Abstract
Lymphangioleiomyomatosis is a rare cystic lung disease primarily affecting premenopausal females and may be exacerbated by pregnancy. We conducted a literature review of lymphangioleiomyomatosis during pregnancy with a specific focus on related maternal morbidity and obstetrical outcomes. We also report a case of lymphangioleiomyomatosis that presented as an acute spontaneous pneumothorax in the third trimester of pregnancy, followed by significant maternal morbidity. A 37-year-old primigravid woman who presented at 29 weeks 5 days gestation with chest pain was diagnosed with spontaneous pneumothorax. Further imaging demonstrated cystic lung lesions and renal angiomyolipomas. She developed severe abdominal pain concerning for placental abruption that led to an urgent cesarean delivery at 30 weeks 2 days gestation. Her course was complicated by recurrent pneumothorax, superimposed preeclampsia, and significant ileus and bowel dilation complicated by bowel perforation. For patients with a clinical suspicion of lymphangioleiomyomatosis in pregnancy, prompt recognition, diagnosis, and referral to appropriate multidisciplinary subspecialists is critical to mitigate complications and optimize outcomes both during and after pregnancy.