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Case Reports
. 2021 Jan 18;14(1):e237824.
doi: 10.1136/bcr-2020-237824.

Initial presentation of lymphangioleiomyomatosis in third trimester of pregnancy

Lily Alkemade  1 , Meike A T Berghuis  2 , Bart Koopman  3 , Mariëlle G van Pampus  2
Affiliations
Case Reports

Initial presentation of lymphangioleiomyomatosis in third trimester of pregnancy

Lily Alkemade et al. BMJ Case Rep. .

Abstract

Lymphangioleiomyomatosis (LAM) is a progressive cystic lung disease which mostly affects premenopausal women and could be exacerbated by pregnancy. Therefore, it is thought that oestrogen plays an important role in LAM pathogenesis. Here, a case of LAM is described in which the first presentation of symptoms occurred during the third trimester of pregnancy. Symptoms included acute onset dyspnoea and chest pain at gestational age of 39 weeks and 2 days. A CT was performed which showed multiple thin-walled cysts and a small pneumothorax. Serum levels of vascular endothelial growth factor-D (VEGF-D) was 1200 pg/mL. The typical cystic lung changes on chest CT in combination with elevated VEGF-D is diagnostic for LAM. Given the risk of respiratory complications, the decision was made to deliver the baby at a gestational age of 39 weeks and 6 days by a planned caesarean section. Both mother and child were discharged home in good condition.

Keywords: gynaecology and fertility; obstetrics; pneumothorax; pregnancy; respiratory system.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Axial chest CT image showing a small cyst in the left lung aligning a small pneumothorax.
Figure 2
Figure 2
Axial chest CT image showing numerous small cysts bilateral.
See this image and copyright information in PMC

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