Related Experiment Videos
Acute jejunal ileus in intestinal lymphangiectasia
R Lenzhofer1, M Lindner, A Moser
1Interne Abteilung, Kardinal Schwarzenberg'sches Krankenhaus Schwarzach.
The Clinical Investigator
|July 1, 1993
Summary
A young patient with severe swelling and abdominal pain was diagnosed with primary intestinal lymphangiectasia. Treatment with a low-fat diet and medium-chain triglycerides led to symptom resolution and recovery.
Area of Science:
- Gastroenterology
- Vascular Medicine
Background:
- Primary intestinal lymphangiectasia is a rare disorder characterized by dilation of intestinal lymphatics.
- It often leads to protein-losing enteropathy, causing hypoproteinemia, edema, and lymphopenia.
Observation:
- A 26-year-old patient presented with acute epigastric pain, severe limb edema, hypoproteinemia, low IgG, and lymphopenia.
- Initial management with a low-fat diet and medium-chain triglycerides showed partial improvement.
- The patient later developed obstructive ileus, necessitating surgical intervention.
Findings:
- Laparotomy revealed yellowish-white deposits in the small intestine, with a resected mass found 100 cm distal to the duodenojejunal flexure.
- Histological examination confirmed dilated submucosal lymphatics, pseudocystic lymph edema, secondary bleeding, and severe stenosis of the jejunal lumen.
Implications:
- This case highlights the complex presentation and management challenges of primary intestinal lymphangiectasia.
- Surgical resection of affected segments may be necessary for obstructive complications.
- Long-term management with dietary modifications can lead to sustained remission and improved quality of life.