Abstract
Lymphangiomatosis – heterogenic disease with generalized or multifocal lymphatic vessels proliferation in bones and extraosseous tissues. We report a case of 2-year monitoring of 8-year-old boy with diffuse lymphangiomatosis. We have described clinical manifestation, surgery, differential diagnosis, Sirolimus treatment, and changes in MRI-images. Prolonged unsuccessful chylothorax, that mimics pneumonia with exudative pleuritis at the onset, treatment was a distinctive feature of this rare disease. The absence of guidelines and certified for children use drugs prompted our multidisciplinary team to make a decision to prescribe off-label Sirolimus as a pathogenetic therapy for this patient. Annual admissions for examination and modulating of drug`s dose allow to correct the treatment according to serum concentration of Sirolimus and MRI changings in the dynamics. Personalized target therapy has resulted in positive outcomes in the treatment of such difficult patient Key words: children, chylotorax, lymphangiomatosis, Sirolimus
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