Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography

Abstract

Historically, congenital pulmonary lymphangiectasia (CPL) has had poor treatment outcomes despite aggressive therapy. There are recent reports of ethiodized oil (Lipiodol) lymphangiography successfully treating lymphatic leakage in adults. In this report, we describe two infants with CPL complicated by chylothoraces successfully treated by instillation of ethiodized oil into the lymphatic system. Congenital atresia of the thoracic duct was demonstrated on the lymphangiogram in both patients before treatment. Both patients have shown good short-term outcomes without supplemental oxygen or fat restricted diets at 9 months of age. Ethiodized oil lymphangiography represents a new treatment modality for some patients with CPL.

Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease

“…Several earlier lymphangiographic studies described the development of chylous effusion caused by lymphatic reflux similar to our observation. 24, 25 Gray et al 26 reported complete occlusion of flow in the TD and retrograde flow of lymph toward lung parenchyma in congenital chylothorax similar to the type 4 pattern. In 5 of 16 patients, the TD was occluded primarily at the distal end.…”

Magnetic Resonance Lymphangiography and Lymphatic Embolization in the Treatment of Pulmonary Complication of Lymphatic Malformation

“…PLPS is a syndrome that was described in several conditions such as cardiac and lymphatic plastic bronchitis and neonatal chylothorax. 8,15,16 In these conditions, there is an abnormal pulmonary lymphatic flow that caries the lymph from the TD toward lung parenchyma. Clinical presentations of PLPS depend on the type of lung surface that is in proximity to the abnormal lymphatic ducts.…”

“…The anatomical substrate of PLPS is a congenital malformation of the thoracic lymphatic ducts and retroperitoneal and mediastinal lymphatic masses. [7][8][9]…”

Lymphatic System: A Forgotten Area?

“…-в работе Вирхова описаны лимфатические кисты в легких ребенка при пато-морфологических исследованиях. С тех пор о мно-гочисленных подобных наблюдениях докладывали на патологоанатомических форумах [12,13]. С раз-витием метода ЛГ некоторые авторы описали фено-мен вытекания лимфы из ГП в легочную паренхиму и назвали его «лимфатический рефлюкс» [14].…”