Sugammadex and amino acid infusion can contribute to safe anesthetic management of variegate porphyria

Abstract

Background: Variegate porphyria (VP) is an inherited type of porphyria characterized by cutaneous manifestations and/or acute neurovisceral attacks. We report successful anesthetic management of VP. Case presentation: A 66-year-old woman with VP was scheduled to undergo distal pancreatectomy for pancreatic cancer. Medical history was unremarkable except for sudden onset of abdominal pain that occurred every few months, possibly due to VP. There was no abnormality in laboratory data except for a mild increase in creatinine levels. General anesthesia was induced and maintained with total intravenous anesthesia using propofol, remifentanil, and rocuronium. Blood glucose levels were measured every hour and glucose administered to prevent hypoglycemia. Amino acids were also infused to treat hypothermia. Upon completion of distal pancreatectomy, sugammadex was administered to reverse neuromuscular blockade. She was neurologically intact and discharged on postoperative day 15 with no acute attack. Conclusions: Sugammadex and amino acids can be used safely in patients with VP.