Anesthetic considerations in Wilson’s disease for fess: A case report

Alisha Margarette*

Department of Anaesthesiology, Kauvery Hospital, Hosur

*Correspondence: medicaladmin.hosur@kauveryhospital.com

Abstract

Wilson’s disease (WD) is an autosomal recessive disorder characterized by the accumulation of copper in various organs and tissues especially in liver, brain, kidney, and cornea- consequently causing hepatic and neurological impairment. Anesthetic management for patients with WD presents a challenge as it requires monitored care in the peri operative period to avoid further worsening of the condition.

Background

Wilson’s disease or hepatolenticular degeneration is a rare, hereditary, disease of copper metabolism characterized by reduction in the synthesis of the copper transporter protein ceruloplasmin. WD has a prevalence of 1 in 30,000 population. It is caused due to a mutation in ATP7B gene, where there is a loss of ability to export copper from liver into bile. This, in turn, causes accumulation of copper in the body tissues especially in liver and brain tissues, leading to hepatic and neurological degeneration.

In patients with Wilson’s disease, there is an increased risk of cirrhosis and liver failure that naturally affect metabolism of many drugs. Apart from this, persistent neurological symptoms, and a variety of psychiatric illnesses are other important concerns during anesthetic planning. Only a few reports on anesthetic management on patients with WD has been documented in our literature. Hence here we present a case of a 25 year old male with Wilson’s disease who was electively taken up for functional endoscopic sinus surgery. We also briefly discuss its anesthetic management.

Case Presentation

A 25 year-old-male, diagnosed with Wilson’s disease at the age of 10 years, on regular treatment with D-penicillamine and zinc sulphate, with compensated chronic liver failure and mild splenomegaly, who presented with sinusoidal polyps, was electively posted for FESS.

Preoperative evaluation: He was clinically stable with no signs of hepatic failure or neurological deficit.

Blood investigations showed thrombocytopenia (platelets: 68000), mildly deranged liver function (total bilirubin: 2.0, direct bilirubin: 0.7, indirect bilirubin: 1.3, SGOT: 69, SGPT: 88) and coagulation parameters on the higher side (PT: 16.0, INR: 1.48). Renal function was normal. Ophthalmology consultation was sought and found to have Kayser-Fleischer (KF) rings on slit lamp examination.

Preoperatively he was well hydrated and 1-unit PRBC and 4 units platelets were arranged in view of any untoward incidents that may occur intra-op.

Intra-OP: On the day of surgery his general condition was re-accessed, the patient was planned for general anesthesia and was taken up for surgery. He was induced with Inj. Fentanyl 50 mcg iv, Inj. Propofol 50 mg iv, Inj. Atracurium 30 mg iv and airway secured. TIVA (Inj. Propofol infusion 3 ml/hr) was preferred to maintain the depth of anesthesia. A very minimal inhalational agent (Sevoflurane 0.4%) was used throughout the surgery. He was hemodynamically stable throughout the procedure, well hydrated, blood loss was minimal and output was adequate.

Postoperative period: He had a smooth emergence from anesthesia and was extubated when he was fully awake and conscious. He was shifted to ICU for further postoperative care and management. There were no signs of hepatic or renal failure. D-penicillamine was started 12 h prior to surgery and his general condition was stable in the postoperative period.

Discussion

Wilson’s disease is a congenital multisystem disorder which requires a clear preoperative assessment and a planned anesthetic approach individually planned for each patient based on their clinical condition.

Pathology

Wilson’s disease is an autosomal recessive disorder originating from a genetic defect in the copper transporting ATPase ATP7B that is required for biliary copper secretion. Toxic copper deposits may induce oxidative stress, modify expression of genes, directly inhibits proteins and impair mitochondrial functions

Hepatocyte dysfunction initially manifests as steatosis and progress gradually to hepatitis, fibrosis, cirrhosis and acute liver failure.

In brain, copper accumulates in astrocytes, cause impairment of blood brain barrier and damage the neurons and oligodendrocytes mainly in the basal ganglia and brainstem, leading to various movement and psychiatric disorders.

Accumulation of copper in the Descemet’s membrane of the cornea causes Kayser-Fleischer rings.

Various possible features of WD

Wilsons-disease-1

Anesthetic considerations

Under normal conditions, during general anesthesia, the effects of anesthetics are naturally toxic to the liver due to decrease in total hepatic blood flow, decreased blood pressure and decreased tissue perfusion as a result of surgery, further disrupting the hepatic function.

When GA is planned in cases with WD, care is to be taken in terms of which drugs are to be used as impaired hepatic failure may cause alterations in drug metabolism. And the effects of anesthetics may further worsen the hepatic functions which are already impaired in WD causing hepatic injury.

Inducing and inhalational agents interfere with the central nervous system, further exacerbating neurological symptoms post operatively. Most of the general anesthetic drugs have hepatic metabolism and inhalational agents decrease hepatic blood flow further exacerbating hepatic failure post operatively.

As of now there is no specific anesthetic drug which can be used safely in Wilson’s disease. Still, propofol is considered to be safe in most of the cases as inducing agent.

Fentanyl and isoflurane are some of the agents that improve hepatic blood flow, can be used safely, and seem to have decreased incidence of postoperative hepatic failure.

In our case, we preferred Total intravenous anesthesia (TIVA) as the main mode of maintaining depth of anesthesia, minimizing the use of inhalational agents. This can be hepatoprotective.

When patients are on long term D-penicillamine treatment, and have cirrhosis of liver, it may prolong the effect of muscle relaxants postoperatively. Atracurium which we have used in our case can be used safely but has been reported to have interactions with D-penicillamine. Post operative monitoring for worsening of neurological, and hepatic failure should be done closely and medical management of Wilson’s disease should be re-initiated as soon as possible in the post operative period.

Conclusion

General anesthesia can be successfully administered in asymptomatic Wilson’s disease patient using an anesthetic agent, the metabolism of which is least affected by the liver disease and one that causes least hepato-toxicity. By proper planning of anesthesia according to the individual patient’s symptomatic profile and pre-operative workup, and close follow-up of patients clinically and biochemically in the postoperative period, it is possible to reduce the complications rate to a minimum.

References

  1. Bayakal M, et al. Anesthetic management of a pediatric patient with Wilson’s disease. 2010.
  2. Athar M, et al. Anesthetic management of a patient with Wilson’s disease: factors to be considered. 2017.
  3. Saito K, et al. Perioperative anesthetic management for cesarian delivery of severe Wilson’s disease with liver failure: a case report. JA Clin Rep. 2019;5:75.

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