Chylothorax and constrictive pericarditis in a woman due to generalized lymphatic anomaly: a case report.

BACKGROUND: Generalized lymphatic anomaly (GLA) is characterized by diffuse or multicentric proliferation of dilated lymphatic vessels resembling common lymphatic malformations. Compared with soft tissue or bone involvement, thoracic involvement may be associated with a worse prognosis.

CASE PRESENTATION: We reported a case of GLA with chylothorax and constrictive pericarditis in a 29-year-old woman. This patient exhibited remarkable features, including a continuously hemorrhagic chylothorax, constrictive pericarditis, and involvement of bone and neck lymph nodes. After attempting to manage her condition with conservative treatment, the patient underwent pericardial stripping surgery. Exploration revealed abundant hyperplasia of tubular tissue in the aortopulmonary window in both pleural cavities.

CONCLUSIONS: This case highlights the importance of maintaining the clinical suspicion of GLA during the follow-up of chylothorax patients. Aggressive pericardial surgery, which is important for both diagnosis and treatment, should be performed in patients with GLA with constrictive pericarditis.