Neonatal Lymphatic (Chylous) Disorders

Abstract

Lymphatic (chylous) disorders are rare in neonates, and most clinicians will only experience a handful of cases during their careers. Chylous effusions of the pleura, peritoneum and pericardium, and lymphedema are not diagnoses but rather symptoms of an underlying disease of the lymphatics. Although it is relatively easy to establish whether an effusion is of chylous origin or not, expertise in different imaging techniques of the lymphatic system does not appear to be universally available. This makes it difficult to determine the cause of the effusion and thereby arrive at a prognosis, and whether and when to recommend surgical treatment. The goal of current medical management is to treat symptoms by reducing overall lymphatic flow and to allow enough time to resolve the lymphatic leakage (whatever the underlying cause) without simultaneously risking infectious complications. Most current therapies are not evidence-based. A prospective registry of patients who have chylous effusions and a multicenter randomized controlled trial to evaluate different treatment options are needed.