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 with forme fruste of LAM, who presented with bilateral pneumothoraces requiring both surgical and medical pleurodesis. Clinical examination and investigations revealed hepatic and renal angiomyolipomas, a left retinal hamartoma, subcortical tubers and subependymal nodules in the brain as well as diffuse bilateral thin-walled lung cysts of varying sizes bilaterally. The histopathological examination (HPE) of the lung biopsy confirmed LAM. She was treated with 12 months of sirolimus with sustained effects on lung function and cystic lung lesions. Eight months post cessation of sirolimus, she conceived and delivered twins at 30 weeks. We describe the efficacy and safety of sirolimus and the successful twin pregnancy in a patient with LAM. This case highlights that treatment with sirolimus and surgical and medical pleurodesis was successful in preventing any further worsening of lung function.
Twin Pregnancy Following Sirolimus Therapy in Lymphangioleiomyomatosis
Abstrak
Lymphangioleiomyomatosis (LAM) adalah penyakit sista paru-paru di mana ianya terjadi secara sporadik atau berkaitan dengan penyakit “tuberous sclerosis complex”. Kami melaporkan satu kes “tuberous sclerosis complex” pada wanita berusia 26 tahun yang mengalami pneumothoraces serentak pada kedua-dua paru-paru dan memerlukan “pleurodesis” secara perubatan dan juga pembedahan. Pemeriksaan klinikal dan hasil siasatan mendapati beliau mempunyai angiomyolipoma pada hati dan ginjal, hamartoma pada retina kiri dan “subcortical tubers” dan “subependymal nodules” di otak berserta sista paru-paru berdinding nipis yang berlainan saiz pada dua-dua bahagian paru-paru. Hasil ujian histopatologi menunjukkan penyakit LAM. Beliau telah diberikan ubat sirolimus selama 12 bulan di mana tiada kemerosotan pada fungsi paru-paru atau peningkatan saiz sista paru-paru. Beliau mengandung 8 bulan selepas pemberhentian ubatan sirolimus dan melahirkan anak kembar pada minggu ke-30 kehamilan. Dalam kes ini, kami menekankan bahawa penggunaan ubat sirolimus berserta pleurodesis secara perubatan dan pembedahan berjaya mengelakkan kemerosotan fungsi paru-paru.
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