Lymphangiomatosis of the ileum with perforation: A case report and review of the literature

Abstract

Lymphangiomatosis is a benign proliferation of lymph vessels. Lymphatic diseases can vary from small lymphangioma to generalized lymphangiomatosis, which is a rare condition and can have several clinical manifestations. The gastrointestinal tract may be affected, but the incidence in the intestinal wall is very low. We propose in our study a case of ileal lymphangiomatosis presenting with perforation, in which the diagnosis was made after the pathological analysis of the resected intestinal tract. Although rare and not described in the literature, intestinal lymphangiomatosis could manifest itself with acute abdomen and could be a surgical urgency. This disease should be considered when intestinal perforation is observed.

Keywords: Acute abdomen; Bowel perforation; Bowel resection; Lymphangiomatosis; Small intestine.

Fig. 1

Microscopic findings. (A) Hematoxylin and eosin staining of numerous dilated lymphatic vessels (4× magnification). (B) Immunohistochemical D2-40 expression (brown color) in dilated lymphatic vessels of the submucosa (10× magnification). (C) Subserous dilated lymphatic vessels with discontinuity of the muscular layer and serositis (H&E, 10× magnification). (For interpretation of the references to color in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

(A) D2-40 immunostaining shows positive reactivity for lining endothelial cells of lymphatic spaces in the muscular layer (10× magnification). (B) Numerous submucosal dilated lymphatic vessels (H&E, 10× magnification). (C) Lymphatic vessels that interrupt muscular layer (D2-40 stain, 10× magnification).