Plastic bronchitis chylous reflux and lymphatic imaging: a continuing story.

In 1973, during the 4th International Congress on Lymphology in Tucson, thoracic surgeon Herbert Maier, a devout cardcarrying lymphologist, chatted in our office about his latest mission – to raise awareness of what he coined “chyloptysis” and the neglected role of the “(cardio)thoracic lymphatic system” in a variety of rare and common lymphatic disorders. As a daily reminder of the body’s vulnerability to dysfunction of the interstitial fluid drainage system, he himself had suffered from a recurrent craniopharyngioma for more than 20 years plagued by a continuous nasal leak of cerebrospinal fluid through his re-operated and chronically infected cribiform plate. In a visionary yet long buried paper (1) from his 1966 Presidential Address to the American Thoracic Society, he called attention to the lymphatics of the chest as the next frontier for thoracic surgeons. Further, he confessed to us his frustrating encounters with a little known usually fatal lung disease without a name (now called lymphangioleimyomatosis or LAM), which he viewed as a prime example of disordered pulmonary lymphatic proliferation, influenced by female hormones, and commonly manifesting as chylous effusions and occasionally as “chyloptysis” – expectoration of milky intestinal lymph. He cared compassionately and alone for a group of these LAM patients and improved their outlook with a variety of operative approaches to lymphatic complications, rerouting the lymph circulation, and suppressing hormonal influences. At the same time, the newly formed International Society of Lymphology (ISL) in its earliest Congresses (2) was also focusing on improved visualization of the previously inaccessible peripheral and central lymphatic system by invasive lipid-soluble contrast lymphography (LAG) (Kinmonth, Viamonte, Ruttimann, Wallace, Kandl) and also featuring visceral chylous reflux syndromes and their treatment including dietary and operative approaches [surgeons from Belgium (Godart, Gruwez), France (Servelle), Italy (Tossati) and Brazil (Degni, Cordeiro) among others]. In this issue of Lymphology, in an era where cardiac surgery has reached new heights in rescuing infants born with previously unsurvivable complex congenital cardiac anomalies, Herbert Maier’s simple and timeless observations resonate with the case report of a child successfully treated for life-threatening “plastic bronchitis” manifesting as chyloptysis after a series of cardiovascular operations leading up to a Fontan procedure for hypoplastic left ventricle (3). Indeed, the Pediatric Cardiology and Pulmonary Divisions at the University of