b'Most interdisciplinary teams function as vascularpatient with caloric support. anomaly clinics and are well equipped to treat patientsIn all these cases, it is recommended that a highly with a wide spectrum of vascular disorders 137 .trained nutritionist be an active member of the Treatment approaches typically include medicalmultidisciplinary team. management and minimally invasive and interventional radiologic techniques, with operative interventionD. MEDICAL MANAGEMENT: USE OFreserved for only the most severe cases.MTOR INHIBITORS AND OR OTHER Diagnosis will trigger management. Therefore,PHARMACOTHERAPIES FOR LYMPHATIC the correct diagnosis is essential for timely andMALFORMATIONS AND COMPLEX appropriate treatment. VASCULAR LESIONSClinical Example:To date, medical therapy is institution-dependent and, unfortunately, not yet standardized. We have foundUsing the term hemangioma for a venous orthat sirolimus 138and sometimes everolimus, both lymphatic malformation will automatically result inmTOR inhibitors, are safe and effective for lymphatic the initiation of propranolol, which not only has noand vascular anomalies even in infants with multiple efficacy on malformations but may cause significantcomorbidities 139 . Other agents used include side effects.trametinib, octreotide, propranolol, etc. Also, not recognizing a PIK3CA-related overgrowth In recent years, lymphatic malformations were found to syndrome or generalized lymphatic anomaly delaysbe caused by somatic activating mutations of PIK3CA, the initiation of the appropriate targeted medicaland targeted therapy with PIK3CA inhibitor, alpelisib, therapy that the patient needs to stabilize thereceived FDA approval in 2022, marketed as VIJOICE. disease and, therefore, improve outcomes. Supportive care for a patient with lymphatic effusions C. MANAGEMENT OF PROTEIN-LOSINGincludes close monitoring and management of ENTEROPATHIES, CHYLOTHORAX, ANDhypoalbuminemia and hypogammaglobulinemiaCHYLOPERITONEUM that may predispose to recurrent and difficult-to- In patients with protein-losing enteropathies,treat infections. chylothorax, and/or chyloperitoneum, symptomDeep lymphatic imaging, such as MRL with contrast, severity reflects the amount of accumulated fluid andmay be utilized to localize the source of the leakage, the location of the lymphatic leakage. Congenital/ and more aggressive interventions may be indicated in gestational disease, where lymph leaks into thecases that fail to respond to conservative medical pleural, pericardial and/or peritoneal spaces, can causemanagement 140 . lung compression, impaired cardiovascular function, CAUTION when using mTOR Inhibitorsmay be or abdominal compartment issues for the fetus. responsible for causing lymphedema.In neonatal disease, similar features may be present, and if left untreated, result in respiratory failureAnother consideration in treating lymphatic requiring ventilatory support or cardiac tamponade.malformations and complex vascular lesions involves Conservative management can take many forms, careful management of mTOR inhibitors, such as with several therapeutic measures implementedsirolimus and everolimus. mTOR inhibitors are narrow simultaneously or sequentially.therapeutic drugs meaning that small differences in In some cases, support and observation may be thedose or blood concentrations may be a cause of appropriate approach to allow the development ofserious therapeutic failures and/or adverse drug collateral lymphatic circulation and the possibility ofreactions that can be life-threatening or result in spontaneous closure.persistent or significant disability or interfere with activities of daily living. In cases where chyle leakage is not self-limited,Education of the patient and their familiesreplenishment of necessary fluid losses in the form ofon proper medication dosing and monitoringenteral or total parenteral nutrition (TPN) is essential.of these drugs is essential.Enteral nutrition, which contains a low-fat formula It is known in the transplant and oncology literature of medium-chain triglycerides (MCT), may promotethat the use of mTOR inhibitors can carry undesirable decreased chyle production and spontaneous closureside effects, such as unilateral or bilateral upper and/or of the leak.lower extremity edema or facial/eyelid edema. TPN, which must contain lipid emulsions, can be usedThe mean interval between symptom onset andin patients with massive chyle leakage to provide themTOR inhibitor initiation is approximately 12 months. | Standardized Approach for the Diagnosis and Management of Lymphedema (LE) and Lymphatic Diseases (LD)|35'