Ya-nan Zhang, Zhen Yuan, Jin-chen Du, Bin Liu, Fu Li, Shou-Cai Hu, Qing-Xin Li
940th Hospital of Joint Logistics Support Force of Chinese People’s Liberation Army. Gansu University of Traditional Chinese Medicine.
China
Frontiers in Pediatrics
Front Pediatr 2026; 13:
DOI: 10.3389/fped.2025.1591611
Abstract
We report a rare case of generalized lymphatic anomaly (GLA) in a 12-year-old male, presenting with intermittent cough, expectoration, and hemoptysis exacerbated by strenuous activity. Key features included bilateral pleural/pericardial effusions; chest/abdominal CT showed diffuse mediastinal infiltration, bronchovascular/interlobular septal thickening, and extrathoracic extension to periaortic abdominal tissues. Definitive diagnosis was confirmed by thoracoscopic biopsy and immunohistochemistry (CD31+, D2-40+, Ki-67 ≈ 3%), differentiating it from mimics like pulmonary lymphangiectasia. The patient achieved symptomatic remission after pericardiectomy, thoracic catheter drainage, and postoperative sirolimus. This case enriches pediatric GLA literature, highlights multidisciplinary diagnosis value, and supports mTOR inhibitors’ role in pediatric GLA management.
Category
Primary Pulmonary Lymphatic Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes