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

Year 2019, Volume: 16 Issue: 1, 51 - 54, 01.01.2019

Özkan İlhan Sinem Akbay Senem Alkan Özdemir Berat Kanar Şeyma Memur Meltem Bor Esra Arun Özer

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

References

• Altunhan H, Annagür A, Ertuğrul S, Yüksekkaya HA, Örs R. Coexistence of congenital chylous ascites and congenital hypothyroidism: case report. Turkiye Klinikleri J Med Sci 2012;32:1486-9.
• Mouravas V, Dede O, Hatziioannidis H, Spyridakis I, Filippopoulos A. Diag- nosis and management of congenital neonatal chylous ascites. Hippokra- tia 2012;16:175-80.
• Bellini C, Ergaz Z, Radicioni M, Forner-Cardero I, Witte M, Perotti G. Con- genital fetal and neonatal visceral chylous effusions: neonatal chylothorax and chylous ascites revisited. A multicenter retrospective study. Lympho- logy 2012;91-102.
• Rerksuppaphol S, Rerksuppaphol L. Neonatal chylous ascites success- fully treated with mct-based formula and octreotide: a case report. Int J Clin Pediatr 2012;1:30-3.
• Romaska-Kita J, Borszewska-Kornacka MK, Dobrzaska A, Rudziska I, Czech-Kowalska J, Wawrzoniak T. Congenital chylous ascites. Pol J Ra- diol 2011;76:58-61.
• Poralla C, Specht S, Born M, Müller A, Bartmann P, Müller A. Treatment of congenital generalized lymphangiectasia with propranolol in a preterm infant. Pediatrics 2014;133:438-43.
• Ozeki M, Fukao T, Kondo N. Propranolol for intractable diffuse lymphangiomatosis. N Eng J Med 2011;364:1380-2.
• Jeltsch M, Kaipainen A, Joukov V. Hyperplasia of lymphatic vessels in VEGF-C transgenic mice. Science 1997;276:1423–5.
• Ingle SB, Hinge CR. Primary intestinal lymphangiectasia: minireview. World J Clin Cases 2014;2:528-33.
• Kuroiwa M, Toki F, Suzuki M, Suzuki N. Successful laparoscopic ligati- on of the lymphatic trunk for refractory chylous ascites. J Pediatr Surg 2007;42:15-8.