Treatment of Refractory Congenital Chylous Ascites Caused by Congenital Lymphangiectasia with Propranolol

Abstract

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.

Keywords

• Chylous ascites,infant,newborn,lymphangiectasis,propranolol

Konjenital Lenfanjiektazinin Neden Olduğu Dirençli Şilöz Asidin Propanolol İle Tedavisi

Abstract

Şilöz asit, şilusun periton boşluğuna sızması sonucunda oluşan nadir bir klinik durumdur. Bir çok nedeni olmakla birlikte genellikle lenfatik kanallara ait patolojilerden kaynaklanır. Tanı ve tedavi yöntemlerinin henüz standardize edilmemiş olması nedeniyle tedavinin hastaya göre belirlenmesi önemlidir. Oktreotid uygulaması, total parenteral nutrisyon ve orta zincirli trigliseridlerden zengin diyet tedavinin ilk aşamasını oluşturmakta olup, medikal tedaviye dirençli olgularda cerrahi girişim uygulanabilir. Literatürde yenidoğan döneminde klasik tedavilere yanıt vermeyen, ancak propranolol tedavisi ile düzelen yalnızca bir lenfanjiektazi olgusu mevcuttur. Bu yazıda, non-immun hidrops fetalis tanısı ile yenidoğan yoğun bakıma kabul edilen, izlemde lenfosintigrafi ile lenfanjiektazi tanısı alan, klasik tedavi yöntemleri ile şilöz asidi gerilemediği için propranolol tedavisi uygulanan bir yenidoğan sunulmaktadır.

Keywords

• Şilöz asit,infant,yenidoğan,lenfanjiektazi,propranolol

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