Case ReportA 20-year-old man complained of exertional dyspnea for two months, cough and asthenia. He had no fever, chest pain or lower limb edema. Past medical history, physical examination and lab results were unremarkable. Cardiac ultrasound showed an abundant compressive pericardial effusion and a “mediastinal mass” that was present on the topographic scan of the chest computed tomography (CT) as a mediastinal widening (Figure 1). The contrast-enhanced chest CT itself comprehensively displayed the pericardial effusion and the diffusely hypodense infiltra-tion of the mediastinum extending to hila (Figure 2). There was diffuse thickening of the lung interstitium and patchy ground glass opacities (Figure 3). A thoracoscopy with pleuro-pericardial window and biopsy was performed and led to the diagnosis of pulmonary lymphangiomato-sis. The patient’s symptoms subsided after this surgery followed by multiple pleural punctures. An immuno-suppressive treatment with Sirolimus (Rapamicyn) was initiated, and the patient has now had good clinical and radiological evolution for four years.

DiscussionDiffuse pulmonary lymphangiomatosis is a rare pulmo-nary lymphatic system disorder characterized by the pro-liferation and distension of lymphatic vessels. It occurs in

CHR Liège, BECorresponding author: Laurent Médart (laurent.medart@chrcitadelle.be)

IMAGES IN CLINICAL RADIOLOGY

Diffuse Pulmonary LymphangiomatosisCaroline Ernotte, Laurent Médart and Laurent Collignon

Keywords: lymphangiomatosis; thoracic malformation

Ernotte, C, et al. Diffuse Pulmonary Lymphangiomatosis. Journal of the Belgian Society of Radiology. 2018; 102(1): 64, 1–2. DOI: https://doi.org/10.5334/jbsr.1603

Figure 1.

Figure 2.

Figure 3.

Ernotte et al: Diffuse Pulmonary LymphangiomatosisArt. 64, page 2 of 2

children and young adults with equal sex prevalence. It is more aggressive in patients who present at a younger age. Symptoms are nonspecific, including dyspnea, cough and wheezing, hemoptysis, chyloptisis and chest pain. The major symptoms are directly related to pleural and/or pericardial chylous effusions.

The CT findings are quite typical: diffuse interstitial thickening (interlobular, peribronchovascular and pleu-ral), patchy ground glass opacities, pleural thickening, pleural and/or pericardial effusion and mediastinal soft tissue infiltration with no mass effect on mediastinal vessels. Differential diagnosis is limited to other pulmo-nary lymphatic disorder [1].

Lymphatic imaging is complex, either by invasive technique (lymphangiography associate or not to cross-sec-tionnal imaging, lymphoscintigraphy) or by non- invasive technique (Magnetic resonance lymphangiography,

T2-weighted). Those radiologic exams are not much avail-able except in specialized centers.

Different therapies have been tried like various systemic or chemotherapies, radiological embolization, radiation therapy and surgical approaches including lung transplan-tation, but with inconstant results. There is no standardized treatment protocol. The prognostic is poor, with progres-sive dyspnea leading to death from respiratory failure.

Competing InterestsThe authors have no competing interests to declare.

Reference 1. Xiaoli, S, Wenbin, S, Song, X, et al. Diffuse

Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography. AJR, 2017; 208: 300–305. DOI: https://doi.org/10.2214/AJR.16.16589

How to cite this article: Ernotte, C, Médart, L and Collignon, L. Diffuse Pulmonary Lymphangiomatosis. Journal of the Belgian Society of Radiology. 2018; 102(1): 64, 1–2. DOI: https://doi.org/10.5334/jbsr.1603

Submitted: 05 July 2018 Accepted: 19 September 2018 Published: 04 October 2018

Copyright: © 2018 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC-BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See http://creativecommons.org/licenses/by/4.0/.

OPEN ACCESS Journal of the Belgian Society of Radiology is a peer-reviewed open access journal published by Ubiquity Press.