A Rare Lung Disease Diagnosed by CT in a Young Pregnant Woman

Questions and Text

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that predominantly affects women in the reproductive age group, many of whom may present with a pneumothorax1. High-resolution CT is known to provide a correct diagnosis in over 80% of patients.

Q1. What is the diagnosis based on the Chest X- ray
shown in Figure 1?

Q2. What is the diagnosis based on the high-resolution CT shown in Figure 2?

A 38-year- old woman in her second pregnancy at 20 weeks gestation presented to our maternity unit with a history of shortness of breath and chest tightness.1 Physical examination showed decreased air entry on the left side of the chest and her oxygen saturation was 90% on room air. A chest X- ray (Figure 1) revealed a left- sided pneumothorax, and she went on to have a CT scan of the chest (Figure 2). This showed multiple thin-walled cystic lesions

CT scan showing characteristic appearance of LAM in both lung fields with a large left-sided pneumothorax as well as a chylothorax. These appearances are consistent with a diagnosis of lymphangioleiomyomatosis, a rare multisystem disease that can occur either sporadically or in association with tuberous sclerosis complex. In 2016, the American Thoracic Society and the
Japanese Respiratory Society published clinical practice guidelines that state a definitive diagnosis of LAM can be established if the patient has a characteristic high-resolution CT of the chest.2 Therefore, LAM is one of the few diseases in which a diagnosis can be made on solely on imaging, even in the absence of a confirmatory histopathological lung biopsy.

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