title: Airway response to sirolimus therapy for the treatment of complex pediatric lymphatic malformations.
abstract: Head and neck lymphatic malformations can create airway management challenges requiring tracheotomy. Sirolimus, an inhibitor of mammalian target of rapamycin (mTOR), may inhibit growth of lymphatic malformations. We describe two patients born with large lymphatic malformations with improved airway symptoms following sirolimus therapy. Patient #1 underwent tracheotomy and multi-modal therapy including sirolimus with reduction in airway involvement but regrowth after discontinuation of sirolimus. Patient #2 also experienced a significant response to sirolimus allowing for extubation and discharge without tracheotomy. Early initiation of sirolimus therapy should be considered as a means to avoid tracheotomy in complex head and neck lymphatic malformations.
authors.affiliation: Department of Otolaryngology - Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA. Electronic address: sean.alemi@ucsf.edu.
authors.forename: A Sean
authors.initials: AS
authors.lastname: Alemi
authors.affiliation: Department of Otolaryngology - Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA; Division of Pediatric Otolaryngology, Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA.
authors.forename: Kristina W
authors.initials: KW
authors.lastname: Rosbe
authors.affiliation: Department of Otolaryngology - Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA; Division of Pediatric Otolaryngology, Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA.
authors.forename: Dylan K
authors.initials: DK
authors.lastname: Chan
authors.affiliation: Department of Otolaryngology - Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA; Division of Pediatric Otolaryngology, Head & Neck Surgery, University of California, San Francisco, San Francisco, CA, USA.
authors.forename: Anna K
authors.initials: AK
authors.lastname: Meyer
mesh_terms: D000402:Airway Obstruction / Q000209:etiology; D003131:Combined Modality Therapy; D005260:Female; D006257:Head; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D007231:Infant, Newborn; D044148:Lymphatic Abnormalities / Q000150:complications / Q000628:therapy*; D008297:Male; D009333:Neck; D012008:Recurrence; D015911:Sclerotherapy; D020123:Sirolimus / Q000627:therapeutic use*; D014140:Tracheotomy
chemical_list: D007166:Immunosuppressive Agents; D020123:Sirolimus
keywords: Lymphatic malformation; Sirolimus; Tracheotomy