Young patient with generalized lymphangiomatosis: Differentiating the differential

Abstract

We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices. There were a few dilated retroperitoneal lymphatics and well-defined lytic lesions in the bones. In this case report, we aim to systematically discuss the relevant differentials and arrive at a diagnosis. We also briefly discuss the treatment options and prognosis along with our patient's course in the hospital and final outcome.

Keywords: Cystic mediastinal mass; generalized lymphangiomatosis; pulmonary interstitial thickening; pulmonary lymphangiectasia.

Figure 1

Chest radiograph showing mediastinal widening, bilateral pleural effusion (open arrows), prominent interstitial lung markings (oval), and Kerley B lines (curved arrows)

Figure 2 (A-C)

Images of CT thorax in mediastinal window, axial (A, B) and coronal (C) images showing confluent, sheet-like, hypodense (HU-28) mediastinal lesion (straight solid arrows) encasing the mediastinal viscera with no mass effect. Left pleural effusion (star) and pericardial effusion (curved arrow) are also evident on coronal image (C). A well-defined lytic lesion is seen in the sternum (open arrow) in image 2A

Figure 3 (A-C)

Coronal (A, B) and axial (C) CT images of chest in lung window, showing interlobular septal thickening (solid arrows), peribronchovascular interstitial thickening (open arrows) and patchy lobular areas of ground glass density (oval) in both lungs with relative sparing of the lung apices

Figure 4 (A-C)

Axial (A, B) and coronal (C) CT images in bone window, showing the lytic lesion in sternum (open arrow in A), mottled lucencies with coarse trabecular pattern in vertebrae and pelvic bones (solid arrows in B and C)

Figure 5 (A-C)

Axial (A, B) and coronal (C) CT images of abdomen showing dilated lesser omental lymphatics (open arrow in A) and retroperitoneal lymphatics (solid arrows in B and C)