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Primary intestinal lymphangiectasia

N Suresh1, R Ganesh, Janani Sankar

  • 1Departments of Pediatrics and Gastroenterology, Kanchi Kamakoti CHILDS Trust Hospital, Nungambakkam, Chennai, Tamil Nadu, India. drsuresh30@rediffmail.com

Indian Pediatrics
|November 6, 2009
PubMed

Insights

Primary intestinal lymphangiectasia (PIL) is a rare condition affecting intestinal lymphatics. This study reports on four children in India diagnosed with PIL, highlighting key clinical and diagnostic features.

Area of Science:

  • Gastroenterology
  • Pediatric Medicine
  • Rare Diseases

Background:

  • Primary intestinal lymphangiectasia (PIL) is a rare congenital disorder characterized by dilated intestinal lymphatics.
  • It leads to protein-losing enteropathy, hypoproteinemia, and edema.
  • Diagnosis often requires invasive procedures like endoscopy and biopsy.

Observation:

  • This case series describes four children from Chennai, India, with symptoms suggestive of PIL.
  • Patients presented with anasarca (generalized edema), recurrent diarrhea, and severe hypoproteinemia.
  • Clinical presentation was consistent with protein-losing enteropathy.

Findings:

  • Endoscopic examination revealed characteristic findings of intestinal lymphangiectasia.
  • Histopathological analysis of intestinal biopsies confirmed the diagnosis of PIL.
  • The findings underscore the importance of considering PIL in children with unexplained edema and hypoalbuminemia.

Implications:

  • Early diagnosis and management of PIL are crucial to prevent complications like malnutrition and growth failure.
  • This report expands the understanding of PIL presentation in a South Asian pediatric population.
  • Further research into the genetic and environmental factors of PIL in diverse populations is warranted.

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