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

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


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.

Publication types

  • Case Reports

MeSH terms

  • Antibiotics, Antineoplastic / therapeutic use*
  • Diagnosis, Differential
  • Head and Neck Neoplasms / diagnosis
  • Head and Neck Neoplasms / drug therapy*
  • Humans
  • Infant
  • Lymphangioma / diagnosis
  • Lymphangioma / diagnostic imaging
  • Lymphangioma / drug therapy*
  • Lymphoma / diagnosis
  • Lymphoma / drug therapy*
  • Magnetic Resonance Imaging
  • Male
  • Sirolimus / therapeutic use*
  • Ultrasonography


  • Antibiotics, Antineoplastic
  • Sirolimus