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A systematic review of the literature published in the journal Cureus found that there are only 19 case reports about the anesthetic management of patients with Wilson disease for surgeries other than a liver transplant. “The paucity of scientific data in this regard is concerning,” the authors of the review said and suggested that an international registry of Wilson disease is necessary and will facilitate the gathering of information that can help patients as well as the clinicians caring for them.

Read more about the epidemiology of Wilson disease

Because Wilson’s disease is a disease affecting many organs and systems in the body, the administration of anesthesia to a patient with the disease can be challenging, the authors noted. 

Here, a team of researchers from India led by Priyankar K. Datta, an anesthesiologist at the All India Institute of Medical Sciences in New Delhi, conducted a systematic search of the literature related to the anesthetic management of patients with Wilson disease.

The team identified 19 articles about the management of patients with Wilson disease for various surgeries other than a liver transplant.

They found that most of the information about the anesthetic management of the disease was from isolated cases, which they said, was related to its relative rarity. 

The authors also provided a summary of perioperative concerns and the safe anesthesia practices in Wilson disease.

Wilson disease is a rare genetic disease that affects around 1 in 30,000 people and is characterized by impaired copper metabolism due to a mutation in the ATP7B gene, which encodes a protein responsible for excreting excess copper into bile and plasma. When the protein does not function properly due to the mutation, copper is deposited in different organs and tissues, including the liver, brain, and eyes. This, in turn, leads to liver disease as well as neurological and psychiatric problems, and Kayser-Fleischer rings, greenish-brown annular copper deposits in the eyes.

A liver transplant is a curative treatment for patients with Wilson disease, which can also reverse the neurological deterioration.

Reference

Aravindan A, Shiva Priya K, et al. Challenges in anesthesia in Wilson’s disease: a systematic review of the existing literature. Cureus. Published online January 4, 2023. doi:10.7759/cureus.33334