Introduction:

Pneumothorax is one of the pleural diseases most frequent in medical practice. Its association with pregnancy is essential. Few cases are reported in worldwide medical literature. Lymphangioleiomyomatosis is a rare multisystem disease that predominantly affects individuals of the female sex and at fertile age; it can be aggravated by pregnancy.

Objective:

To present a case report and a topic review that allow early diagnosis and early management.

Clinical case:

A 15-week-pregnant patient aged 36 years and with a previous health history suddenly presented dyspnea for average efforts, cough, chest pain, and spontaneous right pneumothorax, recurrent in its evolution and, later, bilateral. Its evolution was slow, a reason why it required intensive care for acute respiratory failure. The patient died three months after the onset of symptoms. The patient was performed chest x-ray, which showed honeycomb-type reticular pattern; and chest tomography, which showed multiple cystic images throughout pulmonary parenchyma, predominantly at baseline. Lung biopsy consistent with lymphangioleiomyomatosis was performed.

Conclusions:

Onset of sudden dyspnea, pleuritic pain and pneumothorax in a pregnant woman should always be sufficiently indicative of lymphangioleiomyomatosis. Its initial symptomatic non-specificity is determined by late diagnosis, which impoverishes prognosis.

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