B60-10 When the CT Lies Silent: Edema as the First Clue to Congenital Pulmonary Lymphangiectasis

Abstract Introduction Congenital pulmonary lymphangiectasis (CPL) is a rare developmental disorder involving the lung and characterized by pulmonary subpleural and interlobar lymphatic dilatation. A high-resolution CT (HRCT) scan is the imaging modality of choice when CPL is suspected. Findings typically include septal and peribronchovascular thickening of the interstitium and pleural thickening with or without effusions. We present the case of a 14-month-old patient who presented to our clinic with chronic respiratory distress, edema, and lymphadenopathy. His HRCT showed diffuse nonspecific ground glass opacities (GGO) with no interstitial or pleural changes. Case Our patient is a 14-month-old male born at 29-week of gestation and admitted to the NICU where he was intubated for 24 hours, transitioned to nasal-CPAP then low flow nasal cannula for respiratory support. He was eventually discharged on room air. At home, he continued to have persistent tachypnea not related to feeds and not associated with cough, choking or noisy breathing. He started to develop periorbital, upper and lower extremity edema which prompted him to be admitted at 2 months of age for evaluation of edema. A multidisciplinary evaluation did not uncover an etiology for his edema. Initial evaluation included a HRCT that showed hypodensity within left and right axillary soft tissue, upper lobe predominant perihilar consolidation, diffuse groundglass and linear opacities. He underwent a chest US followed by MRI which were remarkable for bilateral cervical and axillary lymphadenopathies in neck and chest. A lymph node biopsy was completed and showed immature adipose tissue with no significant histopathologic abnormality. Decision was made to perform a lung biopsy that showed CPL. A referral to nutrition was placed for MCT diet. Currently, he continues to have continuous tachypnea and subcostal retractions but is growing appropriately. He is on lasix as needed with improvement of his edema without any effect on his work of breathing. Discussion CPL is a rare congenital disorder characterized by dilatation of the pulmonary lymphatic channels. It’s true incidence remains unknown and is limited to isolated case reports with no established guidelines for management. In our case, chest HRCT findings were not suggestive of CPL, underscoring the importance of maintaining a high index of clinical suspicion even in the absence of characteristic imaging features. Definitive diagnosis, however, continues to rely on histopathologic confirmation via lung biopsy, which remains the diagnostic gold standard. This abstract is funded by: None