Authors
Baburaj P1, Anand R2, Harikrishnan BL3
1Professor and Unit Chief, Department of Medicine, Jubilee Mission Medical College and Research Institiute, Thrissur
2Senior Resident, Department of Medicine, Jubilee Medical College and Research Institute, Thrissur
3Assistant Professor, Department of Medicine, Jubilee Medical College and Research Institute, Thrissur
Abstract
Introduction: Primary intestinal lymphangiectasia (PIL) is a rare disorder causing protein and lymphocyte loss into the GI tract, leading to hypoproteinemia, edema, and lymphopenia. Its diagnosis can be challenging.
Case Presentation: A 20-year-old male presented with progressive bilateral lower limb edema, facial puffiness, ascites, bilateral pleural effusion, and intermittent loose stools over 11 months.
Investigations: Baseline labs showed severe hypoproteinemia and hypocalcemia. Ascitic fluid was milky with high SAAG and elevated triglycerides. Upper GI endoscopy revealed whitish powdering precipitates in D2 and D3 villi. Histopathology of duodenal and terminal ileum biopsies confirmed mild lymphangiectasia. Lymphoscintigraphy suggested lymphatic leak. Secondary causes were ruled out.
Management & Outcome: Diagnosed with primary intestinal lymphangiectasia, he was prescribed a high protein, low-fat diet with medium-chain fatty acids. Abdominal distension decreased by discharge.
Conclusion: This case highlights the presentation and diagnostic pathway of primary intestinal lymphangiectasia, emphasizing the role of endoscopy and biopsy for confirmation.