The technique of intranodal contrast injection to visualize the lymphatic vessels, along with the use of dynamic contrast-enhanced MR lymphangiography, has led to important insights into the phenotype of patients with complex lymphatic anomalies. The pediatric patient with a complex lymphatic anomaly benefits from an integration of therapy across modalities, including supportive strategies (diet), localized therapy (drainage, embolization), and systemic therapy including targeted molecular therapy in mutation-driven complex lymphatic anomalies. A multidisciplinary collaboration, involving pediatricians, pharmacotherapeutic experts, pediatric radiologists, interventional radiologists, surgeons, geneticists, and nutritionists, is essential to tailor diagnosis to genotype and phenotype, personalize treatment, and monitor response.
Klein et al. (Thu,) studied this question.