Intestinal Lymphangioma With Extensive Secondary Lymphangiectasia Causing Life-Threatening Hemorrhage: First Case...

Intestinal lymphangiectasia is a rare disorder characterized by dilated lymphatic vessels in the gastrointestinal tract, typically presenting with protein-losing enteropathy. Adult-onset cases with severe gastrointestinal bleeding are exceptionally rare.

A 40-year-old male presented with severe recurrent gastrointestinal bleeding and iron-deficiency anemia. Double-balloon enteroscopy revealed diffuse jejunal infiltration with white villi throughout the jejunum, consistent with lymphatic dilation. CT imaging showed mesenteric lymphadenopathy. Technetium-99m-labeled red blood cell scintigraphy demonstrated active bleeding within the superior mesenteric artery distribution. Histopathological examination confirmed intestinal lymphangioma with extensive secondary lymphangiectasia. Despite conservative management, refractory bleeding requiring over 20 units of packed red blood cells necessitated small bowel transplantation with subtotal colectomy. Pathological examination of the resected specimen revealed extensive lymphangiectasia affecting the small intestine, colon, appendix, and gallbladder. Notably, all mesenteric lymph nodes showed only reactive hyperplasia, excluding a lymphoproliferative disorder. The patient developed postoperative complications, including extensive venous thrombosis, empyema, and multiorgan failure, succumbing on postoperative Day 20.

This represents the first reported case of intestinal lymphangioma with extensive secondary lymphangiectasia requiring small bowel transplantation. The atypical presentation without classic protein-losing enteropathy features, multiorgan gastrointestinal involvement, and fatal outcome highlight the variable clinical spectrum and potential severity of this rare condition.