Primary intestinal lymphangiectasia presenting with intussusception in a child

Area of Science:

• Gastroenterology
• Pediatric Surgery
• Vascular Malformations

Background:

• Intestinal lymphangiectasia is a protein-losing enteropathy stemming from lymphatic abnormalities.
• It is categorized as primary (congenital) or secondary (acquired).
• Primary intestinal lymphangiectasia (PIL) is a rare condition with diverse presentations, infrequently involving intussusception in pediatric cases.

Purpose of the Study:

• To report a rare case of intussusception caused by primary intestinal lymphangiectasia in a child.
• To emphasize PIL as a differential diagnosis for intestinal obstruction in pediatric patients.

Main Methods:

• Case report of a 12-year-old male presenting with abdominal pain and a palpable mass.
• Diagnostic workup included emergency laparotomy revealing ileal intussusception.
• Histopathological examination of the resected segment confirmed dilated lymphatic channels consistent with PIL.

Main Results:

• The patient presented with symptoms of acute intestinal obstruction.
• Laparotomy identified intussusception of the distal ileum.
• Histopathology confirmed primary intestinal lymphangiectasia as the underlying cause.

Conclusions:

• Primary intestinal lymphangiectasia is an infrequent cause of intussusception in children.
• This condition should be considered in the differential diagnosis of pediatric intestinal obstruction.
• This is the second reported case in a child from the Indian subcontinent, underscoring its rarity and geographic association.