Conservative management of an unusual cause of breathlessness during pregnancy

A 37 yr old lady in her 14th week of her second pregnancy presented to chest clinic with breathlessness. Over the preceding month she had gradual onset of exertional breathlessness limiting her activities of daily living. Examination and routine blood tests were unremarkable and she declined a chest radiograph. Her symptoms progressed and she allowed us to perform a chest radiograph, which revealed a large right sided pneumothorax. This was aspirated successfully. During the final few months of her pregnancy she had a further episode of symptomatic ipsalateral pneumothorax, which was treated successfully with aspiration. She delivered a healthy baby via uncomplicated caesarean section under a spinal anaesthetic at term. A chest radiograph taken 2 d post-partum showed normal lung fields with no pneumothorax. Unfortunately, 2 weeks later she developed her third ipsalateral pneumothorax and this was treated conservatively pending video-assisted thoracoscopic surgery and pleurodesis, which occurred a few weeks later. At VATS the appearance of her lungs was grossly abnormal and a biopsy was sent for histological analysis. After a HRCT and specialist staining of her lung biopsy the diagnosis of lymphangioleiomyomatosis was established.