Chylothorax and Chylous-Like Diseases in Children: Clinical Management

Abstract

Background: Chylothorax and chylous-like diseases are rare conditions and difficult to treat. But they may represent potentially life-threatening disorders and important causes of morbidity and prolonged hospitalization, especially in critically ill children. Conservative as well as surgical therapeutic management strategies are continuously performed at our institution, however the results have never been evaluated and no guidelines for treatment recommendations have been put into practice so far. The objective of this retrospective study was to present a comprehensive and substantial evaluation of all relevant demographic data from children with the chylothorax and chylous-like diseases and their clinical management. Methods: We retrospectively analyzed data from all children with diagnoses of chylothorax and chylous-like diseases admitted to our pediatric intensive care unit between the years 1999 and 2012. Results: Data of 34 patients were analyzed for this study. Gender distribution (M/F) was almost equal (19/15; 56%/44%). Thirty-one children (91%) developed chylothorax after surgery. Two children (6%) had idiopathic chylothorax and in one child (3%) congenital chylothorax was diagnosed. All study patients ( n = 34; 100%) received MBF/MCT therapy. We were quite successful in treating 14 children who received only this therapy, with chest tube output dropping from 100 to 4.7%. But only 11 (32%) children received somatostatin and 7 (20%) children received beta-isodona. Different surgical interventions were performed in 6 patients (17%). All study patients received chest tubes to drain the pleural fluid and hence to relieve the chyle related symptoms. Conclusion: A combination of different conservative therapies was successful in most of our patients. Prevention, early diagnosis and treatment of potential complications may further improve the success rate of conservative therapy especially in patients with postoperative chylothorax. In summary, appropriate therapy of this condition may be lengthy but can prevent significant morbidity and mortality.

Etiological factors and treatment of chylothorax in paediatric patients - a systematic review

“…The mean gestational age at diagnosis was 31.7 weeks. Infants constituted the largest group of patients in which the first symptoms of chylothorax were observed (45%) [2][3][4], Additional demographic information is shown in Table 1. Causes of chylothorax are extremely diverse.…”

“…However, the analysis of the available literature shows that iatrogenic factors are the most common cause (70.8%). Among surgical procedures contributing to the development of chylothorax, cardiothoracic surgeries are most prevalent, with predominance of Fontan procedures and congenital heart defect corrective surgeries (61.2%) [2], [3], [6]- [30]. A significant group undergoing these procedures were infants with genetic conditions, such as Noonan syndrome, Down syndrome or Gorham disease.…”

“…Chylothorax also occurred post-operatively in patients with congenital diaphragmatic hernia, tracheoesophageal fistula or after other thoracic operations (in 8% of the analyzed cases) [3], [15], [20], [27], [36]-[54]. 12,8% of children developed an effusion prenatally or shortly after birth [3], [20], [27], [32], [33], [37], [39], [52], [55]- [68].…”

“…It is more common to accumulate on the right side of the chest. [1][2][3] The diagnosis of pleural effusion begins with imaging tests. Ultrasound is the most commonly performed test because of its high-accessibility and non-invasiveness.…”

Incidence and Nutritional Management of Chylothorax after Surgery for Congenital Heart Diseases in Children

“…Resolution of chylothorax has been described with TPN (complete oral intake cessation) [Bond 1993], a fat-free/ low-fat diet [Chan 2010;Büttiker 1999], or an MCT plan [Chan 2010;Biewer 2010;Densupsoontorn 2005;Cormack 2004;Hermon 2019;Chan 2006] in children with variable success rates, but comparisons of the therapeutic effectiveness of nutrition support modalities in children are few and inconsistent. Nguyen et al [1995] reported that in 15 patients on TPN or a low-fat enteral diet, the average duration of lymph leak was 13.7 versus 13.2 days, respectively.…”