The Knee
Volume 17, Issue 3 , Pages 252-254, June 2010

Total knee arthroplasty and Crigler–Najjar syndrome: A case report

  • David Walmsley

      Affiliations

    • Division of Orthopedic Surgery, Department of Surgery, University of Toronto, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, Canada, M5T 2S8
    • ,
  • Khalid Alzaharani

      Affiliations

    • Division of Orthopedic Surgery, Department of Surgery, University of Toronto, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, Canada, M5T 2S8
    • ,
  • William J. Coke

      Affiliations

    • Department of Medicine, University of Toronto, Toronto General Hospital, Toronto, ON, Canada, M5G 2C4
    • ,
  • Rajiv Gandhi

      Affiliations

    • Division of Orthopedic Surgery, Department of Surgery, University of Toronto, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON, Canada, M5T 2S8
    • Corresponding Author InformationCorresponding author. Tel.: +1 416 603 5642; fax: +1 416 603 3437.

Received 6 April 2009; received in revised form 21 August 2009; accepted 25 August 2009. published online 14 September 2009.

Abstract 

Crigler–Najjar (CN) syndrome is a rare genetic disease characterized by hyperbilirubinemia due to a deficiency in the hepatic enzyme UDP-glucuronosyl-transferase. We describe the first case of total knee arthroplasty in a patient with CN syndrome (type II). This procedure was complicated by kernicterus 1 week after hospital discharge. He also developed Klebsiella bacteremia and sepsis, requiring a brief ICU stay. He was discharged in good condition 2 months later. It is evident that physicians involved in the care of patients with CN syndrome in the peri-operative period need to have a high index of suspicion for the development of severe hyperbilirubinemia and kernicterus in order to appropriately manage and, possibly, prevent this complication. A literature review and intra-operative observations provide insight into the possible relationship between hyperbilirubinemia and osteoarthritis as well as the peri-operative considerations to be made for this group of patients.

Keywords: Crigler–Najjar syndrome, Osteoarthritis, Total knee arthroplasty, Kernicterus

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PII: S0968-0160(09)00166-5

doi:10.1016/j.knee.2009.08.012

The Knee
Volume 17, Issue 3 , Pages 252-254, June 2010

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