The successful management of diffuse lymphangiomatosis using sirolimus: A case report

Authors

  • Jason Reinglas BSc,

    1. Faculty of Medicine, University of Ottawa, Ottawa
    2. Department of Otolaryngology, Childrens Hospital of Eastern Ontario (CHEO), Ottawa, Ontario, Canada
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  • Raveena Ramphal MD, MBChB,

    1. Department of Pediatric Hematology and Oncology, Childrens Hospital of Eastern Ontario (CHEO), Ottawa, Ontario, Canada
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  • Matthew Bromwich MD, FRCSC

    Corresponding author
    1. Department of Otolaryngology, Childrens Hospital of Eastern Ontario (CHEO), Ottawa, Ontario, Canada
    • Pediatric ENT, Division of Otolaryngology–Head and Neck Surgery, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa ON K1H 8L1, Canada
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  • The authors have no funding, financial relationships, or conflicts of interest to disclose.

Abstract

Lymphangiomatosis is a rare and fatal congenital lymphatic malformation. Because the natural course of the disease affects multiple body systems, the management can be challenging. This article presents a novel approach to the treatment of diffuse lymphangiomatosis using sirolimus. The reported case involves a 4-month-old male with a known lymphatic malformation who presented to the emergency department with respiratory difficulties. Sirolimus was successful at significantly reducing our patient's mass at a relatively low target level of 5 to 10 μg/L. The use of sirolimus for the treatment of lymphangiomatosis should be studied further in the setting of a formal trial.

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