Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study

Area of Science:

• Gastroenterology
• Pediatrics
• Vascular Biology

Background:

• Primary intestinal lymphangiectasia is a rare condition characterized by lymphatic system abnormalities in the gut.
• It often presents with protein-losing enteropathy, leading to hypoalbuminemia and edema.

Observation:

• A 16-year-old male presented with a 6-week history of peripheral edema.
• Clinical findings included lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin, and imaging showing dilated intestinal lymphatics and lymph node defects.

Findings:

• Small intestinal biopsies revealed dilated lacteals and enterocyte abnormalities.
• Electron microscopy confirmed enlarged lacteals and intercellular vacuoles.
• Exploratory laparotomy identified dilated serosal lymphatic vessels but no masses.

Implications:

• Dietary management, specifically restricting long-chain triglycerides, effectively treated the patient's edema.
• This case highlights the importance of nutritional intervention in managing intestinal lymphangiectasia.
• Early diagnosis and appropriate treatment can lead to significant clinical improvement and normalization of activity.