Atypical manifestation of pulmonary lymphatic perfusion syndrome treated with percutaneous lymphatic embolization

We report a case of a 62-year-old male with a history of obesity, COPD, diabetes, and smoking, who presented with progressive dyspnea on exertion. CT chest imaging demonstrated interlobular septal and peribronchovascular thickening in a lymphatic distribution, lung biopsy demonstrated dilation of the pulmonary lymphatics with lobular septal fibrosis and he was diagnosed with diffuse pulmonary lymphangiomatosis. A pedal MR lymphangiogram failed to visualize the thoracic duct, prompting further evaluation with intranodal MR lymphangiography, which revealed markedly dilated pulmonary lymphatics and retrograde lymphatic flow. The patient underwent successful thoracic duct embolization using N-butyl cyanoacrylate and coils. Follow-up imaging showed resolution of interstitial thickening, and the patient reported significant symptomatic improvement. This case underscores the diagnostic value of intranodal MR lymphangiography over pedal techniques and highlights an uncommon imaging and clinical presentation of pulmonary lymphatic perfusion syndrome. Early identification and targeted embolization of lymphatic conduction anomalies can offer significant clinical benefit.