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Fibrotic entrapment of the small bowel in congenital intestinal lymphangiectasia

C Rust1, E Pratschke, W Hartl

  • 1Department of Medicine II, Klinikum Grosshadern, University of Munich, Germany.

The American Journal of Gastroenterology
|October 15, 1998
PubMed

Insights

Congenital intestinal lymphangiectasia, a rare protein-losing enteropathy, can cause severe abdominal pain due to fibrotic entrapment. Surgical intervention successfully treated this rare complication in a long-standing case.

Area of Science:

  • Gastroenterology
  • Rare Diseases
  • Surgical Pathology

Background:

  • Congenital intestinal lymphangiectasia is a rare protein-losing enteropathy presenting with diarrhea and peripheral edema.
  • This condition can lead to significant morbidity if not properly managed.

Observation:

  • A 37-year-old woman with a long history of congenital intestinal lymphangiectasia experienced severe abdominal pain.
  • Diagnostic workup, including endoscopy, histology, and radiography, confirmed intestinal lymphangiectasia.
  • Laparotomy revealed extensive fibrotic entrapment of the small bowel, causing partial mechanical obstruction.

Findings:

  • The patient's condition was attributed to fibrotic entrapment of the small bowel, a previously unreported complication of congenital intestinal lymphangiectasia.
  • Surgical decortication was performed to relieve the mechanical obstruction.

Implications:

  • This case highlights fibrotic entrapment as a potential complication of long-standing congenital intestinal lymphangiectasia.
  • Surgical management, specifically decortication, can be effective in treating this rare presentation.
  • Further research into the pathogenesis and management of fibrotic complications in intestinal lymphangiectasia is warranted.

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