ISSN 1004-4140
CN 11-3017/P
Volume 31 Issue 4
Aug.  2022
Turn off MathJax
Article Contents
DONG J, XIN J F, YUAN G X, et al. CT lymphangiography (CTL) in different type of primary intestinal lymphangiectasia (PIL): A comparative study[J]. CT Theory and Applications, 2022, 31(4): 469-477. DOI: 10.15953/j.ctta.2022.115. (in Chinese)
Citation: DONG J, XIN J F, YUAN G X, et al. CT lymphangiography (CTL) in different type of primary intestinal lymphangiectasia (PIL): A comparative study[J]. CT Theory and Applications, 2022, 31(4): 469-477. DOI: 10.15953/j.ctta.2022.115. (in Chinese)

CT Lymphangiography (CTL) in Different Type of Primary Intestinal Lymphangiectasia (PIL): A Comparative Study

doi: 10.15953/j.ctta.2022.115
  • Received Date: 2022-06-12
  • Accepted Date: 2022-06-30
  • Rev Recd Date: 2022-06-25
  • Available Online: 2022-07-06
  • Publish Date: 2022-08-01
  • Objective: To explore the clinical and imaging characteristics and application value CT lymphangiography (CTL) in patients with different types of primary intestinal lymphangiectasia (PIL). Methods: Patients diagnosed as PIL in our center were recruited in this retrospective study from January 2016 to December 2019, All CTL data were blindly reviewed by two radiologists separately, and the evaluation indicators included: sex, onset age, symptoms and signs, serum albumin, wall thickening, serous cavity effusion, abnormal distribution of lymphatics, lymph reflux, lymph nodes, fistula and abnormal lymphatics around neck area. Based on the abnormal lymphatics in intestinal wall and/or mesentery, PIL was classified into type Ⅰ (positive type) and type Ⅱ (negative type). The clinical and imaging features were compared between the two groups. Results: 34 PIL patients were recruited in this study, including 15 cases of Ⅰ and 19 cases of Ⅱ. Type I showed older age of first onset (15.3±9.4 vs. 8.3±3.8), higher rate of diarrhea, and lower rate of limb and/or facial edema than type Ⅱ, with no statistical difference in sex and serum albumin. For imaging features comparisons, type Ⅰ demonstrated higher rate of abnormal dilated lymphatics and fistula, lower rate of increased attenuation of mesentery and lymph nodes, while no statistical difference was found between wall thickening, serous cavity effusion, lymph reflux and abnormal dilated lymphatics around neck area. Conclusion: CTL demonstrated capability of evaluation in detection of location, distribution and range of abnormal lymphatics in PIL. Based on CTL, the imaging classification of PIL was proposed. Different types of PIL showed different clinical and imaging features, which was useful for therapeutic adoptions.

     

