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Thoracic lymphangiomatosis presenting as chylothorax

Imaging findings

CT scan shows diffuse thickening of the interlobular septa, prominent peribronchovascular fluid cuffs, and bilateral pleural effusions. Thoracentesis confirmed a chylothorax. Biopsy of the lung showed abnormal lymphatic vessel proliferation consistent with lymphangiomatosis.

Key takeaways

Thoracic lymphangiomatosis is a rare lymphatic malformation that can present as diffuse interstitial thickening, peribronchovascular fluid cuffing, and recurrent chylothorax. It behaves as a slowly progressive, low-grade process.

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