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

Abstract 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 contr...

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Main Authors: Yusen Ou, Yanqiu Xie, Xinyue Zhang, Weilin Wang, Yun Lin, Yequn Chen, Haiying Wang, Yang Zeng
Format: Article
Language:English
Published: BMC 2025-08-01
Series:Journal of Medical Case Reports
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Online Access:https://doi.org/10.1186/s13256-025-05465-y
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Summary:Abstract 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.
ISSN:1752-1947