A Rare Case of Acute Intermittent Porphyria


Dr. Vasanthi Vidyasagaran*

Department of Anaesthesiology, Kauvery Hospital, Chennai, Tamilnadu, India


A 28 years old man fit and well, with no known medical illness, was posted for inguinal hernioplasty. Airway and spine were normal. Blood investigations were also normal. The surgery was carried out uneventfully under spinal anaesthesia and the patient was shifted to the ward after 2 hours. In the post- operative ward, the patient became very restless and started complaining of pain.

The on-duty staff nurse administered Injection Diclofenac 75 mg IM. The discomfort did not subside. He was further given 100 mg Tramadol IM. Over the next half an hour, the patient’s anxiety was getting worse. Doctors and staff could not have a meaningful conversation with him. The operated site was normal. The surgeons thought it could be due to a full bladder and hence catheterised. He was then shifted to the surgical ICU.

Investigations including x-ray chest, arterial blood gas, and CT Brain were planned after stabilising the patient. At that time, a resident noticed that the urine in the urobag had changed to a deep red colour. Differential diagnoses at that time were drug interactions, acute renal impairment or a metabolic disorder. General physician’s team was involved. There was increased levels of porphobilinogen in the spot urine test. A rare case of acute intermittent porphyria in a post-operative patient was confirmed. He responded well to supportive therapy and a precautionary note was given at discharge to prevent any future complications.


In this case, establishment of diagnosis was by chance. Metabolic disorders though a rare entity, must be kept in mind to clinch the diagnosis.

Diagnosing a case of AIP without any symptom or sign is almost impossible. High degree of suspicion must arise in the mind of the anaesthesiologist if they find a patient to be restless in the post-operative period. All other causes of restlessness should be ruled out or treated. Arriving at a diagnosis, and management of an acute state of unexpected confusion in a completely normal patient can be very challenging. Of relevance to the anaesthesiologist is that Thiopentone is an absolute contra indication in a patient with AIP. (There have been reports of acute intermittent porphyria being precipitated after induction with thiopentone in abdominal emergencies.)


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A Simple Procedure Small? Mistake – Grave Consequence

A 60-year-old man post-CABG was recovering very well in the intensive care unit. His central line was to be removed before shifting him to the ward on the 3rd post-operative day. A nurse happened to remove the line quite suddenly, in the sitting position and he collapsed in front of our eyes. He was immediately resuscitated, and recovered. This came as a shock to the relatives, staff and doctors involved in the team.

It was speculated that a gush of air got sucked into the vein due to improper technique of removing the central venous catheter, or it could have been pain shock, as the nurse was quite rough at handling. This incident could have been prevented if the central line was removed slowly in the supine or head down position, and pressure applied over the site.


  1. Removal of central line may seem like a simple procedure, but if not done with care may turn catastrophic.
  2. Safety procedures to follow include:
  3. Ensure patient is not dehydrated Low CVP may allow air entrapment into systemic circulation
  4. Position the patient in Trendelenburg position This allows venous pressure above atmospheric pressure
  5. Remove the line with patient performing Valsalva like manoeuvre (forced expiration) – Intrathoracic and intravenous pressures are high, hence air cannot enter venous system
  6. Apply gentle pressure at the site Prevents blood loss and air entry
  7. Apply tight dressing for 24 hours and encourage patient to lie down for 30 minutes
  8. We work in a complex system where medical care provider of varying levels of expertise are interdependent in the care of the patient. These providers use highly sensitive and potentially dangerous technologies and medications. This system provides breeding ground for error. It is essential to ensure that people who know the technique are delegated to certain jobs. Or else the whole system fails.


  1. Pronovost PJ, Wu AW, Sexton JB; Acute decompensation after removing a central line: practical approaches to increasing safety in the intensive care unit Ann Intern Med. 2004 Jun 15; 140(12):1025-33.
  2. Kim SK et al. Cerebral air embolism and subsequent transient neurologic abnormalities in a liver transplant recipient following the removal of the pulmonary artery catheter from the central venous access device: a case report. Korean J Anesthesiol. 2016 Feb; 69(1):80-3
  3. Sarah R Drewett Central venous catheter removal: procedures and rationale. British Journal of nursing, 2000, Vol9,No22