Protein loss in the small intestine detected by capsule endoscopy

Abstract

A 32-year-old woman presented with leg edema and low serum albumin. Although she had experienced intermittent episodes of leg edema since childhood, she had not been examined for this condition. Laboratory data showed hypoproteinemia (3.8 g/dL) and hypoalbuminemia (1.8 g/dL). The result of serology for infectious enteropathy was negative. Gastroduodenoscopy and total colonoscopy showed no abnormal findings. The result of Sudan III staining of the stool was positive. A fecal α-1-antitrypsin clearance test showed abnormal leakage (128 mL/day), and double-balloon enteroscopy showed white villi, white nodules (A), and chylorrhea in the jejunum. Capsule endoscopy showed apparent leakage of white lymphatic fluid from the white villi, where the internal pressure must have been physiologic. It showed leakage of protein from the villi to the intestinal lumen (B). It may be difficult to see a spontaneous leak with double-balloon enteroscopy when the intraluminal pressure is high. Biopsy specimens from the jejunal mucosa demonstrated characteristic lymphangiectasia (C, H&E and D2-40, orig. mag. ×40; D, H&E and D2-40, orig. mag. ×40 D2-40) of the lamina propria. Albumin scintigraphy showed hot spots in the small intestine. These data confirmed a diagnosis of primary intestinal lymphangiectasia and showed that the hypoalbuminemia was secondary to protein-losing enteropathy. The patient was given a low-fat diet with medium-chain triglyceride supplementation, and her clinical condition improved. All authors disclosed no financial relationships. Commentary Primary intestinal lymphangiectasia with protein-losing enteropathy is a disorder wherein ectasia of the enteric lymphatics develops. This can occur at almost any layer of the GI lumen wall. This disorder is usually seen in young patients but can be seen in adults as well (as was the case with this patient). Intestinal lymphangiectasia can be seen in the context of other conditions, including Noonan syndrome, von Recklinghausen syndrome, and Turner syndrome, among others. This patient also had symptoms that suggested this condition had been present for many years. This case illustrates the value of a well-timed capsule endoscopy study, whereby a key finding was detected that allowed elucidation and/or confirmation of a diagnosis for the patient. The findings seen in this study could be intermittent or ongoing, so the capsule findings are both as striking as they are rare. Douglas G. Adler, MD, FASGE, GIE Senior Associate Editor, University of Utah School of Medicine, Salt Lake City, Utah Mohamed O. Othman, MD, Associate Editor for Focal Points