Complications of Lymphangioleiomyomatosis in Pregnancy: A Case Report and Review of the Literature

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease primarily affecting premenopausal females and may be exacerbated by pregnancy. We conducted a literature review of LAM during pregnancy, specifically focusing on related maternal morbidity and obstetric outcomes. We also report a case of LAM presenting as acute spontaneous pneumothorax in the third trimester of pregnancy followed by significant maternal morbidity. A 37-year-old primigravid woman presenting at 29 5/7 weeks 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 and resulting in urgent cesarean delivery at 30 2/7 weeks. 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 LAM in pregnancy, prompt recognition, diagnosis, and referral to appropriate multidisciplinary subspecialists is critical to mitigate complications and optimize outcomes, both during and after pregnancy.