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[Congenital intestinal lymphangiectasia]
Dusan D j Popović1, Milan Spuran, Tamara Alempijević
1Klinicki centar Srbije, Sluzba za patohistologiju, Beograd, Srbija. dr.popovic@sezampro.rs
Vojnosanitetski Pregled
|April 30, 2011
Summary
Congenital intestinal lymphangiectasia causes protein losing enteropathy with symptoms like diarrhea and swelling. Early diagnosis and treatment, including diet and medication, are crucial for managing this rare condition.
Area of Science:
- Gastroenterology
- Pediatric Gastroenterology
- Vascular Medicine
Background:
- Congenital intestinal lymphangiectasia is a rare condition characterized by protein-losing enteropathy.
- It involves tortuous, dilated lymphatic vessels in the intestinal wall and mesentery.
- Clinical signs include malabsorption, diarrhea, steatorrhea, edema, and effusions.
Observation:
- A 19-year-old male presented with diarrhea, abdominal swelling, fatigue, growth impairment, ascites, and lymphedema.
- Laboratory findings revealed iron deficiency anemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection.
- Endoscopic evaluations showed dilated lymphatic vessels in the small intestine.
Findings:
- Diagnosis was confirmed via intestinal biopsy.
- The patient received a high-protein diet with medium-chain fatty acids, somatotropin, and supportive care.
- Congenital intestinal lymphangiectasia is typically diagnosed in childhood.
Implications:
- Early recognition and adequate treatment are vital to prevent complications associated with congenital intestinal lymphangiectasia.
- Management strategies include dietary modifications and pharmacotherapy.
- This case highlights the importance of considering this rare diagnosis in adolescents presenting with relevant symptoms.
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