Lymphangiectasia: Dilated Lymphatic Vessels – Etiology, Presentation, Management

Lymphangiectasia = pathological dilation of lymphatic vessels. Can be primary (congenital) or secondary.

Etiology

• Primary: Lymphatic malformation (e.g., intestinal lymphangiectasia, congenital lymphedema).
• Secondary: Obstruction (surgery, radiation, filariasis, tumor), inflammation (infection, IBD), elevated central venous pressure (CHF, constrictive pericarditis), trauma.

Clinical Features

• Swelling (lymphedema) – dependent, pitting → non-pitting.
• Chylous effusion (ascites, pleural, pericardial) if thoracic duct involved.
• Protein-losing enteropathy (intestinal) → hypoalbuminemia, edema, lymphopenia.
• Skin changes: thickening, hyperkeratosis, papillomatosis, lymphorrhea, ulceration.

Diagnosis

• Clinical + imaging (lymphoscintigraphy, MRI lymphangiography, ICG fluorescence).
• Endoscopy + biopsy (intestinal).
• Lymph fluid analysis (triglycerides, lymphocytes).

Treatment

• Compression, elevation, skin care, CDT (complete decongestive therapy).
• Low-fat/MCT diet (intestinal).
• Sirolimus (lymphatic anomalies).
• Surgical: lymphaticovenular anastomosis, debulking.
• Treat underlying cause (infection, tumor, CHF).