Unmasking the invisible: Complex lymphatic anomaly uncovered by bilateral chylothorax

Abstract

Complex lymphatic anomalies (CLA) are an extremely rare group of disorders resulting from embryogenic lymphatic malformations that are characterized by overlapping clinical, anatomic location, imaging features, and complications. Due to their low incidence, these conditions are often reported in case studies and small series Chylothorax, or the accumulation of chyle in the pleural space, is a rare condition typically resulting from thoracic duct rupture or interruption. In CLA, it is one of the most frequent complications that reveal this pathology. It may occur as a result of the involvement of the lymphatic vessels of the pleura or thoracic duct by adjacent osteolysis. Some of the diagnoses include Gorham Stout disease and generalized lymphatic anomaly, which can be differentiated mostly by analyzing the imaging patterns of bone lesions. Diagnosis is based on clinical, analytical, radiological, and histopathological findings. We are reporting a case of a young 22-year-old female that presented with acute bilateral chylothorax with secondary to CLA. The diagnosis was confirmed through radiological findings using the non contrast MR lymphography, that revealed the presence of lymphatic malformations that were responsable for lytic bone lesions, and a splenic cystic lesion that was also compatible with the diagnosis, that was later on confirmed by histopathological of the pleural tissu,. This report aims to increase awareness and improve diagnostic approach of CLA, emphasizing the role of comprehensive imaging.

Keywords

• Chylothorax
• Complex Lymphatic Anomaly
• Generalized Lymphatic Anomaly
• Gorham Stout Disease
• Lymphangiomatosis