2.3.1 Medical Management
Medical management of CLA involves supportive care and targeted drug therapy. Improved understanding of pathogenesis and genetic etiology for lymphatic anomalies lead to use of mTOR inhibitors such as sirolimus and everolimus, bisphosphonates (ex. zoledronic acid) and more recently, MAPK/MEK inhibitors (ex. trametinib). PIK3CA inhibitors are also a potential treatment option in the future. Multiple case series and reports describe treatment algorithms of different institutions, but there is no consensus on indications for starting medication, initial dosing, dosing adjustments, frequency of monitoring or length of therapy. Since no universal guidelines exist, we offer, in Table 2 , our institutional protocols for these medications.