CT Lymphangiography (CTL) in Primary Intestinal Lymphangiectasia (PIL): A Comparative Study with Intraoperative Enteroscopy (IOE).

RATIONALE AND OBJECTIVES: To investigate the clinical feasibility of CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL) by comparison with intraoperative enteroscopy (IOE) during exploratory laparotomy. MATERIALS AND METHODS: Eleven PIL patients (F/M, two/nine, age range 10-37 years) were recruited in this study, and they were performed IOE during exploratory laparotomy for suspected serious lymphatic-intestinal leakages. All the patients were performed CTL before surgery, and the imaging data were reviewed by two radiologists separately. CTL assessments included intestinal lesions, edematous lesions, intestinal and mesenteric lymphangiectasia, lymphaticabdominal leakages, lymph fluid reflux, lymphangioma and abnormal lymphatics in other area. The intestinal lymphangiectasia and lymphaticintestinal leakages were confirmed by histology and IOE. RESULTS: For CTL, (1) nine intestinal wall thickening; (2) eight ascites, complicated with four pleural effusions, (3) eight intestinal and mesenteric lymphangiectasia, (4) six lymph fluid reflux (5) one lymphatic-abdominal leakage, (6) two lymphangioma. While for IOE, intestinal lymphangiectasia has been confirmed in all patients, including five segemental and six diffusive lesions in intestinal mucosa. Besides, one lymphatic-intestinal fistula, one lymphatic-abdominal leakage was confirmed. Compared to IOE and histology, the accuracy of CTL was 72.7% in detecting intestinal lymphangiectasia. CONCLUSION: Compared to IOE, CTL demonstrates feasibility in detection of intestinal lymphangiectasia and other abnormalities in whole lymphatic circulation for PIL. Combination of CTL with IOE accommodates guidance for preoperative evaluation and therapeutic management for PIL.

Unipedal Diagnostic Lymphangiography Followed by Sequential CT Examinations in Patients With Idiopathic Chyluria: A Retrospective Study.

OBJECTIVE: The objective of our study was to investigate the clinical value of diagnostic lymphangiography followed by sequential CT examinations in patients with idiopathic chyluria. MATERIALS AND METHODS: Thirty-six patients with idiopathic chyluria underwent unipedal diagnostic lymphangiography and then underwent sequential CT examinations. The examinations were reviewed separately by two radiologists. Abnormal distribution of contrast medium, lymphourinary leakages, and retrograde flow were noted, and the range and distribution of lymphatic vessel lesions were recorded. The stage of idiopathic chyluria based on CT findings and the stage based on clinical findings were compared. Therapeutic management and follow-up were recorded. Statistical analyses were performed. RESULTS: Compared with CT studies performed after lymphangiography, diagnostic lymphangiography showed a unique capability to depict lymphourinary leakages in three patients. Lymphourinary fistulas and abnormal dilated lymphatic vessels were found in and around kidney in all patients. CT depicted retrograde flow of lymph fluid in 47.2% of patients. The consistency in staging chyluria based on CT findings and clinical findings was fair (κ = 0.455). Twenty-nine patients underwent conservative therapy, and seven underwent surgery. Surgical therapy was superior to conservative management (no recurrence, 85.7% of patients who underwent surgery vs 62.1% of patients who underwent conservative therapy; p = 0.025). CONCLUSION: From assessing the drainage of contrast medium on unipedal diagnostic lymphangiography and the redistribution of contrast medium on sequential CT examinations, it is possible to detect the existence of lymphourinary fistulas, the precise location of lymphatic anomalies, the distribution of collateral lymphatic vessels, and hydrodynamic pressure abnormality in the lymph circulation in patients with idiopathic chyluria. CT staging of chyluria provides additional information that can be used to guide therapeutic management.

Primary intestinal lymphangiectasia: Multiple detector computed tomography findings after direct lymphangiography.

INTRODUCTION: To analyse the findings of multiple detector computed tomography (MDCT) after direct lymphangiography in primary intestinal lymphangiectasia (PIL). METHODS: Fifty-five patients with PIL were retrospectively reviewed. All patients underwent MDCT after direct lymphangiography. The pathologies of 16 patients were confirmed by surgery and the remaining 39 patients were confirmed by gastroendoscopy and/or capsule endoscopy. RESULTS: After direct lymphangiography, MDCT found intra- and extraintestinal as well as lymphatic vessel abnormalities. Among the intra- and extraintestinal disorders, 49 patients had varying degrees of intestinal dilatation, 46 had small bowel wall thickening, 9 had pleural and pericardial effusions, 21 had ascites, 41 had mesenteric oedema, 20 had mesenteric nodules and 9 had abdominal lymphatic cysts. Features of lymphatic vessel abnormalities included intestinal trunk reflux (43.6%, n = 24), lumbar trunk reflux (89.1%, n = 49), pleural and pulmonary lymph reflux (14.5%, n = 8), pericardial and mediastinal lymph reflux (16.4%, n = 9), mediastinal and pulmonary lymph reflux (18.2%, n = 10), and thoracic duct outlet obstruction (90.9%, n = 50). CONCLUSIONS: Multiple detector computed tomography after direct lymphangiography provides a safe and accurate examination method and is an excellent tool for the diagnosis of PIL.

Computed tomography lymphangiography findings in 27 cases of lymphangioleiomyomatosis.

Background Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Material and Methods Twenty-seven patients with LAM underwent direct lymphangiography (DLG), followed by a post-procedural thoracoabdominal CT examination. Results All 27 patients were diagnosed with LAM, including 25 (93%) with chylothorax, eight (30%) with chyloperitoneum, seven (26%) with chylous fluid in the chest and abdomen, and one (4%) with lower-limb lymphedema. The CT lymphangiography showed that 27/27 (100%) patients presented iliac or retroperitoneal lymphatic vessel dilation; 20/27 (74%) presented thoracic duct outlet obstruction; 9/27 (33%) presented pleural lymph leakage; and 13/27 (48%) presented an abnormal distribution of contrast agent in the contralateral lumbar trunk, 3/27 (11%) in the pulmonary field, 1/27 (4%) in the abdominal cavity, and 4/27 (15%) in the pelvic cavity. Five of the 27 patients (19%) had retroperitoneal lymphangioleiomyomas, with contrast agent accumulation in three cases during CT lymphangiography. Two of the 27 (7%) patients had enlarged lymph nodes in the retroperitoneum. All 27 patients (100%) diagnosed with LAM displayed various lung cysts on thoracic high-resolution CT scans. Conclusion CT lymphangiography findings can be used to diagnose most LAM cases accurately. Moreover, these findings also display the various lymphatic vessel abnormalities associated with LAM.