Pelvic Microcystic Lymphatic Malformation.

HomeRadiologyVol. 308, No. 1 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyPelvic Microcystic Lymphatic MalformationDominik Geisel , Thula Cannon Walter-RittelDominik Geisel , Thula Cannon Walter-RittelAuthor AffiliationsFrom the Department of Radiology, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Augustenburger Platz 1, 13353 Berlin, Germany.Address correspondence to D.G. (email: [email protected]).Dominik Geisel Thula Cannon Walter-RittelPublished Online:Jul 18 2023https://doi.org/10.1148/radiol.230364MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Supplemental material is available for this article.A 25-year-old woman presented with a history of vaginal discharge (range, 300–2000 mL/day) beginning at 14 years of age and chronic lymphedema of the left lower limb. Lymphatic origin of the vaginal discharge was confirmed with laboratory analysis. Abdominal and pelvic MRI with intravenous administration of contrast material showed multiple diffuse T2-weighted hyperintense microcystic lesions in the left hemipelvis without enhancement. Dynamic MR lymphography was performed with simultaneous injection of 5.5 mL of a 1:1 mixture of gadobutrol and saline solution into the right and left inguinal lymph nodes (Figure). The maximum intensity projection and time-lapse movie (Movie) show a left-dominant pelvic microcystic lymphatic malformation involving the left parametrium and uterus (Figure). Delayed (30 minutes) contrast-enhanced imaging enabled confirmation of vaginal lymphatic drainage (Figure).Images in a 25-year-old woman with a history of persistent lymphatic vaginal discharge (range, 300–2000 mL per day) that began at 14 years of age and chronic lymphedema of the left lower limb. MRI showed a microcystic lymphatic malformation that was referred for diagnostic work-up. (A) Dynamic MR lymphography with injection of 5.5 mL of a 1:1 mixture of gadobutrol and saline solution into bilateral inguinal lymph nodes was performed with a repeated coronal fat-saturated T1 volumetric interpolated breath-hold examination, or VIBE, sequence over a 30-minute time course after injection. Static image shows appearance after 30 minutes. (B) Delayed (30 minutes) postcontrast image enables confirmation of contrast material in the vaginal canal (arrow) and lymphatic vaginal drainage. (C) Coronal still frames from time-lapse T1-weighted postcontrast (bilateral injection into an inguinal lymph node) imaging show pelvic lymphatic flow from this microcystic lymphatic malformation.Download as PowerPointOpen in Image Viewer Get the Flash Player to see this video.Movie: Coronal time-lapse T1-weighted postcontrast (bilateral injection into an inguinal lymph node) movie shows pelvic lymphatic flow from this microcystic lymphatic malformation.Download Original Video (1.2 MB)Lymphatic vaginal discharge is rare. It can be caused by lymphangiectasis with or without thoracic duct obstruction, intestinal lymphangiectasis, lymphedema, and xanthomatosis (1). Lymphatic malformations involving the genitalia can be treated with sclerotherapy, embolization (2,3), ligation of dilated lymph vessels, and systemic mammalian target of rapamycin inhibitors (mTOR) (4). After treatment with three courses of sclerotherapy, there has been a substantial improvement in the discharge volume. This patient is currently scheduled to undergo further embolization therapy.Disclosures of conflicts of interest: D.G. No relevant relationships. T.C.W.R. No relevant relationships.References1. Karg E, Bereczki C, Kovacs J, et al. Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia. Br J Dermatol 2002;146(1):134–137. Crossref, Medline, Google Scholar2. Alali M, Park R, Shin J, et al. Lipiodol Lymphangiography and Glue Embolization for Vulvar and Vaginal Lymphorrhea. AJIR 2022;06(01):047–049. Google Scholar3. Hur S, Jun H, Jeong YS. Novel interventional radiological management for lymphatic leakages after gynecologic surgery: lymphangiography and embolization. Gland Surg 2021;10(3):1260–1267. Crossref, Medline, Google Scholar4. Wolter JK, Valencia-Sama I, Osborn AJ, et al. Combination mTOR and SHP2 inhibitor treatment of lymphatic malformation endothelial cells. Microvasc Res 2022;143:104397. Crossref, Medline, Google ScholarArticle HistoryReceived: Feb 15 2023Revision requested: Mar 6 2023Revision received: Mar 13 2023Accepted: Mar 31 2023Published online: July 18 2023 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 308, No. 1 Metrics Altmetric Score PDF download