Síndrome Gorham-Stout : um desafio diagnóstico e terapêutico

Abstract

Diffuse lymphangiomatosis, also known as Gorham-Stout syndrome, is a rare disease. The pathology causes morphological alterations caused by the infiltration of lymphatic tissue, as well as the formation of chylous pleural effusion and chylous ascites, which can affect several organs such as bones, spleen, lung and others. Still considered a diagnostic challenge due to the limited knowledge about the disease, the limited number of cases reported, and due to diffuse involvement, with several possibilities of clinical manifestations depending on the affected organ.The gold standard for diagnosis remains the anatomo-pathological, but suggestive radiological findings, along with compatible clinical and evidence of chylous effusions, having other etiologies away, confirm a presumptive diagnosis of diffuse lymphangiomatosis. To date, no therapy has been effective, being a disease with unfavorable evolution and poor prognosis. The present study reports a case of presumed diffuse lymphangiomatosis.