A Case Report of Anesthesia Management in the Liver Transpla
Porphyrias are a group of rare diseases related to an enzyme deficiency in the heme metabolism. In some cases of porphyria, liver transplantation (LT) is considered as the treatment of choice for the life-threatening acute attacks resulting in hepatic failure.

A 23-year-old man (body weight: 60.7 kg, height: 173 cm) visited the emergency room with abdominal pain and jaundice. He had been diagnosed with biliary cirrhosis 4 years ago, while being evaluated with abnormal liver function tests after alcohol consumption. During his workup, hepatosplenomegaly with collateral vessels was found on abdominal computed tomography, and esophageal varices were found on esophagogastroduodenoscopy. He had progressing proximal limb pain that could not be controlled by opioids. He also had skin sensitivity on exposure to sunlight, and there was a family history of multiple skin blisters on exposure to sunlight.

His clinical features were attributed to porphyria, and he was found to have increased coproporphyrin in the stool and uroporphyrin in the urine. His renal function suddenly deteriorated to the point of needing continuous renal replacement therapy. The preoperative electrocardiogram revealed sinus tachycardia, and his echocardiogram was within normal limits. Pulmonary edema and pulmonary effusion were identified on his preoperative chest X-ray. According to the preoperative laboratory data, his model for end-stage liver disease score was 33 points.

He was intubated, and a continuous infusion of norepinephrine (0.9 µg/kg/min) was given in the intensive care unit (ICU). In the operating room, a Bispectral Index® monitor was attached, and ventilator care was continued at the same ICU settings. Arterial cannulation was performed with a 20G catheter in the left radial artery, and central venous cannulation was performed with a 9-Fr Advanced Venous Access catheter in the right internal jugular vein. A multifunction Swan-Ganz catheter was inserted for hemodynamic monitoring, and the endotracheal tube was changed.

General anesthesia was induced and maintained with total intravenous anesthesia, consisting of 2% propofol (100 µg/kg/min) and remifentanil (0.15 µg/kg/min). The continuous infusion of norepinephrine (0.9 µg/kg/min) was maintained. The patient had hypovolemic acute renal failure, and atracuronium (5 µg/kg/min) was infused intravenously. The shadowless surgical lights were covered with special filters , and other lights in the operating room were turned off before the patient was brought in.

The LT operation started 75 minutes after induction of anesthesia. Dopamine (5 µg/kg/min) was added to maintain the mean arterial blood pressure between 60 and 70 mmHg, before the anhepatic phase. During the anhepatic period, the hemodynamic profile was stable. Immediately before the reperfusion, 100% oxygen was given. After reperfusion, the mean blood pressure dropped to about 49 mmHg, but it was normalized within 2 minutes, without bolus injections of inotropics. On the third day after LT, the patient became alert and his renal function improved, so the continuous renal replacement therapy was discontinued. However, his motor function was weak, therefore extubation was delayed.

In conclusion, understanding the patient's status and the disease process and avoiding the triggering factors, based on knowledge of the porphyria attacks, are important for successful LT anesthesia in porphyria patients

Source : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3272535/