Endoscopic and histopathological study on primary and secondary intestinal lymphangiectasia

Abstract

Jejunal endoscopy and histopathological study of biopsied specimens were performed to clarify states of jejunal mucosa and the mechanism of enteric protein loss in six patients with protein-losing enteropathy, including four patients with intestinal lymphangiectasia, one patient with constrictive pericarditis associated with dilated lymphatics of the intestine, and one patient with Budd-Chiari syndrome. Three cardinal endoscopic findings, scattered white spots, white villi, and chyle-like substances covering the mucosa, were demonstrated in protein-losing enteropathy. Scattered white spots indicated markedly dilated lymphatics in the stroma of the villi. White villi seemed to be due to fats including chylomicrons or fat droplets in the absorptive cells, interepithelial spaces, and/or stroma, even though the biopsies were obtained in the fasting state. Therefore, white villi suggest impaired transport of fats from intestinal epithelial cells to intestinal lymphatics. These three cardinal findings are thought to be characteristic for protein-losing enteropathy secondary to lymphatic disorders.

Immune-mediated Protein-losing Enteropathy with Down Syndrome

“…1A, B). However, endoscopy did not reveal scattered white spots, white villi, or chyle-like substances covering the mucosa, which are typical findings of PLE due to lymphangiectasia (7). The mucosal biopsy specimens of the duodenum and jejunum revealed villous atrophy, moderate inflammatory cell infiltration (T lymphocytes and plasma cells), and intraepithelial lymphocytes without lymphangiectasia or vasculitis ( Fig.…”

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

“…One type had a diameter of less than 1 mm, while the other exceeded 3 mm in diameter. Asakura et al [21] reported three cardinal endoscopic findings in protein-losing enteropathy: scattered white spots, white villi and chyle-like substances covering the mucosa in the jejunum. In cases of sustained protein-losing enteropathy, capsule endoscopy may assist diagnosis.…”

Late-Onset Primary Intestinal Lymphangiectasia Successfully Managed with Octreotide: A Case Report

“…2). Biopsies from the duodenal wall did not show a typical picture of dilated lymphatics in the lamina propria, 3) but instead showed mildly dilated lymphatics in the deeper layer. Since no other underlying diseases, including inflammatory bowel disease, infection, celiac disease, etc., were found in the gastrointestinal system of the patient, his condition was diagnosed as PLE due to PIL associated with primary lymphedema in the extremities.…”