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Primary intestinal lymphangiectasia in a 23-month- old girl

Lava Mohammad1, Razan Omran1, Alexandr Ibrahim2

  • 1Department of Pediatrics, Tishreen University Hospital, Lattakia, Syria.

Oxford Medical Case Reports
|July 24, 2019
PubMed

Insights

Primary intestinal lymphangiectasia (PIL), a rare protein-losing condition, involves lymphatic dilation in the GI tract. This case report details a toddler

Area of Science:

  • Gastroenterology
  • Pediatric Medicine
  • Rare Diseases

Background:

  • Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy.
  • It is characterized by lymphatic dilation within the gastrointestinal tract, leading to protein and lymph fluid loss.
  • PIL is typically diagnosed in children under three years old, with adult-onset being uncommon.

Observation:

  • A 23-month-old female presented with peripheral edema and diarrhea.
  • Diagnostic evaluation included upper gastrointestinal endoscopy and histological analysis.
  • The patient exhibited symptoms consistent with protein-losing enteropathy.

Findings:

  • Histological examination confirmed primary intestinal lymphangiectasia.
  • The patient experienced hypoproteinemia, edema, and immunologic anomalies due to lymph fluid loss.
  • The diagnosis was established through endoscopic and pathological findings.

Implications:

  • Early diagnosis and management of PIL are crucial for preventing complications.
  • Dietary modifications, including medium-chain triglycerides and high protein intake, are effective treatments.
  • This case highlights the importance of considering PIL in young children presenting with edema and diarrhea.

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