Safe anesthesia practice in patients with lipin-1 deficiency: a case report and narrative review

Lipin-1 deficiency is a rare recessive autosomal disorder which causes recurrent episodes of rhabdomyolysis. These episodes are frequently initiated by a triggering event such as febrile illness, exercise or fasting. Anesthesia has also been claimed to provoke rhabdomyolysis in these patients. These episodes start in early childhood and often require intensive treatment which is complicated by a high morbidity and mortality. We present a review of the available literature and analyzed the data of 80 lipin-1 deficient patients. We found 79 cases published in 24 articles. We also report our own patient. Analysis of this data could not provide documentation proving a causal relationship between anesthesia and a rhabdomyolysis episode. We found four case reports of anesthetic procedures in lipin-1 deficient patients. These patients had been given an intravenous glucose infusion to avoid the induction of a catabolic state. The procedures were uncomplicated and did not provoke rhabdomyolysis. We present the case of a 14 year old boy requiring several surgical and anesthetic procedures. Administration of intravenous glucose was part of our strategy to prevent the perioperative occurrence of rhabdomyolysis. We provide detailed information about the agents we used for the anesthetic management of this patient. We hypothesize that not the anesthetic exposure but other factors might cause rhabdomyolysis in the perioperative period. These factors include fasting, inflammation, the surgical stress response and a mismatch in metabolic supply and demand. We provide recommendations for anesthetic procedures in lipin-1 deficient patients.