Wilson’s Disease

  • Stephanie I. ByerlyEmail author


Wilson’s disease (hepatolenticular degeneration) is a rare autosomal recessive inherited disorder with abnormal hepatic metabolism and biliary excretion of copper. Symptoms usually appear in the second and third decades of life. The liver can be affected to varying degrees, but the disease can also affect the renal, cardiac, and neurologic systems. Additionally, patients may experience pancreatitis, hypothyroidism, and infertility.

The progression of Wilson’s disease does not seem to be affected by pregnancy, and some patients may go into remission. Women must be maintained on treatment throughout their pregnancies; otherwise fulminant liver failure may occur. Treatments include d-penicillamine, trientine, zinc, antioxidants, and diet.

Anesthetic choice will be based on current status and organ involvement of the patient. Regional anesthesia is the preferred method for labor, vaginal delivery, and cesarean section in patients without coagulopathy.


Wilson’s disease Ceruloplasmin Copper Pregnancy Obstetrical Anesthesia Regional General 


  1. 1.
    Dobyns WB, Goldstein NP, Gordon H. Clinical spectrum of Wilson’s disease. Mayo Clin Proc. 1979;54:35.PubMedGoogle Scholar
  2. 2.
    Roberts EA, Schilsky MI. Diagnosis and treatment of Wilons’s disease: an update. American Association for the Study of Liver Diseases. Hepatology. 2008;47:2089–111.CrossRefGoogle Scholar
  3. 3.
    Mustafa MS, Shamina AH. Five successful deliveries following 9 consecutive spontaneous abortions in a patient with Wilson’s disease. Aust N Z J Obstet Gynaecol. 1998;38:312–4.CrossRefGoogle Scholar
  4. 4.
    Schilsky, MI. Wilson disease: diagnostic tests. Up to Date; 2016.Google Scholar
  5. 5.
    Brewer GJ, Johsnon VS, Dick RD, Fink JK, Kluin KJ. Treatment of Wilson’s disese with zinc XVII: treatment during pregnancy. Hepatology. 2000;31:364–70.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Anesthesiology and Pain ManagementUniversity of Texas SouthwesternDallasUSA

Personalised recommendations