Intestinal Lymphangiectasia in Children: A Study of Upper Gastrointestinal Endoscopic Biopsies

Abstract

From 1980-1986 intestinal mucosal lymphangiectasia was diagnosed histologically in eight patients (6 weeks to 16 years; four males/four females; seven white). The presenting features were diarrhea (six/eight), vomiting (four/eight), and growth deficit (seven/eight). Additional conditions in these patients included asthma, urinary tract infection, esophageal atresia, hydrops fetalis, inflammatory bowel disease, malabsorption syndrome, and thymic hypoplasia. Hypoalbuminemia and edema (four/eight) were more prominent in those patients under 5 years of age. Two had systemic lymphangiectasia and lymphopenia. The patients responded variably to hyperalimentation and dietary supplements, depending on the extent of their lymphangiectasia and the age at onset of symptoms. Dilated lymphatics were seen in the small intestinal mucosa under the surface epithelium. Lesions were often focal, requiring several biopsies or serial sections for detection. Other common findings were mild to moderate lymphoplasmacytic inflammation and mild to moderate villous injury with blunting and edema. Mild inflammation without lymphangiectasia was also present in esophageal, gastric, or colonic biopsies. Diagnosis should be made on the basis of endoscopic findings or in small-intestinal inflammatory conditions even in the absence of a classic clinical picture. Histologic confirmation may require more than one serially sectioned biopsy. This study confirms the diversity of disorders that may be associated with intestinal lymphangiectasia and shows that the disease in infants is more severe and generalized.

The pathology of malabsorption: current concepts

“…Abnormal dilation of the intestinal lymphatics/lacteals is associated with malabsorption, resulting in protein‐losing enteropathy, steatorrhoea, peripheral oedema and lymphyocytopenia 86,100–102 . The condition is commonly seen secondarily in a variety of disorders, including carcinoma, lymphoma, IBD and others 103,104 .…”

“…92 Intestinal lymphangiectasia Abnormal dilation of the intestinal lymphatics ⁄ lacteals is associated with malabsorption, resulting in proteinlosing enteropathy, steatorrhoea, peripheral oedema and lymphyocytopenia. 86,[100][101][102] The condition is commonly seen secondarily in a variety of disorders, including carcinoma, lymphoma, IBD and others. 103,104 However, a rare primary form of the disorder occurs also, known as primary intestinal lymphangiectasia (PIL).…”

Primary intestinal lymphangiectasia with lymphedema of lower extremities

“…Serous effusions in more severe cases are also common -pleural effusion, chylous ascites, and pericarditis may be present in patients and may be life-threatening. Other concomitant symptoms are abdominal pain, nausea, vomiting, moderate diarrhea, fat-soluble vitamin deficiencies, and weight loss [2][3][4][5]. In our patient, there were no digestive symptoms all the time.…”

Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

“…Small bowel biopsies are required to confirm the diagnosis. 15 Biopsies show prominent and dilated lymphatics in the lamina propria region, often with extension into submucosa. 15 All 4 patients in our series showed characteristic histology (Figure 2).…”