Value of CT lymphangiography combined with direct lymphangiography in diagnosing primary intestinal lymphangiectasia

Objective: To investigate the clinical value of CT lymphangiography (CTL) combined with direct lymphangiography (DLG) in primary intestinal lymphangiectasia (PIL). Methods: Sixteen patients diagnosed as PIL with intestinal enteroscopy were recruited in this retrospective study. All the patients were performed DLG and CTL one week before exploratory laparotomy. Subjective assessment in DLG included weak lymphatic fluid drainage, lymphangiectasia, lymphatic reflux, fistula and thoracic outlet reflux or obstruction. While for CTL combined with DLG, the intestinal and extra-intestinal lesions were evaluated, including lymph node, edema, lymphangiectasia and abnormal distribution, fistula, and lymphangiomatosis. All the diagnosis was compared with intestinal endoscopy results. Results: For DLG, 16 weak lymphatic fluid drainages, 9 lymphangiectasia, 1 fistula with abdomen and 14 thoracic outlets weak lymphatic fluid drainage or obstruction were found. For DLG combined with CTL, 16 intestinal lumens dilatation and 14 circumferential intestinal thickening were found in intestinal lesions. While for extra-intestinal lesions, the imaging features included edematous findings (12 in mesentery, 7 ascites only, 2 hydrothorax and ascites, and 3 pericardial, thoracic and abdominal effusions), abdominal lymph nodes (6 cases), lymphangiectasia and abnormal distributions (14 cases), fistulas (lymph-intestinal luminal fistula in 4 cases, and lymph-abdominal fistula in 3 cases), lymphangiomatosis (3 cases), and thoracic duct outlet dysfunction and reflux (14 cases). The number of cases diagnosed as intestinal lymphangiectasia, intestinal luminal lymph exudation and lymph fistula were 16, 10 and 6 with intestinal endoscopy, while the number were 11, 0, and 4 with CTL combination with DLG. Conclusion: Combination of CTL with DLG is valuable in the diagnosis of PIL. (authors)