Kim Na Rae, Lee Suk-Koo, Suh Yeon-Lim
Department of Pathology, Gil Medical Center, Gachon University of Medicine and Science, Incheon 405-760, Korea.
J Pediatr Surg. 2009 Oct;44(10):e13-7. doi: 10.1016/j.jpedsurg.2009.06.034.
Primary intestinal lymphangiectasia is a rare cause of protein-losing enteropathy and usually presents with intermittent diarrhea or malnutrition. Diagnosis depends largely on its pathologic condition demonstrating greatly dilated lymphatics mainly in the lamina propria of the mucosa. We report a case of primary intestinal lymphangiectasia, of the diffuse type, presenting with abdominal pain and voluminous diarrhea in a previously healthy 8-year-old boy. He had periumbilical pain for 3 months before presentation. He was managed by segmental bowel resections and end-to-end anastomoses. The histopathologic condition of the resected small intestine showed lymphatic dilation limited mainly to the subserosa and mesentery but was not prominent in the mucosa. Abdominal pain and diarrhea subsided postoperatively. The present case is the fourth report describing a response to operative resection.
原发性肠淋巴管扩张症是蛋白丢失性肠病的罕见病因，通常表现为间歇性腹泻或营养不良。诊断主要依赖于其病理状况，即主要在黏膜固有层显示淋巴管显著扩张。我们报告一例弥漫型原发性肠淋巴管扩张症，发生在一名既往健康的8岁男孩身上，表现为腹痛和大量腹泻。他在就诊前脐周疼痛3个月。通过节段性肠切除和端端吻合术进行治疗。切除的小肠组织病理学状况显示淋巴管扩张主要局限于浆膜下和肠系膜，但在黏膜中不明显。术后腹痛和腹泻症状缓解。本病例是描述手术切除反应的第四例报告。
