Successful live birth via in vitro fertilization in a patient with lymphangioleiomyomatosis: a case report.

Background: Lymphangioleiomyomatosis is a rare, estrogen-sensitive multisystem disorder that predominantly affects women of reproductive age. Pregnancy and fertility interventions present a unique challenge in patients with lymphangioleiomyomatosis, as an elevated estrogen level during controlled ovarian hyperstimulation and pregnancy may exacerbate the disease.

Case presentation: We describe a 43-year-old Han Chinese female patient diagnosed with sporadic lymphangioleiomyomatosis because of recurrent pneumothorax and diminished ovarian reserve who had a strong desire for pregnancy. In vitro fertilization was considered the most effective treatment to improve pregnancy rate, with low-estrogen protocols recommended following a comprehensive multidisciplinary evaluation. In the first controlled ovarian hyperstimulation cycle, the patient was treated with a microstimulation protocol, resulting in a peak estradiol level of 621 pg/ml. Two fresh embryos were transferred but did not achieve pregnancy. In the second controlled ovarian hyperstimulation cycle, a combination of letrozole and antagonist protocol was used, with a peak estradiol level of 248 pg/ml. Endometrial preparation for the frozen embryo transfer was conducted using a natural cycle, leading to a successful pregnancy. Throughout the pregnancy, multidisciplinary collaboration ensured continuous monitoring of both lung function and fetal development. Eventually, she achieved a full-term live birth via elective cesarean section, and pulmonary parameters remained stable after pregnancy.

Conclusion: This is the first case report presenting a female diagnosed with sporadic lymphangioleiomyomatosis and diminished ovarian reserve who successfully achieved a live birth via in vitro fertilization with no lymphangioleiomyomatosis-related complication. The use of low-estrogen protocols and multidisciplinary collaboration are essential to ensure maternal-fetal safety and to minimize the risk of lymphangioleiomyomatosis progression during controlled ovarian hyperstimulation and pregnancy.