Complex lymphatic anomalies are congenital diseases of the lymphatic
circulation system that are associated with significant morbidity and
early mortality. While guidelines for the comprehensive evaluation of
the complex lymphatic anomalies were recently published, the diagnostic
approach and medical management are not standardized. The current
manuscript presents the clinical features of 4 complex lymphatic
anomalies: Gorham-Stout disease, generalized lymphatic anomaly,
kaposiform lymphangiomatosis, and central collecting lymphatic anomaly.
We also offer three cases from the authors’ practice and our views on
diagnostic testing and disease management including supportive care,
medical therapies and other interventions.