  • loading
  • [1]
    GONZALEZ N R, DOMINGUEZ C A, ALCOBA V L, et al. Primary intestinal lymphangiectasia: A rare disease as a cause of protein-losing enteropathy[J]. Revista Espanola de Enfermedades Digestivas, 2022. DOI:10.17235/reed.2022.8673/2022. (Online ahead of print).
    [2]
    KHAYAT A A. Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: A case report and literature review[J]. BMC Gastroenterol, 2021, 21(1): 225. doi: 10.1186/s12876-021-01813-6
    [3]
    BORZUTZKY A, ESPINO A, ALBERTI G, et al. Primary intestinal lymphangiectasia (Waldmann's Disease)[J]. American Journal of Gastroenterology, 2019, 114(2): 197. doi: 10.1038/s41395-018-0413-0
    [4]
    YANG C, DEHNER L P. Protein-losing enteropathy with intestinal lymphangiectasia in skeletal dysplasia with Lys650 Met mutation[J]. American Journal of Medical Genetics Part A, 2016, 170(11): 2993−2997. doi: 10.1002/ajmg.a.37756
    [5]
    CRUTZEN B, PONCELET P A. Protein-losing enteropathy in primary lymphangiectasia[J]. Journal of the Belgian Society of Radiology, 2020, 104(1): 34. doi: 10.5334/jbsr.2136
    [6]
    LOPEZ R N, DAY A S. Primary intestinal lymphangiectasia in children: A review[J]. Journal of Paediatrics and Child Health, 2020, 56(11): 1719−1723. doi: 10.1111/jpc.14837
    [7]
    MANORIA P, GULWANI H V. Primary intestinal lymphangiectasia in a middle-aged female[J]. Saudi Jorunal Medicine & Medical Sciences, 2021, 9(3): 280−281.
    [8]
    HUBER R, SEMMLER G, MAYR A, et al. Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature[J]. World Journal of Gastroenterology, 2020, 26(48): 7707−7718. doi: 10.3748/wjg.v26.i48.7707
    [9]
    PRASAD D, SRIVASTAVA A, TAMBE A, et al. Clinical profile, response to therapy, and outcome of children with primary intestinal lymphangiectasia[J]. Digestive Diseases, 2019, 37(6): 458−466. doi: 10.1159/000499450
    [10]
    CRAVEN M D, WASHABAU R J. Comparative pathophysiology and management of protein-losing enteropathy[J]. Journal of Veterinary Internal Medicine, 2019, 33(2): 383−402. doi: 10.1111/jvim.15406
    [11]
    KAKIUCHI T, MIZUOCHI T, KOJI A, et al. Long-term endoscopic findings in pediatric primary intestinal lymphangiectasia[J]. Clinical Case Reports, 2021, 9(2): 1029−1030. doi: 10.1002/ccr3.3619
    [12]
    CUNNINGHAM J M, NEPAL S, TRUESDALE A E. Primary intestinal lymphangiectasia diagnosed by video capsule endoscopy in a patient with immunodeficiency presenting with morganella morganii bacteraemia[J]. BMJ Case Reports, 2020, 13(9): e235898. doi: 10.1136/bcr-2020-235898
    [13]
    TOMINAGA K, TSUCHIYA A, KAWATA Y, et al. Novel magnified single-balloon enteroscopy enables observation of jejunal white spots associated with lymphangiectasia[J]. Digestive Diseases, 2019, 37(2): 170−174. doi: 10.1159/000493578
    [14]
    Van der REIJDEN S M, van WIJK M P, JACOBS M, et al. Video capsule endoscopy to diagnose primary intestinal lymphangiectasia in a 14-month-old child[J]. Journal of Pediatric Gastroenterology and Nutrition, 2017, 64(6): e161. doi: 10.1097/MPG.0000000000001586
    [15]
    BROWNELL J N, BIKO D M, MAMULA P, et al. Dynamic contrast magnetic resonance lymphangiography localizes lymphatic leak to the duodenum in protein-losing enteropathy[J]. Journal of Pediatric Gastroenterology and Nutrition, 2022, 74(1): 38−45. doi: 10.1097/MPG.0000000000003287
    [16]
    DONG J, XIN J, SHEN W, et al. Unipedal diagnostic lymphangiography followed by sequential ct examinations in patients with idiopathic chyluria: A retrospective study[J]. American Journal of Roentgenology, 2018: 210(4): 792-798.
    [17]
    董健, 信建峰, 霍萌, 等. 直接淋巴管造影和CT淋巴管成像在乳糜尿中的诊断价值[J]. 临床放射学杂志, 2018,37(5): 798−802. doi: 10.13437/j.cnki.jcr.2018.05.020

    DONG J, XIN J F, HUO M, et al. Direct lymphangiography with sequential CT lymphangiography in chyluria: A retrospective study[J]. Clinical Radiology, 2018, 37(5): 798−802. (in Chinese). doi: 10.13437/j.cnki.jcr.2018.05.020
    [18]
    董健, 沈文彬, 信建峰, 等. 联合应用CT淋巴管成像与直接淋巴管造影诊断原发性小肠淋巴管扩张症的价值[J]. 中华放射学杂志, 2017,51(5): 362−365. doi: 10.3760/cma.j.issn.1005-1201.2017.05.008

    DONG J, SHEN W B, XIN J F, et al. Value of CT lymphangiography combined with direct lymphangiography in diagnosing primary intestinal lymphangiectasia[J]. Journal of Chinese Radiology, 2017, 51(5): 362−365. (in Chinese). doi: 10.3760/cma.j.issn.1005-1201.2017.05.008
    [19]
    DONG J, XIN J, SHEN W, et al. CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL): A comparative study with intraoperative enteroscopy (IOE)[J]. Academic Radiology, 2019, 26(2): 275−281. doi: 10.1016/j.acra.2018.04.023
    [20]
    董健, 信建峰, 张春燕, 等. 直接淋巴管造影术后CT在原发性小肠淋巴管扩张症中的回顾性研究[J]. 临床放射学杂志, 2018,37(9): 1510−1514.

    DONG J, XIN J, ZHANG C Y, et al. Post-lymphangiographic CT (PLCT) in primary intestinal lymphangiectasia: A retrospective study[J]. Clinical Radiology, 2018, 37(9): 1510−1514. (in Chinese).
    [21]
    董健, 信建峰, 霍萌, 等. 直接淋巴管造影与CT淋巴管成像在继发性小肠淋巴管扩张症的回顾性研究[J]. 临床放射学杂志, 2018,37(3): 419−423. doi: 10.13437/j.cnki.jcr.2018.03.014

    DONG J, XIN J, HUO M, et al. Direct lymphangiography with sequential CT lymphangiography in secondary intestinal lymphangiectasia: A retrospective study[J]. Clinical Radiology, 2018, 37(3): 419−423. (in Chinese). doi: 10.13437/j.cnki.jcr.2018.03.014
  • 加载中

Catalog

    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Figures(3)  / Tables(2)

    Article Metrics

    Article Views(108) PDF Downloads(6) Cited by()
    Proportional views
    Related

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return