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Intestinal Lymphangiectasia

Hauser, Bruno*; Moreels, Tom; Urbain, Daniel; Van Marck, Veerle§; Pletincx, Michel||; Devreker, Thierry*; Vandenplas, Yvan*

Journal of Pediatric Gastroenterology & Nutrition: February 2009 - Volume 48 - Issue 2 - p 125
doi: 10.1097/MPG.0b013e318192418e
Image of the Month

*Unit of Paediatric Gastroenterology, Universitair Ziekenhuis Brussel Kinderen, Brussels, Belgium

Department of Gastroenterology and Hepatology, Universitair Ziekenhuis Antwerpen, Antwerp, Belgium

Department of Gastroenterology, Universitair Ziekenhuis Brussel, Brussels, Belgium

§Department of Pathologic Anatomy, Universitair Ziekenhuis Antwerpen, Antwerp, Belgium

||Department of Pediatrics, Clinique Saint Anne–Saint Rémi, Brussels, Belgium

A boy presented at the age of 6 weeks with edema, hypoproteinemia, and increased fecal α1-antitrypsin, suggestive of protein-losing enteropathy due to intestinal lymphangiectasia (IL). A treatment was started with a diet high in medium-chain triglycerides and proteins, and low in long-chain triglycerides, resulting in disappearance of the edema and improvement of the laboratory findings. An esophagogastroduodenoscopy performed at the age of 4 years could not confirm the diagnosis of IL. He was referred at the age of 7 years to undergo a video capsule endoscopy (VCE) to confirm the still suspected diagnosis of IL. The VCE (Given Imaging, Yoqneam, Israel) showed ileal mucosa with a whitish aspect suggestive of IL and erosive lesions, which seemed uncharacteristic for IL (Fig. 1). For this reason, a double-balloon enteroscopy was also performed using the Fujinon EN-450 P5/20 pediatric double-balloon enteroscope (Fujinon Corporation, Saitama, Japan) under general anesthesia and fluoroscopic guidance, allowing complete visualization of the small bowel by a combined oral and anal approach. Both in the proximal and distal small bowel, mucosal villi tips were broadened and whitish, characteristic for diffuse IL. The lesions were more pronounced in the distal small bowel with elevated hyperemic lesions and spontaneous leakage of chylous liquid (Fig. 2). These more pronounced lesions most probably corresponded to the erosive lesions observed with the VCE. Histologic analysis of the small bowel biopsies showed dilated lymphatic vessels in the mucosa confirming the diagnosis of IL (Fig. 3).

The authors report no conflicts of interest.

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