Intestinal lymphangiectasia: Understanding the bigger picture

Abstract

Intestinal lymphangiectasia (IL) is characterized by the dilation of intestinal lymphatic vessels, which can rupture and cause loss of lymph into the intestine. Due to the high content of proteins, lipoproteins, and lymphocytes in the intestinal lymph, loss of lymph might result in hypoproteinemia, hypoalbuminemia, hypogammaglobulinemia, and lymphocytopenia. In addition, there may be a depletion of minerals, lipids, and fat-soluble vitamins. IL can be primary due to inherent malfunctioning of the lymphatic system, or secondly, a result of various factors that may hinder lymphatic drainage either directly or indirectly. This condition has emerged as a subject of significant clinical interest. Given that the intestinal lymphatic system plays an important role in the body’s fluid homeostasis, adaptive immunity, nutrient and drug absorption, intestinal transport, and systemic metabolism, its dysfunction may have wider implications. Although primary IL is rare, with varied clinical features, complications, treatment response, and outcomes, secondary IL is more common than previously believed. The definitive diagnosis of IL requires endoscopic demonstration of whitish villi (which frequently resemble snowflakes) and histological confirmation of dilated lacteals in the small intestinal mucosa. Treatment of IL is challenging and involves dietary modifications, managing underlying medical conditions, and using medications such as sirolimus and octreotide. Recognizing its prevalence and diverse etiology is crucial for targeted management of this challenging medical condition. This article provides a comprehensive exploration of the clinical implications associated with IL. In addition, it offers valuable insights into critical knowledge gaps in the existing diagnostic and management landscape.

Keywords: Intestinal lymphatics; Lymphangiectasia; Waldman's disease; Lacteals; Lymph

Core Tip: Exploring the intricate relationship between the intestinal lymphatic system and clinical outcomes, particularly in the context of intestinal lymphangiectasia, reveals crucial knowledge gaps that require attention. There is a broader implication of intestinal lymphatic dysfunction than is typically talked about. This article delves into the existing voids in our understanding, emphasizing the significance of future research to refine diagnostic approaches, establishing treatment guidelines, and uncovering novel therapeutic interventions for individuals affected by this complex condition. Bridging these knowledge gaps is essential to advancing patient care and improving outcomes in the realm of intestinal lymphatic disorders.