Konjenital Lenfanjiektazinin Neden Olduğu Dirençli Şilöz Asidin Propranolol İle Tedavisi: Olgu Sunumu

Şilöz asit, şilusun periton boşluğuna sızması sonucunda oluşan nadirbir klinik durumdur. Bir çok nedeni olmakla birlikte genellikle lenfatikkanallara ait patolojilerden kaynaklanır. Tanı ve tedavi yöntemlerininhenüz standardize edilmemiş olması nedeniyle tedavinin hastaya görebelirlenmesi önemlidir. Oktreotid uygulaması, total parenteral nutrisyonve orta zincirli trigliseridlerden zengin diyet tedavinin ilk aşamasınıoluşturmakta olup, medikal tedaviye dirençli olgularda cerrahi girişimuygulanabilir. Literatürde yenidoğan döneminde klasik tedavilereyanıt vermeyen, ancak propranolol tedavisi ile düzelen yalnızca birlenfanjiektazi olgusu mevcuttur. Bu yazıda, non-immun hidrops fetalistanısı ile yenidoğan yoğun bakıma kabul edilen, izlemde lenfosintigrafi ilelenfanjiektazi tanısı alan, klasik tedavi yöntemleri ile şilöz asidi gerilemediğiiçin propranolol tedavisi uygulanan bir yenidoğan sunulmaktadır.

Treatment of Refractory Congenital Chylous Ascites Caused by Congenital Lymphangiectasia with Propranolol: Case Report

Chylous ascites is a rare clinical condition occurring as the result of the extravasation of the chyle into the peritoneal cavity and caused by numerous factors. The most common cause of underlying pathologies are lymphatic vessel disorders. Owing to the lack of the therapeutic and diagnostic standards, individual therapy seems to be extremely important. Octreotide administration, total parenteral nutrition and a diet rich in medium-chain triglycerides constitute first step of the treatment, but surgical intervention may be performed in case of failure of the medical treatment. In literature, there is only one neonate with refractory generalized lymphangiectasia who is treated with propranolol. A newborn baby was admitted to our clinic after delivery due to nonimmune hydropsfetalis and he was diagnosed as lymphangiectasia with lymphoscintigraphy. Herein, we describe the use of propranolol in the therapy of lymphangiectasia in an infant with refractory chylous ascites.

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