From the Guidelines
Steatorrhea in lymphangiectasia is primarily caused by the malabsorption of dietary fats due to the obstruction and dilation of intestinal lymphatic vessels. This condition is characterized by the disruption of normal fat absorption, leading to the excretion of undigested fat in stool. The pathophysiology of lymphangiectasia involves the blockage or malformation of lymphatic vessels that transport chylomicrons from the intestinal cells to the bloodstream, resulting in the inability to properly absorb long-chain fatty acids and fat-soluble vitamins 1.
Key Factors Contributing to Steatorrhea in Lymphangiectasia
- Obstruction and dilation of intestinal lymphatic vessels
- Disruption of normal fat absorption
- Malabsorption of long-chain fatty acids and fat-soluble vitamins
- Protein loss into the intestinal lumen, contributing to hypoproteinemia and sometimes lymphopenia
As noted in the study published in Gut in 2012 1, intestinal lymphangiectasia is a common physical cause of steatorrhea, highlighting the importance of considering this condition in patients presenting with greasy, foul-smelling stools. The condition may be primary (congenital) or secondary to conditions like constrictive pericarditis, lymphoma, or tuberculosis that obstruct lymphatic flow. Management of steatorrhea in lymphangiectasia often involves a low-fat diet supplemented with medium-chain triglycerides, which can be absorbed directly into the portal circulation, bypassing the lymphatic system 1.
From the Research
Reasons for Steatorrhea in Lymphangiectasia
Steatorrhea, or the presence of excess fat in the stool, is a common symptom of lymphangiectasia. The reasons for steatorrhea in lymphangiectasia include:
- Malabsorption of dietary fats due to abnormal lymphatic drainage in the small intestine 2, 3
- Impaired digestion and absorption of fats due to pancreatic insufficiency 2
- Bacterial overgrowth in the small intestine, which can contribute to malabsorption of fats 2
Treatment Options for Lymphangiectasia
Treatment options for lymphangiectasia include:
- Dietary modifications, such as a low-fat diet supplemented with medium-chain triglycerides (MCTs) 2, 3, 4
- Pancreatic enzyme supplements to improve digestion and absorption of fats 2
- Octreotide therapy, although its effectiveness is unclear and may not alter lymphatic function 5, 6
- Surgery, such as pericardial window and lymphaticovenous anastomoses, in some cases 5
Diagnosis of Lymphangiectasia
Diagnosis of lymphangiectasia can be made using: