Researchers from India summarized concerns about anesthesia and surgery in patients with Wilson disease in a review article they published in the journal Cureus.
Based on 19 published case reports, the authors of the review concluded that there is little scientific data on the anesthetic management of the disease, which is mostly based on isolated case reports, the study authors.
Read more about the treatment of Wilson disease
“We, therefore, believe that an international registry of this rare condition is needed,” the authors concluded. This, they said, “will facilitate the gathering of useful information, which can help both patients and peri-operative clinicians involved in their care.”
According to the team led by Priyankar K. Datta from All India Institute of Medical Sciences in New Delhi, the most common need for anesthesia reported in the literature was obstetric and gynecological procedures, including cesarean section and termination of pregnancy. Others included correction of scoliosis, orthopedic procedures for trauma, and neurosurgical procedures for traumatic brain injury. Most patients were between 4 and 53 years of age.
Anesthetic management included general anesthesia, spinal anesthesia, and regional anesthesia, and no intraoperative or postoperative complications were reported in any of the cases.
Preoperatively, a general physical examination with emphasis on relevant systemic involvement must be conducted, the authors wrote. The function of the liver and kidney must be assessed carefully, as should the cardiovascular system.
In the case of general anesthesia, care must be taken in terms of the choice and dosage of the drug used as reduced protein binding and slow metabolism in Wilson disease can increase the effect of anesthetic agents, the authors wrote.
In the postoperative period, dystonia may be exaggerated, the researchers noted. A worsening in neuropsychiatric symptoms following general anesthesia has also been reported. “To prevent worsening of symptoms, copper levels need to be maintained in the safe range by continuous pharmacotherapy,” the authors wrote.
They concluded that Wilson disease is a challenge for anesthesiologists. “Appropriate management requires detailed knowledge of the organ systems involved and needs to be tailored according to the individual patient’s clinical profile.”
Reference
Aravindan A, Shiva Priya K, Roy Chowdhury S, Datta PK. 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
