Pregnancy and lymphangioleiomyomatosis: anaesthetic management

Lymphangioleiomyomatosis is a rare, progressive cystic pulmonary disease related to tuberous sclerosis complex, affecting almost exclusively females of childbearing age. Progression of the disease is variable but may lead to terminal respiratory failure. As the disease may be oestrogen-dependent, it can arise de novo in pregnancy or established disease may undergo exacerbation. We report on the successful labour and delivery of such a patient and present the results of an internet survey of 30 pregnancies in 15 patients worldwide with lymphangioleiomyomatosis conducted by our patient before delivery.