Perioperative management of thoracic surgery in patients with lymphangioleiomyomatosis

BACKGROUND: General surgery for patients with lymphangioleiomyomatosis (LAM) is infrequent, however, general surgeons also occasionally experience it. Only a few reports have described the specific perioperative management appropriate for patients with LAM. Hence, in this case series, we aimed to investigate the surgical outcomes of LAM patients and their characteristics. CASE PRESENTATION: Medical records of 4482 patients who underwent thoracic surgery between 2009 and 2017 at our institution were assessed. Twelve patients were diagnosed with LAM. Details of the postoperative courses and surgical outcomes of LAM patients were retrospectively examined. All LAM patients were female (age 41.3 ± 10.6 years). Surgeries were performed for patients undergoing biopsy (n = 4) and those with pneumothorax (n = 3), lung cancer (n = 2), and other conditions (n = 3). The mortality rate was 0% and the length of hospital stay was 27.4 ± 8.9 days. Ten postoperative complications occurred in six patients (50%): hypoxemia (n = 5), chylothorax (n = 2), and prolonged air leakage (n = 3). CONCLUSIONS: Thoracic surgery may pose a risk of postoperative complications and long hospitalization for patients with LAM, although it lowers the risk of fatality. Management of perioperative air and chyle leakages and lymphatic stasis in the lungs is important for preventing morbidities.