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Laryngoscope. 2011 Sep;121(9):1851-4. doi: 10.1002/lary.21927. Epub 2011 Aug 16.

The successful management of diffuse lymphangiomatosis using sirolimus: a case report.

Author information

1
Faculty of Medicine, University of Ottawa, Ottawa, Canada.

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.

PMID:
22024836
DOI:
10.1002/lary.21927
[Indexed for MEDLINE]

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