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[Primary intestinal lymphangiectasia: first case report in Peru]
Katherine M Usnayo Usnayo1, Alejandro Piscoya2, Raffo Escalante Kanashiro3
1Escuela de Medicina, Universidad Peruana de Ciencias Aplicadas. Lima, Perú.
Summary
Primary intestinal lymphangiectasia, a rare condition causing edema and diarrhea, was diagnosed in a 1-year-old boy. Prompt treatment with a specialized diet led to his recovery.
Area of Science:
- Gastroenterology
- Pediatrics
- Rare Diseases
Background:
- Primary intestinal lymphangiectasia (PIL) is a rare congenital disorder.
- It involves the abnormal dilation of intestinal lymphatic vessels, leading to protein and lymph loss.
- Unknown etiology contributes to diagnostic and therapeutic challenges.
Observation:
- A 1-year-old male presented with generalized edema and diarrhea.
- Laboratory tests revealed significant hypoproteinemia.
- Endoscopic examination and duodenal biopsy were performed for diagnosis.
Findings:
- Histopathological examination of the duodenal biopsy confirmed primary intestinal lymphangiectasia.
- The patient exhibited classic symptoms of protein-losing enteropathy.
- Clinical presentation included edema and diarrhea, consistent with lymphatic malabsorption.
Implications:
- Early diagnosis and appropriate management are crucial for improving outcomes in pediatric PIL.
- Dietary modification, specifically a low-fat, medium-chain triglyceride (MCT) diet, is the cornerstone of treatment.
- This case highlights the importance of considering PIL in children with unexplained edema and hypoalbuminemia.
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