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Case Reports
. 2021 Jan 18;14(1):e237331.
doi: 10.1136/bcr-2020-237331.

Systemic generalised lymphangiomatosis: unknown aetiology and a challenge to treat

Tess Van Meerhaeghe  1 , Frederik Vandenbroucke  2 , Brigitte Velkeniers  3
Affiliations
Case Reports

Systemic generalised lymphangiomatosis: unknown aetiology and a challenge to treat

Tess Van Meerhaeghe et al. BMJ Case Rep. .

Abstract

We describe a case of a woman diagnosed at the age of 35 years with a generalised mediastinal and abdominal lymphangiomatosis associated with a protein losing enteropathy, who successfully improved when treatment with sirolimus was initiated.

Keywords: cardiovascular system; malabsorption.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Coronal view of the contrast enhanced CT scans with the selected content of the peritoneal and retroperitoneal cysts, marked in blue. A, B illustrates the lymphangiomatosis at the start (A) and 24 months after start of sirolimus therapy (B). (C, D): Three-D image of the calculated volumes of the selected content as shown in (A, B). This was done for both scans with an interval of 24 months. (C) Before start of therapy—(D) 24 months after start of sirolimus therapy).
See this image and copyright information in PMC

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