b'Additionally, patients typically exhibit lymphedema inAt the time, the belief was that a TDE would closestages II or III according to ISL staging criteria. Patientsthe lymphatic feeders responsible for the accumulation exhibiting lymphedema secondary to mTOR inhibitorof chylothorax. However, unfortunately, whileuse may benefit from dose reduction or if necessaryTDE resulted in immediate, short-term benefits, careful consideration of the discontinuation of thesurgeons and interventional radiologists soon learned offending agent.that these procedures can sometimes cause disruption Furthermore, earlier detection of lymphedema andof the normal lymphatic drainage into the main cessation of mTOR inhibitors is more likely to preventcirculation, leading to significant long-term permanent limb changes. complications and morbidity. E. INTERVENTIONAL THERAPIES IN SECTION 3: THE TREATMENT OF LYMPHATIC AND VASCULAR ANOMALIES CONSULTATIVE Venous and Lymphatic Sclerotherapy SERVICES FOR For focal venous and lymphatic malformations, sclerotherapy is considered first-line therapy.LYMPHATIC DISEASESSclerotherapy involves injecting the lesion with sclerosing agents to induce damage to theA. GENETICS (SCREENING FOR KNOWN endothelium, causing a cascade of inflammation,GENES AND MUTATIONS, GENETIC vascular occlusion, and sclerosis. The most common sclerosing agents used today include doxycycline,COUNSELING TO PATIENTS AND FAMILIES)sodium tetradecyl sulfate (STS), absolute ethanol, Author: Salma Adham, MDand bleomycin 141 .Primary lymphedema (LE) is thought to be causedAll sclerosing agents are considered effective, with by defects in genes involved in the developmenta mean overall response rate from 71% to 100%.of lymphatic vessels. The Lymphatic Education & Complications such as cellulitis and skin necrosis haveResearch Network (LE&RN) endorses the utilizationbeen seen following sclerotherapy, with a higherof St. Georges University Hospitals Classification frequency of 18% occurring after ethanol sclerotherapyAlgorithm of Primary Lymphatic Anomalies, described compared to the other sclerosing agents of 06%. previously 145 , for the diagnosis of primary lymphedema Less common complications include facial nerveand guide for genetic testing and management.paralysis after OK-432 and ethanol use 142 . The St. Georges classification is meant to help Larger or syndromic vascular malformations mayphysicians better categorize their patients and offer benefit from a combined approach, including the possibility of molecular diagnosis. The St. Georges medical therapy, endovascular, laser, and surgicallymphedema team performed careful phenotyping and interventions 143 . identified five subgroups of primary lymphedema. Thoracic Duct Embolization for Plastic Bronchitis,1.Lymphedema associated with other genetic Chylothorax, and Chyloperitoneum syndromes (where lymphedema is not the predominant feature of the syndrome)Thoracic duct embolization (TDE) is a historic minimally2.Lymphedema with systemic, or internal, lymphatic invasive procedure, infrequently used now for theproblems (i.e., pleural effusions, pericardial percutaneous treatment of plastic bronchitis,effusions, ascites, chylous reflux, protein-losing chylothorax, and chyloperitoneum. The procedureenteropathy/intestinal lymphangiectasia, orinvolves a diagnostic pedal lymphangiography tofetal hydrops)identify the location of the chyle leak along with 3. Congenital lymphedema (present at birth oranatomical variations, followed by transabdominal catheterization and embolization of the thoracic duct.within the first few months of life but with no Although identifying the cause of chyle leak hassystemic involvement)proven to be difficult, with one study reporting a4.Lymphedema that occurs later in life (after 1 yearsuccess rate of 65%, TDE was clinically successful of age, but with no systemic involvement)in 73% of patients with nontraumatic chylothoraces5. L ymphedema may be associated with lymphatic with thoracic duct occlusion. However, in cases ofmalformations, vascular malformations, or nontraumatic chylothorax with a normal thoracic segmental overgrowth problems.duct, TDE was largely unsuccessful, emphasizing the importance of identifying the cause of the lymphaticWithin these subgroups, genetic mutations commonleak to achieve better procedural outcomes 144 . to the cohort were successfully identified. A molecular | Standardized Approach for the Diagnosis and Management of Lymphedema (LE) and Lymphatic Diseases (LD)|36'