Gross Description -- End-stage Restrictive Lung Disease and Liver Disease


Lungs: The right lung weighed 780 grams and the left 870 grams. The pleural surfaces of both lungs showed diffuse retraction and scarring with widespread fibrous adhesions and mild anthracotic pigment deposition. Multiple bands of fibrosis and cystic cavities were palpable from the external surfaces. The cut surfaces revealed diffuse thick fibrosis, bronchiectasis, and multi-loculated cysts containing clear yellow serous fluid (Image 01). Large areas of hemorrhage and clotted blood were present, and grossly were found within poorly demarcated ectatic bronchi or vascular structures. Relatively uninvolved parenchyma was red-brown and edematous, with bile stained yellow-green septa (Image 02). The pulmonary arterial trunk and branches were free of thrombi.

Liver: The liver was enlarged and weighed 2,340 grams. The capsular surface showed patchy focal gray-tan fibrosis. The common bile duct was widely patent, and on cut surface the right and left hepatic ducts were dilated with inspissated black-green bile (Image 03, arrow). The parenchyma was deeply bile-stained and diffusely replaced with pink-tan fibrosis. No nodules or masses were identified, and the portal and hepatic veins were free of thrombi.

Brain: The brain weighed 1,290 grams (formalin-fixed), with transparent meninges and intact symmetrical gyri without softening or hemorrhage. The optic chiasm was enlarged to 1.5 cm with involvement of the adjacent optic nerves (Image 04). Cut sections showed the optic chiasm to be widened and enlarged by a green-tan mass that extended superiorly to the hypothalamus (Image 05). The cerebral cortex, cerebellum, and spinal cord were unremarkable.




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