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Tortuous lymphatic vessels

MedGen UID:
1788937
•Concept ID:
C5543367
•
Anatomical Abnormality
Synonym: Tortuous lymphatics
 
HPO: HP:0033986

Definition

The presence of an increased number of twists and turns of lymphatic vessels. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTortuous lymphatic vessels

Conditions with this feature

Lymphatic malformation 9
MedGen UID:
1779656
•Concept ID:
C5543365
•
Disease or Syndrome
Lymphatic malformation-9 (LMPHM9) is an autosomal dominant disorder characterized by the onset of lower-extremity lymphedema in the first decades of life. Imaging may show lymph backflow and defects in lymph channel valves. There is variable expressivity and incomplete penetrance; female mutation carriers are more likely to show disease manifestations than male carriers (summary by Erickson et al., 2019). For a discussion of genetic heterogeneity of lymphatic malformation, see 153100.
See: Condition Record
Lymphatic malformation 9

Professional guidelines

PubMed

MR imaging for preoperative evaluation of primary rectal cancer: practical considerations.
Kaur H, Choi H, You YN, Rauch GM, Jensen CT, Hou P, Chang GJ, Skibber JM, Ernst RD
Radiographics 2012 Mar-Apr;32(2):389-409. doi: 10.1148/rg.322115122. PMID: 22411939
Normalization of the vasculature for treatment of cancer and other diseases.
Goel S, Duda DG, Xu L, Munn LL, Boucher Y, Fukumura D, Jain RK
Physiol Rev 2011 Jul;91(3):1071-121. doi: 10.1152/physrev.00038.2010. PMID: 21742796Free PMC Article

Recent clinical studies

Diagnosis

Lymphangioleiomyoma of the Uterus and Pelvic Lymph Nodes: A Report of 3 Cases, Including the Potentially Earliest Manifestation of Extrapulmonary Lymphangioleiomyomatosis.
Ando H, Ogawa M, Watanabe Y, Tsurunaga K, Nakamura C, Tamura H, Ikeda K, Takahashi R, Nakajima R, Yoshida S, Tsujie T, Doi R, Wakimoto A, Adachi S
Int J Gynecol Pathol 2020 May;39(3):227-232. doi: 10.1097/PGP.0000000000000589. PMID: 30789500
Near-Infrared Fluorescence Lymphatic Imaging in Lymphangiomatosis.
Rasmussen JC, Fife CE, Sevick-Muraca EM
Lymphat Res Biol 2015 Sep;13(3):195-201. Epub 2015 Aug 19 doi: 10.1089/lrb.2015.0013. PMID: 26287470Free PMC Article

Prognosis

Blockade of VEGF receptor-3 aggravates inflammatory bowel disease and lymphatic vessel enlargement.
Jurisic G, Sundberg JP, Detmar M
Inflamm Bowel Dis 2013 Aug;19(9):1983-9. doi: 10.1097/MIB.0b013e31829292f7. PMID: 23835443Free PMC Article

Clinical prediction guides

Lymphangioleiomyoma of the Uterus and Pelvic Lymph Nodes: A Report of 3 Cases, Including the Potentially Earliest Manifestation of Extrapulmonary Lymphangioleiomyomatosis.
Ando H, Ogawa M, Watanabe Y, Tsurunaga K, Nakamura C, Tamura H, Ikeda K, Takahashi R, Nakajima R, Yoshida S, Tsujie T, Doi R, Wakimoto A, Adachi S
Int J Gynecol Pathol 2020 May;39(3):227-232. doi: 10.1097/PGP.0000000000000589. PMID: 30789500
Near-Infrared Fluorescence Lymphatic Imaging in Lymphangiomatosis.
Rasmussen JC, Fife CE, Sevick-Muraca EM
Lymphat Res Biol 2015 Sep;13(3):195-201. Epub 2015 Aug 19 doi: 10.1089/lrb.2015.0013. PMID: 26287470Free PMC Article
Blockade of VEGF receptor-3 aggravates inflammatory bowel disease and lymphatic vessel enlargement.
Jurisic G, Sundberg JP, Detmar M
Inflamm Bowel Dis 2013 Aug;19(9):1983-9. doi: 10.1097/MIB.0b013e31829292f7. PMID: 23835443Free PMC Article

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