Bilateral pneumothorax in pregnancy unmasking lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare idiopathic lung disease which almost exclusively affects pre-menopausal women [1]. It is generally progressive, which can potentially lead to respiratory failure and death [2]. Pregnancy may be implicated in disease pathogenesis, progression and subsequent complications. An 18-year-old female of 20-weeks gestation in her first pregnancy (G1P0) was admitted with a 2-day history of increasing dyspnoea and fatigue. She was otherwise well with no other medical co-morbidities but was a smoker of 15 cigarettes per day. Respiratory examination revealed reduced breath sounds on the left side with associated hyperresonance. An urgent chest X-ray (CXR) confirmed a 70% left pneumothorax. A 16F seldinger intercostal drain (Portex, Smiths Medical International, Kent, UK) was subsequently inserted under local anaesthetic without difficulty and placed on low grade suction. Despite the insertion of a further 16F seldinger intercostal chest drain, the lung failed to expand with the continued presence of persistent air leak. A larger apical 24F intercostal drain (Portex, Smiths Medical International, Kent, UK) was inserted following transfer to the regional thoracic surgery unit and placed on suction. A persistent air leak with no evidence of radiological improvement necessitated bilateral thoracoscopy demonstrating grossly abnormal lungs with multiple cystic lesions at sequential stages. Several of the larger bullae were stapled bilaterally and lung biopsies were taken. Histopathological examination confirmed lymphangioleiomyomatosis (LAM) (Fig. 1). A foetal ultrasound scan at 29 weeks gestation confirmed poor in utero foetal growth compared to 2 weeks previously. A caesarean section was subsequently performed under general anaesthetic. Jet ventilation was administered throughout the procedure to minimise the risk of further pneumothoraces and intercostal drainage continued throughout the peri-partum period. A female baby, weighing 1,115 g, was delivered without complication. The patient was advised to avoid any future pregnancy. The patient made an uneventful recovery and was discharged. Two years after discharge, thromboembolisation was performed for an incidental progressively enlarging right kidney angiomyolipoma. Respiratory function continued to deteriorate which necessitated referral to a national lung transplant unit. Unfortunately she died from complications relating to transplant 4 years after her initial LAM presentation. Lymphangioleiomyomatosis (LAM) is a rare clinicopathological abnormality characterised by inappropriate hamartomatous proliferation of smooth muscle [3]. Johnson et al. [1] reported predominance in pre-menopausal females with a median age of symptom onset of 35 years (22–50 years) and a 5-year prevalence of 1 per 1.1 million population in the United Kingdom. Although older patients C. R. Johnston A. N. Graham Department of Thoracic Surgery, Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, Northern Ireland, UK