PROGESTERONE TREATMENT IN CHYLOTHORAX ASSOCIATED WITH PULMONARY TUBEROUS SCLEROSIS

We report the case of a young woman with tuberous sclerosis who develo ped a chylous pleural effusion after several invasive procedures for t reatment of recurring pneumothoraces, Oophorectomy was rejected by the patient and progesterone therapy initiated. Medroxyprogesterone aceta te administration led to a complete disappearance of the chylothorax i n 8 months, The patient was kept on therapy, and no recurrence of pleu ral effusion has been observed up to the present (22 months follow-up) , However,a progressive deterioration in pulmonary function was observ ed, with a significant decrease in pulmonary transfer factor and incre ase in airway obstruction. In conclusion, this report demonstrates an objective benefit of progesterone therapy on chylous effusion associat ed with pulmonary tuberous sclerosis.