 7th ed. Elsevier Inc; 2018:377-384.

6. Findley H, Philips A, Cole D, Nair A. Porphyrias: implications for anaesthesia, critical care, and pain medicine. 
Continuing Educ Anes Crit Care Pain. 2012;12(3). 128-133. 

Conclusions and Recommendations
• A thorough perioperative workup must be performed in 

order to prevent an acute attack of porphyria which can be 
life-threatening. 

• Determination of safe vs unsafe anesthetic medications is 
key. 

• No evidence suggests a general anesthetic is safer than a 
regional anesthetic. 

• Further investigation of anesthetic management of the 
patient with HCP is required because most of the current 
clinical reports are anecdotal and/or outdated.

• 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.

Pathophysiology and Manifestations
• Heme is a vital porphyrin to hemoglobin because it is 

required to transport O2, remove CO2, and 
biotransform essential enzymes such as CYP450; see 
Figure 2.

• The porphyrias, a group of metabolic disorders, each 
lack any 1 of the 8 enzymes in the heme synthetic 
pathway resulting in the accumulation of porphyrin 
and porphyrin precursors ALA and PBG, subsequently 
producing a form of porphyria; see Figure 1. 

• HCP lacks CPO, the 6th enzyme in the heme 
biosynthesis pathway, which originates from a genetic 
mutation of the CPOX gene on chromosome 3; see 
Figure 3.

• Manifestations: acute abdominal pain, cutaneous 
lesions, neuropsychiatric abnormalities such as 
confusion, seizures, mood disturbance, hysteria, 
syncope, autonomic instability, peripheral neuropathy, 
sensory loss and ascending muscle weakness 
mimicking Guillain-Barre; see Figure 4.

CPOX GENE MUTATION ON CHROMOSOME 3 AT 
POSITION 12

VS

Safe Agents

•Propofol
•Nitrous Oxide
•Volatile Agents
•Depolarizing and 
Nondepolarizing 
NMB

•Reversal Agents
•Antiemetics 
(excluding Reglan)*

Unsafe agents

•Barbiturates
•Ketamine
•Etomidate
•Decadron*
•Calcium Channel 
Blockers*

•Ketorolac*

Potential of Drugs to Provoke HCP Attacks

*majority consensus

Table 1.  Triggering Agents 

Figure 1. Heme Synthesis Pathway 

Figure 2. Hemoglobin Molecule 

Figure 3. Location of CPOX Gene Mutation  

Figure 4. Apparent vs Nonapparent Symptoms 


