A Case of Intestinal Lymphangiectasia

장림프관 확장증 1례

Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as diffuse nodular thickening of the small bowel and as linear hypodense streaking densities in the small bowel caused by dilated lymphatic channels. Demonstration of increased enteric protein loss using $^{51}Cr-$, $^{131}I-$ or $^{99m}Tc-labeled$ albumin, timed measurement of fecal excretion of radioactivity or by measuring fecal clearance of alpha 1-antitrypsin can also help the diagnosis. We experienced a rare case of intestinal lymphangiectasia in an eight year old boy who presented with facial edema, abdominal distension and intermittent diarrhea. We report a patient with intestinal lymphangiectasia, in whom abdominal CT, $^{99m}Tc-labeled$ albumin scintitigraphy, and stool alpha 1-antitrypsin measurement played key roles in determining the diagnosis. A brief review of literature was made.

저자들은 얼굴 부종, 복부 팽만 및 저알부민혈증을 주소로 내원한 8세 환아에서 원발성 장림프관 확장증으로 진단된 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.