Twin Pregnancy Following Sirolimus Therapy in Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is an orphan cystic lung disease which can occur sporadically or in association with tuberous sclerosis complex (TSC). We report a 26-year-old woman diagnosed forme fruste of LAM, who presented bilateral pneumothoraces requiring both surgical and medical pleurodesis. Clinical examination investigations revealed hepatic renal angiomyolipomas, left retinal hamartoma, subcortical tubers subependymal nodules the brain as well diffuse thin-walled cysts varying sizes bilaterally. The histopathological (HPE) biopsy confirmed LAM. She was treated 12 months sirolimus sustained effects on function lesions. Eight post cessation sirolimus, she conceived delivered twins at 30 weeks. describe efficacy safety successful twin pregnancy patient This case highlights that treatment pleurodesis preventing any further worsening function.