Pediatric Idiopathic Chylous Leak: A 15-Year Single-Center Experience

Objective: Idiopathic chylous leak (ICL) is defined by the accumulation of chyle in the pleural or peritoneal space, for which there is no underlying etiology. Given the low incidence and heterogeneity of presentation, evaluation, and management guidelines lack consensus. The purpose of this study is to provide insights regarding the clinical manifestations and propose a multidisciplinary treatment approach in ICL. Methods: A single-center retrospective chart review was conducted on patients with a diagnosis of idiopathic chylous ascites and/or chylothorax from January 1, 2008 to December 31, 2023. Data pertaining to the evaluation and treatments was abstracted. Results: A total of 18 patients with ICL were identified. Median age at diagnosis was 1 month (interquartile range [IQR]: 0–6.0) with 3 patients diagnosed prenatally. Clinical presentation was chylothorax for 78% and chylous ascites for 22% of patients. Most patients were initially managed conservatively with nil per os (NPO) and total parental nutrition (83%). Octreotide was utilized in 72% of patients. Sirolimus was used in 22% of patients. Seven patients required surgical management due to failure of medical management. All surgical patients underwent a magnetic resonance lymphangiogram before intervention. Nonoperative patients had fewer total NPO days (14.0, IQR: 0.0–45.0) relative to patients who required surgery (44, IQR: 20.0–48.0). However, median NPO days postoperatively were 12 days (IQR: 1.0–34.0). Conclusion: We describe our 15-year experience on the evaluation and management of ICL. We advocate a multidisciplinary management approach to promptly optimize both conservative measures and medical treatment. The use of dedicated lymphatic imaging and surgical intervention should be considered for intractable cases of ICL refractory to medical treatment. Level of Evidence: Level III.